Nutritional management and home monitoring programs (HMPs) may be beneficial for improving interstage morbidity and mortality following stage I Norwood palliation (S1P) for hypoplastic left heart syndrome (HLHS). We recognized an increasing trend towards early feeding gastrostomy tube (GT) placement prior to discharge in our institution, and we aimed to investigate the effect of HMPs and GTs on interstage mortality and growth parameters. Single-institutional review at a tertiary referral center between 2008 and 2018. Individual patient charts were reviewed in the electronic medical record. Those listed for transplant or hybrid procedures were excluded. Baseline demographics, operative details, and interstage outcomes were analyzed in GT and non-GT patients (nGT). Our HMP was instituted in 2009, and patients were analyzed by era: I (early, 2008–2012), II (intermediate, 2013–2016), and III (recent, 2017–2018). 79 patients were included in the study: 29 nGTs and 50 GTs. GTs had higher number of preoperative risk factors more S1P complications, longer ventilation times, longer lengths of stay, and shorter times to readmission. There were no differences in interstage mortality or overall mortality between groups. There was one readmission for a GT-related issue with no periprocedural complications in the group. Weight gain doubled after GT placement in the interstage period while waiting periods for placement decreased across Eras. HMPs and early GTs, especially for patients with high-risk features, provide a dependable mode of nutritional support to optimize somatic growth following S1P.

Study Objective: This study evaluated self-reported discussions with health care providers (HCPs) among adolescent and young adult (AYA) women with congenital heart disease (CHD). Design: Data were collected through a one-time survey of AYA women. Setting: Participants were recruited from pediatric cardiology clinics. Participants: AYA women with CHD, ages 14-21 (N = 107) Interventions: None Main Outcome Measures: Questionnaires assessed adolescent characteristics and specific HCP discussions regarding transmissibility of a cardiac condition to the infant, risk of pregnancy, and hormonal contraception. Outcome measures were self-reported discussions with HCPs about these reproductive health topics. Results: Mean age was 16.8 years (SD = 2.1). Self-reported reproductive health discussions were infrequent, including discussions on transmissibility of a heart condition to their offspring (37%), risk of pregnancy to their offspring (34%), risks of pregnancy to their health (46%), and risks of hormonal contraception given their heart condition (21%). Reported discussions were most commonly with a cardiologist. Conclusions: AYA women with CHD reported limited discussions about reproductive health topics important to those with CHD. Lack of appropriate and timely counseling could lead to poor maternal and child health outcomes. Targeted interventions that improve reproductive health discussions between HCPs and AYA women with CHD are needed to close critical information and service gaps.

Background: Prolongation of the ECG derived QT interval predicts increased risk for sudden life-threatening arrhythmias. Many medicines are known to prolong the QT interval though current data demonstrate that progesterone given to premenopausal females <18 years shortens the QT interval by six msec. This effect may be used to treat individuals with abnormal prolongation of the QT interval. Hormone progestin is used to treat a number of conditions in post-menarche females >18 years old. The effects of this treatment on the corrected QT (QTc) in this population are unknown. Objective: To describe the effect of exogenous progestin on the QTc in adolescent females. Methods: In post-menarcheal females, <18 years old, using no medications with known ECG effects, we compared QTc in milliseconds (ms) on ECG evaluations in those taking exogenous progestin vs those who are not. Descriptive statistics were used to compare the groups. Results: There were 40 controls and 21 treated participants. The age range was 10 - 17 years. There were no differences between the groups with regard to race, height, weight, BMI, or blood pressure. In the controls, the mean QTc was 403 +/-19 milliseconds (ms) vs. 397 +/-15 ms in those treated (p=0.22). Those on progestin therapy had a shorter QTc by the same magnitude difference (six ms) as the hormonal naive group in the adult literature. Conclusions: We report no adverse effects of progestin associated with QTc prolongation and a trend suggesting a decreased QTc in a population of post menarcheal-adolescent females.

Background: Reproductive issues as related to congenital heart disease (CHD) such as recurrence risk in offspring, safety of hormonal birth control, and safety of pregnancy for mother and baby must be discussed in the clinic and at home. Providers can ensure correct information to assist the adolescent in making informed decisions about reproductive health and allow the parent to provide proper 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 …

Objective: Stage I Norwood palliation (S1P) for functional single ventricle physiology carries a mortality risk of 15%, and patients remain at risk for adverse outcomes during the interstage period prior to second stage palliation (S2P). Feeding dysfunction (FD) is common in this population and may be associated with poorer outcomes. While there is wide institutional variation in nutritional management, there have been increasing efforts to ameliorate interstage mortality over the past decade emphasizing nutrition, feeding delivery, and home monitoring programs (HMPs). In 2009, we implemented an HMP, and by …

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.

Despite prenatal diagnosis, prenatal intervention, and immediate postnatal intervention, patients with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have the highest risk for mortality. Charts for all infants diagnosed with hypoplastic left heart syndrome from 2009 to 2017 were retrospectively reviewed and compared, including pulmonary vein Doppler patterns on fetal echocardiogram and evidence of pulmonary lymphangiectasia on fetal MRI. Of the 81 newborns with hypoplastic left heart syndrome, we defined two groups. Group 1 patients had an adequate atrial septal communication (n = 69), while Group 2 met criteria for intact/restrictive septum (n = 12). No patient in Group 1 had a type C pulmonary vein Doppler pattern, while no patient in Group 2 had a type A pulmonary vein Doppler pattern. The two patients with pulmonary lymphangiectasia had type C pulmonary vein Doppler pattern and an intact atrial septum and did not survive. Survival to discharge for Group 1 was 83% compared to 58% for Group 2 (p = 0.116). Survival to stage 2 palliation was 71% for Group 1 compared to 50% for Group 2 (p = 0.186). Only 4 of the initial 12 patients from Group 2 are alive, which is an overall survival of 33%. Our experience supports previous evidence that fetal echocardiography can identify those patients with the greatest likelihood for postnatal intervention as well as those at highest risk for mortality. Fetal MRI is a novel imaging modality that may help providers separate patients at highest risk for mortality, regardless of pulmonary vein Doppler pattern.

A 4-year-old female underwent surgical repair of an unusual variation of an aorto-left ventricular tunnel (ALVT). Serial echocardiograms had demonstrated previous spontaneous closure of a perimembranous ventricular septal defect (VSD). The patient presented with concern for residual VSD which was later demonstrated to be an eccentric jet through an ALVT. This case emphasizes early and accurate diagnosis for ALVTs and how they can be misdiagnosed as VSDs.