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Chapter

37 
Pediatric Cardiopulmonary Bypass
Richard M. Ginther, Jr., and Joseph M. Forbess

popularized by Dr. F. John Lewis, used total body hypothermia


PEARLS and vena cava inflow occlusion to achieve direct visualization
• Cardiopulmonary bypass (CPB), which originated in the of atrial septal defects.2 Although this technique proved to be
mid-twentieth century, was designed to allow for the repair a fairly safe technique for simple atrial septal defects, failure
of congenital heart defects. Its history has since been was often the result when more complex defects were
characterized by perpetual technological advancements attempted.3,4 Surgeons needed a way to safely perfuse the
that have been instrumental in sustaining the momentum of patient’s circulatory system and extend the “safe” surgical
clinical progress of this field. time. In the late 1930s, Dr. John Gibbon and his wife Mary, a
• Because of the morbidity associated with the “time nurse and research assistant, began developing a heart-lung
on-pump,” many early surgeries were performed at machine to do just this. By the early 1950s, Dr. Gibbon, in an
profoundly hypothermic temperatures by utilizing circulatory interesting collaboration with International Business Machines
arrest. Corporation (IBM), reported promising success in the labora-
• The current philosophy underpinning the use of pediatric tory using a heart-lung machine on cats and dogs.5-7 After a
CPB is to meet the metabolic demands of the patient previous fatal attempt to repair an atrial septal defect (ASD)
throughout the repair while minimizing the impact of in a 15-month-old child in February 1952, Dr. Gibbon suc-
associated nonphysiologic effects. cessfully closed an ASD in an 18-year-old patient using his
• All aspects of CPB have experienced major technological heart-lung machine on May 6, 1953.8 Unfortunately, Dr.
improvements. Circuits are miniaturized and cause less Gibbon was not able to repeat the same success with the heart-
blood trauma, blood component therapy is highly directed, lung machine on subsequent cases, and his next four patients
and on-pump patient monitoring techniques have died. Other surgical teams devised their own versions of car-
advanced. diopulmonary bypass but were unable to replicate laboratory
• The progress of pediatric CPB has played a major role in successes, and no other human survivors were reported. It was
the steady reduction of morbidity and mortality associated theorized that perhaps these hearts were too sick to be repaired
with cardiac surgery in children. Pediatric mortality rates are and that it was unrealistic to expect that these hearts
now comparable to those in adult patients. could recover. Cardiopulmonary bypass became a widespread
disappointment, and most investigators abandoned the tech-
nique. While others were reporting their attempts using the
heart-lung machine, however,9-12 Dr. C. Walton Lillehei and
Background his colleagues at the University of Minnesota introduced a new
approach for supporting patients during surgery: controlled
History cross circulation. During cross circulation, the patient’s parent
Surgery for congenital heart disease has evolved into a rela- was used as the “heart-lung machine” and supported the
tively safe intervention considering its brief history and count- patient during the operation (Fig. 37.1). Considering the
less hurdles. This historical journey is of course filled with potential for a 200% operative mortality, this was a highly
triumphs and tragic failures and tells a story of progressive controversial technique. However, using this method, Dr.
intuition and challenges steadily surmounted. This has culmi- Lillehei was able to effectively close an ASD on March 26,
nated in the generally successful model that is used today 1954.13 Dr. Lillehei and his colleagues14 continued a remark-
(Table 37.1). The early years of cardiac surgery spawned many able series of successes using cross circulation by performing
novel techniques for operations that did not rely on cardio- 45 operations for anomalies including ventricular septal
pulmonary bypass as used today. Surgeons initiated their defect, atrioventricular canal, and tetralogy of Fallot, with an
efforts in cardiovascular surgery with attempts to repair extra- operative mortality of only 38%. This progress with more
cardiac vascular anomalies such as patent ductus arteriosus complex lesions prompted investigators to rethink their
and coarctation of the aorta. On August 26, 1938, at the options for supporting, repairing, and recovering these
Boston Children’s Hospital, Dr. Robert Gross performed the patients. Two surgical camps ignited the resurgence of the
world’s first successful patent ductus arteriosus closure on a artificial heart-lung machine: Dr. Lillehei and his colleagues at
7-year-old girl.1 Soon, exposing the heart and attempting to the University of Minnesota and Dr. John Kirklin and his col-
correct life-threatening cardiac defects became a reality. In leagues at the nearby Mayo Clinic. Dr. Kirklin and colleagues15
the early 1950s, surgeons began to explore several different reported a 50% mortality among eight patients using a modi-
approaches to repairing intracardiac defects. One technique, fication of the Gibbon-IBM pump oxygenator in the spring of

430
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Pediatric Cardiopulmonary Bypass Chapter 37 431

techniques with limited periods of extracorporeal circulation


TABLE 37.1  Successful Congenital Cardiac Surgery Milestones were popularized in the early 1970s by Dr. Barratt-Boyes and
proved to dramatically extend the “safe” period of support.17
Year Event Surgeon
Surgeons began to perform increasingly complex congenital
1938 Patent ductus arteriosus Gross heart repairs. Pediatric cardiac surgical care was further refined
ligation over the subsequent several decades. The development of
1944 Coarctation repair Crafoord smaller, more efficient, and customizable heart-lung machine
1944 Blalock-Taussig shunt Blalock, Taussig hardware and components, as well as improvements in myo-
cardial protection, have allowed surgical teams to move away
1946 Potts shunt Potts
from the concept of limited cardiopulmonary bypass and
1947 Closed pulmonary valvotomy Sellors toward a more “full-flow” philosophy wherein the metabolic
1948 Atrial septectomy Blalock, Hanlon demands of the body are continuously met while the patient
1951 Pulmonary artery band Muller, Dammann is on the heart-lung machine. This chapter explores the con-
cepts that form the basis of this philosophy and the techniques
1952 Atrial septal defect closure Gross
using atrial well
that surgical teams currently use to support pediatric patients
during cardiovascular surgery.
1952 Atrial septal defect closure Lewis
using hypothermia Surgical Team
1953 Atrial septal defect closure Gibbon The surgical team consists of highly trained specialists, each
using cardiopulmonary bypass
of whom plays a vital role in the safety and success of the
1954 Ventricular septal defect Lillehei surgical procedure. This specialized team is led by the cardiac
closure using cross circulation surgeon and typically includes an assistant surgeon or physi-
1958 Superior cavopulmonary shunt Glenn cian assistant, an anesthesiologist, a perfusionist, as well as
(Glenn shunt) several nurses, surgical scrub technologists, anesthesia assis-
1958 Senning operation for Senning tants, and perioperative surgical assistants.
transposition of great arteries A perfusionist is a health care professional who specializes
1963 Mustard operation for Mustard in all aspects of extracorporeal circulation. The primary focus
transposition of great arteries of a perfusionist is to support the cardiac surgical patient
1968 Fontan procedure for tricuspid Fontan during cardiopulmonary bypass. Because of this, the perfu-
atresia sionist’s clinical expertise is a critical component of operative
1975 Arterial switch for transposition Jantene success. Perhaps the first perfusionist was Mary Gibbon, Dr.
of great arteries Gibbon’s wife. In addition to helping design the Gibbon-IBM
heart-lung machine, she assembled and operated it as well.
1981 Norwood procedure for Norwood
hypoplastic left heart syndrome The term perfusionist did not emerge until the early 1970s, and
in the early days of cardiac surgery, surgical groups would
1985 Neonatal heart transplantation Bailey
typically use any locally available combination of physiolo-
gists, biochemists, cardiologists, or surgical residents to help
operate the heart-lung machine. Now, cardiovascular perfu-
1955. Months later, Lillehei and colleagues16 reported a 29% sionists are highly trained, nationally certified (CCP; Certified
mortality among seven patients using their own heart-lung Clinical Perfusionist), state-licensed allied health profession-
machine and the groundbreaking DeWall Bubble Oxygenator. als. The common scope of practice for a perfusionist consists
These two groups demonstrated that surgical repair of complex of cardiopulmonary bypass (CPB), extracorporeal membrane
congenital defects could be performed in a more controlled oxygenation (ECMO), isolated limb/organ chemoperfusion,
environment than cross circulation or inflow occlusion, with ventricular assist device (VAD), autotransfusion, and intra-
promising results. What followed were many groups initiating aortic balloon counterpulsation.
open-heart programs primarily addressing congenital heart
disease. Despite significant improvements in survival rates,
congenital cardiac repairs remained a daunting undertaking
with significant risk. Bypass circuits were enormous when Equipment and Preparation for
compared with the patient blood volume, the systemic Cardiopulmonary Bypass
response was an extreme shock, and the understanding of the
physiologic response to this “nonphysiologic” extracorporeal Heart-Lung Machine Console and Pumps
circulation was quite limited. Investigators sought to use car- The cardiopulmonary bypass (CPB) machine, commonly
diopulmonary bypass but limit the actual cumulative time referred to as the heart-lung machine, is the mechanical hard-
that nonphysiologic blood flow is provided to the patient— ware that a perfusionist uses to support the patient during
with its attendant risk. The bypass circuit could be used to surgery. Until the late 1950s, the CPB hardware and circuitry
cool the patient down to profound hypothermia, after a were typically handmade, and many of the components had
lengthy period of topical cooling. The circulation of the to be hand washed and sterilized for reuse. The hardware
patient could then be safely terminated for lengthy periods of components were designed at that time with two objectives:
time, allowing for complex cardiac repairs. At the conclusion to pump blood through the patient’s cardiovascular system
of the repair, the heart-lung machine could be used to fully and to successfully perform respiratory gas exchange, hence
warm the patient. These hypothermic circulatory arrest the term “heart-lung machine.” Unfortunately, this heart-lung

