Pulmonary Atresia With Intact Ventricular Septum Clinical Presentation

Updated: Dec 27, 2020
  • Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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History and Physical Examination


Infants with pulmonary atresia with intact ventricular septum (PAIVS) are usually born at term, and cyanosis is apparent within hours. The diagnosis can be made prenatally using fetal echocardiography. These babies develop early progressive cyanosis and tachypnea associated with closure of the patent ductus arteriosus.

Physical examination

The most common finding upon physical examination is central (perioral and periorbital) cyanosis. Following ductal closure, profound generalized cyanosis is present. Also note the following:

  • Apical left ventricular impulse may be pronounced.

  • The first and second heart sounds are single.

  • A pansystolic murmur is often heard at the left lower sternal border, consistent with tricuspid regurgitation. If severe, the murmur of tricuspid regurgitation may be associated with a thrill and a diastolic rumble.

  • A systolic ejection murmur of the patent ductus arteriosus may be heard at the left second or third intercostal space, particularly after initiating prostaglandin infusion.

  • Normal arterial pulses are usually present but can be bounding in the setting of a large patent ductus arteriosus and increased pulmonary blood flow.

  • Hepatomegaly is uncommon unless the atrial septal defect is restrictive (rare).