MIXED BLOOD FLOW DEFECTS BLOOD FLOW PATTERN

TRANSPOSITION OF THE GREAT VESSELS Pulmonary artery leaves L ventricle, aorta exits R ventricle NO communication systemic & pulmonary circulations

TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION Pulmonary veins fail to join L Atrium & empty into R Atrium Mixed blood returned to R Atrium & shunted right to left thru ASD

TRUNCUS ARTERIOSIS Single large vessel instead of aorta & pulmonary artery blood from both ventricles mix in great vessel blood flows to lungs or aorta

HYPOPLASTIC LEFT HEART SYNDROME Inadequate development of L side of heart results in a hypoplastic L ventricle and aortic atresia one effective ventricle (R) blood flows from L atrium across patent foramen ovale to R ventricle. The descending aorta gets blood from the PDA. n/a -If s/s not present @ birth, will be in 1st wk. -Mild cyanosis & CHF until PDA closes. -Deterioration with cyanosis and decreased cardiac output. -Cardiovascular collapse Echo

MURMUR SYMPTOMS

n/a Cyanosis **Hypoxemia w/ minimal response to O2 Tachypnea (>60 rpm) Poor feeding Cardiomegaly CXR (**egg-shaped heart) Echo Prostaglandin E1 Cardiac Cath (balloon) Surgical: arterial switch Intraatrial baffle Rastelli procedure **Life-threatening at birth Assoc. defects: (rqd for survival & mixing of circ) Patent FO

n/a Cyanosis (early) CHF Sx w/ pulmonary vascular resistance

Holosystolic @ left sternal border Variable cyanosis Mod severe CHF Poor growth Activity intolerance

DIAGNOSIS

TREATMENT

CXR (Right-sided hypertrophy) Echo **Surgery early in infancy

CXR Echo Pharm:

-

Dig for CHF Sx Surgical repair of all defects

MISCELLANEOUS

**Rare

VSD usually present Higher mortality rate (>10%)

Mechanical Ventilation Prostaglandin E1 Surgery: 3 stages 1. Norwood proc. 2. Bidirectional Glenn Shunt 3. Fontan proc. Transplant **More frequent in males Must treat w/in a few months or will be fatal. Rare

PDA VSD

Mortality risks vary greatly. 10-30% with early surgeries and transplantation.