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A premature male infant was born at the gestational age of 31 weeks (birth weight, 1,478 g). Resuscitation with chest compressions was performed after birth due to profound bradycardia. Congenital heart disease was suspected because of persistent cyanosis and a grade 3/6 systolic murmur at the left sternal border. Echocardiography was performed immediately, with use of a Philips HP SONOS 5500® imaging system with 12-MHz transducer (Koninklijke Philips Electronics N.V.; Eindhoven, The Netherlands). The right ventricle (RV) and RV outflow tract were structurally intact. However, the pulmonary valve leaflets did not open during systole. No antegrade flow could be seen on the Doppler color-flow map (Fig. 1). Severe tricuspid regurgitation and a large patent ductus arteriosus with left-to-right shunt were noted. The atrial septum bulged from the right atrium into the left atrium. Initially, critical pulmonary stenosis was suspected, because of the presence of pulmonary regurgitation. After the administration of mechanical ventilation and prostaglandin E1 for 1 day, repeat echocardiography showed good pulmonary valve leaflet motion and good antegrade flow (Fig. 2).
Functional pulmonary atresia can be seen in certain congenital heart diseases, such as Ebstein anomaly of the tricuspid valve, tricuspid valve dysplasia, Uhl anomaly, and RV hypoplasia.1 However, functional pulmonary atresia is only rarely reported in newborn infants who have anatomically normal hearts.2–6 In our patient, the condition was caused by concomitant severe pulmonary hypertension, RV dysfunction, and tricuspid regurgitation. Performing follow-up echocardiography after providing good general care can avoid unnecessary cardiac catheterization.7 The presence of pulmonary regurgitation is an important diagnostic clue.
Address for reprints: Yun-Ching Fu, MD, PhD, Department of Pediatrics, Taichung Veterans General Hospital, 160, Sec. 3, Chung-Kang Rd., Taichung 407, Taiwan, ROC