Pallister-Killian syndrome (PKS) is a sporadic multisystem genetic diagnosis characterized by facial dysmorphia, variable developmental delay and intellectual impairment, hypotonia, hearing loss, seizures, differences in skin pigmentation, temporal alopecia, diaphragmatic hernia, congenital heart defects, and other systemic abnormalities. Although congenital heart defects have been described in...

The neuromuscular blocking agent cisatracurium is frequently used adjunctively in anesthesia to facilitate endotracheal intubation and to provide muscle relaxation during surgery. We aimed to determine the pharmacokinetics (PK)/pharmacodynamics (PD) of cisatracurium in patients with congenital heart defects (CHDs), such as ventricular septal defects and atrial septal defects, and to assess the ...

Many surgical approaches have been taken when closing ventricular septal defects after correcting transposition of the great arteries in order to avoid postoperative complete heart block. We describe a residual ventricular septal shunt that developed after repair using de Leval's method in a patient with congestive heart failure. The defect was closed transaortically without a complete heart bl...

The neuromuscular blocking agent cisatracurium is frequently used adjunctively in anesthesia to facilitate endotracheal intubation and to provide muscle relaxation during surgery. We aimed to determine the pharmacokinetics (PK)/pharmacodynamics (PD) of cisatracurium in patients with congenital heart defects (CHDs), such as ventricular septal defects and atrial septal defects, and to assess the ...

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...

BACKGROUND Kabuki syndrome (KS) is an entity of multiple congenital malformations with mental retardation with undetermined etiology. Congenital heart defects are one of the clinical manifestations of KS with insufficient elucidations. METHODS Literature of congenital heart defects associated with KS was comprehensively retrieved, collected and reviewed. The clinical features of the congenita...

BACKGROUND Congenital heart diseases cause significant childhood morbidity and mortality. Several restricted studies have been conducted on the epidemiology in Nigeria. No truly nationwide data on patterns of congenital heart disease exists. OBJECTIVES To determine the patterns of congenital heart disease in children in Nigeria and examine trends in the occurrence of individual defects across...

Background Dextrocardia is a malposition of the heart defined as the right-sided development of the heart. It can increase the likelihood of congenital heart defects or diseases (CHD) and the risk of related morbidities and mortalities. We aimed to determine the frequency of CHDs among Dextrocardia patients. Materials and Methods In a retrospective cross-sectional study the records of patients ...

Background: Patients with Down’s syndrome are prone to have congenital heart defects. This study was conducted to evaluate the frequency of various congenital heart defects in children with Down’s syndrome in Khyber Pukhtunkhwa province. Material & Methods: This descriptive study was conducted at Department of Pediatrics, Lady Reading Hospital Peshawar, from July 2011 to June 2012. Fifty-five p...

Anatomically true cross-sectional ultrasonic images of the heart have demonstrated ventricular septal defects in 25 patients with this lesion as an isolated anomaly or in conjunction with other defects. In two additional patients a defect was visualized but confirmation was not obtained. Ventricular septal defects were not demonstrated in 13 other patients in whom this lesion was identified by ...