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432 Section II Cardiovascular System

Sigmamotor pump

Patient

Donor

Defect

Fig. 37.1. Controlled cross circulation. (From Stoney WS. Evolution of cardiopulmonary bypass. Circulation. 2009;119:2844-2853.)

apparatus was large, difficult to move, had no safety features,


and was not available to other institutions eager to operate.
Surgeons interested in these handcrafted devices would often
visit the surgical groups at the University of Minnesota
and Mayo Clinic, but few could replicate their expensive and
intricate systems. Eventually, industry developers began to
commercially release heart-lung machines with hardware
components consolidated onto a wheel-mounted console.
Interestingly, although cardiac surgery began with the pediat-
ric patient population, heart-lung machines were developed
as “one-size-fits-all” units and were not customizable for
smaller patients.
Modern heart-lung machine consoles are mobile, offer
many pump configuration options, and are loaded with safety
features. These design improvements allow for better configu-
ration options for the pediatric surgical population. An ideal
heart-lung machine for pediatric CPB is customizable for
circuit miniaturization and offers safety devices and hardware
that accommodate both smaller tubing sizes and circuitry.
Customizations such as mast mounting pumps in various
configurations and incorporating mini−roller pumps with
shorter raceway lengths are two popular heart-lung machine
configurations.18,19 Fig. 37.2. Roller pump with 1
-inch tubing placed in the raceway.
4
Several different types of mechanical pumps have been
used to substitute the function of the heart, and interest-
ingly, the roller pump has remained a standard pump mech- creating a continuous nonpulsatile flow. The flow output is
anism since the beginning of cardiopulmonary bypass. A controlled by changing the revolution per minute (RPM) of
roller pump functions by positive fluid displacement. Tubing the pump. Roller pumps are the most commonly used arte-
is placed in a curved raceway, and as occlusive rollers rotate rial (heart) pump in pediatrics (Fig. 37.2).20 While roller
over the compressible tubing, blood is pushed forward pumps are used as the arterial pump, the heart-lung

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Pediatric Cardiopulmonary Bypass Chapter 37 433

machine console also holds several other roller pumps used


for cardiotomy field suction, venting the heart, and cardio- Cardiopulmonary Bypass Circuit
plegia delivery. The handmade circuits used on children in the mid-1950s
The centrifugal pump is another type of arterial pump that were elaborate, and the large blood volume required to prime
has gained significant popularity since the mid-1970s. A cen- them was a burden on the blood bank. Perfusionists would
trifugal pump uses an impeller cone and rotational kinetic have to spend the evening of surgery assembling the circuit
energy to propel the blood, and because it is nonocclusive, it and then tackle the tedious task of dismantling, rewashing,
is thought to be safer and cause less hemolysis than roller and sterilizing the same circuitry after surgery. Fortunately,
pumps. Centrifugal blood flow is controlled by the impeller manufacturers now offer a wide variety of disposable circuit
cone RPMs and is also dependent on preload and sensitive to components that are fairly simple to assemble. The modern
resistance distal to the pump. Because the pump is not occlu- CPB circuit is a series of components consisting of cannulas,
sive, any resistance or occlusion will result in a reduction or tubing, venous reservoir, filters, oxygenator, heat exchanger,
cessation of flow. These pumps require the use of a flow probe hemoconcentrator, suction, and cardioplegia delivery system.
to measure actual flow, and the nonocclusive property is con- Deoxygenated blood from the superior vena cava (SVC)
sidered a safety feature in the event of cannula obstruction or and inferior vena cava (IVC) travels down a venous line,
accidental arterial line occlusion. The use of centrifugal pumps usually pulled by simple gravitational siphon effect, and into
during ECMO has become increasingly popular due to the a venous reservoir. The deoxygenated blood in the reservoir is
suggested hemolytic and safety benefits; however, these ben- pumped through the oxygenator and then back to the patient’s
efits have often been refuted.21-24 Roller pumps remain the aorta (or other major artery) via the arterial line (Fig. 37.3).
main arterial pump type in pediatric CPB because they are This blood pathway diverts blood away from the heart and
simple, inexpensive, and, importantly, require a much smaller lungs, creating a bloodless operative field. In the adult patient
prime volume than centrifugal pumps. population, where the circuit prime volume is typically no

Venous line; Gravity drainage

SVC & IVC


bicaval
cannulation Cardiotomy;
Venous

Hemoconcentrator
Level sensor

reservoir

Arterial line
Cardiotomy
field suction

del Nido
Cardioplegia
1:4
Bubble
Temp mm Hg sensor Oxygenator;
4°C Heat exchanger; mm Hg
Integrated
arterial filter

Water
Gas heater/cooler
filter
Field Field Vent; Arterial pump
suction suction Cardioplegia; Gas flow
Mini roller Isoflurane meter Blender
pumps Air
O2

CO2

Fig. 37.3. Schematic of the cardiopulmonary bypass circuit at Children’s Health Dallas.

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434 Section II Cardiovascular System

greater than 25% of the patient’s blood volume, a single circuit cardiac surgery to be performed more safely, in progressively
size can be used for almost all patient sizes. The small circuit smaller patients.
prime-to-patient blood volume ratio helps to minimize The first oxygenators used in the early days of cardiac
patient hemodilution during CPB and ultimately reduces the surgery were hardware units that either used rotating discs or
likelihood of donor blood exposure. The same adult circuit, large mesh screens. These oxygenators worked by creating a
with a prime volume of about 1 L, would be approximately large surface area film of blood, either over rotating discs in a
500% of the circulating blood volume in a neonate. This dis- pool of venous blood or trickling over large mesh screens, and
crepancy would seem outrageous considering current circuit exposing the film of blood to an oxygenated atmosphere.25,26
options, but the prime-to-blood volume ratio was even higher Though these units were successful at oxygenating blood, they
before manufacturers began to release pediatric oxygenators required extremely large priming volumes; were not dispos-
in the mid-1980s. Since the oxygenator is one of the largest able; were difficult to assemble, operate, and clean; and lost
volume components of the CPB circuit, any significant reduc- significant efficiency during hemodilution. In addition to
tion in size would result in large prime volume reductions. A these disadvantages, these oxygenators were not commercially
circuit miniaturization movement began, and the new clinical available to clinicians looking to operate beyond the Univer-
challenge in pediatric CPB was to reduce both circuit prime sity of Minnesota and Mayo Clinic.
volume and surface area. The goal of circuit prime and surface The University of Minnesota team dramatically changed
area reduction is to minimize hemodilution and the deleteri- this landscape in the late 1950s by releasing the simple, dispos-
ous effects of foreign surface blood contact activation. Strate- able, inexpensive, and commercially available DeWall-Lillehei
gies such as using smaller diameter and shorter tubing lengths bubble oxygenator.27 Though the safety of actively adding
and incorporating neonatal and pediatric CPB components bubbles to the blood was debated, the commercial availability
have allowed clinicians to reach this goal. At Children’s Health of this device contributed to a rapid global expansion of
Dallas, the perfusionists have made many circuit modifica- cardiac surgery. The bubble oxygenator was a distinct improve-
tions to achieve a static prime volume of approximately ment over the previous unwieldy direct blood contact oxygen-
165 mL in our neonatal circuit. This prime volume lowers the ators, yet it was still limited in that the direct blood air interface
circuit size to approximately 45% of the blood volume of a could produce significant blood trauma. This trauma accrues
3-kg patient (Fig. 37.4). over time, so the safety margin for longer pump runs was
diminished for longer, complex cases.
Oxygenators The next generation of oxygenators, membrane oxygen-
An oxygenator, the artificial lung of the CPB circuit, might be ators, better mimicked the function of the lungs. These micro-
considered the most important component of the circuit. It is porous, gas-permeable membranes eliminated direct contact
responsible for oxygen and carbon dioxide (CO2) gas exchange, between gas and blood, thus reducing blood trauma.26 The
as well as volatile anesthetic administration. A heat exchanger, concept of a microporous membrane separating the gas and
used for cooling and warming the perfusate, and hence the blood was sound, but it took decades of research to find a
patient, is housed inside the oxygenator, and certain newer suitable membrane material before these oxygenators could
models now integrate the arterial filter, to reduce particulate replace bubble oxygenators commercially. Initial success with
matter, into the oxygenator. A venous reservoir, which includes silicone membranes was observed with long-term support
both venous line and cardiotomy suction filters and various during ECMO; however, in the operating room these mem-
ports for drug and fluid administration, is typically packaged branes proved to be less efficient, prone to plasma leakage and
with an oxygenator. Currently, hollow fiber membrane oxy- thrombus formation.26,28 The development and release of
genators, which fully separate the blood flow from gas flow by polypropylene microporous membranes allowed for efficient
a thin polymer membrane, are used during CPB. A brief gas exchange over a wide range of temperatures and pump
history of oxygenator development reveals much about some flow rates and soon replaced the bubble oxygenator during
of the important “engineering” solutions that have allowed for CPB in the mid-1980s. In these oxygenators, CO2 and O2 flow
meters and a gas blender control gas and volatile anesthetic
flow through the inside of the hollow polypropylene fibers.
The gas within the hollow fibers passively diffuses into the
blood flowing on the outside of the fibers.
In 1985 Cobe released the popular Variable Prime Cobe
Membrane Lung (VPCML) designed for the pediatric market.
This oxygenator was divided into separate compartments and
gave clinicians three maximum blood flow options, 1.3, 2.6,
and 4.0 L per minute (LPM), depending which compartments
were opened.29 The VPCML also tried a new concept with the
heat exchanger. Once a separate external CPB component, the
stainless steel heat exchanger was placed inside the venous
reservoir. The stainless steel coil wrapped around the inside of
the reservoir was not efficient unless a large amount of volume
was held in the reservoir. This was counter to the efforts to
reduce the overall circuit prime volume. Despite this short-
Fig. 37.4. Sorin S5 heart-lung machine with mast mounted arterial coming in the VPCML model, the move toward integration
pump and Terumo Baby FX reservoir and oxygenator at Children’s and consolidation of functionalities continued. These heat
Health Dallas. exchangers are now integrated within the oxygenator housing.

