PHACE syndrome is a neurocutaneous syndrome with the following features: posterior fossa malformations of the brain, large facial hemangiomas, arterial anomalies, cardiac anomalies and aortic coarctation, and eye abnormalities. We report a rare case of bilateral internal carotid artery agenesis with transcranial collaterals from the external carotid arteries and agenesis of the vertebrobasilar ...

Coronary anomalies are less frequent than acquired coronary diseases, such as atherosclerosis, and have been implicated a cause of cardiovascular events. This case report describes an incidental finding on angiography anomalous right artery with origin in the left sinus intra-arterial course, after episode aborted sudden death. The Heart Team analysis indicated percutaneous approach drug-elutin...

The trigeminal artery is the largest of the fetal carotid-basilar anastomotic arteries, and it persists for the longest embryonic period. The artery usually involutes after the development of the posterior communicating artery. The exact causes of persistence of this primitive vessel into adulthood are not completely clear. Angiographic and anatomical descriptions of the various persistent trig...

The pulmonary sequestration spectrum and related congenital lung anomalies present an extremely complex and varied group of bronchopulmonary-vascular malformations. Previous attempts at nomenclature and classification have proved inadequate. In this article we present a classification of the newly named pulmonary malinosculation spectrum, which includes all congenital lung anomalies where there...

Dear Editor, We read with interest the article of Chou et al. (1). The authors reviewed the multidetector computed tomography (CT) findings of congenital coronary sinus anomalies and reported two cases of coronary sinus anomaly. We would like to share our observations, with respect to coronary sinus anomalies, with our colleagues. For better clinical outcomes, coronary venous variations should ...

BACKGROUND Aberrant coronary arteries represent a diverse group of congenital disorders. Post-mortem studies reveal a high risk of exercise-related sudden cardiac death in those with an anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an inter-arterial course. There is little documentation of lifetime history and long-term follow-up of patients with coronar...

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenge...

Doerr's theory of the morphogenesis of transposition is discussed with special reference to recent studies by Goor and co-workers and Anderson and associates. The views advanced by all these authors coincide in three points: (a) the description of the reorganisation process occurring at the arterial end of the embryonic heart (a process called by Doerr vectorial bulbus rotation); (b) the pathog...

BACKGROUND AND PURPOSE PHACE is an acronym for posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Several case reports of arterial ischemic stroke (AIS) in individuals with PHACE have been published, but risk factors for AIS in PHACE have not been clearly defined. The objective of this article is to review a...

Conotruncal and ventricular septal congenital heart anomalies result from defects in formation and division of the embryonic outflow tract. Cardiac remodeling during outflow tract and ventricular septation converts the tubular embryonic heart into a parallel circulatory system with an independent left ventricular outlet and right ventricular inlet. Tbx3 encodes a T-box-containing transcription ...