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Abstract

Background: HCM is an autosomal dominant complex disease and considered the commonest cause of sudden cardiac death. Hypertrophic cardiomyopathy frequently associated with left ventricular outflow tract (LVOT) obstruction. It is clearly demonstrated that this is due not only to septal hypertrophy but also to systolic anterior motion (SAM) of mitral valve leaflets secondary to mitral valve/subvalvular apparatus abnormalities. Surgical treatment involves performing an extended septal myectomy which is the gold standard for relief of outflow tract obstruction and SAM.
Patients & Methods: This retrospective study in Kasr Alainy Hospital in the period of January 2000 until December 2023 included 40 patients who had HOCM with moderate to severe mitral regurgitation were scheduled for extended septal myectomy using on pump method, patients were selected with septal wall thickness ≥ 1.5 cm in adults and Resting or provoked peak systolic LVOT gradient of 50 mmHg or more. Specimens were examined for any histopathological changes &patients were followed up by clinical examination &investigations during surgery, ICU stay, hospital stay &follow up periods data collected and analyzed in comparison with pre-operative data as well as at these different follow up periods.
Results: Mean age was (37.93±16.13), with 52.5% males. All patients suffered from dyspnea FC III, IV despite maximally tolerated medication. Family history of HCM was positive in 27.5%. Pre-operative mean PG was (105.8±22.9) mmHg, mean SWT was (2.30±0.66) cm, and 100% have moderate to severe MR. Intra-operative assessment showed significant reduction of PG to (14.27±8.32). mmHg, and 72.5%free of MR and only 25% have only mild MR. There was also significant improvement in 92.5% of SAM, clinical improvement in dyspnea FC. With 4 deaths at mean follow up period (3.67±5.2) ranges from 1-21 years. All survivors showed sustained clinical and echocardiographic improvements without recurrence of obstruction or SAM
Conclusions: We concluded that Extended myectomy is sufficient and efficient in treatment of HOCM and associated SAM &mitral regurgitation with preservation of mitral valve keeping replacement only for patients with intrinsic mitral valve abnormalities.