Thomas J. Starc

Cardiac dysfunction and mortality in HIV-infected children: The Prospective P2C2 HIV Multicenter Study. Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV Infection (P2C2 HIV) Study Group.

BackgroundLeft ventricular (LV) dysfunction is common in children infected with the human immunodeficiency virus (HIV), but its clinical importance is unclear. Our objective was to determine whether abnormalities of LV structure and function independently predict all-cause mortality in HIV-infected children. Methods and ResultsBaseline echocardiograms were obtained on 193 children with vertically transmitted HIV infection (median age, 2.1 years). Children were followed up for a median of 5 years. Cox regression was used to identify measures of LV structure and function predictive of mortality after adjustment for other important demographic and baseline clinical risk factors. The time course of cardiac variables before mortality was also examined. The 5-year cumulative survival was 64%. Mortality was higher in children who, at baseline, had depressed LV fractional shortening (FS) or contractility; increased LV dimension, thickness, mass, or wall stress; or increased heart rate or blood pressure (P ≤0.02 for each). Decreased LV FS (P <0.001) and increased wall thickness (P =0.004) were also predictive of increased mortality after adjustment for CD4 count (P <0.001), clinical center (P <0.001), and encephalopathy (P <0.001). FS showed abnormalities for up to 3 years before death, whereas wall thickness identified a population at risk only 18 to 24 months before death. ConclusionsDepressed LV FS and increased wall thickness are risk factors for mortality in HIV-infected children independent of depressed CD4 cell count and neurological disease. FS may be useful as a long-term predictor and wall thickness as a short-term predictor of mortality.

Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival

Left-ventricular noncompaction (LVNC) is an echocardiographic finding of increasing frequency in pediatrics; however, predictors of outcomes have been difficult to identify. We conducted a retrospective review of pediatric patients at the Morgan Stanley Children’s Hospital of New York from January of 1993 to September of 2009 to identify predictors of the primary outcome of death or heart transplantation. LVNC was identified in 50 patients, 34 of themxa0<xa01xa0year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17xa0years after presentation. Patients surviving 1xa0year after presentation had 75% conditional survival, and patients surviving 2xa0years after presentation had 92% conditional survival. Hemodynamic instability, poor ventricular function, and LV dilatation were each independent predictors of poor outcome. Of the 21 patients who presented with hemodynamic instability, 17 died or underwent transplantation at a median of 0.08xa0years after presentation. In conclusion, LVNC is recognized more in younger patients; however, age is not a predictor of outcome. Patients who present with hemodynamic instability and poor ventricular function have decreased transplant-free survival, and most poor outcomes occur within the first year after presentation. Therefore, early listing for transplant may lead to better outcomes in this population.

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

BACKGROUNDnChildren 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.nnnMETHODSnA 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.nnnRESULTSnThere 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).nnnCONCLUSIONSnLadd procedure is associated with increased odds of adverse events for children with heterotaxy and IRA.

Percutaneous Coronary Intervention With Bioresorbable Scaffolds in a Young Child

Importance Although much less frequent than in adults, coronary artery disease requiring revascularization may develop in children because of homozygous familial hypercholesterolemia or other underlying conditions. Percutaneous coronary intervention (PCI) with a bioresorbable scaffold (BRS) may have advantages over metallic coronary stents in this population. Objective To present a case of the successful treatment of unstable, multivessel coronary artery disease in a child with PCI with BRS implantation. This case highlights the limitations of conventional metal stents and the potential benefits of using BRSs in children. Design, Setting, and Participants This is a case report from an academic tertiary care institution of a 3-year-old boy with homozygous familial hypercholesterolemia and unstable coronary artery disease requiring revascularization. We also briefly review the related literature. Interventions/Exposures Intravascular imaging-guided PCI of the proximal right coronary artery and the left main and proximal left circumflex arteries was performed with BRSs. Main Outcomes and Measures The primary outcomes were acute procedural success and survival to liver transplant (3 months after PCI). Results Following BRS implantation, the patient recovered and remained free of cardiovascular symptoms 3 months after PCI. He subsequently underwent an orthotopic liver transplant for definitive treatment of homozygous familial hypercholesterolemia but died of noncardiac complications. A postmortem examination, including a histological assessment, revealed both BRSs to be patent with nonobstructive neointimal hyperplasia. Conclusions and Relevance To our knowledge, this is the first report of PCI with BRSs in a child. This represents an application of a BRS with potentially important implications for the future treatment of coronary artery disease in children and warrants further study.

