Assuntos
Síndrome de Brugada/embriologia , Síndrome de Brugada/patologia , Crista Neural/anormalidades , Potenciais de Ação , Animais , Síndrome de Brugada/etiologia , Síndrome de Brugada/fisiopatologia , Movimento Celular , Conexina 43/biossíntese , Eletrocardiografia , Junções Comunicantes , Cardiopatias Congênitas/embriologia , Cardiopatias Congênitas/etiologia , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Humanos , Crista Neural/metabolismo , Crista Neural/fisiopatologia , Fenótipo , Canais de SódioRESUMO
BACKGROUND: Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction. Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm. An even more rare presentation is the coexistence mid-ventricular and apical HCM. This case is a combination of obstructive HCM with mid-ventricular HCM and an apical aneurysm, which to date, has not been reported in the literature. CASE PRESENTATION: The patient is a 49 year-old lady who presents a combination of septal asymmetric hypertrophic cardiomyopathy (HCM) and midventricular HCM, a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, plus an apical aneurysm. Her clinical presentation was an acute myocardial infarction in June 2005. One month after hospital discharge, the electrocardiogram (ECG) showed a right bundle branch block (RBBB) with no Q waves or ST segment elevation. Coronary angiography revealed normal coronary arteries, left ventricular hypertrophy and an apical aneurysm. CONCLUSION: This case is a rare example of an asymptomatic patient with subaortic and mid-ventricular hypertrophic cardiomyopathy, who presents a myocardial infarction and normal coronary arteries, and during the course of her disease develops an apical aneurysm.