Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery.

We hypothesized that enthusiasm for surgery increased for infants with hypoplastic left heart syndrome (HLHS) at Columbia University Medical Center (CUMC) between 1995 and 2012. We sought to identify factors that engendered this paradigm shift. Confidential surveys were distributed to providers at CUMC in 1995 and 2012 to measure enthusiasm for surgical intervention for HLHS. Surgical preference scores are presented as median [interquartile range]. Surveys were completed by 99/176 providers (56 % response rate) in 1995 and 153/267 (57 %) in 2012. The median surgical preference score for infants with HLHS increased from 35 [25-45] in 1995 to 45 [35-50] in 2012, P < 0.001. 53 %, 95 % CI [42, 64] of respondents recommended surgical intervention for a ward of the court in 1995 compared to 81 % [73, 89] in 2012, P < 0.001. In 2012, 64 % [53, 75] of respondents were more likely to recommend surgery than 10 years prior. The percentage of respondents who saw good outcomes following three-stage repair increased from 49 % [38, 60] in 1995 to 84 % [78, 90] in 2012, P < 0.001. The majority believed that parents should have the option of comfort care, 91 % [85, 97] in 1995 and 85 % [79, 91] in 2012, P = 0.06. In both eras, prematurity and additional surgical problems dissuaded providers from recommending surgical intervention. Despite the fact that most providers have seen good outcomes and now recommend surgery for infants with HLHS, the majority of providers still believe that the option of comfort care should be available to families.

Heterotaxy syndrome and intestinal rotational anomalies: Impact of the Ladd procedure.

BACKGROUND: Children with heterotaxy syndrome and intestinal rotational anomalies (IRA) are at risk for midgut volvulus and ischemia. Controversy exists regarding risks and benefits of prophylactic Ladd procedures. The purpose of this study is to assess the impact of Ladd procedures on adverse events for children with heterotaxy and IRA. METHODS: A retrospective cohort study using the Pediatric Health Information System (PHIS) database was performed. All children with heterotaxy and IRA admitted at age ≤ 30 days and discharged between 1/1/2004 and 1/1/2011 were included with a minimum 2-year follow-up period. The primary outcome was major morbidity, defined as a composite variable for intestinal obstruction/volvulus, ischemia, or resection, or inhospital mortality. Univariable and multivariable analyses were performed. RESULTS: There were 325 patients who met the inclusion criteria, including 92 (28%) patients with single ventricles. Mean gestational age was 38.0 ± 2.1 weeks and birth weight was 3.1 ± 0.6 kg. Ladd procedure was performed during initial hospitalization on 188 (58%) children. In multivariable analyses, Ladd procedure on initial hospitalization was associated with a 2.2 times increased odds of adverse events on subsequent admissions (95% CI 1.3-4.0, p = 0.007). CONCLUSIONS: Ladd procedure is associated with increased odds of adverse events for children with heterotaxy and IRA.

Aggregate risk of cardiovascular disease among adolescents perinatally infected with the human immunodeficiency virus.

BACKGROUND: Perinatally HIV-infected adolescents may be susceptible to aggregate atherosclerotic cardiovascular disease risk, as measured by the Pathobiological Determinants of Atherosclerosis in Youth (PDAY) coronary arteries and abdominal aorta risk scores, as a result of prolonged exposure to HIV and antiretroviral therapy. METHODS AND RESULTS: Coronary arteries and abdominal aorta PDAY scores were calculated for 165 perinatally HIV-infected adolescents, using a weighted combination of modifiable risk factors: dyslipidemia, cigarette smoking, hypertension, obesity, and hyperglycemia. Demographic and HIV-specific predictors of scores ≥1 were identified, and trends in scores over time were assessed. Forty-eight percent and 24% of the perinatally HIV-infected adolescents had coronary arteries and abdominal aorta scores ≥1, representing increased cardiovascular disease risk factor burden. Significant predictors of coronary arteries scores ≥1 included male sex, history of an AIDS-defining condition, longer duration of use of a ritonavir-boosted protease inhibitor, and no prior use of tenofovir. Significant predictors of abdominal aorta scores ≥1 included suppressed viral load, history of an AIDS-defining condition, and longer duration of boosted protease inhibitor use. No significant changes in coronary arteries and abdominal aorta risk scores were observed over the 4-year study period. CONCLUSIONS: A substantial proportion of perinatally HIV-infected youth have high PDAY scores, reflecting increased aggregate atherosclerotic cardiovascular disease risk factor burden. High scores were predicted by HIV disease severity and boosted protease inhibitor use. PDAY scores may be useful in identifying high-risk youth who may benefit from early lifestyle or clinical interventions.

