Key result
Extended myectomy and mitral repair resolves LVOT obstruction, reduces MR, and improves walk distance.
Why the study?
The combination of hypertrophic cardiomyopathy with outflow tract obstruction, severe pulmonary hypertension, and severe primary mitral regurgitation is rare and presents distinct management challenges.
Does extended septal myectomy and complex mitral valve repair improve functional capacity and hemodynamics in a patient with hypertrophic cardiomyopathy with outflow tract obstruction, severe primary mitral regurgitation, and severe combined pre- and post-capillary pulmonary hypertension?
Case Report (n=1)
No
Does extended septal myectomy and complex mitral valve repair improve functional capacity and hemodynamics in a patient with hypertrophic cardiomyopathy with outflow tract obstruction, severe primary mitral regurgitation, and severe combined pre- and post-capillary pulmonary hypertension?
Answers are grounded in this paper and cite their sources.
This case demonstrates that concomitant extended septal myectomy and mitral valve repair can be safely and effectively performed to improve functional capacity and relieve obstruction in a patient with the rare triad of obstructive hypertrophic cardiomyopathy, flail mitral valve, and severe combined pre- and post-capillary 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. Background and Clinical Significance: 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. Case Presentation: 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. Conclusions: 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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Sunga et al. (2024) conducted a case report in Hypertrophic cardiomyopathy with outflow tract obstruction, severe pre-capillary and post-capillary pulmonary hypertension, and severe primary mitral regurgitation due to flail anterior mitral leaflet (n=1). Extended septal myectomy and complex mitral valve repair with preoperative sildenafil stabilization was evaluated on Resolution of left ventricular outflow tract obstruction, improvement in mitral regurgitation, and functional capacity at 5 months. Extended septal myectomy and mitral valve repair resolved LVOT obstruction, reduced mitral regurgitation to mild, and improved walking distance from 611 to 741 feet at 5 months.
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