Redefining the Relationship: Palliative Care in Critical Perinatal and Neonatal Cardiac Patients.

Patients with perinatal and neonatal congenital heart disease (CHD) represent a unique population with higher morbidity and mortality compared to other neonatal patient groups. Despite an overall improvement in long-term survival, they often require chronic care of complex medical illnesses after hospital discharge, placing a high burden of responsibility on their families. Emerging literature reflects high levels of depression and anxiety which plague parents, starting as early as the time of prenatal diagnosis.

When the course deviates from expected: Misplacement of an epicutaneo-caval catheter in a neonate.

Central venous access, a common and essential component of the care of the critically ill neonate, is associated with complications such as infection, thrombosis, and bleeding. Unintentional arterial cannulation of a venous catheter is a rare but potentially dangerous complication. In the report, we describe the accidental cannulation of an artery with an epicutaneo-caval catheter in an extremely low birth weight infant.

Hand-held echocardiography in children with hypoplastic left heart syndrome.

Background: When performed by cardiologists, hand-held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.

Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled.

Isolated coarctation of the aorta in the fetus: A diagnostic challenge.

Isolated coarctation of the aorta (CoA) is estimated by the Centers for Disease Control and Prevention to account for 4%-6% of all congenital heart disease (CHD) in the United States, with a reported prevalence of ~4 per 10 000 live births. Prenatal recognition of coarctation is important as it may improve neonatal survival and reduce morbidity. However, despite advances in imaging and the trend toward detailed aortic arch assessment as part of a comprehensive fetal echocardiogram, isolated CoA may still elude prenatal detection, with potentially lethal consequences if the diagnosis is not suspected and the patent ductus arteriosus (PDA) closes spontaneously in postnatal life.

The Care of Children With Congenital Heart Disease in Their Primary Medical Home.

Congenital heart disease (CHD) is the most common birth anomaly. With advances in repair and palliation of these complex lesions, more and more patients are surviving and are discharged from the hospital to return to their families. Patients with CHD have complex health care needs that often must be provided for or coordinated for by the primary care provider (PCP) and medical home.

A genome-wide association study of congenital cardiovascular left-sided lesions shows association with a locus on chromosome 20.

Congenital heart defects involving left-sided lesions (LSLs) are relatively common birth defects with substantial morbidity and mortality. Previous studies have suggested a high heritability with a complex genetic architecture, such that only a few LSL loci have been identified. We performed a genome-wide case-control association study to address the role of common variants using a discovery cohort of 778 cases and 2756 controls.

D-transposition of the great arteries: the current era of the arterial switch operation.

This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients.

Type B interrupted left aortic arch with isolated right subclavian artery.

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left-sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).

Acute hemodynamic decompensation following patent ductus arteriosus ligation in premature infants.

Introduction: Patent ductus arteriosus (PDA) ligation can lead to postoperative hemodynamic instability requiring inotropic support, termed hemodynamic decompensation. The purpose of this study was to prospectively determine the incidence, predictors, and clinical impact of hemodynamic decompensation after PDA ligation in preterm infants.

Methods: All infants undergoing PDA ligation were eligible for this prospective cohort study.

Perinatal pseudocoarctation: echocardiographic findings in vein of Galen malformation.

Objective: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital malformations thought to develop during weeks 6 to 11 of fetal life. Although they represent less than 1% of all cerebral vascular malformations, they constitute up to 30% of all pediatric vascular malformations. Vein of Galen aneurysmal malformations cause high-output heart failure in the fetus and neonate secondary to the decreased resistance and high blood flow in the lesion.

What is new in pediatric cardiology.

Enormous advances in the diagnosis and management of heart disease in pediatric patient have taken place during the last-four decades. In this review the authors will concentrate on the developments within the last five to ten years. It will deal with what may be called medical advances.