PMC5896257_01
Patient's abnormal cardiac anatomy (picture courtesy of cardiologist Dr. Tan Ju Le). (1) Transposition of great arteries: the morphologic left ventricle is connected to the pulmonary artery (PA) and pulmonary circulation; the hypoplastic right ventricle is connected to the aorta and systemic circulation. (2) The tight pulmonary artery band limits blood flow from the left ventricle into the pulmonary circulation to prevent development of pulmonary hypertension and to direct oxygenated blood from left ventricle to right ventricle through the large nonrestrictive ventricular septal defect (VSD) and then from right ventricle to systemic circulation. (3) Systemic venous blood from the superior vena cava enters the pulmonary circulation for oxygenation via the bidirectional cavopulmonary connection. (4) Systemic venous blood from the inferior vena cava returns to the right atrium and enters left atrium via the large nonrestrictive atrial septal defect (ASD), from left atrium to the morphologic left ventricle. Due to the congenital tricuspid atresia, there is no blood flow form right atrium to right ventricle. (5) Oxygenated blood from the lungs enters the pulmonary veins, flows to left atrium and left ventricle and then through the ventricular septal defect to right ventricle. Both the hypoplastic right ventricle and the left ventricle (via the ventricular septal defect) eject blood into the aorta. Bidirectional shunting occurs across the ventricular septal defect with mixing of oxygenated and deoxygenated blood.
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fig1
She had pulmonary artery banding at 6 weeks of age and bidirectional cavopulmonary connections (also known as Glenn's shunt) were performed at the age of 18 (see Figure 1.) Completion of Fontan's procedure was held off indefinitely because of an incidental finding of cerebral arteriovenous malformation (AVM).