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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 progressi
SUBMITTER: Murakawa K
PROVIDER: S-EPMC12861618 | biostudies-literature | 2025 Dec
REPOSITORIES: biostudies-literature