Hypertrophic Cardiomyopathy

Heart, 1977

Attempts to identify a pathognomonic abnormality in 'idiopathic hypertrophic subaortic stenosis' date back to the time of its recognition. We examined the echocardiographic, haemodynamic, and angiographic features of 14 consecutive patients in whom the clinical diagnosis was supported by the presence of both asymmetrical septal hypertrophy and systolic anterior movement of the anterior leaflet of the mnitral valve on the echocardio- gram, or an intraventricular pressure gradient at rest or with provocation. A gradient was shown in 12 cases (86%), the Brockenbrough or postextrasystolic response in 9 (64%), asymmetrical septal hypertrophy in 11 (79%), systolic anterior movement in 7 (50%), and left ventricular cavity obliteration in systole in 12 (86%). movement of the anterior leaflet of the mitral valve 945

Journal of the American College of …, 1983

The distribution of left ventricular hypertrophy was assessed by M-mode and two-dimensional echocardiography in 89 patients with hypertrophic cardiomyopathy. Myocardial thickness was measured in the septum and the free and posterior wall in both the proximal and distal left ventricle. All patients had at least one myocardial region that was hypertrophied. The predominant pattern of hypertrophy was defined as symmetr ic (31%), asymmetric septal (55% ) and distal ventricular (14%). The spectrum of wall thickness measurements between patients with symmetric hypertrophy was wide (1.5 to 4.5 em) and was not related to age. In patients with asymmetric septal hypertrophy , the distribution of hypertrophy conformed to previously described patterns; hypertrophy was localized to the anterior septum (14%) Hypertrophic cardiomyopathy is defined as a heart muscle disorder of unknown origin that is characterized by unexplained hypertrophy of a nondilated left ventricle ( I). During the past decade, the most widely applied diagnostic criteria were derived from the M-mode echocardiogram. These criteria emphasized the demonstration of asymmetric hypertrophy between the upper anterior septum and the left ventricular posterior wall as well as features associated with Icft ventricular pressure gradients, such as systolic anterior motion of the mitral valve and mid-systolic closure of the aortic valve. Recent studies, however, have shown that myocardial regions that are not visualized by the M-mode beam may be hypertrophied in the absence of asymmetric septal hypertrophy (2,3). Thus, using the M-mode technique, the diagnosis and pattern of myocardial hypertrophy may be undetected. Two-dimensional echocardiography permits evaluation of the entire left ventricle (4-6). The or the anterior and posterior septum (35%) or involved both the septum and the left ventricular free wall (51%). The patients with distal ventricular hypertrophy had marked papillary muscle thickening, and only 1 of 12 patients could be correctly diagnosed using M·mode echocardiography ,

2021

Journal of Clinical & Experimental Cardiology, 2017

Hypertrophic cardiomyopathy is a disease of the myocardium characterized by the thickening of the heart walls. It is a genetic disorder transmitted by first-degree relatives, caused by mutations in genes that code for sarcomere protein. A 77-year-old woman presented to the emergency department and complained of continuous vertigos and presyncope with subsequent fall to the floor with a concussive traumatic brain injury. The ECG observed signs of left ventricular hypertrophy. Blood exams highlighted a slight increase of troponin T (25 ng/L). The ultrosonography instead demonstrated a pronounced left ventricular hypertrophy with a 240 mmHg dynamic gradient, a moderate mitral regurgitation secondary to SAM. Lastly, ECG Holter excluded significant arrhythmias (1 couple of ventricular contractions). In the ward, the echocardiographic pattern was repeated and confirmed the clinical picture of a pronounced ventricular hypertrophy at the expense of the IVS (VTD 45 ml), a diastolic dysfunction Grade I with anterior systolic movement of the mitral flap valves with a dynamic obstruction to the left ventricular outflow (DP max 260 mmHg). Moreover, a moderatesevere mitral insufficiency was reported. Vital signs were stable. The antihypertensive therapy was enhanced with added ACE inhibitors, loop diuretics, beta blockers and the disopyramide administration started. The patient was discharged from the hospital and was scheduled a cardiologic echocardiographic follow up in order to evaluate the effectiveness of the therapy. This study has demonstrated the association between the clinical symptom, the clinical objectivity and the echocardiographic anomalies typical of the obstructive hypertrophic cardiomyopathy. Severe blood pressure values of dynamic obstruction to the left ventricular outflow have been recorded and which are not currently known by the scientific literature. Our purpose will be to evaluate the clinical and echocardiographic follow up and the potential response to the pharmacological therapy prescribed.

