Exceptional survival: Double inlet left ventricle presenting with aortic dissection

Exceptional Survival: Double Inlet Left Ventricle with Pulmonary Artery Banding

“…In our current case, for example, the initially reported PA gradient was erroneously higher than actually measured, and initiation of bosentan would not improve our patient's symptoms. 3,15,17 Our patient is not interested in further surgical intervention, and we are unable to substantiate that the benefits outweigh the risks for him. His surgical options are limited, and the current recommendations for final palliation of patients with univentricular hearts are mainly directed at a Fontan-type operation.…”

“…At baseline, his systemic saturation was 89% 6 Although survival into late adulthood without surgical repair has been previously documented, it is exceedingly rare. [2][3][4][7][8][9] The best survival for patients with a single ventricle has been shown in patients with double inlet LV with transposed great arteries and moderate pulmonary stenosis. The combination of these defects is associated with "balanced hemodynamics" and improved survival by allowing: (1) "preferential streaming" of oxygenated blood from the left atrium through a ventricular septal defect to the systemic circulation; (2) delivery of an adequate but not excessive blood flow to the lungs, thereby preventing severe cyanosis and pulmonary overcirculation; and (3) avoidance of ventricular pressure overload.…”

“…The combination of these defects is associated with "balanced hemodynamics" and improved survival by allowing: (1) "preferential streaming" of oxygenated blood from the left atrium through a ventricular septal defect to the systemic circulation; (2) delivery of an adequate but not excessive blood flow to the lungs, thereby preventing severe cyanosis and pulmonary overcirculation; and (3) avoidance of ventricular pressure overload. [2][3][4][7][8][9] However, PAB has been linked to the accelerated development of subaortic stenosis. [10][11][12][13] In the Toronto study, 84.4% of patients with univentricular hearts of LV morphology and transposition of the great arteries that underwent PAB developed subaortic stenosis after a mean of 2 years.…”

“…15 However, these surgeries have been associated with significant morbidity and mortality. 3,15 Long-term complications can arise, including ventricular dysfunction, atrioventricular valve regurgitation, thromboembolism, supraventricular arrhythmias, and protein-losing enteropathy. 3,15,17 Our patient is not interested in further surgical intervention, and we are unable to substantiate that the benefits outweigh the risks for him.…”

“…Single ventricle is a rare and complex malformation reported to represent approximately 1–3% of congenital heart disease and is associated with extremely poor survival 2–4 . Moodie et al.…”