Clinical Analysis And Surgical Treatment Of Hypertrophic Obstructive Cardiomyopathy

Hypertrophic Obstructive Cardiomyopathy is a primary sarcomere disease of myocardium, it can be caused by several genes mutation and it is an autosomal- dominant inherited disease characterized by heterogeneity of clinical coures and genotype. Researchers found thatβmyosin heavy chain gene and cardiac troponin T chromosomes mutation. Pathology showed the left ventricle or right ventricular hypertrophy, myocardial fibrosis, myocardial fibers disordered can be seen under the microscope. HOCM Mainly show ventricular septum and Left ventricular hypertrophy, the location and extent of hypertrophy is different ,and the pathophysiology is as follows: 1, left ventricular outflow tract obstruction caused by systolic left ventricular outflow tract pressure gradient; 2, the diastolic ventricular Dysfunction, ventricular compliance, diastolic blood pressure, resulting in reduction of diastolic volume and pulmonary congestion; 3, there compensatory myocardial hypertrophy, myocardial ischemia and arrhythmia; 4, before systolic anterior mitral leaflet Movement or the valve itself, mitral valve regurgitation caused. HOCM clinical symptoms vary, mild symptoms can be chest tightness, chest pain, shortness of breath; severe symptoms can also be manifested as severe cyanosis, syncope, arrhythmia, heart failure and sudden death.Hypertrophic obstructive cardiomyopathy can occur at any age, more common 20 to 30 years old, about 1 / 3 of patients with hypertrophic cardiomyopathy have a clear family history, often accompanied with mitral systolic anterior motion phenomenon (SAM sign),The annual mortality rate is 1%, and about 10% patients eventually develop ventricular dilatation, congestive heart failure, called end-stage HOCM. The largest scale of echocardiography-based survey show that the prevalence of HCM is about 0.16%, and it suggest that at least 100 million patients with hypertrophic cardiomyopathy. However, confirmed cases of clinical practice and theoretical data gaps exist, more patients did not receive a clear diagnosis and correct treatment, including some patients with risk factors for sudden death. Therefore, the full understanding of hypertrophic cardiomyopathy with clinical features, combined with echocardiography, cardiac magnetic resonance imaging, cardiovascular imaging, electrocardiogram, genetic diagnosis, examination methods, early diagnosis and treatment, has very important significance.Treatment of HOCM include: medical drugs to control symptoms,β-blocker or disopyramide to reduce left ventricular outflow tract pressure gradient, anti-arrhythmic drugs to prevent sudden cardiac death; install implantable cardioverter defibrillator ( ICD) to reduce severe arrhythmia; Percutaneous septal myocardial ablation or hypertrophic ventricular septum myectomy to lift the left ventricular outflow tract obstruction. HOCM treatment options currently mostly based on observational results, medical drugs no one can change the disease progression and clinical outcome, hypertrophic ventricular septum myectomy which can significantly reduce left ventricular outflow tract pressure gradient is the gold standard for the treatment of HOCM. In this thesis, we collect HOCM patients who have accepted hypertrophic ventricular septum myectomy from March 2002 to October 2010 in our department, clinical analysis, and follow-up, summarize the results of hypertrophic ventricular septum myectomy for HOCM, and investigate surgical treatment strategies.Objective1 To analyse clinical characteristics of 33 patients with hypertrophic obstructive cardiomyopathy. 2 To summarize the results of hypertrophic ventricular septum myectomy for HOCM, and investigate surgical treatment strategies.Method1 collected clinical data of 33 received treatment patients who have diagnosed with HOCM from March 2002 to October 2010 in Nanjing Drum Tower Hospital. The clinical diagnostic criteria: left ventricular hypertrophy more than 13mm by echocardiography, and precluded high blood pressure or other diseases that could cause cardiac hypertrophy; or ventricular hypertrophy diagnosed by angiography. Analyze and summarize the clinical features of patients with HOCM.2 diagnosed patients under general anesthesia and hypothermic cardiopulmonary bypass to complete resection of septal myocardium, analysis and comparison of preoperative echocardiography (UCG), intraoperative transesophageal echocardiography (TEE), and postoperative 1 week, 3 months, 6 Months, 1 year echocardiography.Results1 Among those 33 patients with hypertrophic obstructive cardiomyopathy, all were because of the clinical symptoms to hospital, accepted treatment, and eventually were diagnosed. There were 16 males and 17 female patients. The age ranged from 13 to 59 years old with a mean of (42.7±13.6) years old. The main symptoms were chest tightness, palpitations, chest pain, syncope, etc., most patients takedβ-blockers and calcium channel antagonists, because the symptoms could not control and choosed the surgical treatment. Physical examination showed left sternal border 3 to 4 intercostal rough systolic murmur associated with tremor. ECG showed left ventricular hypertrophy before surgery, X-ray showed slight Hypertrophy. Preoperative Echocardiography showed that left ventricular and ventricular septum asymmetric hypertrophy, anterior mitral leaflet systolic typical forward movement (SAM sign), left ventricular outflow tract pressure (LVOTPG) 70 ~ 120 mmHg, mean (95.0±22.6) mmHg. There were 24 cases of mitral valve regurgitation, 7 cases with aortic insufficiency, 3 cases with ascending aortic widened, 2 cases with coronary heart disease.2 Intraoperative TEE, 1 week, 3 months, 6 months and 1 year were prompted echocardiographic left atrial dimension (LAD), left ventricular end diastolic diameter (LVDd), left ventricular outflow tract pressure (LVOTPG), Interventricular septum thickness (IVST) decreased significantly compared with the preoperative (P <0.05); In addition to the preoperative, the other time points of the LAD, LVDd, LVOTPG, IVST was no significant difference.Conclusion1 Among 33 patients with HOCM, there were varying degrees of chest tightness, palpitations, chest pain, fainting and other symptoms of cardiovascular disease, lesions mainly in ventricular septum hypertrophy; clinical onset age was mainly concentrated between 20 to 50 years old. 60.6% of patients were with high risk of sudden death.2 Surgical treatment of hypertrophic obstructive cardiomyopathy with good outcome. Understand the pathophysiological process, careful exploratory and radical surgery is the key to successful operation.