Abstract
47 years old male smoker with no co morbidities, presented with history of chest pain in the retrosternal area for the 3 days. The Pain was described as sharp, non-exertional and intermittent. Patient did not have any family history of known cardiac disease or sudden cardiac death. A Clinical examination failed to reveal any abnormalities. An ECG revealed deep T wave inversion in the anterolateral chest leads with left ventricular hypertrophy (LVH). Bloods analysis showed normal Complete blood count, liver function and mildly increased cardiac troponins. The Patient was admitted to the CoronaryCare Unit with a diagnosis of acute coronary syndrome. Patient was initiated dual antiplatelets, fondaparinux and high intensity atorvastatin. Following this, an echocardiogram revealed severe Left ventricular hypertrophy and reduced LV (left ventricular) cavity dimensions. Good LV systolic function with grade 3 diastolic dysfunction was noted. Coronary angiogram showed a normal right and left coronary system. Patient was diagnosed with hypertrophic cardiomyopathy. Cardiac MRI Showed severe Left ventricular hypertrophy with interventricular septum thickness 3.7 cm with rest of the walls hypertrophied. There was evidence of severe fibrosis of the septum, anterior and lateral wall. On the basis of severe left ventricular hypertrophy (especially septal thickness >3.5 cm) and myocardial fibrosis, Patient was started on beta blockers and ICD was inserted for primary prevention of arrhythmias.
Highlights
Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most commonly caused by mutations in sarcomere genes
Hypertrophic cardiomyopathy is characterized by left ventricular hypertrophy of various morphologies including Left ventricular outflow obstruction, apical or septal hypertrophy
Hypertrophy Cardiomyopathy is genetic cardiomyopathy caused by mutations in sarcomere genes
Summary
Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most commonly caused by mutations in sarcomere genes. Prior epidemiological studies didn’t take into account autosomal dominant transmission of HCM For these reason, Prevalence of HCM in general population has been estimated to closer to 0.5 percent [5]. Advanced heart failure symptoms of orthopnea, paroxysmal nocturnal dyspnea, and oedema are uncommon This case is important as the patient’s presentation was highly suggestive of acute coronary syndrome because of his history, ECG changes and mild increase in troponins. It is important to possess a wide range of experience when dealing with cases This is a classic example of a common presentation of a relatively uncommon disease. Other common presentation of HCM is syncope, sudden death and arrhythmias This case pertains to a 47 years old male with a history of chest pain lasting for three days.
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