Biventricular Conversion of Borderline Hypoplastic Left Heart Complex Facilitated by Spontaneous Closure of the Atrial Septum.

We present the case of a child with borderline hypoplastic left heart complex who initially received Norwood and Glenn operations without atrial septectomy and was later converted to a biventricular circulation after progressive growth of the left ventricle as assessed by serial echocardiography and cardiac magnetic resonance imaging.

Improving maternal-infant bonding after prenatal diagnosis of CHD.

Background: Infants with prenatally diagnosed CHD are at high risk for adverse outcomes owing to multiple physiologic and psychosocial factors. Lack of immediate physical postnatal contact because of rapid initiation of medical therapy impairs maternal-infant bonding. On the basis of expected physiology, maternal-infant bonding may be safe for select cardiac diagnoses.

PRKAG2 mutations presenting in infancy.

PRKAG2 encodes the γ2 subunit of AMP-activated protein kinase (AMPK), which is an important regulator of cardiac metabolism. Mutations in PRKAG2 cause a cardiac syndrome comprising ventricular hypertrophy, pre-excitation, and progressive conduction-system disease, which is typically not diagnosed until adolescence or young adulthood. However, significant variability exists in the presentation and outcomes of patients with PRKAG2 mutations, with presentation in infancy being underrecognized.

Associations Between Day of Admission and Day of Surgery on Outcome and Resource Utilization in Infants With Hypoplastic Left Heart Syndrome Who Underwent Stage I Palliation (from the Single Ventricle Reconstruction Trial).

Newborns with hypoplastic left heart syndrome and other single right ventricular variants require substantial health care resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations but has not been studied in patients with single ventricle. Subjects of the Single Ventricle Reconstruction trial were classified by whether they had a weekend admission and by day of the week of Norwood procedure.

Right ventricular echocardiographic indices predict poor outcomes in infants with persistent pulmonary hypertension of the newborn.

Aims: Infants with persistent pulmonary hypertension of the newborn (PPHN) have elevated pulmonary vascular resistance that can lead to right ventricular (RV) failure and death. Clinicians must decide which infants will fail conventional therapy and require transfer to extra corporeal membrane oxygenation (ECMO) centres, but accurate echocardiographic predictors have not been identified. We assessed echocardiographic measurements of RV pressure and function in predicting progression to death or ECMO in infants with PPHN.

Left ventricular myocardial performance index change for detection of acute cellular rejection in pediatric heart transplantation.

EMB, the gold standard for diagnosis of ACR, poses unique risks in children. Limited cross-sectional data have associated LV MPI with ACR. We hypothesize that a relative change in MPI from baseline without ACR to the time of ACR will better detect ACR than an absolute threshold LV MPI value.

Prognostic significance of recurrent grade 1B rejection in the first year after pediatric cardiac transplantation: a case for reinstatement of the 1B rejection grade.

The 2005 ISHLT rejection grading system merged grades 1A, 1B, and 2 into a single grade (1R) assuming equivalent prognostic significance. We hypothesized that recurrent 1B ACR is associated with adverse outcomes. Data on all heart transplant recipients at our center from 1990 to 2007 were reviewed.

Effect of dexmedetomidine on pulmonary artery pressure after congenital cardiac surgery: A pilot study.

Objective: To characterize the effects of dexmedetomidine on the pulmonary artery pressure in patients after congenital cardiac surgery.

Design: Prospective observational pilot study.

Setting: Pediatric cardiac intensive care unit at a university hospital.

Neonatal congestive heart failure due to a subclavian artery to subclavian vein fistula diagnosed by noninvasive procedures.

Unlabelled: Congestive heart failure in the neonate is usually due to intracardiac anomalies or cardiac dysfunction. Extracardiac causes are rare.

Patient: We report a newborn infant who presented with respiratory distress and cardiomegaly.

The management of hypoplastic left heart syndrome with a right aortic arch.

The combination of hypoplastic left heart syndrome and a right-sided aortic arch is extremely rare and lethal. To the best of our knowledge, no patient with this combination has previously been reported as surviving initial palliation. The anatomic variant is associated with abnormalities in the arteries branching from the aortic arch, making it difficult to construct a reliable source of flow of blood to the lungs.