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Hypertrophic obstructive cardiomyopathy was referred to as subaortic muscular obstruction. Davies in 1952 reported a nine brothers and sisters who have the disease in 5, including 3 cases of sudden death. 1958 Teare described a high degree of ventricular septal hypertrophy, which far exceeds the left ventricular free wall thickness. And myocardial cells thick and short, messy arrangement, the lateral connections between cells called rich asymmetric ventricular septal hypertrophy Medical Education Network. After 1960, considered a type of primary cardiac disease, various types of cardiomyopathy in about 20%, which is called idiopathic obstructive cardiomyopathy, idiopathic hypertrophic subaortic stenosis or hypertrophic obstructive cardiomyopathy. The disease about 30% of cases have a family history, may have genetic factors. Time of onset from infants to 60 years old, but the most common is 10 to 30 years old, suggests the likelihood of congenital malformations, also may be acquired. 1960 Goodwin, Kelly, Morrow, Brockenbrough, Braunwald, Wigle carried out successively on the surgical treatment of this disease.
Treatment collected Medical Education Network
Hypertrophic obstructive cardiomyopathy can show symptoms at any age, the most common age of onset before 20 years of age. Transcatheter examination of cases diagnosed in the 10 years of age only 10% presented with severe symptoms, more than 50 years of age increased to 70%. In some cases, the condition ca


n be stable or sustainable development for many years become increasingly serious. After atrial fibrillation or congestive heart failure often show circulation embolism. Without showing clinical symptoms and arrhythmia surgery cases, about 15% at 5 years after the death of 25% in 10 years after the death. Most patients with sudden death, only a few cases, died of heart failure or infective endocarditis. Clinical symptoms, medical therapy does not work, resting left ventricular cavity and outflow tract systolic blood pressure difference exceeds 6.6kPa (50mmHg) should be the purposes of surgical treatment, removal of ventricular septal myocardial hypertrophy to remove the obstruction. Commonly used surgical methods are: (a) After aortic and left ventricular incision cardiac surgery sternal incision, application of cardiopulmonary bypass with hypothermia, decompression of the left atrium into the drainage tube, blocking the ascending aorta, increase in the roots pressure injection of cold cardioplegia and local reduction of myocardial temperature, transverse incision aortic root, right coronary valve with a retractor to move forward traction, with a round knife blade removed from the front U-ventricular septal myocardium, cut right below the crown flap began to extend to the left and right coronary valve below the junction of the left coronary valve. Must not be extended to the right ventricular septal incision to avoid damage caused by complete left atrioventricular bundle block. The ventricular septal myocardium rectangular piece to the bottom of elongation under direct vision, but not cut too deep. Also at the bottom of the left ventricular anterior wall for support and the minimum angle of oblique parallel incision about 4cm into the bottom of the left ventricular papillary muscle before the cavity, the incision will be before the valve leaflets to the ventricular septum of the left side of pulling from the bottom up with knife to cut cardiac ventricular septal hypertrophy, and myocardial removed by the aorta to link the pieces, then cut off the entire piece of myocardial hypertrophy, notch depth of about 15 ~ 20mm trim pieces to prevent the occurrence of cardiac embolism. Interrupted suture full thickness myocardial incision, suture aortic incision (Figure 1). Discharge of residual gas left ventricular cavity and aorta, aortic cross-clamping pliers and get higher than body temperature, heart bypass effectively stops. Figure 1 The left ventricular incision in the aorta and resection of left ventricular hypertrophy
left index finger inserted into the left ventricular cavity from the aorta for left ventricular incision; the proposed removal of the left ventricular hypertrophy; hypertrophic left ventricular wall and cardiac septal resection (b) of the myocardium by aortic resection and ventricular septal incision incision establishment of cardiopulmonary bypass and myocardial protection measures to block the ascending aorta after aortic root blood flow transverse incision, pulling the right lobe revealed ventricular septal crown, crown with small round knife in the right upper lobe below the ventricular septal incision for the two parallel and cut Happy ventricular pressure when the lower part of right ventricular free wall, interventricular septum toward the left ventricular cavity to the heart to improve the exposure, and then cut between two parallel rectangular incision hypertrophic myocardium. Press the ventricular septal incision with your fingers to increase the ventricular septal notch depth and width, take heart fragments in addition, suture aortic incision, the left ventricular cavity and aortic exhaust gases, to take except aortic occlusion clamp. Rewarming to a temperature of 35 or more, a strong heart beat, stop the pump, such as cardiac hypertrophy that the ventricular septum resection remains less satisfactory and it has to be the other way by the complete removal of the left ventricular incision (Figure 2). Figure 2 ventricular septal incision of left ventricular hypertrophy
