**9. Physical findings**

Growth and development are generally normal. Physical signs vary depending on the severity of pathology and magnitude of the right-to-left atrial shunt, which may lead to cyanosis and digital clubbing in patients with interatrial communication. Cyanosis is typically pronounced in the neonate and infants, whereas it is milder (sometimes only exertional) in older children. Many have an unusual facial coloration, described as violaceous hue, red-cheeked, or malar rush. Usually these patients have an associated mild polycythemia. Asymmetry of the chest is a frequent finding secondary to the right heart dilatation. Arterial and venous pulsations are usually normal, even in the presence of tricuspid insuficiency. The jugular venous pulsations may not have a large V wave because of poor transmission of the venous pulse wave in the presence of a dilated and compliant right atrium [41, 42]. Jugular venous and hepatic distention may be present in advanced cases. The praecordium is usually not overactive. After the neonatal period, it is auscultation that often alerts the physician to the diagnosis of EA. The systolic murmur of tricuspid regurgitation is variable and its intensity depends on the degree to which contractility of the fRV is preserved. Multiple first heart sounds are heard because the highly mobile anterosuperior valvar leaflets and anterior leaflet mimic ejection clicks. Occasionally, the heart sounds are soft, but usually they are of normal intensity. The first heart sound is widely split because of the increased excursion of the anterosuperior leaflet and the subsequent delayed closure of the abnormal TV. The second heart sound is often split owing to the late closure of the pulmonary valve as a result of the conduction delay associated with severe RV enlargement. A holosystolic murmur is found along the left sternal border in those with an organized jet of tricuspid regurgitation. Diastolic murmurs are rare, unless there is coexisting pulmonary regurgitation. Low-intensity diastolic murmurs can be auscultated in the same location as a result of antero-grade flow across the TV [42]. Importantly, murmurs may be very soft or absent if the coaptation gap is very large; the velocity of to-and-fro flow is low, and rapid equalization of pressure across the functional TV does not result in blood flow turbulence.
