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    REVIEW

    Severe Right Ventricular Dysplasia with Absent Pulmonary Valve Syndrome and Tricuspid Atresia: A Literature Review

    Kinjal Parikh1,2,*, Juan Carlos Muniz1,2, Elizabeth Welch1,2, Abdul Aldousany1,3, Nao Sasaki1,2

    Congenital Heart Disease, Vol.15, No.5, pp. 339-346, 2020, DOI:10.32604/CHD.2020.012910

    Abstract This is a newborn male prenatally diagnosed with severe right ventricular (RV) hypertrophy and depressed function, aneurysmal dilation of the main pulmonary artery and tachyarrhythmia. Postnatally, he required immediate intubation and inotropic support. Echocardiogram revealed a large dysplastic RV, absent pulmonary valve syndrome (APVS), markedly dilated pulmonary arteries and tricuspid atresia (TA). The trabecular portion of the RV was excessively trabeculated and severely dilated. Inflow and infundibular walls were thin, with multiple infundibular aneurysms. There was APVS with free regurgitation and massively dilated pulmonary arteries. The RV bulged into the LV, though there was no outflow tract obstruction. The LV… More >

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