Ebstein’s anomaly accounts for 0.5% of all congenital heart diseases. The condition is characterized by >8 mm/m2 displacement of the septal and posterobasal tricuspid leaflets toward the apex of the right ventricle (RV). This causes “atrialization” of the RV inflow tract, with decreased size of the RV and right atrial (RA) dilatation. The anterior valve is rarely displaced, but tends to be larg...

Hemodynamics influence cardiac development, and alterations in blood flow may lead to impaired cardiac growth and malformations. The developing myocardium adapts to augmented workload by increasing cell number (hyperplasia). The aim of this study was to determine the influence of alterations in ventricular preload on fetal myocyte proliferation by manipulation of intracardiac shunting at the at...

BACKGROUND Cdc42 is a member of the Rho GTPase family and functions as a molecular switch in regulating cytoskeleton remodeling and cell polarity establishment. Inactivating Cdc42 in cardiomyocytes resulted in embryonic lethality with heart developmental defects, including ventricular septum defects and thin ventricle wall syndrome. FINDINGS In this study, we have generated a Cdc42 cardiomyoc...

A 16-year-old male patient who underwent bilateral modified Blalock-Taussig shunt operation at the age of 6 was admitted to our clinic with cyanosis. The oxygen saturation was 60% at time of admission. Echocardiography and computed tomography revealed situs solitus dextrocardia with concordant atrioventricular (AV) discordant ventriculoarterial relation and obstructed bilateral Blalock-Taussig ...

Mandibular coronoid aplasia is defined as the absence of process and hypoplasia defective or underdeveloped process. Hypoplasia mandibular condyle means that smaller than normal. Both these situations are very rare in literature. In literature, condylar usually associated with hyperplasia seen together. The case report we prepared a unilateral hypoplasia, which not syndrome, second literature t...

Received March 20, 2017; revised manuscript received July 16, 2017; accepted August 1, 2017; released online September 1, 2017 Time for primary review: 45 days Department of Cardiovascular Medicine (K.M., S.F., K.S., A.T., T.O., N.N., S.Y.), Department of Pediatric Cardiology (H.O.), National Cerebral and Cardiovascular Center, Suita, Japan Mailing address: Shigefumi Fukui, MD, PhD, Department ...

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were...

Hypoplastic left heart syndrome (HLHS) comprises 2 to 3 percent of all congenital heart disease. HLHS is uniformly lethal if left untreated; however, there is currently an estimated 70 percent 5-year survival rate for infants who undergo surgical correction. The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment...