Coronary artery disease in a child with homozygous familial hypercholesterolemia: Regression after liver transplantation.

Children with homozygous familial hypercholesterolemia are at risk for early cardiovascular events secondary to coronary artery disease. Current medical therapy does not ameliorate this risk. Liver transplantation offers the most effective option to reduce circulating levels of low-density lipoprotein cholesterol and thereby reduce risk of cardiovascular events. Angiographic evidence of regression of coronary artery disease is presented.

Outcomes in adults with congenital heart disease and heterotaxy syndrome: A single-center experience.

BACKGROUND: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with congenital heart disease (CHD). Little is known about the adult HS population with CHD. OBJECTIVE: To describe the outcomes and sociodemographics of the adult CHD population with HS. METHODS: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 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, respectively. 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, respectively. 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, respectively. CONCLUSIONS: At a tertiary referral center, adult patients with CHD and HS 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.

Functional classification of heart failure before and after implementing a healthcare transition program for youth and young adults transferring from a pediatric to an adult congenital heart disease clinics.

OBJECTIVE: To describe changes in functional status between the last pediatric and first adult congenital heart disease (CHD) clinic visits in patients with moderate to severe CHD after implementing a healthcare transition (HCT) planning program. DESIGN: Quasi-experimental design. Patients were followed prospectively following the implementation of the intervention; Control patients transitioned from the Pediatric CHD Clinic into Adult CHD Clinic before the intervention. SETTING: Texas Children's Hospital (TCH). PATIENTS: Sixteen to 25-year-olds, cognitively normal, English speaking patients with moderate to severe CHD who transitioned from the Pediatric to the Adult CHD clinic. INTERVENTIONS: An EMR-based transition planning tool (TPT) was introduced into the Pediatric CHD Clinic. Two nurses used the TPT with eligible patients. Independent of the intervention, two medicine-pediatric CHD physicians and one nurse practitioner were added to the ACHD Clinic to address growing capacity needs. OUTCOME MEASURES: The New York Heart Association Functional Classification of Heart Failure (NYHAFS). RESULTS: Control patients waited 26 ± 19.2 months after their last pediatric clinic visit for their first adult visit. Intervention patients waited 13 ± 8.3 months (P = .019). Control and Intervention patients experienced a lapse in care greater than two (50% vs 13%, P = .017) and three (30% vs 0%, P = .011) years, respectively. The difference between the recommended number of months for follow-up and the first adult appointment (15.1 ± 17.3 Control and 4.4 ± 6.1 Intervention months) was significant (P = .025). NYHAFS deteriorated between the last Pediatric visit and the first ACHD visit for seven (23%) Control patients and no Intervention patients (P = .042). Four of seven Control patients whose NYHAFS declined had a lapse of care of more than two years. CONCLUSIONS: There is a need for improved HCT planning for patients with moderate to severe CHD, otherwise, lapses of care and adverse outcomes can ensue.

Lifestyle Modification for the Prevention of Morbidity and Mortality in Adult Congenital Heart Disease.

Patients with adult congenital heart disease are now living longer due to the advancements in medicine. As such, these patients are now experiencing morbidities that are commonly seen in the general population such as myocardial infarction, heart failure, and arrhythmias. Often times these problems can be attributed to the underlying adult congenital heart disease; however, a patient making poor lifestyle choices only compounds their risk for these life-threatening comorbidities. The aim of this article is to propose recommendations for health care providers to follow with this specific patient population. These recommendations encompass the importance of proper caloric intake, methods of weight loss (including behavioral therapy, drugs, and surgeries), practical recommendations for physical activity, and the implications of substance abuse. Being proactive and addressing important lifestyle choices in this population can reduce comorbidities and, therefore, medical cost.

Coronary artery anatomy in congenital heart disease.

Expanded surgical options and improved outcomes for children born with structural heart defects have ushered a greater clinical interest in the normal and abnormal development of the coronary circulation. Anatomic variations of the coronary system may impact surgical candidacy or operative technique during neonatal life, while others may impact long-term clinical management and planning for subsequent interventions. This review aims to characterize coronary artery anatomy in symptomatic congenital heart disease, emphasizing the clinical consequence of these variations and anomalies.

Outcomes of heart failure-related hospitalization in adults with congenital heart disease in the United States.