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Pediatric Cardiopulmonary Bypass Chapter 37 435

Considering that pediatric cardiac surgery is more likely than


adult surgery to use moderate to deep hypothermia, these Tubing
heat exchangers need to be extremely efficient with a small The tubing used to connect the various components of the
surface area. CPB circuit to the patient is made of a medical-grade polyvinyl
As technology relentlessly improved, membrane hollow chloride (PVC). Tubing length and diameter are the two main
fibers were wrapped into tighter configurations. This eventu- factors to consider when designing a circuit. Shorter tubing
ally allowed for a priming volume low enough to release a with the smallest internal diameter will reduce prime volume,
dedicated neonatal oxygenator. In 2006, Dideco released the but the tubing must also be large enough to safely manage
first neonatal oxygenator with a prime volume of 31 mL and required blood flows and line pressures for a given patient. In
max rated flow of 700 mL/min.30 The new generation of the past, 1 4-, 3 8 -, and 1 2 -inch tubing were the only tubing
neonatal and pediatric oxygenators achieves much higher options and thus made circuit miniaturization a difficult task.
maximum flow rates while keeping prime volumes appropri- Currently, a wide range and selection of pediatric tubing and
ate for neonates. This has allowed clinical teams to achieve connector sizes are available, and tubing sizes such as 1 8 , 3 16 ,
consistent physiologic outcomes after pump runs in neonates and 1 4 inch have become the new standards in pediatrics.
and small infants. A modern pediatric device like the Terumo Changing the internal diameter of tubing affects blood flow
Baby FX oxygenator with integrated arterial filter (Terumo resistance and must not impede venous drainage or arterial
Cardiovascular Group, Ann Arbor, Michigan, USA) offers a blood flow. At our institution, we select arterial-venous line
low total prime volume and a high maximum blood flow (Fig. sizes that accommodate gravity venous drainage and do not
37.5). With arterial filter integration, this oxygenator has a exceed an arterial line pressure of 350 mm Hg (Table 37.2).
total prime volume of 43 mL and a maximum rated blood Large reductions in tubing length have been made possible by
flow of 1.5 LPM. This low prime oxygenator is suitable for positioning the smaller new-generation pump consoles close
neonates but also accommodates patients up to approximately to the patient and using mast mounted pumps to bring com-
15 kg. This wide range of blood flow and low prime improves ponents closer together. The bioreactivity of blood coming
the likelihood of bloodless surgery—wherein an asanguineous into contact with artificial surfaces, such as tubing, is known to
prime is used—for the larger patients in range for this device. exacerbate the systemic inflammatory response and disrupt
The Maquet Quadrox-i Neonatal oxygenator (Maquet Holding hemostasis. A major advancement has been the development
B.V. & Co. KG, Rastatt, Germany) is another oxygenator with of surface coatings that attempt to mimic the endothelial
an integrated arterial filter that has a 40-mL total prime surface of blood vessels. These coatings have been shown to
volume and 1.5-LPM maximum flow. When considering the attenuate the increase of cytokines and inflammatory markers
additional volume of an external arterial line filter, this high- and preserve platelets.32,33 When selecting tubing for the pedi-
efficiency oxygenator with an integrated filter offers the lowest atric circuit, the goal is to safely achieve maximum blood flows,
total prime volume unit on the market today.31 Current trends decrease prime volume, and attenuate blood trauma.
in oxygenator design and development include integration of
the arterial line filter, biocompatible surface coatings for Hemoconcentrators
circuit tubing, decreasing flow resistance, and more efficient A hemoconcentrator is an ultrafiltration device that consists
heat exchange. of semipermeable membrane fibers that remove plasma water

TABLE 37.2  Tubing Specifications and Maximum Blood Flow


Ranges Tested at Children’s Health Dallas
CHILDREN’S HEALTH DALLAS
TUBING SPECIFICATIONS PROTOCOL

Internal Max Art Max Gravity


Diameter Flow (mL/ Drainage
(inches) mL/ft mL/rev min) (mL/min)
1/8 2.5 3.5 ~450
5/32 3.7 5 ~750
3/16 5 7 ~1300 500-650
1/4 9.65 13 ~3000 1300-
1500
5/16 13.5 18 ~5500 2000-
2200
3/8 21.71 27 >5000 4000-
4500
7/16 28.5 38 5000-
5500
1/2 38.61 45 >5000
5/8 55.77 65
Fig. 37.5. Picture of the Terumo Baby FX05 pediatric oxygenator.

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436 Section II Cardiovascular System

and solutes. They function similarly to hemodialysis units but


TABLE 37.3  Cardiopulmonary Bypass Circuit Prime Drugs
are simpler in that they do not require a dialysate solution.
Blood flows through microporous membrane fibers, and since
Drug Action Prime Dose
the hydrostatic pressure is higher inside the membrane fibers,
effluent fluid permeates the membrane and can be removed. Heparin Anticoagulant Calculated by
The membrane pore sizes are typically less than 55,000 daltons, Medtronic HMS and
varies per patient
which preserve plasma proteins such as albumin (65,000
daltons), and maintain the colloid oncotic pressure. The ultra- Sodium bicarbonate Buffer Achieve pH 7.40
filtration rate of a hemoconcentrator is dependent on the Mannitol Osmotic diuretic; 0.5 mg/kg; 12.5 g
hydrostatic pressure gradient across the membrane, blood oxygen radical max dose
flow rate through the membrane fibers, membrane pore size, scavenger
and the hematocrit. Ultrafiltration is useful for increasing Furosemide Loop diuretic 0.25 mg/kg; 20 mg
hematocrit, reducing high potassium levels after cardioplegia max dose
delivery, and removing harmful inflammatory mediators. Methylprednisolone Corticosteroid 30 mg/kg; 1 g max
Hemodilution during pediatric CPB is difficult to avoid, and dose
a 2004 survey of pediatric cardiac surgery centers report that 25% Albumin Plasma protein 10% circuit prime
98% of perfusionists routinely use a hemoconcentrator during volume
the conduct of CPB.20 Tranexamic acid Antifibrinolytic 20 mg/kg; 20 g
max dose
Circuit Prime
The CPB circuit is primed with a crystalloid replacement fluid.
Common solutions include Plasma-Lyte A, lactated Ringer, antithrombin III (ATIII), which then inactivates thrombin
and Normosol-R.34 Lactated Ringer is a replacement fluid that and other proteases involved in coagulation. Heparin is used
contains 29 mEq/L of lactate but lacks magnesium. Plasma- because it is fast-acting, and anticoagulation reversal can easily
Lyte A and Normosol-R both closely mimic human physio- be achieved by administering protamine. Anticoagulation
logic plasma electrolyte concentrations, osmolality, and pH. helps prevent circuit thrombus formation and avoid the
However, these two solutions do not contain calcium. At Chil- devastating effects of potential arterial thromboembolism.
dren’s Health Dallas, the perfusionists use Plasma-Lyte A Heparin was the anticoagulant used during Dr. Gibbon’s first
because it does not contain lactate or calcium, thus allowing successful cardiac surgery in 1953, and its use during CPB has
the perfusionists to lower CPB perfusate calcium levels, which continued for more than 60 years. Before heparin administra-
is desirable, as is discussed later. tion and dosing protocols were available, anticoagulation
Once the CPB circuit is primed with a crystalloid solution methods were cumbersome and unsafe. The dosing was
and cleared of any air, the total prime volume of the circuit is empiric, and the only methods for testing heparinization were
estimated. The perfusionist must then choose between initiat- lengthy laboratory heparin concentration tests. Fortunately,
ing CPB with or without adding heterologous blood. Unlike the activated clotting time (ACT) test was introduced in 1966
the adult patient population, blood products are often added and this bedside whole blood test became the foundation of
to the neonatal and pediatric CPB circuits due to the small how heparinization is monitored in the cardiac operating
patient blood volume-to-circuit prime volume ratio. The dilu- room today.35 Traditional laboratory tests such as partial
tional effect of the crystalloid prime is determined by calculat- thromboplastin time (PTT) and prothrombin (PT) time are
ing the patient resultant hematocrit (HCTr). The HCTr sensitive to low doses of heparin and therefore are not useful
formula, HCTr = (Patient blood volume × HCT)/(Patient during CPB. The ACT is a point-of-care test that measures the
blood volume + Circuit prime volume), is calculated once the time (in seconds) needed for activated whole blood to form
patient hematocrit value is measured in the operating room thrombin. In 1975, Bull et al.36 reported a heparin manage-
before surgery. The institutional protocol at Children’s Health ment approach using the ACT test and the technique quickly
Dallas is to maintain a CPB HCTr above 30%. If that value became universally accepted. The report describes the heparin
cannot be reached, then packed red blood cells (PRBCs) are dose response curve technique and suggests an optimal ACT
added to the circuit. The institutional protocol also directs range of 480 seconds during CPB. In this technique, ACTs are
that a half unit of fresh frozen plasma, approximately 100 mL, run on various whole blood samples containing different
will be added to the circuit prime for all patients less than 6 kg. heparin concentrations and results are plotted versus the
The pre-CPB circuit prime drug additives at our institution heparin concentration. The heparin dose response curve,
include heparin (1000 units/mL), 8.4% sodium bicarbonate, commonly referred to as the Bull curve, demonstrates the indi-
20% mannitol, furosemide (10 mg/mL), methylprednisolone, vidualized ACT response to different levels of heparinization
tranexamic acid, and 25% albumin (Table 37.3). The ideal and is a useful tool in estimating the concentration of heparin
prime solution should be “physiologic” and also attempt to necessary to achieve an ACT of 480 seconds (Fig. 37.6). Main-
attenuate the adverse response to artificially supporting a taining ACT results of at least 480 seconds during CPB remains
patient with an extracorporeal circuit. the standard of care today. Though most clinicians will agree
that 480 seconds is acceptable during CPB, there is debate
Anticoagulation whether or not that value should be universally applied, con-
Due to the foreign surface contact and resultant intrinsic acti- sidering that not all ACT analyzers operate and activate blood
vation of the coagulation cascade, the patient must be antico- in exactly the same manner.
agulated before CPB. Heparin is the most widely used Pediatric patients undergoing cardiac repair suffer dispro-
anticoagulant during CPB, and it acts by super-activating portionate postoperative bleeding complications after CPB,

