Browsing by Author "Ilisic, Tamara (8285901300)"

A double-chambered right ventricle (DCRV) is an uncommon congenital anomaly: the right ventricle (RV) is divided into two chambers due to the presence of an abnormally located muscular band or anomalous muscle hypertrophy in the subinfundibular part of RV outflow tract, with a variable degree of obstruction. Generally, DCRV is well recognized in childhood and misdiagnosed in adult patients. Transthoracic and/or transesophageal echocardiography are the mthods of choice for the diagnosis of DCRV. Due to limitations of echocardiography in adult patients, this entity may be missed, particularly if it presents concomitant with other congenital defects, and therefore additional imaging methods such as MRI or cardiac catheterization are required for a definitive diagnosis. © 2016 S. Karger AG, Basel.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with scimitar syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome. © 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc.

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The lesser form is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the branch pulmonary arteries is often associated with other congenital heart defects and extracardiac anomalies, as well as with 22q11.2 microdeletion. We report three infants with crossed pulmonary arteries and one adolescent with lesser form of the malformation. The results suggest that diagnosis of malposition of the branch pulmonary arteries could be challenging if based solely on echocardiography, whereas modern imaging technologies such as contrast computed tomography and magnetic resonance angiography provide reliable establishment of diagnosis. In addition, we performed the first molecular characterisation of the 22q11.2 region among patients with malposition of the branch pulmonary arteries and revealed a 3-megabase deletion in two out of four patients. © 2012 Cambridge University Press.