Congenital cutaneous hemangioma causing cardiac failure: a case report and review of the literature.

We present a case of a large congenital hemangioma (CH) on the neck causing cardiac failure and thrombocytopenia in a female neonate. A trial of medical therapy with corticosteroids and propranolol was attempted, but the patient ultimately underwent definitive treatment with embolization and surgical resection with a positive outcome. A review of the English language literature revealed 16 previously reported cases of CHs complicated by congestive heart failure. This series supports known demographic features of CHs, including a lack of gender discrepancy and a predilection to affect the head and neck. These CHs are rarely diagnosed in utero; most patients present with a mass at birth. Cardiac failure is identified prenatally or in the first days of life. A mild to moderate thrombocytopenia and coagulopathy, which is likely transient and distinct from classic Kasabach-Merritt phenomenon, accompanies many of these cases. There is a 30% associated mortality rate. Both medical and interventional treatment modalities have been reported. Steroids are the most commonly used medication, but without any clear benefit. We hypothesize that, based on its possible mechanisms of action,propranolol may be a more effective treatment for CHs requiring treatment. As surgical intervention may be necessary, we recommend a multidisciplinary approach to treating patients with problematic CHs.

Predictors for hemodynamic improvement with temporary pacing after pediatric cardiac surgery.

OBJECTIVES: Temporary epicardial pacing wires are commonly placed during pediatric cardiac surgery. Data are sparse on postoperative pacing in this population. The objective of this study was to determine the frequency of use and identify predictors for the use of temporary epicardial pacing wires. METHODS: Perioperative data were prospectively collected on all patients who underwent cardiac surgery at our institution (n = 162). RESULTS: A total of 117 (72%) patients had temporary epicardial pacing wires placed. Postoperatively, 23 (20%) of 117 patients had hemodynamic improvement with the use of temporary epicardial pacing wires. Indications for pacing were slow junctional rhythm (11/23 [48%]), junctional ectopic tachycardia (7/23 [31%]), pace termination of supraventricular tachycardia (3/23 [13%]) and atrial flutter (1/23 [4%]), and complete heart block (1/23 [4%]). By using univariate analysis, single-ventricle anatomy, heterotaxy, the Fontan procedure, use of circulatory arrest, intraoperative arrhythmia, pacing in the operating room, and use of vasoactive medications were predictors for hemodynamic improvement with the use of temporary epicardial pacing wires (P < .05). On multivariate analysis, the Fontan procedure, circulatory arrest, and intraoperative arrhythmias were independent predictors (P < .01). When excluding all patients with any of these 3 risk factors, only 2% were paced. Patients with clinically significant pacing had longer chest tube drainage (P < .01) and intensive care unit length of stay (P < .01). There were no complications associated with temporary epicardial pacing wires. CONCLUSIONS: The Fontan procedure, use of circulatory arrest, and intraoperative arrhythmias were associated with hemodynamic improvement with postoperative pacing and might represent indications for empiric intraoperative placement of temporary epicardial pacing wires. Patients without these risk factors were less likely to require pacing. Temporary epicardial pacing wires were safe and useful in the management of arrhythmias after pediatric cardiac surgery.

