Mulibrey Nanism Syndrome: A Case for Heart Transplantation.

Anwer, Mohammad; Bin Mahmood, Syed Usman; Stawiarski, Kristin; Elder, Robert; Ali, Ayyaz; Jacoby, Daniel · Ann Thorac Surg · 2020

case_report · Level V

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Abstract

Mulibrey nanism syndrome is a rare genetic disorder affecting multiple organ systems. The cardiovascular system is one of the most significantly affected, with simultaneous myocardial and pericardial disease. These patients are usually managed by pericardiectomy to resolve the milieu of hemodynamic problems ensuing due to concurrent constrictive and restrictive pathologies. We highlight the use of cardiac transplantation as a definitive management for a hemodynamically decompensated patient with Mulibrey nanism syndrome.

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