BACKGROUND Fetal cardiac intervention represents a potential advance in the treatment of congenital cardiac lesions that increase in complexity during development. Prenatal repair of a primary defect might prevent pathologic blood-flow patterns that can result in hypoplasia of a cardiac chamber or great vessel. However, strategies to optimize fetal myocardial protection have not been studied. A...

OBJECTIVE We have utilized subclavian flap angioplasty (SFA) frequently in infants with coarctation particularly in patients with arch hypoplasia which is quite frequent. We have followed these patients with serial echocardiography and have analyzed our results in this study to determine recoartation rates, recurrent hypertension and left arm development. METHODS Thirty eight infants less tha...

HAND2 is an essential transcription factor for cardiac, pharyngeal arch, and limb development. Apoptosis in the HAND2-null embryo causes hypoplasia of the right ventricle and pharyngeal arches leading to lethality by embryonic day (E)10.0 from heart failure. In order to investigate the role of apoptosis in inducing the HAND2-null phenotype, we generated mouse embryos lacking both HAND2 and Apaf...

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 ...

introduction left ventricular (lv) twist is believed to store potential energy and plays an important role in generating diastolic suction. recent advances in echocardiography techniques have allowed quantification of lv twist. the aim of the present study was to compare lv twist and torsion in healthy human subjects determined by velocity vector imaging (vvi) and tissue doppler imaging (tdi) a...

Abstract Background Joubert syndrome (JS) is a rare autosomal recessive genetic heterogeneously inherited disorder characterized by neurological features that include hypotonia, ataxia, developmental delay, intellectual disability, abnormal eye movements, and neonatal breathing dysregulation. Case presentation The main purposes of the case report are to highlight benefit multidisciplinary rehab...

The purpose of this study was to assess the longitudinal relationships between enamel hypoplasia and caries experience of primary second molars. The study sample was 491 subjects who received dental examinations at both age 5 and 9 by the calibrated examiners. Four primary second molars (n = 1,892) were scored for the presence of enamel hypoplasia for each participant. Caries presence and numbe...

OBJECTIVES To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible. METHODS We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and...

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...

Renal hypoplasia refers to a congenitally small kidney where there is essentially normal residual parenchyma but smaller calyces, lobules, and papillae. This anomaly can be divided into two broad groups: complete (global) renal hypoplasia and segmental renal hypoplasia. In this study, a 3 days-old simmental calf was diagnosed with renal hypoplasia and unreconstructed exstrophic bladder. It pres...