INTRODUCTION Macrocephaly-capillary malformation syndrome is characterized by cutaneous vascular lesions, including cutis marmorata telangiectatica and hemangiomas, associated with congenital anomalies, including macrocephaly, macrosomia, asymmetry and mental retardation. In addition to these cardinal signs, several other clinical conditions have been reported in people with this condition. How...

THE electrocardiogram in patients with tetralogy of Fallot usually shows right axis deviation and right ventricular hypertrophy. After creation of a systemic-pulmonary artery anastomosis, the work of the left ventricle increases, usually accompanied by a corresponding increase in the pattern of left ventricular loading." 2 Following openheart surgery, with closure of the ventricular septal defe...

Migration of cardiac neural crest cells into the pharyngeal arches and the pharyngeal and splanchnic mesoderm contributes to the development of the cardiac outflow tract. The adrenal cortex is derived from the splanchnic mesoderm. Neuroblastoma is more prevalent in patients with congenital heart disease than in the general population, because both originate from embryonal neural crest-derived c...

BACKGROUND Although "squatting" in patients with tetralogy of Fallot is famous, the hemodynamic mechanism of the phenomenon has not been fully elucidated. To clarify the mechanism of squatting, the change in aortic pulsatile property induced by squatting was investigated. METHODS Twelve healthy young adults were enrolled in this study. The change in central pressure waveform induced by squatt...

Myhre syndrome is a rare autosomal dominant disorder caused by a narrow spectrum of missense mutations in the SMAD4 gene. Typical features of this disorder are distinctive facial appearance, deafness, intellectual disability, cardiovascular abnormalities, short stature, short hands and feet, compact build, joint stiffness, and skeletal anomalies. The clinical features generally appear during ch...

A 24-year-old woman with a history of unrepaired tetralogy of Fallot was scheduled to undergo laparoscopic cholecystectomy. Her significant history included tetralogy of Fallot with pulmonary atresia, hypoplastic left pulmonary artery, pulmonary vascular obstructive disease, a functioning right subclavian artery to right pulmonary artery shunt (modified Blalock-Taussig palliative procedure) wit...

The heart rate of ven-tricular tachycardia following an old myocardial infarction is inversely related to the size of scarring. resonance-based anatomical analysis of scar-related ventricular tachycardia: implications for catheter ablation. Cardiovascular magnetic resonance by non contrast T1-mapping allows assessment of severity of injury in acute myocardial infarction. Aletras AH et al. Myoca...