engleski

An inverse variant of hypertrophic cardiomyopathy : first case reports in Croatia

Hypertrophic cardiomyopathy (HCM) is a primary disease of the heart and it appears in a number of variants. Hypertrophy most frequently and most extensively involves the IVS with the LVPW not affected or slightly thickened. The inverse variant of HCM is rare form of this disease, characterized by a dominant hypertrophy of the LVPW and a normal or mild tickening of IVS. In most patients sinus rhythm is preserved. Medicamentous therapy is efficacious in only one third of the patients. In our 2 patients (male, age 18 and 14 yr.) hypertrophy most severely affected the LVPW (20 mm in both) and the IVS was of normal tickness (8 and 7 mm respectively), so that the IVS/LVPW ratio was 0.4 and 0.3 respectively. In the first patient only the LA was dilated, and in the second all four cavities were dilated. The possible cause of global heart dilatation in the second patient was an unrecognized oligosymptomatic myocarditis. The "SAM" phenomenon was not observed in either of the patients. Mitral and tricuspid regurgitation was present in both. EF was normal in the patient with non-dilated LV (62%), and reduced in the patient with dilated LV (28%). The sinus rhythm was preserved in both patients and ECG demonstrated signs of LV hypertrophy. The chest x-ray in both patients showed an enlarged heart shadow and signs of pulmonary congestion. Medical therapy in the first patient (ACE-inhibitors, diuretics, calcium antagonists) caused regression from stage NYHA IV to II, and in the second patient (ACE-inhibitors, diuretics, digitalis) from stage NYHA IV to stage NYHA III. To the best of our knowledge, these are the first reported cases of the inverse variant oh HCM in Croatia.

hypertrophic cardiomyopathy; inverse variant; case report

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