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Pediatric Cardiopulmonary Bypass Chapter 37 437

600 determine the unit/mL heparin concentration. The HPT test


is used frequently during CPB to maintain heparin concen-
D
500 trations suggested by the HDR and also run post CPB to
C verify proper heparin reversal after protamine administra-
400 tion. Without heparinization, hemodilution and the degree
of hypothermia alone could extend the ACT beyond 480
ACT (sec)

B
300 seconds and this effect is amplified in the pediatric patient.
Administering heparin to maintain a patient heparin concen-
200 tration calculated by the HDR, despite an ACT above 480,
D will help to reduce consumptive coagulopathy, thrombin gen-
100 A eration, fibrinolysis, neutrophil activation, and the need for
C transfusions.40,41
0
Once the patient is ready to be cannulated for CPB, a
heparin bolus is administered to the patient by the anesthesi-
0 100 200 300 400
ologist into an intravenous line or by the surgeon directly
Heparin dose (U/kg)
into the right atrium. In general, the patient receives a 400 U/
Fig. 37.6. An example of a heparin dose response curve wherein the kg dose of heparin minutes before arterial cannulation. Once
patients baseline activated clotting time (ACT) is shown at point A. An the heparin has circulated within the patient for approxi-
initial heparin dose of 200 units per kilogram resulted in an ACT shown mately 5 minutes, a blood sample from an arterial or intrave-
at point B. A linear extension of points A and B is drawn with an inter-
nous line is used to run an ACT and HPT. If the ACT reaches
section at 400 (point C) and 480 seconds (point D), and these target
intersects can be used to estimate further heparin doses to administer
480 seconds or the HPT confirms an adequate heparin con-
to the patient. (From Bull BS, Huse WM, Brauer FS, Korpman RA. centration, cardiotomy pump suction may be used and CPB
Heparin therapy during extracorporeal circulation. II. The use of a dose- may be initiated when the surgeon inserts the arterial and
response curve to individualize heparin and protamine dosage. J Thorac venous cannulas. ACT and HPT tests are run every 30
Cardiovasc Surg. 1975;69:685-689.) minutes during CPB, and heparin is administered if the ACT
falls below 480 seconds or the heparin concentration falls
below the maintenance value calculated by the HDR. If a
likely because of their size and immature coagulation system. parameter is low, the Hepcon HMS PLUS uses a formula
Contributing factors to postoperative bleeding are dilution of based on the HDR, blood volume of the patient, and circuit
coagulation factors during CPB, induction of the systemic prime volume to calculate the amount of heparin needed to
inflammatory response, hematologic changes in cyanotic adequately raise the ACT or HPT.
patients, hypothermia, and numerous coagulation factor defi-
ciencies. All of these situations can inhibit adequate antico- Cannulation
agulation with heparin and ultimately lead to the generation Cannulation refers to the process in which the surgeon
of thrombin. It has been shown that prolonged ACT results of attaches the venous limb of the CPB circuit to the systemic
pediatric patients poorly correlate with the plasma levels of venous circulation of the patient, while attaching the arterial
heparin during CPB.37 Reports have shown that pediatric limb to the systemic arterial system of the patient. This is
patients require higher plasma heparin concentrations than most commonly accomplished by placing an arterial cannula
adults because they metabolize heparin faster, have a larger in the distal ascending aorta and venous cannulas in the SVC
blood volume-to-body weight ratio, and have lower ATIII and IVC, respectively. The cannulas are inserted through
levels.38,39 Weight-based heparin doses and ACT monitoring appropriately sized purse-string sutures and secured with
used with adult patients are therefore not recommended for tourniquets. This bicaval configuration allows for the achieve-
use in pediatric patients. ment of “total” CPB, and the vast majority of cardiac repairs
The potential variability of a pediatric patient’s response to can be accomplished using this technique. In pediatric cardiac
heparin necessitates an individual dosing regimen and the programs, patients ranging in weight from approximately
use of different coagulation tests. A useful bedside hemostasis 1000 gm up to adulthood are placed on CPB. Therefore a
management tool in pediatric cardiac surgery is the Hepcon wide range of cannula sizes must be kept in stock. Arterial
HMS PLUS (Medtronic Inc., Minneapolis, Minnesota, USA). cannulas range from as small as 8 French (Fr) (2.67 mm) in
The Hepcon HMS PLUS is fully automated and is used to run diameter up to over 20 Fr. Venous cannulas for CPB are avail-
the following tests: ACT, heparin dose response (HDR) to able in straight and angled varieties and range down to as
identify individual heparin needs, and heparin-protamine small as 10 Fr. Inserting these cannulas into the diminutive
titration (HPT) to verify heparin concentration. A baseline aorta and vena cavae of neonates is a taxing technical exercise
sample is collected from the arterial line before hepariniza- that must be accomplished without complication in order to
tion and is used to test the HDR. The HDR test determines appropriately support the patient during the repair and leave
the baseline ACT and patient response to increasing amounts the patient with undamaged vessels at the cannulation sites
of heparin. Results are used to identify heparin-resistant or postoperatively.
heparin-sensitive patients and determine the patient heparin Exceptions to standard bicaval cannulation are frequently
concentration needed to achieve appropriate anticoagulation. seen in pediatric practice. First, patients can have anomalies
To test the blood heparin concentration with the HPT test, of systemic venous return, such as bilateral superior vena
blood is added to tubes containing different mg/mL concen- cavae, ipsilateral hepatic veins, or interrupted inferior vena
trations of protamine. Heparin and protamine bind in a 1 : 1 cava with azygous continuation to the SVC. All these anom-
ratio, so the tube that produces a clot can be used to alies have to be assessed, and an appropriate venous

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438 Section II Cardiovascular System

cannulation strategy must be devised. Occasionally, if these


TABLE 37.4  Differences Between Adult and Pediatric
anomalies are prohibitive for selective cannulation, or the
overall patient size is so small that the cavae are too small to Cardiopulmonary Bypass
cannulate, the right atrial appendage is cannulated in isola-
tion, and periods of circulatory arrest, wherein venous Parameter Adult Pediatric
return is not required, are used as to accomplish intracar- Hypothermic Rarely below Commonly
diac portions of the repair. temperature 25°C-32°C 15°C-20°C
Alternatives to standard ascending aortic cannulation are Use of circulatory Rare Common
also used. In order to accomplish aortic arch reconstructions arrest
without resorting to circulatory arrest, a small prosthetic vas- Pump prime
cular tube graft is anastomosed to the innominate artery and
  Dilution effects on 25%-33% 150%-300%
the arterial cannula is inserted into this “chimney” graft. Alter- blood volume
natively, if the patient is large enough, the innominate artery
  Additional additives Blood, albumin
can be cannulated directly. These innominate artery cannula-
tion techniques allow the brain to be perfused up the right Perfusion pressures 50-80 mm Hg 25-50 mm Hg
carotid artery while the aortic arch is being repaired. Influence of α-stat Minimal at Marked at deep
Reoperations are common in congenital heart surgery. A versus pH-stat moderate hypothermia
number of these patients have pulmonary outflow conduits management strategy hypothermia
that are densely adherent to the sternum. Patients with trans- Measured PaCO2 30-45 mm Hg 20-80 mm Hg
position of the great arteries have an abnormally anteriorly differences
located ascending aorta that can also be adherent to the chest Glucose regulation
wall in the midline. Peripheral cannulation via a femoral   Hypoglycemia Rare—requires Common—
artery is sometimes necessary in these instances. Peripheral significant reduced hepatic
arterial cannulation in children should only be performed hepatic injury glycogen stores
when absolutely necessary and converted to the ascending   Hyperglycemia Frequent— Less common—
aorta as soon as possible. The obturation of the femoral artery generally easily rebound
by the cannula almost always causes hypoperfusion of the controlled with hypoglycemia
lower extremity. With longer cannulation times, the leg can be insulin may occur
at significant risk for ischemic complications. From Greely WJ, Cripe CC, Nathan AT. Anesthesia for pediatric cardiac surgery.
In: Miller RD, Cohen NH, Eriksson LI, et al. Miller’s Anesthesia. 8th ed.
Philadelphia: Elsevier Saunders; 2015:2820.

