Authors
Praveen Kerala Varma1, Narayanan Namboodiri2
1Division of Cardiac surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India
2Division of Cardiology and Electrophysiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India
Abstract
Introduction: Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, often presenting as an autosomal dominant condition. While most cases remain undiagnosed, it is a significant cause of sudden cardiac death. Obstruction to the left ventricular outflow tract (LVOT) occurs in 70% of patients, leading to severe symptoms. Medical therapy is the initial approach, but invasive options like surgical myectomy are considered for refractory cases.
Case Presentation: We report a case of a 51-year-old female with a family history of HCM (mother had sudden cardiac death, brother also diagnosed). She presented with recurrent palpitations, multiple syncopal episodes, exertion angina, and shortness of breath (NYHA Class 3 symptoms) despite maximal medical therapy. Echocardiography revealed asymmetric LV hypertrophy with significant LVOT and mid-cavity gradients.
Intervention: Due to persistent severe symptoms and mid-cavity obstruction unsuitable for alcohol ablation, she underwent extended myectomy via cardio-pulmonary bypass. Post-procedure trans-esophageal echocardiography showed a residual gradient of 6mm of Hg, resolution of systolic anterior motion (SAM), and mild mitral regurgitation.
Conclusion: This case highlights the clinical presentation and successful surgical management of familial obstructive HCM. Surgical myectomy remains the gold standard for patients with significant obstruction and symptoms refractory to medical therapy, offering excellent outcomes, particularly in complex cases involving mid-cavity obstruction.