After establishing cardiopulmonary bypass, the aortic incision into the surgeons fingers and a knife; ventricular hypertrophy in the vertical shear happy myocardial treatment: surgical mortality rate of about 10%. Common cause of death was low cardiac output and left ventricular incision bleeding. About 5% of cases after concurrent complete block, left bundle branch or right bundle branch block were more common. In addition a small number of patients complicated with perioperative myocardial infarction, ventricular septal perforation, iatrogenic left ventricular aneurysm and aortic valve or mitral regurgitation. Ventricular septal myocardial hypertrophy after removal of the cases the symptoms disappeared completely or significantly reduced systolic blood pressure difference disappears, aortic pressure waveform returned to normal. Echocardiography and selective left ventricular angiography showed left ventricular cavity increases, the systolic anterior mitral valve leaflet gone forward, but atrial fibrillation is still present. About 90% of patients improved heart function after surgery to 1 ~ 2. Postoperative long-term survival of 70% of cases followed up more than 10 years, 50% survive more than 15 years. The main cause of death was congestive heart failure or serious arrhythmia, atrial fibrillation caused by cerebral thrombosis or myocardial infarction. The incidence of sudden death is about 25%, compared with patients without surgery was significantly reduced. Pathological changes
Hypertrophic obstructive cardiomyopathy degree of left ventricular obstructive lesions of varying severity. The typical lesions in the upper ventricular septal hypertrophy the most significant, the vertical cut happy septum, hypertrophy of the heart that is to the left and right ventricular cavity bulging. Ventricular septum is the thickest part of the free edge of anterior mitral valve leaflet below the ventricular septum in the area due to collision with each other before the valve leaflets and showed the limitations of fibrosis intimal thickening (Figure 1). Myocardial hypertrophy in ventricular septal thickness up (aortic rings), down (apex) decreased, lower left ventricular outflow tract obstruction in cardiac hypertrophy with a ventricular septal leaflet between the free edge. Cardiac contraction, hypertrophy of the heart into the ventricular septum convex cavity, close to the forward of the anterior mitral valve leaflets, resulting in left ventricular outflow tract obstruction, sometimes associated with regurgitation. Early systolic outflow tract obstruction to a lesser extent, at this time of ventricular cardiac output more. Anterolateral part of the left ventricular free wall and apex uniform hypertrophy, left ventricular posterior wall thickness is less, ventricular septum and left ventricular posterior wall thickness ratio up to 3:1, smaller left ventricular cavity. Middle of the ventricular septal thickening was dumbbell-shaped ventricular cavity. Advanced stage disease, due to severe myocardial infarction or chronic heart failure, left ventricular possible expansion of the left atrial cavity often enlarged heart wall thickening, thickened anterior mitral valve leaflets, chordae rupture or may be associated with congenital malformations. Right ventricular hypertrophy due to heart broke into the right ventricle can cause ventricular outflow tract obstruction. Right ventricular free wall course of the elderly may be due to obstructive lesions or thickening of the pulmonary circulation rising pressure. Ventricular septal and ventricular wall of the coronary artery wall thickening often, the lumen is small, may lead to transmural myocardial obstruction. Clinical manifestations
Clinical symptoms are shortness of breath after exertion, syncope or dizziness, and angina after the event, and is similar to aortic stenosis. About 10% of cases because of paroxysmal or persistent atrial fibrillation, or palpitations caused by circulation embolism. Appeared in advanced cases of congestive heart failure, orthopnea and pulmonary edema. Common symptoms are increased apical pulse, the shift to the lower left, lifting impulse or dual common sexual impulses. The lower left sternal border or apex can be heard mid-ejection systolic murmur transmitted to the base of the heart, often accompanied by tremor. Cases were associated with mitral regurgitation systolic murmur apical area were completely, 2 split heart sounds, heart sounds can be heard 3 or 4 heart sounds. But can not hear systolic ejection sound like s. Strong shock waves around the artery, disappearance of smaller waves, and water will pulse similar. Auxiliary examination
Chest X-ray: increased heart shadow, left ventricular increased, but no aortic enlargement or signs of calcification in the leaflets. Advanced cases, the left atrium and right ventricle can increase lung field of blood vessels Yu. ECG: shows left ventricular hypertrophy and strain, and sometimes chest leads aVL and I showed abnormal Q waves. Some patients showed complete right bundle branch, left bundle branch or left anterior half branch block and left atrial hypertrophy. Cardiac catheterization: right heart catheterization showed pulmonary artery pressure can be increased or signs of right ventricular outflow tract obstruction. Cardiac catheterization showed the left ventricular end-diastolic pressure increased significantly, the left ventricular cavity and outflow tract systolic pressure gradient between. Aortic or peripheral arterial pressure waveform shows increased support rapid rise, showing two peaks, and then slowly decline. Ventricular extrasystoles after the aortic pulse pressure decreased. Taking nitroglycerin, amyl nitrite, isoproterenol, digitalis, and manual labor, and Valsalva maneuver to enhance myocardial contractility, increased left ventricular outflow tract obstruction, can lead to noise loudness strengthened, increasing systolic pressure gradient . Selective angiography of left ventricular outflow tract can be displayed before the top of the uplift of the ventricular septal hypertrophy and outflow tract of the posterior wall of the anterior mitral valve leaflet, left ventricular cavity bending, end-systolic left ventricular volume small and thick papillary muscle. Left ventricular angiography with or without mitral regurgitation can still be ascertained. Adult patients should undergo coronary angiography, in order to understand whether coronary artery disease. Echocardiography: shows marked thickening of left ventricular wall, ventricular septal hypertrophy more than the ventricular wall, left ventricular cavity is small, outflow tract obstruction and systolic anterior mitral valve leaflet when the forward displacement. Medical Education Network
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