Abstract
BACKGROUND: Survival to adulthood with an unrepaired double-inlet left ventricle is rare and typically associated with pulmonary stenosis, which provides protective hemodynamic effects. CASE SUMMARY: We report a 70-year-old woman with an unrepaired double-inlet left ventricle without pulmonary stenosis, the longest documented instance of this condition. She presented with mild symptoms (NYHA functional class I and II) and underwent pharmacological treatment for chronic heart failure. Six months later, she developed pulmonary thromboembolism and died of respiratory failure. Postmortem examination revealed significant atherosclerosis as well as hypertensive changes of the pulmonary vasculature. DISCUSSION: The absence of pulmonary stenosis may have contributed to progressive pulmonary arterial hypertension while facilitating long-term survival. The autopsy results provided critical insights into the unique pathophysiology of this rare congenital heart disease. TAKE-HOME MESSAGES: This case exemplifies a rare congenital heart disease. The detailed autopsy results significantly enhance understanding of the condition's natural history and clinical course.