Surgery of Hypertrophic Obstructive Cardiomyopathy, Kasr El-Aini limited experience Elsayed K. AKL, Tarek A. Mohsen, Amal Khalifa and Sameh Zaghloul. Faculty of medicine, Cairo university
Background Hypertrophic obstructive cardiomyopathy ( HOCM) is the most common genetic cardiovascular disease. It affects 1:500 of population in different parts of the world. However, it did not attain enough attention for many years. There is a definite recent increasing interest in this disease because of the dramatic fatal events of sudden death in young patients.
Aim of work We sought to : 1- Evaluate our learning curve of septal myectomy 2- Investigate whether Egyptian patients have different phenotypic characteristics from other reported large series.
Patients and materialAll patients with: symptomatic obstructive cardiomyomathy with maximum medical treatment and a resting or provoked peak systolic gradient > 60 mmHg. were enrolled in this study. All patients were operated by one surgeon .
Preoperative data Between Jan. 2000 & Dec. 2010 Limited experience ( 19 patients ) Age ( years ) Sex 2-47 (23.4 + 12.7) Male63.2% 12 9 10 12 10 8 6 4 7 2 0 Female 36.8% < 18 y > 18 y
Preoperative data 17 patients had SOB III-IV & 2 had dizziness & syncope 10 8No. of Patients 6 4 2 0 0 1 2 3 4 NYHA Functional Class
Operative Technique All patients underwent cautious septal myectomy through transaortic approach. Excision extends to the base of the papillary muscles. Attention to release of both fibrous trigones was added during the last 3 years. Septal endocardium is cautiously peeled off if thickened Only one patient required mitral repair excising prolapsing p1. Immediate postoperative peak systolic geardient was measured in each case by pull back or simultaneous left ventricular / aortic pressure tracing. TEE was used in most of the cases.
RESULTS ( 19 patients ) NO op. mortality Immediate peak gradient dropped from a mean of 105 + 30 to a mean of 11.5 + 6.1 mmHg. Significant reduction in pressure gradient was achieved in every patient. All patients came out of OR with no or trivial mitral incompetence. No incidence of iatrogenic VSD, aortic valve injury or complete heart block.
Examples of excised septal muscle P1Thickened septal endocardium
Follow Up is 100% 2 w -132 m ( mean 34.4 + 31.9) One patient ( 11 y ) died 3 years after operation out of chronic renal failure. He had family history of sudden death, was born with one kidney and had ICD 3 years before operation. One patient ( 35 y ) with preoperative hepato- renal failure died 5 weeks after operation during dialysyis session. One patient ( 16 y ) developed an attack of VT 5 years later and treated medically. All survivors are well and free of SOB (FC 0-1)
Symptomatic improvement Pre. & postoperative SOB Preoperative 12 Postoperative 10No. of Patients 8 6 4 2 0 0 1 2 3 4 NYHA Functional Class
CONCLUSIONS 1 – Awareness of the disease and its consequences is mandatory. 2 – Training with experts allows safe learning to properly perform septal myectomy that gives excellent short and long term results with excellent relief of gradient and symptoms. 3 –Patients with preoperative renal problems seem to be at higher risk after operation. 4- Compared to large reported series (1,2&3), Egyptian patients with HOCM are younger and present with higher gradients. This may be explained by particular genetic variations which needs to be explored.
References1 ) Mahboob Alam, Hisham Dokanish, and Nasser M. Lakkis.European Heart Journal (2009) 30, 1080–10872 ) Nicholas G. Smedira, MD, Bruce W. Lytle, MD, et al. Ann Thorac Surg 2008;85:127–343 ) J. Dearani, S. Ommen, B. Gersh et al. Nature Clinical Practice, cardiovascular medicine. 2007; 4 (9) : 503- 512