Key result
Unoperated double-inlet left ventricle without pulmonary stenosis permits survival to age 70 despite progressive PAH.
Why the study?
Survival to adulthood with unrepaired double-inlet left ventricle is rare and typically associated with protective pulmonary stenosis.
Case Report (n=1)
No
This rare autopsy case highlights the unique pathophysiology of an unoperated double-inlet left ventricle without pulmonary stenosis, demonstrating that survival to 70 years is possible despite the development of progressive pulmonary artery hypertension.
Rare survival to 70 without pulmonary stenosis in unrepaired double-inlet left ventricle requires PAH vigilance; leaves open mechanisms of balanced flow beyond typical stenosis.
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.
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Murakawa et al. (2025) conducted a case report in Unrepaired double-inlet left ventricle without pulmonary stenosis (n=1). Unrepaired double-inlet left ventricle without pulmonary stenosis was evaluated on Clinical course and autopsy findings. The rare absence of pulmonary stenosis in an unoperated double-inlet left ventricle promoted progressive pulmonary artery hypertension while facilitating a delicate blood flow balance that supported survival to age 70.
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