Serial Magnetic Resonance Imaging for Aortic Dilation in Tetralogy of Fallot With Pulmonary Stenosis.

Aortic dilation occurs in patients with repaired tetralogy of Fallot (TOF), but the rate of growth is incompletely characterized. The aim of this study was to assess the rates of growth of the aortic root and ascending aorta in a cohort of pediatric and adult patients with sequential magnetic resonance angiography Magnetic Resonance Imaging (MRI) data. Using serial MRI data from pediatric and adult patients with repaired TOF, we performed a retrospective analysis of the rates of growth and associations with growth of the aortic root and ascending aorta. Patients with pulmonary atresia or absent pulmonary valve were excluded. Between years 2005 to 2021, a total of 99 patients were enrolled. A follow-up MRI was performed an average of 5.9 ± 3.7 years from the initial study. For the cohort aged ≥16 years, the mean rate of change in diameter was 0.2 ± 0.5 mm/year at the ascending aorta and 0.2 ± 0.6 mm/year at the sinus of Valsalva. For the entire cohort, the mean change in cross-sectional area indexed to height at the ascending aorta was 7 ± 12 mm2/m/year and at the sinus of Valsalva was 10 ± 16 mm2/m/year. Younger age was associated with higher rates of growth of the sinus of Valsalva while the use of ß blockers or angiotensin-converting enzyme inhibitors was associated with a slower rate of growth. There were no cases of aortic dissection in this cohort. We conclude that serial MRI demonstrates a slow rate of growth of the aorta in the TOF.

Clinical response to lifestyle counseling for dyslipidemia and elevated blood pressure in childhood cancer survivors.

BACKGROUND: Childhood cancer survivors (CCS) have increased risk of premature cardiovascular disease. Whether they respond similarly to lifestyle changes for elevated blood pressure (BP), body mass index (BMI), and dyslipidemia to those without history of childhood cancer is unknown. PROCEDURE: This retrospective cohort study included CCS and 3:1 age- and sex-matched controls treated at Boston Children's Hospital Preventive Cardiology (2010-2019) using lifestyle management based on National Heart, Lung, and Blood Institute (NHLBI) guidelines. Change in BMI, BP, and lipids were analyzed. RESULTS: We included 52 CCS and 162 controls with a median age of approximately 16 years. More CCS (84.3%) had elevated baseline fasting triglycerides (TG) than controls (49.4%) (p < .001). More CCS (62.5%) also had abnormal baseline high-density lipoprotein cholesterol (HDL-C) compared to controls (35.2%) (p = .001). Baseline BMI, BP, total cholesterol (TC), and low-density lipoprotein cholesterol (LDL-C) were similar between groups. Over 15 weeks [IQR: 10.5-26], CCS had greater decrease in TG than controls (72.5 vs. 17 mg/dl decrease, p = .095). BP improved in 5% of CCS versus 38% of controls (p = .008). For both, BMI, TC, LDL-C, and HDL-C remained stable. CCS with stem cell transplantation (SCT) had a TC increase of 5% (6 mg/dl) compared to a decrease of 9% (19 mg/dl) among CCS without SCT (p = .02). CONCLUSIONS: CCS demonstrated similar improvement in lipids, but impaired BP lowering in response to lifestyle management compared to controls. Further prospective studies are needed to determine if earlier pharmaceutical treatment is warranted in this higher risk population and for the long-term risk reductions of these approaches.

Reproductive health conversations in adolescents with CHD.

BACKGROUND: Reproductive issues as related to CHD must be discussed in the clinic and at home. Providers can ensure that correct information is imparted to the adolescent and encourage mothers to provide support and guidance to the adolescent. The level to which these conversations occur is unknown. METHODS: A survey distributed to female adolescent/mother dyads assessed self-reported conversations with the healthcare provider and between each other about reproductive health topics. A clinician survey was completed to assess CHD diagnosis, risk of hormonal contraception, and pregnancy risk. RESULTS: Among 91 dyads, 33.0% of adolescents and 42.9% of mothers reported discussing recurrence risk of CHD with the provider. In regard to the cardiac lesion affecting a baby, 30.7% of adolescents and 28.7% of mothers reported discussing this with a provider. Significantly less adolescents and mothers reported discussing the risks of hormonal contraception and pregnancy with a provider. In assessing conversations between adolescents and mothers, only 44.2% of adolescents and 52.3% of mothers reported discussing with each other the safety of using birth control and 46.5% of adolescents and 64.0% of mothers reported discussing the safety of pregnancy. CONCLUSIONS: Adolescents with CHD and their mothers report low rates of reproductive health-related conversations with the healthcare provider, and mothers report low rates of having these conversations with their daughters. These topics should be discussed at each appointment with the cardiologist and must be encouraged to continue at home.

Successful Allogeneic Stem Cell Transplant in Pediatric Patients With Diminished Left Ventricular Ejection Fractions.

Chemotherapy-associated cardiotoxicity may delay or impair the ability to administer fully myeloablative chemotherapy for stem cell transplant in those with reduced left ventricular ejection fraction. Studies in adults have been inconsistent regarding the value of ejection fraction in predicting cardiotoxicity in the posttransplant period. Recent publications, however, have demonstrated successful stem cell transplantation in adults despite low ejection fractions. This case series highlights 2 pediatric patients who were successfully treated with stem cell transplantation without posttransplant cardiac complications, despite pretransplant ejection fractions of 38% and 29%.

Unusual interatrial fistula discovered during device closure of an atrial septal defect.

Atrial septal defect (ASD) closure is a common reason for referral to the cardiac catheterization laboratory. We report a case in which a relatively large Qp:Qs of 1.85:1 was demonstrated in the face of a small secundum ASD. This led to further investigation, ultimately leading to the discovery of an unusual atrial fistula. Rare cases of interatrial tunnels have been described in the literature, however, this is a unique case of a left atrial appendage to right atrial appendage fistula in the setting of a right juxtaposed left atrial appendage.