It has been recognized that patients with Cyanotic Congenital Heart Disease (CCHD) show significant bleeding tendency which can be secondary to coagulopathies in these patients. Some coagulation abnormalities are thrombocytopenia, factor deficiencies, fibrinolysis and Disseminated Intravascular Coagulation  (DIC). According to high prevalence of CCHD and major operations in theses patients, th...

Patients with the third and fourth pharyngeal pouch syndrome, thymic and parathyroid aplasia or hypoplasia, have a very high incidence of aortic arch anomalies and congenital heart disease. These patients present with a unique syndrome characterized by profound hypocalcemia, defective thymic-mediated cellular immune function, and cardiovascular anomalies. The cardiac abnormalities most frequent...

A 22 year old man developed symptoms of left ventricular failure secondary to atrial fibrillation and congenital mitral regurgitation. After operation for mitral valve repair he was unable to be successfully weaned from cardiopulmonary bypass and this was ascribed to poor left ventricular function. He therefore underwent emergency cardiac transplantation but again was unable to be weaned from b...

Coronary artery problems in children usually have a significant impact on both short-term and long-term outcomes. Early and accurate diagnosis, therefore, is crucial but technically challenging due to the small size of the coronary artery, high heart rates, and limited cooperation of children. Coronary artery visibility on CT and MRI in children is considerably improved with recent technical ad...

Pulmonary hypertension complicates the course of many newborns with congenital diaphragmatic hernia. In the most severe cases, the fetal condition of markedly elevated pulmonary vascular resistance persists after birth and is associated with hypoxemic respiratory failure and severe disturbances in cardiac performance. Late pulmonary hypertension (weeks to months after birth) is increasingly rec...

Congenital long QT syndrome is an inherited disorder of cardiac repolarization that predisposes to syncope and to sudden death from polymorphic ventricular tachycardia. The disorder should be suspected when the electrocardiogram shows characteristic QT abnormalities, or when there is a family history of long QT syndrome or of an event that raises suspicion of long QT syndrome, such as sudden de...

A 21-year-old woman presented with clinically classic signs of Ullrich congenital muscular dystrophy (figure). Genetic testing of collagen VI genes revealed a homozygous mutation c.2329T.C, p.Cys777Arg in the COL6A2 gene, consistent with the clinical diagnosis. Collagen type VI–related disorders represent a spectrum of overlapping phenotypes: Bethlem myopathy at the milder end, and Ullrich cong...

This study briefly reviews the heart abnormalities in diverse intracranial pathologies, including strokes, spontaneous and traumatic subarachnoid hemorrhage and intracranial hemorrhage, and presents the results of a one-year prospective study of heart abnormalities in patients with moderate to severe head injuries and subarachnoid hemorrhage. Different abnormalities such as: QT -interval, T...

Patients with the third and fourth pharyngeal pouch syndrome, thymic and parathyroid aplasia or hypoplasia, have a very high incidence of aortic arch anomalies and congenital heart disease. These patients present with a unique syndrome characterized by profound hypocalcemia, defective thymic-mediated cellular immune function, and cardiovascular anomalies. The cardiac abnormalities most frequent...

A non-invasive method using continuous wave Doppler shift ultrasound and spectral analysis was used as a screening test for severe carotid artery disease in patients undergoing cardiopulmonary bypass operations. One hundred and eighty-eight patients were examined before cardiac surgery (91 for ischaemic heart disease, 17 for ischaemic heart disease and valve replacement, 66 for valve replacemen...