A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension

The combination of hypertrophic cardiomyopathy with outflow tract obstruction, severe pre-capillary and post-capillary pulmonary hypertension, and severe primary mitral regurgitation is rare and presents distinct management challenges. <b>Background and Clinical Significance</b>: Pulmona...

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Main Authors: Cass G. G. Sunga, Kai-Chun Yang, Shakirat Oyetunji, Erik R. Swenson, Kavita Khaira
Format: Article
Language:English
Published: MDPI AG 2024-12-01
Series:Reports
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Online Access:https://www.mdpi.com/2571-841X/7/4/116
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author Cass G. G. Sunga
Kai-Chun Yang
Shakirat Oyetunji
Erik R. Swenson
Kavita Khaira
author_facet Cass G. G. Sunga
Kai-Chun Yang
Shakirat Oyetunji
Erik R. Swenson
Kavita Khaira
author_sort Cass G. G. Sunga
collection DOAJ
description The combination of hypertrophic cardiomyopathy with outflow tract obstruction, severe pre-capillary and post-capillary pulmonary hypertension, and severe primary mitral regurgitation is rare and presents distinct management challenges. <b>Background and Clinical Significance</b>: Pulmonary hypertension is an independent predictor of all-cause mortality in patients with hypertrophic cardiomyopathy managed medically and often precludes patients from undergoing cardiopulmonary bypass due to increased surgical morbidity and mortality. In studies specifically evaluating surgical myectomy, however, survival is favorable in patients with moderate-to-severe pulmonary hypertension. <b>Case Presentation</b>: We present a case of a 74-year-old male with six months of dyspnea with minimal exertion. A diagnostic work-up with transthoracic echocardiogram showed asymmetric left ventricular hypertrophy, left ventricular outflow tract obstruction with a peak gradient of 200 mmHg, right ventricular systolic pressure of 99 mmHg, systolic anterior motion of the mitral valve and flail anterior mitral leaflet. The patient was evaluated by a multi-disciplinary team and underwent extended septal myectomy and mitral valve repair with significant improvement in functional capacity post-operatively. <b>Conclusions</b>: While pulmonary hypertension increases the risk of morbidity and mortality during cardiopulmonary bypass, moderate-to-severe pulmonary hypertension in hypertrophic cardiomyopathy with outflow tract obstruction is a unique indication for septal reduction therapy that may not be associated with higher surgical mortality.
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spelling doaj-art-bc200bb113de442bae099230d398a14b2025-08-20T02:01:19ZengMDPI AGReports2571-841X2024-12-017411610.3390/reports7040116A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary HypertensionCass G. G. Sunga0Kai-Chun Yang1Shakirat Oyetunji2Erik R. Swenson3Kavita Khaira4General Internal Medicine, Department of Medicine, University of Washington, Seattle, WA 98195, USADivision of Cardiology, Department of Medicine, University of Washington, Seattle, WA 98195, USAVeteran’s Health Administration Puget Sound Health Care System, Seattle, WA 98108, USAVeteran’s Health Administration Puget Sound Health Care System, Seattle, WA 98108, USADivision of Cardiology, Department of Medicine, University of Washington, Seattle, WA 98195, USAThe combination of hypertrophic cardiomyopathy with outflow tract obstruction, severe pre-capillary and post-capillary pulmonary hypertension, and severe primary mitral regurgitation is rare and presents distinct management challenges. <b>Background and Clinical Significance</b>: Pulmonary hypertension is an independent predictor of all-cause mortality in patients with hypertrophic cardiomyopathy managed medically and often precludes patients from undergoing cardiopulmonary bypass due to increased surgical morbidity and mortality. In studies specifically evaluating surgical myectomy, however, survival is favorable in patients with moderate-to-severe pulmonary hypertension. <b>Case Presentation</b>: We present a case of a 74-year-old male with six months of dyspnea with minimal exertion. A diagnostic work-up with transthoracic echocardiogram showed asymmetric left ventricular hypertrophy, left ventricular outflow tract obstruction with a peak gradient of 200 mmHg, right ventricular systolic pressure of 99 mmHg, systolic anterior motion of the mitral valve and flail anterior mitral leaflet. The patient was evaluated by a multi-disciplinary team and underwent extended septal myectomy and mitral valve repair with significant improvement in functional capacity post-operatively. <b>Conclusions</b>: While pulmonary hypertension increases the risk of morbidity and mortality during cardiopulmonary bypass, moderate-to-severe pulmonary hypertension in hypertrophic cardiomyopathy with outflow tract obstruction is a unique indication for septal reduction therapy that may not be associated with higher surgical mortality.https://www.mdpi.com/2571-841X/7/4/116cardiomyopathypulmonary hypertensionmitral valvevalve repair
spellingShingle Cass G. G. Sunga
Kai-Chun Yang
Shakirat Oyetunji
Erik R. Swenson
Kavita Khaira
A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
Reports
cardiomyopathy
pulmonary hypertension
mitral valve
valve repair
title A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
title_full A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
title_fullStr A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
title_full_unstemmed A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
title_short A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension
title_sort triple threat a case report detailing surgical management for hypertrophic cardiomyopathy flail mitral valve and severe pulmonary hypertension
topic cardiomyopathy
pulmonary hypertension
mitral valve
valve repair
url https://www.mdpi.com/2571-841X/7/4/116
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