Cardiopulmonary Bypass
Pediatric Versus Adult Considerations and infants also differs from adults in that they have quantita-
Although many of the management techniques governing tive deficiencies of coagulation factors at baseline. These defi-
pediatric and adult CPB are similar, several differences do exist ciencies of the immature coagulation system coupled with
(Table 37.4). The small size of the pediatric patient and nature hemodilution discussed earlier result in significant postopera-
of the surgical repair often expose these patients to moderate tive coagulopathies and anemia that must be addressed with
or deep hypothermic temperatures, wide ranges of perfusion blood products much more commonly than in adult cases.
flow rates, and hemodilution. These management techniques
represent extreme shifts from normal physiologic parameters, Initiation of Cardiopulmonary Bypass
and the harmful effects are potentially more pronounced in Before starting a planned operation, the surgical team will
these small patients. Low-flow perfusion or circulatory arrest discuss the procedure and form a detailed management plan
at deep hypothermia(15°C–20°C) is often required because of for each team member. The perfusionist must understand the
the complexity of the repair, significant aortopulmonary col- type and complexity of the surgery and discuss the proper
lateral blood flow returning to the operative field from the cannula section, degree of hypothermia, myocardial protec-
pulmonary veins, or simply because position of the perfusion tion technique, and any other unique patient variables that
cannulas interferes with access to the surgical site. might affect perfusion management. The fundamental con-
Compared with adults, hemodilution of the pediatric cepts of managing CPB for congenital patients are similar to
patient during CPB has a larger impact on the concentration adults, but anatomic variations and physiologic extremes
of blood components, and the blood-to-foreign surface area complicate the approach. Once the surgical plan is established,
exposure is much greater. This relatively greater exposure to the patient is prepped and draped for the skin incision and
nonendothelialized surfaces can lead to an increased inflam- sternotomy. With the chest open, the surgeon exposes the
matory response and damage the formed elements of blood. heart and major vessels and then directs the anesthesiologist
Another important contrast between pediatric and adult to administer heparin before cannulation. Alternatively, the
support involves calcium management. The immature myo- surgeon can administer the heparin directly to the right
cardium is susceptible to exacerbated postischemic injury due atrium. Next, the arterial and venous cannulas are inserted,
to overly rapid calcium “loading” at reperfusion.42,43 Because but before it is safe to initiate CPB, it is important to confirm
of this, at Children’s Health Dallas a perfusate that is relatively an adequate anticoagulation level by obtaining an ACT and
depleted of calcium is used until well after cross-clamp heparin concentration and that the arterial cannula is unob-
removal. Calcium is restored in a stepwise fashion before structed. Congenital patients, especially cyanotic patients,
weaning from the circuit. The coagulation system of neonates often demonstrate variable dose responses to heparin. In

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Pediatric Cardiopulmonary Bypass Chapter 37 439

addition, it cannot be assumed that the CPB dose of heparin


TABLE 37.5  Recommended Pump Flow Rates at Normothermia
is circulating in the patient, as intravenous line malfunction
can occur. Initiating CPB on a pediatric patient with an
Patient Weight (kg) Pump Flow Rate (mL/kg/min)
obstructed aortic cannula could quickly exsanguinate the
patient, as venous drainage commences without return of this <3 150-200
blood to the patient, and cause severe hypotension. Once these 3-10 125-175
two safety checks are complete, the patient is ready for CPB. 10-15 120-150
The venous line is unclamped to siphon deoxygenated
blood from the patient, and the arterial flow is slowly increased 15-30 100-120
as the heart begins to empty. Bicaval cannulation is often used 30-50 75-100
in congenital surgery, but it is common to initiate CPB with >50 50-75
only one cannula. This is done to verify adequate drainage
from one cannula, as it would be difficult diagnose poor From Jaggers J, Shearer IR, Ungerleider RM. Cardiopulmonary bypass in
infants and children. In: Gravelee GP, Davis RF, Kuruz M, Utley JR. Cardiopul-
drainage from a single cannula if both were open. Once both monary Bypass Principles and Practice. 2nd ed. Philadelphia: Lippincott
cannulas are open, adequate venous drainage is confirmed Williams and Wilkins; 2000:637.
when the central venous pressure (CVP) falls to zero and the
SVC, IVC, and right atrium are collapsed. Total (also termed
full or complete) CPB is achieved when all of the systemic m2. Factors such as the degree of hypothermia, acid-base
venous blood is being diverted to the heart lung machine and balance, depth of anesthesia and neuromuscular blockade,
full arterial flow can be achieved. When on total CPB, the hematocrit, venous saturation, lactate level, urine output, and
mean arterial pressure (MAP) and CVP should confirm that near infrared saturation trends are used to guide perfusion
the heart is empty by showing a flat tracing. However, the flow rates. Patient temperature is the greatest factor affecting
many anatomic variations of the congenital patient can lead perfusion flow, and rates as low as 40–50 mL/kg are routinely
to blood returning to the heart despite adequate drainage. used at core temperatures in the 20°C range.
Variations such as a patent ductus arteriosus, major aortopul-
monary collateral arteries, an unrecognized left SVC draining Arterial Pressure
to the coronary sinus, and aortic insufficiency can return The MAP will slowly lose its pulsatile trace and flatten out as
blood to the heart and should be considered when evaluating the heart empties on CPB. Though the MAP is calculated by
venous drainage. Assessment of adequate venous drainage and factoring the systolic and diastolic pressures, the value of the
perfusion flow rate is critical before proceeding to the surgical flat tracing is referred to as the MAP and perfusion pressure
repair. A poorly positioned venous or arterial cannula can during CPB. The transition to CPB often leads to hypotension,
restrict optimal perfusion flow and addressing this issue and in contrast to adult cases, vasopressors (eg, phenyleph-
during the aortic cross-clamp period would waste unneces- rine) are not typically administered in the early phase of CPB
sary myocardial ischemic time. Once cannula placement is in young patients. The goal in the early phase of CPB is to cool
deemed acceptable, full perfusion flow is achieved, and oxy- the patient and reduce the metabolic demands. Low perfusion
genation from the oxygenator is confirmed, the anesthesiolo- pressure, 20–30 mm Hg, is accepted during the cooling phase,
gist can turn off the ventilator. and vasodilators (eg, phentolamine) are actually used to
reduce arterial tone and increase uniformity of perfusion and
Determining and Monitoring Effective Perfusion Flow Rate improve cooling. Vasodilation has been shown to improve
The fundamental goals of bypass are to meet the metabolic temperature distribution and reduce lactate production in
demands of all tissues and to attenuate the deleterious patho- pediatric deep hypothermic CPB.44 Hemodilution, hypocalce-
physiologic effects of artificially supporting a patient. Once mia, and the inflammatory response are also factors that cause
the patient is transitioned to cardiopulmonary bypass, several hypotension at the onset of CPB. Hemodilution will lower the
management techniques are used to safely optimize the level perfusion pressure because of the viscosity reduction, and
of support. Perfusion flow rate, which represents the cardiac hemoconcentration performed by the perfusionist with the
output during CPB, is altered to meet the oxygen consump- hemoconcentrator can easily increase perfusion pressure by
tion needs of the patient. Global adequacy of flow is estimated raising the hematocrit. The systemic inflammatory response
in real time by the display of oxygen saturation by a sensor in is triggered by the foreign surface contact of blood and bypass
the venous return line. Assessing regional oxygen consump- circuitry. This response releases many vasoactive mediators,
tion to the brain, kidneys, or bowel, for instance, is a challenge. which can quickly drop the perfusion pressure, and highlights
Additionally, due to age-related differences of body surface the importance of minimizing circuit surface area for the
area (BSA)-to-blood volume ratios, flow rate indexes are pediatric patient. The decrease in pressure in an adult patient
higher in neonates than adults. The optimal effective flow rate with coronary or carotid stenosis would likely be treated with
or cardiac index for any size patient remains unclear; however, a vasopressor, while increasing perfusion flow is the preferred
recommended flow ranges at normothermia are listed in method in young patients.
Table 37.5. Considering that the perfusion flow rate is not
fixed during the different phases of CPB, several variables are Arterial and Venous Oxygen Saturation
helpful in determining a safe minimal rate. Initial normother- Most oxygen saturation monitoring techniques are noninva-
mic target rates are calculated for CPB initiation by weight sive and inexpensive and can be used in real time. Changing
and BSA. At Children’s Health Dallas, in addition to the CPB perfusion flow rate and oxygen delivery will have immediate
initiation flow rates calculated by weight, the perfusionist cal- and direct effects on oxygen saturation levels. Pulse oximetry
culates several cardiac indexes ranging from 2.4–3.0 L/min/ is a clinical mainstay and is used to monitor oxygen delivery

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440 Section II Cardiovascular System

to the extremities during the preoperative and postoperative Near-Infrared Spectroscopy


