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BACKGROUND: Heterotaxy syndrome is a condition in which the thoracoabdominal organs demonstrate abnormal lateral arrangement and is often associated with congenital heart disease. Little is known about the adult heterotaxy syndrome population with congenital heart disease (CHD). OBJECTIVE: Describe the outcomes and sociodemographics of the adult CHD population with heterotaxy syndrome. METHODS: Records of patients 18 years of age or older with diagnoses of both congenital heart disease and heterotaxy syndrome at Texas Children’s Hospital from 1964–2018 were reviewed. RESULTS: Sixty-two patients met inclusion criteria. Median age was 22.7 [IQR 19.6–30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap in care of >3 years. Median follow-up time in adulthood was 2.9 [IQR 1.3–8.2] years. Forty-three (69%) of patients had single ventricle heart disease, 31 (71%) of whom completed Fontan circulation. A total of 36 interventions occurred in 24 patients which included 16 cardiac catherization interventions, 13 electrophysiology related procedures and 18 surgical procedures including 2 orthotopic heart transplants. The median age for death or heart transplant was 45.3 (95%CI 34.3–56.1) years. Heart failure-free survival was 80.8 ± 5.2%, 58.7 ± 11.0% and 31.1 ± 15.7% at 20, 30 and 40 years old. Cerebrovascular accident-free survival was 84.3 ± 5.1%, 54.2 ± 11.3%, and 40.6 ± 14.5% at 20, 30 and 40 years old. Tachyarrhythmia-free survival was 54.0 ± 7.1%, 29.2 ± 8.3%, and 19.5 ± 9.7% at 20, 30 and 40 years old and bradyarrhythmia-free survival was 66.0 ± 6.3%, 41.7 ± 9.4%, and 33.4 ± 10.6% at ages 20, 30 and 40 years. CONCLUSIONS: At a tertiary referral center, adult patients with CHD and heterotaxy syndrome have high rates of comorbidities and early death or heart transplant. Longitudinal surveillance and further exploration into factors associated with improved survival in this population are warranted.

Objective The purpose of this study was to explore the transition process in congenital heart disease (CHD) care through the perceived needs and concerns of adolescents (pretransition) and the experiential insight from adults (post-transition), in order to inform future transition initiatives and information delivery methods. Design In this cross-sectional study, adolescents and adults with moderate or complex CHD participated in semistructured telephone interviews. Interview transcripts were coded using NVivo qualitative data software. Setting Single large urban tertiary care CHD center Patients Adolescents (between 16 and 20 years of age) and adults (21–40 years of age) with moderate or complex CHD. Patients with global developmental delay or known chromosomal abnormalities were excluded. Twenty adolescents and 20 adults participated in the study; each interview allowed for participants to discuss issues relevant to them. Results Among adolescents, half wanted more CHD information and half were concerned about becoming adults with CHD. All adolescents had access to the Internet via a mobile phone, but only 55% had searched online for CHD information. Adolescents were interested in mentorship with other adolescents (90%) and adults (60%) with CHD either in person or via protected social media. Among adults, 55% were satisfied with the amount of information they had received as adolescents. Areas in which adults would have preferred additional information include longer-term implications of CHD, employment, insurance, family planning, and mental health. Conclusions There are similarities and differences in the perceived transition needs of a diverse group of adolescents and adults with CHD. Both cohorts desire additional information during the transition process, including interest in use of a transition checklist to learn about managing their CHD. Adolescents are interested in interactions with other teens and adult mentors with CHD and are open to Internet-based interventions. Web-based initiatives could deliver CHD information and provide a platform for social media mentorship.

Public health research is an integral part of the study of congenital heart disease. While this type of research has become more popular, particularly over the past decade, it has a history that stretches back to almost the beginnings of pediatric cardiology as a field. This review aims to introduce the concepts and methodologies of public health and how they relate to congenital heart disease, describe some of the challenges of traditional research methods in congenital heart disease, describe the history of public health research, and demonstrate the relevance of public health research, particularly databases, to pediatric cardiology fellows.

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).

Obesity is pandemic in Western society. Currently, approximately 100 million Americans are overweight (body mass index > 25 kg/m2) or obese (body mass index > 30 kg/m2). The pandemic is largely attributable to the relatively recent (from an evolutionary perspective) adoption of a sedentary lifestyle, coupled with the high availability of foods with high caloric content in Western cultures. These factors superimposed on dated genotypes have given rise to the global obesity epidemic. Over the past two decades, the discovery of leptin and other new molecules (e.g., adiponectin, resistin, ghrelin) has shed significant light on the pathophysiologic mechanisms of obesity-related morbidities, many of which became apparent through human epidemiologic studies during the last half of the 20th century. Of high concern for modern Western societies is the pediatric obesity epidemic, which stands to cripple Western cultures, both literally and financially in terms of health care costs and exhaustion of finite medical resources. The prevalence of childhood obesity has more than tripled since the 1960s, and 12.5 million (~17%) of children and teenagers are obese in the United States today. The rate of increasing prevalence of childhood obesity is staggering, and the collective efforts of the pediatric medical community and scientists are essential for battling the epidemic.