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Ductus arteriosus
Fetal circulation
Present of PFO and ductus arteriosus; shunting R L
High peripheral pulmonary resistance Upper limbs/brain receive more oxygenated blood
Umbilical arteries
Changes at birth
PVR falls (with first breath and lung expansion pulmonary vasoconstriction ceases) System vascular resistance rises (removal of placental circulation) PDA closes (o in aortic oxygen saturation) PFO closes (o in pulmonary venous return) Ductus venosus closes Note: PA pressure qqq in the first 2 - 3 days of life gradual fail to normal adult level by 2 weeks
Normal circulation
Left ventricle (systemic) with high systolic pressure Right ventricle (venous) lower systolic pressure L R shunt in septal or arterial defects
What are the types of congenital heart disease? (IJN 1996-1997 n =3284)
Acyanotic defect (78.2%)
Septal defects : (45%)
Ventricular septal defect Atrial septal defect Atrioventricular septal defect
Figure 9.6 : Blood supply to the lungs in pulmonary atresia with ventricular septal defect. - low resistance vascular bed. (a) the central pulmonary arteries are present and supply to the lungs is through the patent ductus arteriosus only. (b) The central pulmonary arteries are absent and supply to the lungs is through major aorto-pulmonary collateral arteries which arise from the aorta and have stenosis on them as they enter the lungs
Simple transposition
Aorta
Pulmonary artery
2 separate circulation Survival depends on mixing of blood between the pulmonary circulation of oxygenated blood and systemic deoxygenated blood through the PDA or foramen ovale Severe cyanosis develops with closure of PDA and PFO
Right superior vena cava Right atrium Enlarged right atrium Coronary sinus Enlarged right ventricle
Hepatic vein
Cyanotic heart disease in the newborn: Clinical presentation Anatomical physiologic entities
Cyanotic heart disease with reduced pulmonary blood flow Cyanotic heart disease with increase pulmonary blood flow
Electrocardiography (ECG)
QRS axis Ventricular hypertrophy Dilated right atrium
Chest x-ray
Cardiac position - possible dextrocardia Heart size, great vessels (pulmonary artery size, position of aortic arch) Cardiac chambers Lung fields plethora - excessive pulmonary blood flow oligemia - reduced pulmonary blood flow
Characteristic features
Bootshaped heart TOF/PAVSD Egg on side - TGA Narrow pedicle Snow man heart - total anomalous pulmonary venous drainage (TAPVD)
TGA
Cardiomegaly with egg on side appearance
Diagnosis of CHD
Symptoms Clinical examination ECG, Chest x-ray Echocardiography CT scan, MRI Cardiac catheterization
Cyanotic
Severe critical PS Pulmonary atresia, intact septum Pulmonary atresia, VSD TGA Obstructed TAPVD Complex univentricular heart
Cardiovascular collapse, acidosis + hypoxia May be PDA dependent systemic/pulmonary circulation Treatment: Prostaglandin, correction of acidosis, stabilize haemodynamics definitive treatment depending on the lesion
Issues?
Correct diagnosis and associated problems. Possible options for either catheter intervention or surgery Age and weight at diagnosis Ideal timing for intervention or surgery Result and long-term problems.