periods. However, due to the nonpulsatile flow pattern gener- Near-infrared spectroscopy (NIRS) is a noninvasive optical
ated during CPB, this technology is ineffective. sensor that can measure cerebral and somatic tissue oxygen-
As mentioned earlier, a mainstay monitoring technique ation. NIRS monitoring is gaining considerable popularity
during CPB is to track the oxygen saturation of the venous line because sensor pads may be placed over various regional tissue
blood draining into the venous reservoir. As a general guide- beds, particularly both cerebral hemispheres, and display real-
line, perfusion flow rate is adjusted to maintain this mixed time results. Somatic monitoring sites such as flank, abdomi-
venous saturation (SvO2) greater than 70%. While this guide- nal, and muscle are suggested to help broaden the assessment
line is helpful during “normal” physiologic conditions, the of systemic hypoperfusion. The technology works by bounc-
many nonphysiologic variables of CPB cause shifts in the oxy- ing various wavelength arcs of near-infrared light from a
hemoglobin dissociation curve (Fig. 37.7), and these venous sensor emitter and detector. These photodetectors allow for
oxygen saturations may fail to represent satisfactory oxygen selective measurement of tissue oxygenation. This technology
delivery to the tissues. Leftward shifts in the curve prevent is widely used in the operating room and ICU, although inter-
oxygen from being released from hemoglobin, which could preting the results has been a topic of debate. A validation
deceivingly demonstrate an acceptable SvO2 in the presence of study performed at Children’s Health Dallas demonstrated
tissue hypoxia. As the patient is cooled during CPB, regional that cerebral NIRS values accurately predicted the oxygen
deoxygenation has been shown to occur despite a normal or saturation in the SVC on CPB and that flank NIRS values were
rising SvO2 without increasing perfusion flow rate.45 Hypo- significantly associated with IVC saturation.47 As increasing
thermia not only strengthens the hemoglobin oxygen affinity evidence validates tissue oximetry against invasive measure-
but also creates an alkaline blood pH and causes a further left- ments, NIRS monitoring has shown its value in quickly detect-
ward oxyhemoglobin shift. In this situation, it is important to ing regional low flow.48-51 At Children’s Health Dallas, the
cool and warm patients methodically to minimize the tem- perfusionists use NIRS trends and values to guide perfusion
perature gradient between the blood and tissues and maintain flow rate, hematocrit, blood gas strategy, temperature, and
perfusion flow so that the SvO2 does not trend downward. As vasomotor tone (Box 37.1). It is important to note that NIRS
tissue temperature decreases, venous line SvO2 can be used to helps to guide rather than dictate perfusion management. The
help guide the reduction of perfusion flow rate. upper limits and critical lower values reported by NIRS are
It is especially critical to meet the metabolic needs of brain poorly defined.
tissue, and global SvO2 values may misrepresent regional Considering the regional venous oxygen saturation varia-
oxygen consumption. It has been shown that considerable tions of the neonatal and infant cardiac surgical patients,
regional differences exist and SvO2 can overestimate regional NIRS provides valuable information and early detection of
saturations from the brain.46 The majority of venous blood poor perfusion in critical organs. NIRS has been particularly
analyzed by the venous line SvO2 comes from IVC cannula, useful as a real-time monitor on patients with hypoplastic left
and IVC saturation is notoriously misleading as an estimate heart syndrome, for example. Cerebral oxygen saturation
of oxygen consumption due to “contamination” by highly measured after stage I palliation has been shown to strongly
saturated renal venous blood. Though the SvO2 does have its correlate with hemodynamic parameters and help to identify
place in guiding perfusion flow rate, more specific, regional early postoperative complications.49,52 Hoffman et al.53 found
oxygenation assessment is currently recommended to ensure that avoiding cerebral hypoxia with the use of NIRS monitor-
adequate perfusion flow distribution, particularly for the ing was the most significant factor in improving childhood
brain. neurodevelopmental outcomes.
Methods to Optimize Physiologic Management
100
Left shift: Target Hematocrit and Ultrafiltration
Decreased 2,3-DPG Despite the progress of reducing the pediatric circuit prime
90
Fetal hemoglobin
Hypothermia
volume, hemodilution during CPB remains difficult to avoid.54
80 Hemodilution can cause edema, coagulopathy, blood and
Hypocarbia
Alkalosis colloid osmotic pressure reduction, and the need to transfuse
70
Oxygen saturation (%)

blood products. Blood product transfusion is the most


60 straightforward solution to address these complications;
Right shift:
Increased 2,3-DPG
however, the risk-benefit assessment of blood transfusion
50 Hyperthermia must be considered. Transfusion-related complications
Hypercarbia include increased postoperative morbidity and mortality, pro-
40 Acidosis longed mechanical ventilation and hospital stay, exacerbation
30
BOX 37.1 Methods to Improve Cerebral Near-Infrared
20
Spectroscopy Values
10
Increase perfusion flow rate
Increase hematocrit
pH-stat blood gas strategy
0 10 20 30 40 50 60 70 80 90 100 Decrease temperature
pO2 (mm Hg) Increase mean arterial pressure; vasopressor
Verify adequate superior vena cava drainage
Fig. 37.7. The oxyhemoglobin dissociation curve.

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Pediatric Cardiopulmonary Bypass Chapter 37 441

of the inflammatory response, and infection.55-58 Modern recirculated through the hemoconcentrator, and volume is
blood bank testing and donor screening have significantly removed until the reservoir is almost empty. The circuit prime
reduced infectious complications, but noninfectious risk is then “washed” by adding approximately 500 mL of Plasma-
remains a major concern. In addition, smaller patients are Lyte A and then removing that volume. This process is known
exposed to a higher transfusion risk because the transfusion as prebypass ultrafiltration (Pre-BUF), and in addition to con-
effects may be more pronounced than in adult patients. With centrating the circuit, Pre-BUF has been shown to reduce high
a goal of minimizing hemodilution and donor blood expo- potassium, glucose, lactate, citrate, and bradykinin levels
sure, the cardiac team must implement a transfusion algo- found in packed red blood cells. CUF is then performed
rithm and define a target hematocrit during CPB. Perioperative during the early phase of CPB to remove any excess circuit
and developmental outcomes data reported from clinical trials volume and maintain a hematocrit of 30%. During the
at the Boston Children’s Hospital demonstrate that, when warming phase of CPB the perfusionist performs CUF and
compared with target CPB hematocrit values of 30%, hema- ZBUF. This combined ultrafiltration strategy, coupled with a
tocrit values at or below 20% are associated with adverse out- miniaturized circuit, allows the perfusionist to filter the blood
comes and that the benefits of hematocrit values higher than and exceed or meet baseline hematocrit values before weaning
25% should be further investigated.59,60 The protocol at Chil- from CPB.
dren’s Health Dallas is to maintain a hematocrit of at least 30%
during cardiopulmonary bypass. Hypothermia
Reducing circuit prime volume and incorporating an ultra- DHCA versus SCP
filtration device can significantly reduce donor blood expo- The therapeutic potential of hypothermia has been known for
sure. Originally, a hemoconcentrator added to the CPB circuit centuries and has been routinely used in cardiac surgery since
was used primarily to remove plasma water and raise the its inception. This concept relies on the fundamental physio-
hematocrit. This process is referred to as “conventional ultra- logic relationship between oxygen consumption and tempera-
filtration” (CUF) and its initial use was met with a few limita- ture. In 1950, Bigelow and colleagues62 compared the use of
tions. Perfusionists were accustomed to using diuretics to help normothermia and topical cooling on dogs and reported
increase the hematocrit; however, this strategy offered little superior ischemic tolerance by surface cooling after 15 minutes
control and required adequate kidney perfusion during CPB. of circulatory arrest. The first clinical application in cardiac
When ultrafiltration emerged as a CPB technique in the mid- surgery was reported 1953 by Lewis and Taufic, who described
1980s, hemoconcentrators were viewed as an expensive option the successful repair of an ASD in a 5-year-old girl using
for removing excess circuit volume. Also, while fluid is removed topical cooling and total body hypothermia.2 To achieve this,
from the circuit during CUF, the volume in the venous reser- patients were submerged in an ice bath to reduce their tem-
voir level diminishes and CUF must be stopped before the perature to approximately 28°C, and then the defect was
reservoir is emptied, which would have catastrophic conse- closed with the aid of inflow occlusion. In 1958, Sealy and
quences. Thus the amount of fluid removed during CUF is colleagues63 successfully reported the use of hypothermia in
dependent on available volume in the venous reservoir, which conjunction with CPB. The use of CPB with various degrees
limits the ability to effectively raise the hematocrit. In 1991 a of hypothermia or deep hypothermic circulatory arrest
report described a modified ultrafiltration (MUF) technique (DHCA) dramatically increased the “safe” period of support,
performed after weaning the patient from CPB and enabled which enabled surgeons to repair increasingly complex anom-
the perfusionist to concentrate the entire circuit and return alies, and allowed cardiac surgery to flourish.
most of that volume back to the patient.61 During this era, Hypothermia suppresses metabolic activity, preserves high
pediatric circuit prime volume was still rather high and energy phosphate stores, and reduces the reaction rate of bio-
various MUF techniques proved to be a valuable resource for chemical reactions. Several factors are used in determining the
reducing total body water post CPB. The popularity of MUF type and degree of hypothermia during CPB. The most sig-
led investigators to explore the potential reduction of proin- nificant factor is the degree of surgical difficulty and antici-
flammatory mediators during ultrafiltration. An additional pated CPB support time. Complex surgical repairs requiring
ultrafiltration technique, zero balance ultrafiltration (ZBUF), lengthy support times would benefit from more pronounced
emerged as alternate method to attenuate the inflammatory hypothermia. The degree of hypothermia varies greatly and is
response. ZBUF is performed by removing the ultrafiltration typically classified as mild, moderate, deep, and profound
effluent from the circuit during CPB while administering a hypothermia (Table 37.6). Deep hypothermia might be seen
replacement solution (eg, Plasma-Lyte A) to the venous reser- as desirable when low flow (≤50 mL/kg/min) or DHCA is
voir in a 1 : 1 ratio. The high-volume filtration of fluid that is desired, as is the case in operations involving complete aortic
able to be exchanged allows the perfusionist to control elec- arch reconstruction. Circulatory arrest is a process in which
trolyte and glucose levels (eg, high potassium levels after deliv-
ering cardioplegia) and more effectively remove inflammatory
mediators. The increasing acceptance of ultrafiltration during TABLE 37.6  Hypothermia Classifications in Cardiac Surgery
CPB led to developing many technique variations during the
preoperative, perioperative, and postoperative phases and can Category Core Temperature
thus be categorized into two groups: blood concentration and
Mild 32-34°C
blood filtration.
In preparation for neonatal CPB at Children’s Health Dallas, Moderate 25-32°C
for example, the perfusionist adds approximately 300 mL of Deep 15-25°C
packed red blood cells to the venous reservoir after priming Profound ≤15°C
the circuit with Plasma-Lyte A. The circuit volume is

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442 Section II Cardiovascular System

