Background: The combination of pulmonary valve atresia and ventricular septal defect accounts for about 2% of cases of congenital heart disease. Most of the cases have intracardiac anatomy of Tetralogy Fallot and present with cyanosis in neonatal age.
Objectives: To report a case of a newborn with rare combination of pulmonary atresia, ventricular septal defect and single ventricle physiology presenting with shock very early following birth.
Methods and results: We describe a newborn infant who developed shock with severe metabolic acidosis and respiratory distress several hours following birth. Cardiac ultrasound showed pulmonary atresia and ventricular septal defect with single ventricle physiology. Cardiac cathetrisation revealed major aorto-pulmonary collaterals with an excessive pulmonary blood flow. Resuscitative measures resulted in hemodynamic stability. However, due to unfavorable prognosis, a decision not to undertake surgical palliation was made. Withdrawal of intensive care led to rapid demise of the patient.
Conclusions: We report a case of a newborn with pulmonary atresia, ventricular septal defect and single ventricle physiology in whom an excessive flow through major aorto-pulmonary collaterals led to shock and death very early following birth (Fig. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk.