Cardiovascular

Congenital Heart Disease

Congenital heart disease comprises structural heart or great-vessel abnormalities present from birth; classify the physiology as shunt, obstruction, abnormal mixing or single-ventricle circulation, then recognise duct-dependent neonatal collapse, cyanotic spells, heart failure and the need for lifelong specialist follow-up.

Definition

Congenital heart disease is a structural or functional abnormality of the heart or great vessels present at birth. Its physiology may involve a left-to-right shunt, right-to-left or mixing circulation, obstruction, abnormal venous or arterial connection, or a single-ventricle pathway. Disease may be diagnosed antenatally, in the newborn period, during childhood or in adulthood.

First principles

Classify the physiology before memorising the anatomy

A left-to-right shunt such as a VSD, ASD or PDA recirculates oxygenated blood through the lungs and may cause pulmonary overcirculation and cardiac volume loading. An obstructive lesion such as coarctation or critical aortic or pulmonary stenosis limits outflow. A right-to-left or mixing lesion causes systemic desaturation. The same named lesion behaves differently according to its size, associated anatomy, pulmonary vascular resistance, ventricular function and previous repair.

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