RÉSUMÉ
Non-compaction cardiomyopathy (NCC) is characterized by trabeculations in either one or both ventricles. Clinical presentation is highly variable: dyspnea, palpitation, thromboembolic events, arrhythmia, or sudden cardiac death. There are currently no universally-accepted criteria for classifying and diagnosing left ventricular non-compaction (LVNC) cardiomyopathy. Transthoracic echocardiography (TTE) is the diagnostic exam of choice. The diagnosis is often missed or delayed because of a lack of knowledge about this uncommon disease. Progression of LVNC is highly variable and prognosis is very difficult to predict. We report a case of a 50-year-old female patient with a history of total thyroidectomy under hormonal supplementation who consults for dyspnea and paroxysmal palpitations revealing an isolated LVNC. This case emphasizes the importance of imaging techniques, which are, TTE and cardiac magnetic resonance imaging (MRI) in early diagnosis, management, and follow-up.
RÉSUMÉ
Abstract Noncompaction cardiomyopathy (NCCM) and left ventricular noncompaction (LVNC), in their isolated form, are rare cardiomyopathies. They are characterized by a thickened myocardium due to the presence of deep trabeculae recesses, and to thick trabeculae. This condition is associated with a variable clinical phenotype including heart failure, thromboembolism, and sudden death. We report a case of LVNC at 26 weeks and 4 days of gestation revised on the basis of what is currently reported in the literature. A review of the literature was performed to better describe this rare condition. Left ventricular noncompaction is a rare fetal condition and it should be suspected in case of cardiomyopathy.
Sujet(s)
Humains , Femelle , Jeune adulte , Échographie prénatale , Non-compaction isolée du ventricule/imagerie diagnostique , Coeur/imagerie diagnostique , Myocarde/anatomopathologieRÉSUMÉ
Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.
Sujet(s)
Adulte , Femelle , Humains , Mâle , Grossesse , Jeune adulte , Biopsie , Cardiomyopathies , Diagnostic , Échocardiographie , Électrocardiographie , Développement embryonnaire , Non-compaction isolée du ventricule , Imagerie par résonance magnétique , Myocarde , Syncope , Tachycardie ventriculaireRÉSUMÉ
Isolated noncompaction of ventricular myocardium, sometimes referred to as spongy myocardium, is a rare congenital cardiomyopathy. Spongy myocardium results from an arrest in normal endomyocardial embryogenesis. The clinical symptoms and electrocardiographic findings of this disorder can be showed variously. The diagnosis is usually made by echocardiography. We report a case of isolated noncompaction of the ventricular myocardium in a 20-year-old male patient initially presented with syncope caused by ventricular tachycardia, who was diagnosed with echocardiography, cardiac MRI and endomyocardial biopsy.