150 perfusion (ACP, also known as selective cerebral perfusion)


Oxygen consumption (mL· min–1· m–2)

37 °C
uses a cannulation technique that directs perfusion flow to
only the brain with the theoretic advantage of protecting it
from hypoxic ischemic injury. Though this technique has been
100 adopted among many surgical centers, investigations compar-
ing DHCA and ACP have failed to definitively demonstrate
30 °C superiority of either technique64-66 and not all of this work is
focused only on neurologic issues. Recent literature has sug-
50 25 °C gested that during ACP, the resultant partial perfusion from
20 °C collateral vessels provides better protection to abdominal
15 °C organs than DHCA.67,68 In addition, the ideal temperature
during ACP remains unknown. Recent reports, however, have
0 demonstrated superior results using moderate to mild hypo-
0.0 0.5 1.0 1.5 2.0 2.5 thermia, in the 25°C–30°C range, rather than deep levels of
Perfusion flow rate (I· min–1· m–2) hypothermia.69,70 Considering the coagulopathy, inflamma-
tory response, as well as the vascular and organ dysfunction
Fig. 37.8. Nomogram relating oxygen consumption (VO2) to perfusion
flow rate and temperature. (From Kirklin JW, Barratt-Boyes BG. Hypo-
associated with deep hypothermia, a lesser degree of hypo-
thermia, circulatory arrest, and cardiopulmonary bypass. In: Kirklin JW, thermia may provide superior clinical outcomes.
Barratt-Boyes BG, eds. Cardiac Surgery. 2nd ed. New York, NY:
Churchill-Livingstone; 1993:91.) pH and PaCO2 Strategy
CO2 concentration and pH are primary determinants of cere-
bral blood flow. As body temperature decreases, the solubility
the perfusion flow is turned off and the patient’s blood volume of CO2 increases, resulting in a decreased PaCO2 and increased
is allowed to drain into the venous reservoir. This dramatic pH. Manipulating the acid-base management during hypo-
application provides an asanguineous and completely motion- thermic bypass can be classified by two mechanisms of control:
less surgical field, facilitating complex repairs. In very small alpha-stat or pH-stat. Both mechanisms have been studied
patients, the venous cannula may be obstructive and DHCA intensely in reptiles (ectotherms) and hibernating mammals
is required in order to remove the cannula and access the (endotherms), looking at their adaptive blood pH alterations
surgical site. Hypothermia also facilitates exposure of the sur- that allow them to withstand extreme temperature fluctua-
gical field by allowing decreased perfusion flow rates, which tions. pH-stat acid-base management is practiced by hibernat-
reduces the amount of collateral blood returning to the heart ing mammals and is accomplished by decreasing ventilatory
via the pulmonary veins (Fig. 37.8). Patients with pulmonary rate and raising PaCO2 while maintaining a constant pH
blood flow restrictions (eg, tetralogy of Fallot, pulmonary during hypothermic conditions. Maintaining pH, while
atresia) can develop major aortopulmonary collateral arteries, varying temperature during hibernation, is thought to pre-
and these collaterals can flood the heart during the aortic serve oxygen stores by decreasing metabolic activity. Alterna-
cross-clamp period if CPB flow is maintained. This excessive tively, reptiles use alpha-stat and allow their pH to enter an
blood return not only obscures the surgical site but may also alkaline state by reducing PaCO2.
warm the cold arrested myocardium or wash out cardioplegia The perfusionist can maintain a pH-stat or “temperature-
from the coronary arteries. Hypothermia and perfusion flow corrected” acid-base strategy during CPB by allowing CO2 to
rate reduction can attenuate this collateral flow while main- passively rise or actually adding CO2 to the CPB circuit. Cere-
taining adequate oxygenation delivery to the patient. bral blood flow has been shown to decrease during hypother-
The rate of cooling varies greatly between different tissue mia using the alpha-stat strategy and demonstrates a linear
beds; thus multiple measurement sites are recommended to relationship to the increased PaCO2 when a pH-stat strategy
ensure uniform cooling distribution (Fig. 37.9). The optimal is used.71 In addition to preserving cerebral blood flow during
temperature measurement site is controversial, but choosing hypothermia, a pH-stat strategy induces a rightward shift of
sites that closely reflect tissue temperatures of vital organs, the oxyhemoglobin dissociation curve (see Fig. 37.7) poten-
particularly the brain, is widely accepted. At Children’s Health tially allowing for increased oxygen “off-loading” from hemo-
Dallas, the patient’s nasopharyngeal, rectal, and bladder tem- globin at the capillary level.
peratures are monitored during surgery. Nasopharyngeal Whether pH-stat or alpha-stat acid-base management
closely correlates with brain temperature; however, it may during hypothermic CPB demonstrates clear benefits on clini-
underestimate the global core temperature considering the cal outcomes has been difficult to demonstrate. However, it is
slower cooling rates of other tissue beds. For this reason, rectal suggested that a pH-stat strategy is optimal for pediatric
and bladder temperature monitoring sites with slower rates of patients and alpha-stat is the optimal strategy on adult
cooling are typically used to guide cooling end points. patients.72
The concept of a “safe” circulatory arrest time is controver-
sial, and most guidelines are met with a degree of uncertainty. Myocardial Protection
A nomogram focused on neurologic protection has been Myocardial protection refers to the strategies and techniques
devised that estimates “safe” circulatory arrest times, but employed to allow for the surgeon to work on the heart in a
values should not be used as absolutes (Fig. 37.10). The his- bloodless and motionless field yet recover the best possible
toric incidence of perioperative cerebral injury during DHCA postischemic myocardial function and cardiac output for the
has led investigators to explore alternative techniques to patient. Strategies for both adults and children attempt to
protect the brain during complex repairs. Antegrade cerebral reduce the cardiac workload and minimize the metabolic

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Pediatric Cardiopulmonary Bypass Chapter 37 443

Cooling Rewarming

41
40
39
38
37
36
35
34
33
32
31
30
29
28
27 Temperature (± SEM)
26 Arterial cannula
25 Myocardial
° Centigrade

24
Brain
23
Nasopharyngeal
22
21 Rectal

20 p < 0.05
19
18
17
16
15
14
13
12
11
10
9
8
7
6
5
0 10 20 30 40 10 20 30 40 50 60 70 80 90
Time (minutes)
Fig. 37.9. Relationships of temperatures measured at various sites over time during cooling and warming from cardiopulmonary bypass. (From
Stefaniszyn HJ, Novick RJ, Keith FM, et al. Is the brain adequately cooled during deep hypothermic cardiopulmonary bypass? Curr Surg
1983;40:294-297.)

demands and consequences of oxygen deprivation during protect the myocardium, but cardioplegia strategies are often
ischemia. Reducing afterload and emptying the heart by ini- the focus when discussing protection techniques. In North
tiating CPB is a myocardial protection technique during America, high potassium depolarizing solutions are the most
beating heart procedures (eg, palliative shunts, bidirectional common type of cardioplegia used.34 Universal agreement on
Glenn procedure). When the heart needs to be stopped and an optimal myocardial protective technique is widely debated,
opened for intracardiac repairs, the aorta is cross-clamped and and most strategies are guided by surgeon or institutional
cardioplegia is delivered to the coronary circulation to cause preference.
prolonged asystole. Cardioplegia is a myocardial arrest- After the first successful cardiac surgical correction using
producing solution that is formulated to prolong the myocar- CPB in 1953, surgeons explored a variety of techniques to
dial tolerance to ischemia. There are many techniques to support the patient during the repair. It did not take long for

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444 Section II Cardiovascular System

