Naslov
Neočekivano ili možda (ipak) ne: prikaz slučaja
(Unexpected or maybe not: a case report)

Autori
Pehar-Pejčinović, Vesna ; Peršić, Viktor ; Buršić, Vedran ; Miškulin, Rajko ; Bursać, Iva Uravić ; Rakić, Marijana ; Travica Samsa, Dijana ; Raljević, Damir ; Rotkvić, Luka

Izvornik
Cardiologia Croatica (1848-543X) 13 (2018), 11-12; 414-414

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, stručni

Ključne riječi
koronarna arterijska fistula, spužvasta kardiomiopatija
(coronary artery fistula, ventricular noncompaction)

Sažetak
Left ventricular noncompaction is a rare congenital cardiomyopathy. It can exist in isolated form (INVM) in adults or neonatal form which is caused by mutations gene located on the X chromosome and associated with other congenital cardiac and neuromuscular disease. Isolated noncompaction of the ventricular myocardium (INMV), first described by Chin et al in 1990, is characterized by persistent embryonic myocardial morphology without other cardiac anomalies. In such cases, deep recesses communicate only with the ventricular cavity, not the coronary circulation, whereas in non-compaction associated with other congenital heart disease (non-isolated non-compaction), the intertrabecular recesses communicate both with the left ventricular cavity and the coronary circulation. During embryonic weeks 5 and 8, the ventricular myocardium transforms from a hypertrabeculated morphology to a compacted layer, and this process is concomitant with coronary artery development. Myocardial remodeling proceeding from the epicardium to endocardium and from the base of the heart to the apex. The coronary circulation develops concurrently during this process, and the intertrabecular recesses are reduced to capillaries. Congenital coronary artery fistulas (CAFs) are abnormal communications between a coronary artery and any cardiac cavity or great vessel. Approximately 20% of patients with coronary artery fistulae have other cardiac anomalies, most frequently aortic and pulmonary atresia and patent ductus arteriosus. Coronary artery fistulae between a coronary artery and a cardiac chamber is a rare condition and is found in approximately 0.2% of patients undergoing cardiac catheterization. Non-compaction ventricular myocardium (NVM) in combination with multiple coronary artery to ventricle fistulae are rare cardiovascular malformations.1-3 We present 53- year-old female patient with INMV, with preserved ejection fraction and functional impairment of left ventricle proved with deformation imaging methods (speckle-tracking echocardiography), and existence of multiple coronary to left ventricle fistulae.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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