Clinical presentation

Symptoms are variable, with a spectrum from pink with normal heart size to profound central cyanosis.

Signs include:

• clubbing and cyanosis, usually progressive with age.
• pulse - can present with supraventricular tachycardias
• jugular venous pressure - an atrial defect if present decompresses the atrium; if absent tricuspid regurgitation causes mild elevation of venous pressure and a systolic wave.
• auscultation - There is a split first heart sound - loud delayed tricuspid closure - split second heart sound - delayed P2 due to right bundle branch block. Often there is a tricuspid opening snap, a short ventricular filling murmur and an atrial (fourth) sound. If there is significant tricuspid regurgitation there is a prominent pansystolic murmur at the left sternal edge.