BACKGROUND: Heart failure (HF) accounts for >3 million hospital admissions annually in adults with acquired cardiovascular disease, but there are limited data on HF admissions in adults with congenital heart disease (ACHD). The purpose of this study was to test the hypotheses that HF admissions are common in ACHD and associated with significant morbidity and mortality. METHODS: The 2007 Nationwide Inpatient Sample was used to assess national prevalence, morbidities, and risk factors for mortality during hospitalizations among ACHD with HF. RESULTS: Of the 84,308 (95% CI 71,345-97,272) ACHD admissions in the United States in 2007, 17,193 (95% CI 14,157-20,229) had a diagnosis of HF (20%). ACHD with HF was associated with an increased risk of death compared to ACHD without HF (OR 3.3, 95% CI 2.6-4.1). On multivariable analysis independent risk factors for mortality included nonoperative intubation (OR 6.1, 95% CI 3.3-11.4), sepsis (OR 4.3, 95% CI 2.4-7.4), and acute myocardial infarction (OR 3.2, 95% CI 1.8-5.7). Cardiac defects associated with an increased risk of mortality included ventricular septal defects (VSDs) (OR 1.8, 95% CI 1.0-3.4). CONCLUSIONS: In this large population-based study, HF-related hospitalizations were common in ACHD and associated with an increased risk of death compared to non-HF admissions. The risk of mortality is increased with the diagnoses of VSDs and the presence of specific comorbidities such as respiratory failure and sepsis.

Variability in delivery of care and echocardiogram surveillance of Kawasaki disease.

OBJECTIVE: The objective of this study is to characterize variability in the acute management of Kawasaki disease and compliance of echocardiogram surveillance with published American Heart Association recommendations. DESIGN: Retrospective review. SETTING: Tertiary care children's hospital. PATIENTS: All patients discharged from our institution with Kawasaki Disease between 1999 and 2007 were identified. Patients meeting any of the following were excluded: presence of a comorbidity that necessitated echocardiogram follow-up independent of Kawasaki disease diagnosis, previous history of Kawasaki disease, or magnetic resonance imaging of the coronary arteries performed in place of echocardiography. Preexisting or comorbid conditions resulting in study exclusion included structural heart disease, arrhythmia, and concomitant severe multiorgan disease at presentation (e.g., sepsis). OUTCOME MEASURES: The time course of echocardiogram surveillance among those with a normal echocardiogram at diagnosis was evaluated for compliance with published American Heart Association recommendations. Coronary artery involvement at presentation was characterized using standardized values. Additional characterization of national care practices for children with Kawasaki disease was obtained via distribution of an internet-based survey to pediatric hospitalists. RESULTS: Overall, only 11 (4%) of 302 patients with a normal study at diagnosis received a total of three studies at recommended intervals. Using standardized values for coronary artery dimensions in place of Japanese Ministry of Health aneurysm criteria, 59 (13%) of patients with Kawasaki disease experienced coronary artery involvement at diagnosis. The majority of the early coronary artery abnormalities detected in these patients using standardized definitions persisted on short-term follow-up. Pediatric hospitalist survey results revealed significant interinstitutional variability in the management of these patients. CONCLUSIONS: Lack of optimal surveillance after a diagnosis of Kawasaki disease may result in the underdiagnosis of coronary artery pathology or other complications. Considerable variability in surveillance and acute management exists, and additional research is needed to determine optimal screening and care delivery models for this population.

Identifying arrhythmias in adults with congenital heart disease by 24-h ambulatory electrocardiography.

Adults with congenital heart disease (CHD) are at risk for the development of arrhythmias. This study aimed to assess the incidence of unsuspected arrhythmias among adults with CHD identified on electrocardiograms (ECGs) and 24-h ambulatory electrocardiographic monitoring (Holter monitoring). A review of the cardiology database at the authors' institution from July 2004 through December 2007 identified all clinic patients 18 years old or older who had a recent ECG and Holter monitoring. Data collection included diagnosis, ECG and Holter monitoring results, arrhythmias, and the presence or absence of symptoms. The review identified 140 patients. Analysis of the ECGs showed that 15% of the patients had an arrhythmia. These arrhythmias consisted of ectopy (6%), supraventricular tachycardia (SVT) (3%), pacemaker issues (2%), and previously unrecognized atrioventricular block (AVB) (1%). The majority of the patients with arrhythmias were asymptomatic (76%). Analysis of the Holter monitoring results showed that 31% of the patients had arrhythmias consisting of ectopy (17%), SVT (12%), ventricular tachycardia (7%), high-grade AVB (5%), and pacemaker issues (3%). Of the patients with arrhythmias, 80% were asymptomatic. Among the patients without arrhythmias on ECG, 26% had arrhythmias noted on Holter monitoring. Of the patients with multiple Holter monitorings performed, 34% had a new arrhythmia noted on repeat monitoring. In conclusion, arrhythmias were present in a significant number of adults with CHD, but the majority were asymptomatic. Among adults with CHD, even those with normal ECGs, arrhythmias were frequently detected on Holter monitoring. In addition, repeat Holter monitoring may identify significant arrhythmias over time.