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- Title
A RARE CAUSE OF GENETIC CARDIOMYOPATHY - CASE REPORT.
- Authors
Munteanu, Marius Andrei; Gorga, Simona Madalina; Nacu, Andrada Georgiana; Rodean, Ioana Patricia; Muresan, Laurentiu; Benedek, Theodora
- Abstract
Background: Left ventricular non-compaction (LVNC) is a very rare congenital cardiomyopathy representing an abnormality in the maturation process of the myocardium. The main characterisctic of this condition is the presence of endomyocardial trabeculations that increase in number and prominence. Due to possible late onset of the symptomatology, the first presentation of this condition can be represented by malignant arrhythmias, thromboembolic phenomenon and left ventricular dysfunction. Objective: Our aim is to present a rare case of LVNC in a young male patient, 23-year-old, with no previous cardiovascular history, who was admitted into our department for marked dyspnoea, fatigue at reduced efforts and a presyncopal condition. Material and methods: The objective clinical examination performed, revealed the presence of peripheral edemas, generalized cyanosis and bilateral basal crackels on auscultation. The blood pressure was also low. ECG assessment revealed sinus tachycardia and the presence of negative T waves in the infero-lateral leads. Furthermore, a transthoracic echocardiography was performed which reveled signifcant lef ventricle (LV) dilatation associated with moderate enlargement of the left atrium and right ventricle. Signifcant systolic dysfunction with poor left ventricular ejection fraction (30%) was also observed. In the apex of the LV, the image of an intracardiac mass was observed. The characteristic aspect of LVNC was also revealed by echocardiography (prominent trabeculation of the LV and deep recesses). In order to elucidate the origin of the apical mass, a transesophageal echocardiography was performed, which confirmed the presence of an apical thrombus. To exclude associated congenital malformations a cardiac CT was performed, which was negative. Results: The LVNC diagnosis was esteablished due to the cardiac imaging techniques in a young patient without any hisorty of cardiovascular disease. Conclusions: To make an early diagnosis and to prevent the possible complications of this disease, it is mandatory to use appropiate cardiac imaging tecniques.
- Subjects
CONGENITAL heart disease; TRANSESOPHAGEAL echocardiography; CARDIOMYOPATHIES; BLOOD pressure; DISEASE complications; CYANOSIS; SINUS thrombosis
- Publication
Acta Medica Marisiensis, 2019, Vol 65, p49
- ISSN
2068-3324
- Publication type
Article