Objectives This paper aims to update clinicians on “Hot Topics” in the management of patients with d-transposition of the great arteries (D-TGA) in the current surgical era. Background The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA and 90% of patients now reach adulthood. Methods The Adult Congenital and Pediatric Cardiology Council (ACPC) of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, prenatal diagnosis, surgical timing, balloon atrial septostomy (BAS), prostaglandin therapy (PGE), intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, aortic dilation and regurgitation (AR), neurodevelopmental (ND) issues and lifelong care of D-TGA patients. Results In simple D-TGA, 1) familial recurrence risk is low; 2) children diagnosed prenatally have improved cognitive skills compared with those diagnosed postnatally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and PGE may not be beneficial in some cases; 5) early ASO improves outcomes and reduces costs with a low mortality. Single or intramural coronary arteries remain risk factors; 6) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 7) coronary insufficiency and arrhythmias are rare but associated with sudden death; 8) early and late-onset ND abnormalities are common; 9) AR and aortic root dilation are well tolerated; and 10) the aging ASO patient may benefit from “exercise-prescription” rather than restriction. Conclusions Significant strides have been made in understanding risk factors for cardiac, ND and other important clinical outcomes after ASO.

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. We identified a genome-wide significant association mapping to a 200 kb region on chromosome 20q11 [P= 1.72 × 10 for rs3746446; imputed Single Nucleotide Polymorphism (SNP) rs6088703 P= 3.01 × 10, odds ratio (OR)= 1.6 for both]. This result was supported by transmission disequilibrium analyses using a subset of 541 case families (lowest P in region= 4.51 × 10, OR= 1.5). Replication in a cohort of 367 LSL cases and 5159 controls showed nominal association (P= 0.03 for rs3746446) resulting in P= 9.49 × 10 for rs3746446 upon meta-analysis of the combined cohorts. In addition, a group of seven SNPs on chromosome 1q21.3 met threshold for suggestive association (lowest P= 9.35 × 10 for rs12045807). Both regions include genes involved in cardiac development-MYH7B/miR499A on chromosome 20 and CTSK, CTSS and ARNT on chromosome 1. Genome-wide heritability analysis using case-control genotyped SNPs suggested that the mean heritability of LSLs attributable to common variants is moderately high ([Formula: see text] range= 0.26-0.34) and consistent with previous assertions. These results provide evidence for the role of common variation in LSLs, proffer new genes as potential biological candidates, and give further insight to the complex genetic architecture of congenital heart disease.

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. This policy statement aims to provide the PCP with general guidelines for the care of the child with congenital heart defects and outlines anticipated problems, serving as a repository of current knowledge in a practical, readily accessible format. A timeline approach is used, emphasizing the role of the PCP and medical home in the management of patients with CHD in their various life stages.

We describe the anaesthetic management of a pair of thoracopagus twins of 14 months of age undergoing complex cardiac evaluation. Synchronous ventilation of the twins, needed for the ECG-gated magnetic resonance imaging-angiography, was achieved through a Carlens (Y) adaptor during procedures and transport. The complex logistical implications are obvious. We also describe the first use of bispectral index monitor for detection of cross-circulation in conjoint twins.

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The echocardiographic findings in fetal and neonatal VGAM may include pseudocoarctation of the aorta. Abnormal fetal cardiac findings should raise the practitioner's suspicion for cerebral malformations and vice versa.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

We describe two distinct cases in which discontinuous pulmonary arteries were identified by echocardiography and color Doppler imaging. In both cases, perfusion of one or both pulmonary arteries was dependent on a patent ductus arteriosus. Establishment of the source of perfusion and the anatomy of the discontinuous pulmonary arteries were evident only after an infusion of prostaglandin had been initiated, thus demonstrating that its use was a key component in the identification of this disease entity.