Thelansis

 Hypertrophic obstructive cardiomyopathy (HOCM), once known as idiopathic hypertrophic subaortic stenosis, is a condition commonly encountered. It stands as a significant contributor to sudden cardiac death among young individuals, including athletes, affecting both genders equally across diverse racial backgrounds. The familial form of HOCM follows an autosomal dominant inheritance pattern, making it the most prevalent genetically inherited cardiomyopathy. Additionally, it can manifest in individuals without a familial history due to de novo mutations. HOCM arises from gene mutations responsible for encoding sarcomere proteins, including B-myosin heavy chain, troponin, actin, and titin. These mutations induce structural irregularities in myofibrils and myocytes, potentially resulting in abnormal force generation and conduction issues. Consequently, in the absence of alternative causes, this leads to the characteristic phenotype of asymmetric left ventricular hypertrophy. Classific...

 Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and dyspnea, angina, and syncope symptoms. Obstructive Hypertrophic Cardiomyopathy (oHCM)—defined as HCM with left ventricular outflow tract obstruction causing a peak left ventricular outflow gradient of at least 30 mm Hg is observed in approximately 70% of patients with HCM and frequently leads to symptoms including dyspnea, fatigue, chest pain, or syncope. The unique pathophysiology underlying the obstruction is its functional dynamic nature, which is greatly influenced by alterations in the load imposed on the left ventricle and its contractility. The initial therapy for symptomatic patients with obstruction is medical therapy with β-blockers and calcium antagonists. However, there remains a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. · ...