Anadolu kardiyoloji dergisi : AKD = the Anatolian journal of cardiology, 2006

Zaporozhye medical journal, 2024

Aim. To describe a clinical case and analyse the challenges in diagnosing hypertrophic cardiomyopathy (HCM) in a patient with concomitant arterial hypertension (AH). Materials and methods. The article presents the clinical case of HCM in a patient with concomitant AH that was observed in an ambulatory setting in the Municipal enterprise «Dnipropetrovsk Regional Clinical Center for Diagnostics and Treatment» of the Dnipropetrovsk Regional Council». Results. Patient N., a 66-year-old woman diagnosed by her family physician with coronary artery disease (CAD): stable angina II FC, arterial hypertension (AH) of 2nd grade, and chronic heart failure II FC NYHA, was referred to a cardiologist because of experiencing exertional dyspnea, chest pain, and uncontrolled blood pressure despite adhering to prescribed treatment. Echocardiography identified concentric left ventricular hypertrophy with the left ventricular outflow tract (LVOT) obstruction (a mean gradient of 35 mmHg as per catheterisation). Cardiac MRI confirmed the diagnosis and coronary angiography ruled out CAD. Adjustments to the treatment regimen, taking into account HCM with LVOT obstruction, effectively alleviated the patient's symptoms and stabilised her blood pressure. Conclusions. In patients with high blood pressure, it is crucial to adhere to guidelines of AH management and perform echocardiography on all patients so as not to miss the signs of concomitant HCM, especially with LVOT. In addition, in the case of HCM, it is imperative to identify and provide prevention and manage patients prone to sudden cardiac death timely. Since HCM encompasses various diagnoses with different pathogenesis and distinct management, according to guidelines, cardiac MRI, enzyme checking or genetic testing may be required. As HCM is a relatively common inherited cardiac disease, physicians could frequently encounter such patients during regular clinical practice. Hence, they need to have some suspicion of this diagnosis in persons with AH whose left ventricular mass meets the criteria for HCM.

EPRA International Journal of Multidisciplinary Research (IJMR)

It is a septal hypertrophy, asymmetric type, causing left ventricular outflow obstruction due to thickening of the left ventricular wall; this pathology has a worldwide prevalence of 0.02-0.23%; and it is the most important cause of sudden death in young people. It is characterised by myocardial hypertrophy leading to diastolic dysfunction; epidemiologically, it occurs equally in men and women, although women tend to be more symptomatic, more disabled and present at a younger age. The patient may be asymptomatic or may present with dyspnoea, precordial pain and syncope. For diagnosis, chest X-ray, electrocardiogram and echocardiography may be used; MRI and cardiac catheterisation only in cases to identify the aetiology and severity of the disease. For treatment, background medication such as beta-blockers may be used, or alcohol ablation techniques may be performed. However, in some cases, pacemakers or implantable defibrillators may be needed. The aim of this literature review is t...

The American Journal of Cardiology, 1982

Clinical and morphologic features are described in a unique subgroup of seven patients with hypertrophic cardiomyopathy. Five patients either died suddenly or are alive but severely symptomatic. In each patient ventricular septal hypertrophy was demonstrated on two dimensional echocardiography or at necropsy to be virtually confined to its apical one-half. However, conventional M mode echocardiography was unreliable in identifying this site of hypertrophy because it was oflen inaccessible to the path of the M mode beam. Apical distribution of septal hypertrophy does not constitute a separate disease entity, but rather appears to be part of the morphologic spectrum of hypertrophic cardiomyopathy, as judged from two findings: (1) genetic transmission of hypertrophic cardiomyopathy in relatives of each study patient; and (2) marked disorganization of cardiac muscle cells in the left ventricular wall of the two patients studied at necropsy.

Circulation, 2002

A 42-year-old woman was referred for evaluation of progressive exertional dyspnea and fatigue over the past 2 years. Both symptoms were greatly intensified during paroxysms of atrial fibrillation, which occur every 3 or 4 months. The patient was asymptomatic until 5 years ago, when she suffered a single syncopal episode precipitated by a bout of coughing in the erect position. She subsequently experienced episodes of presyncope under similar circumstances and at increasing frequency, but she learned to abort frank syncope by immediately lying or sitting down. Examination revealed a grade 3/6 systolic murmur along the left sternal border and at the apex. The diagnosis of obstructive hypertrophic cardiomyopathy (HCM) was established by 2-dimensional and Doppler echocardiography, which showed asymmetric septal hypertrophy, a subaortic systolic pressure gradient at rest estimated at 80 mm Hg, and mild mitral regurgitation. Her symptoms did not respond to the sequential administration of atenolol, verapamil, disopyramide, or the combination of atenolol and disopyramide. Although amiodarone reduced the frequency of the paroxysms of atrial fibrillation, she has required cardioversion for individual episodes and receives warfarin.

Clinical Cardiology, 1989

The severity and site of hypertrophy is important in determining the clinical picture and the natural history of hypertrophic cardiomyopathy (HCM). We evaluated left ventricular hypertrophy by means of twodimensional echocardiographic score and score index, and correlated these findings with symptoms, electrovectorcardiographic data, and ventricular arrhythmias. A total of 42 patients with HCM were studied by clinical examination, ECG, VCG, M-mode and 2D echocardiography, and 24-h Holter monitoring. The extent and severity of the hypertrophic process were calculated by a score system. The left ventricle was divided into 11 segments and a hypertrophic score (HS) was given to each segment. A hypertrophy score index (HSI) was also calculated by dividing the number of hypertrophied segments by 13. No correlation was found between symptoms and HS and HSI, nor ECG-VCG abnormalities and HS and HSI. A statistically significant relationship between the severity of ventricular arrhythmias and HS and HSI was found (p<O.Ol).