Adolescent Women with Congenital Heart Disease: Self-Reported Reproductive Health Discussions with Health Care Providers.

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.

Annuloplasty for Aortic Regurgitation in Infantile Takayasu Arteritis: A Case Report.

Aortic regurgitation (AR) is a life-threatening complication of Takayasu arteritis (TA). Takayasu arteritis is a large-vessel vasculitis with a predominance in young adolescent and adult females. Inflammation involves the aorta and its major branches resulting in arterial dilatation, stenosis, aneurysm, occlusion, and thrombosis formation. Pediatric TA cases have been reported to also cause severe complications such as coronary aneurysms, retinopathy, and hypertension due to renovascular stenosis. In this report, we present a rare occurrence of infantile TA with severe AR requiring surgical intervention after failing medical therapy. Due to the patient's age and clinical status, a valve-sparing aortic annuloplasty with aortoplasty was performed.

The long-term functional outcome in Mustard patients study: Another decade of follow-up.

OBJECTIVE: For over 20 years, we have followed a cohort of patients who underwent the Mustard procedure for d-transposition of the great arteries. The current study follows the same cohort from our last study in 2007 to reassess their functional capacity and quality of life. PARTICIPANTS: Of the original 45 patients, six patients have required cardiac transplant and 10 patients have died, including two of the transplanted patients. Twenty-five of the remaining patients agreed to participate in this current study. DESIGN: Patients underwent comparable testing to the previous studies when possible including exercise stress testing, echocardiography, MRI or CT evaluation of cardiac anatomy and function, Holter monitor, and quality of life questionnaire. RESULTS: Thirty-one percent of patients have experienced cardiac death either in the form of mortality or cardiac transplantation. The major cause of death was systemic right ventricular failure. Sixty-five percent have continuing abnormalities of rhythm. Exercise time and workload showed a statistically significant decrease from the original study (Time 1) to both 10-year (Time 2) and 20-year (Time 3) follow-up points. Right ventricular ejection fraction decreased significantly from the Time 1 to Time 2, and again to this current follow-up. Quality of life measures of energy level decreased significantly from the original study to both the Time 2 and Time 3. CONCLUSION: Cardiac mortality for Mustard patients remains high, and over time, systemic right ventricular ejection fraction, rhythm, exercise tolerance, and quality of life assessments show deterioration. There does not appear to be a single clear predictor of poor outcome.

Bovine Jugular Vein Conduit: A Mid- to Long-Term Institutional Review.

BACKGROUND: Since 1999, we have used the bovine jugular vein conduit for right ventricular outflow tract reconstruction in infants and children. Herein, we review their mid- to long-term outcomes. METHODS: Between 1999 and 2016, 315 bovine jugular vein conduits were implanted in 276 patients. Patients were grouped by age at bovine jugular vein conduit implant: group 1: 0 to 1 years (N = 65), group 2: one to ten years (N = 132), and group 3: older than ten years (N = 118). For survival and hemodynamic analysis, additional group stratification was done based on conduit size. Group small: 12 and 14 mm (N = 75), group medium: 16 and 18 mm (N = 84), and group large: 20 and 22 mm (N = 156). RESULTS: Mean follow-up for groups 1, 2, and 3 was 4.0, 4.9, and 5.9 years, respectively. Early mortality was 9%, 0%, and 1% for groups 1, 2, and 3, respectively ( P < .001). Late mortality was 5%, 2%, and 2% for groups 1, 2, and 3, respectively ( P = .337). Group 1 had the lowest ten-year freedom from conduit failure at 13%, versus 53% and 69% for groups 2 and 3, respectively ( P < .001). A total of 21 (6.6%) patients developed endocarditis, 11 (3.5%) patients required reoperation, and 10 (3.2%) patients required antibiotic therapy alone. CONCLUSIONS: The bovine jugular vein conduit is a useful option for right ventricular outflow tract reconstruction given its easy implantability and acceptable midterm durability.

Consideration of pyloric stenosis as a cause of feeding dysfunction in children with cyanotic heart disease.

Feeding difficulty has been reported at a higher incidence in infants with cyanotic heart disease and single ventricle physiology necessitating specialized feeding strategies. However, structural causes of feed intolerance in this subset of patients should not be ignored. This case series highlights three recent cases of pyloric stenosis in infants with left-sided obstructive lesions at our institution. In all three cases, the initial presumed diagnosis was feeding intolerance related to heart disease, and there was significant clinical improvement following identification and correction of pyloric stenosis.

Significant Lead Migration of a Subcutaneous Implantable Cardioverter-Defibrillator in a Pediatric Patient.

Since its introduction, the subcutaneous implantable cardioverter-defibrillator (S-ICD) has provided the benefit of reduced mortality from ventricular tachyarrythmias without the associated short- and long-term morbidity of transvenous or epicardial implantable cardioverter-defibrillator (ICD) leads. As its name implies, the S-ICD system is implanted in its entirety, including device and lead, just under the skin beginning along the anterior axillary line, with its lead tunneled to the left parasternum and then from the xiphoid to the manubrium-sternal junction. Dislocation of the lead due to migration of the parasternal lead has been described in a minority of patients. Here, we describe an unusual case of a significant lead migration in a pediatric patient.

Recurrent takotsubo cardiomyopathy in a child.

Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.

Thromboexclusion of an atypical left ventricular pseudoaneurysm.

Left ventricular pseudoaneurysm is rare in children. The gold standard for treatment has been surgical repair. Here, we describe a boy in whom an atypical left ventricular pseudoaneurysm was treated through staged transcatheter procedures. In addition, we highlight the importance of the preprocedure cardiac magnetic resonance imaging in providing invaluable information that allowed detailed planning of a management strategy for this unusual pseudoaneurysm.

Long-term glycemic control as a result of initial education for children with new onset type 1 diabetes: does the setting matter?

PURPOSE: The purpose of this study was to examine the role of initial diabetes education delivery at an academic medical center (AMC) versus non-AMCs on long-term glycemic control. METHODS: We performed a retrospective study of children with type 1 diabetes referred to an AMC after being educated at non-AMCs. These children were matched to a group of children diagnosed and educated as inpatients at an AMC. The A1C levels at 2, 3, and 5 years from diagnosis were compared between the 2 groups of children. RESULTS: Records were identified from 138 children. Glycemic control was comparable in the non-AMC-educated versus AMC-educated patients at 2, 3, and 5 years from diagnosis. The A1C was also highly consistent in each patient over time. CONCLUSIONS: Long-term glycemic control was independent of whether initial education was delivered at an AMC or non-AMC. Formal education and location at time of diagnosis do not appear to play a significant role in long-term glycemic control. Novel educational constructs, focusing on developmental stages of childhood and reeducation over time, are likely more important than education at time of diagnosis.