Cardiac effects of antiretroviral therapy in HIV-negative infants born to HIV-positive mothers: NHLBI CHAART-1 (National Heart, Lung, and Blood Institute Cardiovascular Status of HAART Therapy in HIV-Exposed Infants and Children cohort study).

OBJECTIVES: The aim of this study was to investigate the possible effects of antiretroviral therapy (ART) in utero on cardiac development and function in human immunodeficiency virus (HIV)-negative children. BACKGROUND: ART reduces vertical HIV transmission. Long-term cardiotoxicity after in utero exposure to ART is unknown in children but has occurred in young animals. METHODS: Using a prospective multisite cohort study design, echocardiograms taken between birth and 24 months were compared in 2 groups of HIV-negative infants of HIV-positive mothers: 136 infants exposed to ART (ART+) and 216 unexposed infants (ART-). RESULTS: Mean left ventricular (LV) mass z-scores were consistently lower in ART+ girls than in ART- girls: differences in mean z-scores were -0.46 at birth (p = 0.005), -1.02 at 6 months (p < 0.001), -0.74 at 12 months (p < 0.001), and -0.79 at 24 months (p < 0.001). Corresponding differences in z-scores for boys were smaller: 0.13 at 1 month (p = 0.42), -0.44 at 6 months (p = 0.01), -0.15 at 12 months (p = 0.37), and -0.21 at 24 months (p = 0.21). Septal wall thickness and LV dimension were smaller than expected in ART+ infants, but LV contractility was consistently about 1 SD higher at all ages (p < 0.001). In ART+ infants, LV fractional shortening was higher than in ART- infants; girls showed a greater difference. CONCLUSIONS: Fetal exposure to ART is associated with reduced LV mass, LV dimension, and septal wall thickness z-scores and increased LV fractional shortening and contractility up to age 2 years. These effects are more pronounced in girls than in boys. Fetal ART exposure may impair myocardial growth while improving depressed LV function.

Variable morphology wide complex rhythm: what is the etiology?

We report a case of an infant who developed transient complete heart block following aortic and mitral valve surgery. In the course of his recovery, he developed a variable morphology wide complex rhythm with intermittent narrow complex beats. Review of his initial postoperative electrocardiogram (ECG), which demonstrated a right bundle branch block, helped to clarify the rhythm.

Spontaneous regression of left ventricular dilation in children with restrictive ventricular septal defects.

OBJECTIVES: To test the hypothesis that left ventricular (LV) dilation associated with pressure-restrictive ventricular septal defect (VSD) often remains stable or regresses spontaneously, calling into question the role of interventional management for such defects. STUDY DESIGN: We analyzed 96 serial echocardiograms from 33 unoperated patients with a moderate-to-large VSD with LV dilation (LV end-diastolic dimension [LVED] z score >2.0) at enrollment who were followed for more than 2 years. Records of 125 surgical patients also were reviewed. Patients were evaluated for evidence of persistent or progressive LV dilation; signs or symptoms of congestive heart failure (CHF), failure to thrive (FTT), or pulmonary hypertension (PAH); as well as acquired ventricular outflow obstruction or aortic regurgitation. LVED z scores at enrollment versus latest follow-up were compared using paired t tests. A random-effects model with random intercept and slope was fitted to account for repeated observations for each patient. RESULTS: Mean age at enrollment was 4.6 +/- 3.2 years, and mean follow-up was 7.8 +/- 4 years (range, 2.8 to 22 years), during which mean LVED z score decreased from 3.0 +/- 0.6 to 1.2 +/- 1.3 (P < .01). LVED z score decreased in 29 of the 33 patients, and decreased to <2 in 26 of these 29 (79%). CONCLUSIONS: Most patients with pressure-restrictive VSD with moderate-to-severe LV dilation without CHF, FTT, or PAH will experience spontaneous resolution of LV dilation and can avoid cardiac surgery or catheter-based intervention.