Anatomical and pathophysiological classification of congenital heart disease.

Congenital heart diseases (CHD) consist of defects of the cardiac architecture which interfere with the venous drainage, septation of the cardiac segments and their sequences and regular function of the valve apparatuses. In the normal heart the segments are disposed in such a way to allow deoxygenated venous blood to go to the lungs through the pulmonary artery and the oxygenated venous blood to go to the systemic organs through the aorta without mixing. Small and great circulations are in sequence, with no communication to each other. Establishing the sequence of cardiac segments is the prerequisite for planning a surgical repair. We propose a pathyphysiological classification of CHD based upon the clinical consequence of structural defects on the physiology of blood circulation. We divided cardiac anomalies in: (1) CHD with increased pulmonary blood flow (septal defects without pulmonary obstruction and with left-to-right shunt); (2) CHD with decreased pulmonary flow (septal defects with pulmonary obstruction and with right-to-left shunt); (3) CHD with obstruction to blood progression and no septal defects (no shunt); (4) CHD so severe as to be incompatible with postnatal blood circulation; and (5) CHD silent until adult age.