Dyslipidemia in adolescents and young adults with type 1 and type 2 diabetes: a retrospective analysis.

BACKGROUND: Youth onset type 1 diabetes (T1D) and type 2 diabetes (T2D) is increasing and associated with earlier vascular complications and mortality. Dyslipidemia is an important modifiable cardiovascular (CVD) risk factor that is under-recognized and undertreated in youth with T1D and T2D. Given this, we evaluated the prevalence and associations between lipid concentrations and clinical CVD risk factors in youth with T1D compared to T2D at our large ethnically diverse diabetes center. METHODS: A retrospective chart review was performed, evaluating patients with T1D or T2D seen at least once in clinic from 2015 to 2017, age 10-22 years of age, duration of diabetes at least 6 months on the date of most recent LDL-cholesterol (LDL-C) concentration, and not on statin therapy. We performed independent and multivariable linear regressions of LDL-C and HDL-cholesterol (HDL-C) concentrations. RESULTS: There were 32.7% with T1D (n = 1701) and 47.7% with T2D (n = 298) with LDL-C above recommend goal (> 100 mg/dL/2.6 mmol/L). Furthermore, there were 9% with T1D and 16.4% with T2D with LDL > 130 mg/dL (> 3.4 mmol/L), who likely met criteria for starting statin therapy. Higher LDL-C and/or lower HDL-C were associated with increased age, diabetes duration, higher HbA1C, female sex, Hispanic ethnicity, obesity, and T2D. After adjusting for these risk factors in a multivariable linear regression model, the association of higher LDL-C and lower HDL-C was higher with T2D than T1D. CONCLUSIONS: This highlights the need for more aggressive dyslipidemia screening and treatment in youth with diabetes, especially T2D. At our institution we have created and instituted quality improvement algorithms to try to address this need.

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.

Interventions in children with renovascular hypertension: A 27-year retrospective single-center experience.

BACKGROUND: Renovascular hypertension (RVH) can be caused by renal artery stenosis (RAS) and/or middle aortic syndrome (MAS). METHODS: Patients who received surgical or transcatheter treatment for RVH between 1/1991 and 11/2017 were retrospectively reviewed using age = adjusted blood pressure ratio (BPR). RESULTS: Fifty-three patients diagnosed with RVH at a median age of 4.5 (0-18) years were included. Vascular involvement ranged from MAS with RAS (20), RAS only (32), and MAS only (1). The first intervention was transcatheter in 47 patients (transcatheter group: angioplasty = 41, stenting = 5, and thrombectomy = 1), and surgical in 6 patients (surgical group), occurring at a median age of 6.2 (0.1-19.6) years. There was a change toward transcatheter interventions as the first procedure over the study period. First reinterventions in the transcatheter group (27 lesions in 18 patients) were repeat transcatheter (in 20 lesions) and surgery (7 lesions) at a median of 92 (2-2555) days; in the surgical group (5 lesions in 4 patients) first reinterventions were transcatheter (4 lesions) and repeat surgery (1) at a median of 2.2 (1.1-12.0) years. A total of 136 transcatheter and 30 surgical discrete interventions were performed. There was a significant decline in antihypertensive medications and BPR at 4-6 months after the first intervention and on last follow-up in patients initially treated by transcatheter means while the decline was not significant in the surgical group (limited by small sample size). Complications were significantly more common in the surgical group (P < .01), 11/27 (41%) vs 10/136 (7.4%). Four patients died (2 from each group): 2 with congenital renal artery atresia and MAS, 2 with MAS and RAS. The median follow-up interval was 3.6 (0.1-35.2) years. CONCLUSION: Pediatric patients with RVH treated with transcatheter means as the first intervention had significant improvement in BPR, as well as decline in antihypertensive medications and were less likely to suffer major complications.

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.

The effects of lifestyle changes on serum lipid levels in children in a real life setting.

OBJECTIVE: Studies have shown improvement in lipid levels after institution of lifestyle changes in children enrolled in closely monitored programs. There programs are difficult to mimic in real world clinics. We aim to determine if diet and exercise result in improvement in lipid levels in patients seen in a designated lipid clinic in a real life setting. DESIGN: Retrospective review of patients followed for dyslipidemia at the Texas Children's Hospital Lipid Clinic from May 1, 2012 to May 1, 2015. Patients included were seen more than once, had repeat lipid testing, and abnormal baseline lipid levels. Multivariate analysis using mixed models were performed to compare outcomes in patients who did and did not participate in lifestyle change. RESULTS: Of the 268 patients seen within the study period, 174 (56% male, 44% female) met inclusion criteria. Median age was 11 years. Compared to patients who did not make lifestyle changes: patients who made only diet changes demonstrated significant improvement in weight only (slope = -1.55, P-value = .014), and those who made only exercise changes demonstrated significant improvements in serum cholesterol (slope = -22.8, P-value = .017) and non-HDL cholesterol (slope = -28.7, P-value = < .01) levels. Patients who participated in both diet and exercise demonstrated significant improvement in weight (slope = -1.13, P-value = .011), diastolic blood pressure (slope = -1.82, P-value = < .01), and serum lipid levels: LDL (slope = -10.8, P-value = 0.017), HDL (slope = 1.52, P-value = .24), Triglycerides (slope = -0.11, P-value = .033) compared to those who did not make lifestyle changes. CONCLUSIONS: Outpatient management of dyslipidemia is difficult. Only patients who participated in both diet and exercise showed significant improvement in outcomes when compared to those who did not make lifestyle changes.

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. We identified a genome-wide significant association mapping to a 200 kb region on chromosome 20q11 [P= 1.72 × 10-8 for rs3746446; imputed Single Nucleotide Polymorphism (SNP) rs6088703 P= 3.01 × 10-9, 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 × 10-5, 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 × 10-9 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 × 10-7 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.

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.