1.0
TABLE 37.7  Crystalloid Formula of del Nido Solution
Probability of “safe” circulatory arrest

0.9
0.8 Plasma-Lyte A* 1000 mL
0.7 K-Cl 26 mEq
0.6 Na-HCO3 8.4% 13 mEq
0.5
Mannitol 25% 3.25 g
37°C 28°C 18°C
0.4
Lidocaine 2% 130 mg
0.3
Mg-SO4 50% 2 g
0.2
*Baxter Healthcare Corporation, Deerfield, Illinois, USA.
0.1
0.0
0 10 20 30 40 50 60 70 80 90
Duration of total circulatory arrest (minutes)
Fig. 37.10. Probability of a safe (absence of structural or functional magnesium, procaine, lidocaine, mannitol, buffering agents,
damage) circulatory arrest according to duration. Estimate at nasopha- glucose, glutamate, and aspartate has led to much debate and
ryngeal temperatures of 37°C, 28°C, and 18°C. (From Kirklin JW, a plethora of cardioplegia recipes. By the late 1970s, these
Barratt-Boyes BG. Hypothermia, circulatory arrest, and cardiopulmonary crystalloid cardioplegia solutions became the dominant form
bypass. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac Surgery. 2nd ed. of myocardial protection. A major shift occurred in 1978 when
New York, NY: Churchill-Livingstone; 1993:74.) Dr. Buckberg and his group at the University of California,
Los Angeles, suggested that blood was an optimal cardioplegia
vehicle.75 Blood cardioplegia was shown to be superior to a
them to realize that the poor visibility in an open beating heart crystalloid cardioplegia solution because blood provides better
needed to be addressed. It was difficult to operate on a beating oxygen delivery, effective buffering, free radical scavenging,
heart, and lethal air embolism (ie, air ejected out the aortic and ideal oncotic pressure.76 Effective myocardial protection
valve) claimed the lives of many patients. In 1955, Dr. Melrose has allowed surgeons to approach increasingly complex surgi-
and his colleagues73 described a technique of stopping the cal corrections. A consequence of the motivation to optimize
heart to address these dangerous operative conditions. The cardioplegia techniques has led to an incredible variation of
report outlines how potassium citrate is used to achieve elec- myocardial protection techniques. Despite numerous advances
tive cardiac arrest. This sparked the interest of other investiga- and an extensive body of research, myocardial protection tech-
tors, and in 1958 Dr. Sealy and his colleagues at Duke reported niques continue to be largely program based because hard
an additive modification to the Melrose method and coined evidence, from prospective randomized trials, for instance, is
the term cardioplegia.74 It is interesting to note that improving lacking.77
operative visibility, not protecting the heart, was the goal of A depolarizing cardioplegia solution uses a high dose of
arresting the heart. Further investigation began to show that potassium, delivered to the coronary circulation in isolation,
these techniques caused damage to the myocardium and car- to decrease the cardiac membrane resting potential and arrest
dioplegia was abandoned. In the 1960s and early 1970s a the heart in diastole. Delivering a cold dose of depolarizing
number of investigators tried to find an alternative protection cardioplegia does add a degree of protection, but simply
technique. Reports of using direct coronary perfusion with arresting and cooling the heart does not offer optimal protec-
intermittent aortic occlusion, topical hypothermia, and nor- tion. An effective cardioplegia solution should (1) achieve
mothermic ischemia with aortic occlusion failed to achieve quick arrest and minimize ATP depletion, (2) delay the onset
consistent results, presented major time limitations for sur- of irreversible damage and limit reperfusion injury, (3) be
geons, and myocardial damage remained an issue. The obser- reversible with prompt resumption of cardiac function upon
vation of “stone heart” by Dr. Cooley and his colleagues74 washout, and (4) be nontoxic.78 Efforts to optimize an effective
highlighted the need to prioritize the protection of the myo- cardioplegia solution have focused on myocyte calcium man-
cardium, and they proposed that depleting myocardium ATP agement and pH buffering. Modified depolarizing cardiople-
stores would leave the heart frozen in systole. Fortunately, gia, a solution that combines a depolarizing agent with
several European researchers continued to explore cardiople- additional membrane stabilizing additives (eg, magnesium or
gia solutions throughout the 1960s. They discovered that the lidocaine), has gained interest in attempting to optimize car-
chelating action of the citrate ion of the potassium-citrate dioplegia. A modified depolarizing solution that is gaining
solution was responsible for myocardial damage because it popularity in congenital surgery is del Nido cardioplegia
interferes with cellular calcium and magnesium traffic. This (Compass; Baxter Healthcare Inc., Edison, New Jersey, USA)
finding resparked interest into pharmacologic cardiac arrest (Table 37.7).79 Unlike most cardioplegia solutions that require
around the world, with a new focus of protecting the myocar- frequent maintenance dosing to achieve effect protection, del
dium by membrane stabilization and managing calcium and Nido cardioplegia is known to provide excellent myocardial
other ion shifts. During the 1970s, a sequence of landmark protection without the need to frequently redose.80 This
reports hailed the worldwide return of cardioplegia and its use reported advantage allows the surgeon to operate uninter-
in protecting the myocardium. These publications identified rupted while reducing the aortic cross-clamp time. Custodiol
potassium-chloride as a safe arresting agent and, moreover, HTK (Essential Pharmaceuticals, LLC, Newton, Pennsylvania,
showed that cold cardioplegia significantly extends the safe USA) is an intracellular cardioplegia that achieves electrical
ischemic period. The use of cardioplegia additives such as and mechanical arrest by equilibrating the intracellular and

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Pediatric Cardiopulmonary Bypass Chapter 37 445

extracellular ion concentrations. This solution is also gaining postoperative morbidity seen in neonates compared with
popularity in the congenital patient population because it older infants and children.86-88
does not require frequent maintenance doses. A number of contemporary pharmacologic strategies and
At Children’s Health Dallas, the surgical team uses del Nido CPB techniques are designed to attenuate the inflammatory
cardioplegia with a customized pediatric cardioplegia circuit.81 reactions and remove mediators during CPB. Corticosteroids
The cardioplegia is mixed at a 1 : 4 blood-to-crystalloid ratio, have been used during CPB for many years and have been
and upon aortic cross-clamp placement, a single, cold (4°C– shown to mitigate many inflammatory processes such as
6°C), 20 mL/kg antegrade dose is administered via the aortic increased capillary permeability, edema, and leukocyte migra-
root. Although there is no cardioplegia specifically designed tion. Various timing and dosing protocols have been suggested
for neonatal or congenital patients, several considerations are with conflicting results.89,90 At Children’s Health Dallas a
made to optimize protection. As previously discussed, toler- 30-mg/kg (1-g maximum dose) perioperative dose of methyl-
ance of intracellular calcium shifts into the immature myocar- prednisolone is administered to the CPB circuit prime. Addi-
dium is impaired. The additives lidocaine and magnesium in tionally, 10 mg/kg of methylprednisolone is administered
del Nido cardioplegia both help to control intracellular 8–12 hours before the initiation of CPB in neonates. Despite
calcium accumulation. Lidocaine prevents sodium shifts by the large variability of preoperative and perioperative cortico-
blocking fast voltage sodium channels, which in turn limits steroid administration in clinical use, the literature generally
calcium shifting into the myocyte. Magnesium, a calcium demonstrates the benefit of CPB-induced systemic inflamma-
antagonist, inhibits calcium channel pumps and competes tory response attenuation.91-93
with calcium binding to troponin. These additives help the Common CPB-based techniques to attenuate SIRS include
impaired immature myocardium to maintain effective electri- ultrafiltration, circuit miniaturization, and the use of biocom-
cal and mechanical arrest. Hypertrophic ventricles, as seen in patible circuit coatings. Ultrafiltration during CPB has been
tetralogy of Fallot, may not be adequately protected with stan- shown to reduce inflammatory mediators, pulmonary injury
dard cardioplegia doses and may require a higher cardioplegia and edema, postoperative mechanical ventilation support
dose and longer delivery time to properly cool and perfuse the time, and the perioperative need for blood transfusion.94-97
hypertrophied myocardium. Cyanotic patients with inade- CPB circuit miniaturization and the use of biocompatible
quate pulmonary blood flow often have increased bronchial surface coatings provide two main benefits in attenuating the
collateral flow. High collateral flow to the lungs ultimately inflammatory response. First, the smaller surface area of a
returns to the heart via the pulmonary veins and is undesired miniaturized circuit and biocompatible coating reduces the
while the aorta is cross-clamped. Collateral flow can fill the bioreactivity of blood coming into contact with foreign sur-
heart, warm the myocardium, and wash out the cardioplegia faces.32,33 Second, smaller circuits reduce overall hemodilution
from the myocardium. Lowering the systemic temperature not and the need to transfuse donor blood, both of which have
only helps to offset myocardial warming but also allows the been shown to exacerbate the inflammatory response.55,56
perfusionist to lower the perfusion flow and pressure, which Although the inflammatory response to CPB cannot be
in turn reduces this collateral flow. Also, adequate LV venting avoided, a multimodal approach can significantly reduce
helps to minimize the effect of volume returning to the heart. CPB-related SIRS.
High sensitivity to the inflammatory response in the imma-
ture myocardium can cause edema and reduce ventricular Termination of CPB
compliance. Mannitol is an additive in del Nido cardioplegia Once the surgeon has completed the cardiac repair, the entry
and helps reduce intracellular water accumulation. Care must sites into the heart are sutured closed. The venous return
be taken to not perfuse the myocardium at too high a pressure. drainage is retarded, which fills the right heart. The anesthe-
This could injure fragile coronary vessels and create myocar- siologist inflates the lungs, which facilitates the movement of
dial edema. A pressure of 30–50 mm Hg has shown to be this blood across the pulmonary vasculature and back to the
adequate.82 left heart. Any active left heart venting is paused at this point,
and the surgeon massages the filling heart to expel blood
Inflammatory Response to CPB with any entrained air, out of a vent hole in the aortic root.
The systemic inflammatory response syndrome (SIRS) insti- Once there is no longer any air emanating from this site, the
gated by CPB is well documented, and limiting this response patient is placed into the Trendelenburg position and the
is associated with improved outcomes.83-85 Multiple factors cross clamp is removed from the aorta. The perfusionist then
including blood exposure to nonendothelialized surfaces (eg, returns to full venous drainage, and the left heart vent can be
CPB circuit, air); surgical trauma (eg, intubation, sternotomy); placed to gentle suction again. The coronary circulation is
ischemia-reperfusion; hypothermia; and allogenic transfusion now reperfused, washing out the cardioplegia. The heart is
have been linked to this inflammatory activation. This complex observed for any return of electrical activity. Temporary epi-
response includes cascade activations of complement, cyto- cardial pacing wires are routinely placed, and pacing is initi-
kine, coagulation-fibrinolytic, and various cellular activations. ated if the native rhythm does not promptly return. The left
Perioperative and postoperative consequences include multi- heart vent is removed before initiating ventilation to avoid
ple organ failure (eg, myocardium, renal, pulmonary, neuro- entrainment air. Transesophageal echocardiography is then
logic, hepatic), coagulopathy, edema, elaboration of injurious used to evaluate for the presence of air in the left heart as
oxygen-free radicals, and hypotension.85 ventilation is restarted. If air is present, the vent site in the
The SIRS is more pronounced in neonates; the degree of ascending aorta is kept open to evacuate it. Once the heart is
hemodilution, the need for longer CPB and ischemic times, confidently de-aired and the patient has returned to normo-
and the more prevalent use of profound hypothermia are all thermia, the team agrees to wean from CPB. The perfusionist
known to exacerbate SIRS and are contributors to the increased steadily reduces pump flow and venous return until the

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446 Section II Cardiovascular System

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Pediatric Cardiopulmonary Bypass Chapter 37  446.e1

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