• A congenital heart lesion that decreases pulmonary arterial blood flow resulting in a right-to-left shunt
• Cyanosis and decreased oxygen delivery causes compensatory polycythemia (Hct > 70%) and spontaneous thrombosis
• Exercise, acidosis, pain worsens cyanosis, can cause hypoxic spells
• Squatting increases systemic resistance, causing increased pulmonary flow and oxygen saturation
• β-Blockers (decreases spasm), fluid intake, HCO3 administration, norepinephrine (increases systemic resistance) may help decrease hypoxia
• Clubbing due to proliferation of capillaries and AV fistulas in extremities
• Bronchial and mediastinal arteries enlarge
• Ductus arteriosus maintains flow to lungs during fetal development
• Alprostadil early can allow time for optimization before definitive treatment
• 4 anomalies:
• Pulmonary stenosis may involve infundibulum, valve, or main pulmonary artery (PA)
• Extent of pulmonary atresia/stenosis has wide spectrum of severity
• Pulmonary flow depends on aortopulmonary collaterals, can be extensive enough to cause congestive heart failure
• Spasm of infundibular muscle may cause unconciousness and death
• Symptoms related to amount of pulmonary blood flow
• With close of ductus, cyanosis and acidosis may be severe
• Hypoxic spells and cyanosis may lead to cerebral hypoxia and death
• Squatting and clubbing
• Prominent RV impulse, single S2
• Ejection murmur, left third intercostal space
• Murmur softens during cyanotic spell
• Chest film: Small heart size + cyanosis + decreased pulmonary flow
• Echocardiography: Confirms diagnosis
• Catheterization: Necessary to define size of pulmonary arteries and identify aortopulmonary arteries
• Consider other congenital anomalies
• Diminutive PA can lead to right heart failure with operative repair
• McGoon ratio: Diameter of PAs normalized to diameter of aorta > 2.0
• Most symptomatic at diagnosis
• Alprostadil, HCO3, oxygen
• One stage correction: Patch repair of VSD, patch to infundibulum to widen outflow tract, pulmonary insufficiency tolerated in child, may not be long-term
• Two stage: Initial systemic-PA shunt, later stage definitive correction
• Tetralogy of Fallot with pulmonary atresia: Connect large aortopulmonary shunts to RV outflow or to shunt; induces growth of vasculature
• RV dysfunction common
• Mortality < 5% in stenosis; 30-45% in atresia
• Reoperation on outflow tract: 10-15% at 10 years
• Those reaching adulthood: > 90% ...
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