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Hypertrophic Cardiomyopathy with Biventricular Involvement and Coronary Anomaly: A Case Report
Ylenia Bartolacelli1, Simone Bonetti1, Anna Balducci1
1Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio-Thoracic and Vascular Medicine IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40122 Bologna, Italy.
Insights
Hypertrophic cardiomyopathy (HCM) can affect both ventricles, causing right ventricular (RV) outflow obstruction. Surgical relief of this obstruction improved symptoms and reduced gradients in a patient with biventricular HCM.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is typically a left ventricular disease.
- Right ventricular (RV) involvement in HCM is less understood.
- Biventricular HCM presents unique diagnostic and therapeutic challenges.
Observation:
- A case of biventricular HCM with significant RV hypertrophy and outflow tract obstruction was observed.
- The patient presented with near obliteration of the RV cavity and no left intraventricular gradient.
- Symptoms included potential for heart failure, arrhythmias, and pulmonary thromboembolism due to RV dysfunction.
Findings:
- Surgical intervention successfully eliminated the RV outflow tract obstruction.
- The patient experienced improved symptoms and a significant reduction in postoperative gradients.
- Direct relief of RV outflow obstruction demonstrated low morbidity and favorable intermediate- to long-term outcomes.
Implications:
- Significant RV hypertrophy in HCM can lead to severe outflow obstruction and diastolic dysfunction.
- Conventional surgery is a viable option for relieving RV outflow obstruction in HCM.
- Effective treatment of RV obstruction can lead to significant symptomatic improvement in biventricular HCM patients.
Abstract:
Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) involvement has also been reported, though still not extensively characterized. We present a case of biventricular HCM with significant RV involvement in the absence of a left intraventricular gradient: RV outflow tract gradient due to hypertrophy and near obliteration of the RV cavity. Significant RV hypertrophy may cause reduced RV diastolic filling and/or RV outflow obstruction, with potentially increased incidence of symptoms of heart failure, arrhythmias, and pulmonary thromboembolism. The optimal treatment for these patients is unclear. Our patient underwent complete treatment and elimination of right ventricular obstruction, resulting in improved symptoms and a significant reduction in postoperative gradients. Direct relief of outflow tract obstruction can be achieved with low morbidity and good intermediate- to long-term results. Conventional surgery may provide significant symptomatic improvement and should thus be considered in the setting of HCM with outflow obstruction.
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