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The paper describes a clinicoanatomic case of two deceased neonates with fibromyxomatous hyperplasia of mitral and tricuspid valve leaflets with vascular malformations of the lung, lower extremity, spermatic cord, and thrombotic and hemorrhagic complications. The cases are considered in the context of congenital connective tissue dysplasia.

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A ten-year-old girl with severe subaortic stenosis was found to have relatively mature valvular endocardial cushion tissue (fibromyxomatous sheets with a chorda tendinea attached to a left ventricular papillary muscle) immediately beneath the aortic valve. This structure behaved like a valve mechanism, obstructing the left ventricular outflow tract during ventricular systole. This anomaly is an extreme on the spectrum of obstructive endocardial cushion malformations.

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