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 (56xa0% response rate) in 1995 and 153/267 (57xa0%) in 2012. The median surgical preference score for infants with HLHS increased from 35 [25–45] in 1995 to 45 [35–50] in 2012, Pxa0<xa00.001. 53xa0%, 95xa0% CI [42, 64] of respondents recommended surgical intervention for a ward of the court in 1995 compared to 81xa0% [73, 89] in 2012, Pxa0<xa00.001. In 2012, 64xa0% [53, 75] of respondents were more likely to recommend surgery than 10xa0years prior. The percentage of respondents who saw good outcomes following three-stage repair increased from 49xa0% [38, 60] in 1995 to 84xa0% [78, 90] in 2012, Pxa0<xa00.001. The majority believed that parents should have the option of comfort care, 91xa0% [85, 97] in 1995 and 85xa0% [79, 91] in 2012, Pxa0=xa00.06. 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.

Prevalence of congenital cardiovascular malformations in children of human immunodeficiency virus-infected women: the prospective P2C2 HIV Multicenter Study. P2C2 HIV Study Group, National Heart, Lung, and Blood Institute, Bethesda, Maryland.

OBJECTIVESnThe purpose of the study was to assess the effects of maternal HIV-1 (human immunodeficiency virus) infection and vertically transmitted HIV-1 infection on the prevalence of congenital cardiovascular malformations in children.nnnBACKGROUNDnIn the United States, an estimated 7000 children are born to HIV-infected women annually. Previous limited reports have suggested an increase in the prevalence of congenital cardiovascular malformations in vertically transmitted HIV-infected children.nnnMETHODSnIn a prospective longitudinal multicenter study, diagnostic echocardiograms were performed at 4-6-month intervals on two cohorts of children exposed to maternal HIV-1 infection: 1) a Neonatal Cohort of 90 HIV-infected, 449 HIV-uninfected and 19 HIV-indeterminate children; and 2) an Older HIV-Infected Cohort of 201 children with vertically transmitted HIV-1 infection recruited after 28 days of age.nnnRESULTSnIn the Neonatal Cohort, 36 lesions were seen in 36 patients, yielding an overall congenital cardiovascular malformation prevalence of 6.5% (36/558), with a 8.9% (8/90) prevalence in HIV-infected children and a 5.6% (25/449) prevalence in HIV-uninfected children. Two children (2/558, 0.4%) had cyanotic lesions. In the Older HIV-Infected Cohort, there was a congenital cardiovascular malformation prevalence of 7.5% (15/201). The distribution of lesions did not differ significantly between the groups.nnnCONCLUSIONSnThere was no statistically significant difference in congenital cardiovascular malformation prevalence in HIV-infected versus HIV-uninfected children born to HIV-infected women. With the use of early screening echocardiography, rates of congenital cardiovascular malformations in both the HIV-infected and HIV-uninfected children were five- to ten-fold higher than rates reported in population-based epidemiologic studies but not higher than in normal populations similarly screened. Potentially important subclinical congenital cardiovascular malformations were detected.

Usefulness of High-Dose Oral Flecainide for Termination of Recent-Onset Atrial Fibrillation in Children

A high dose of oral flecainide has been used for acute termination of atrial fibrillation (AF) and atrial flutter or intra-atrial re-entry tachycardia (AFL-IART) in adults. The use of flecainide for these conditions in children has not been well described. We describe our institutional experience on acute termination of AF or AFL-IART in children with a single high dose of oral flecainide in a hospital setting. All patients who received a single high dose of oral flecainide from 2009 to 2016 who were <21 years of age were included. Patients were treated only if AF or AFL-IART was less than 24 hours of duration. The dose was 300u2009mg for patients >70u2009kg, 200u2009mg for patients 40 to 70u2009kg, and 5u2009mg/kg for patients <40u2009kg. Charts were reviewed to determine demographic information, flecainide dose, termination of arrhythmia, and time to termination. There were 22 patients identified. The median age was 16 years (range 4.6 to 20.3) with a median weight of 75u2009kg (range 19 to 112). There were 13 patients with AF (11 with a normal heart, 85%) and 9 patients with AFL-IART (1 with a normal heart, 11%) (pu2009<0.05). The median dose of flecainide given was 3.6u2009mg/kg (range 2.7 to 6.1) or 136u2009mg/m2 (range 90 to 171). AF in all patients (13/13, 100%) and AFL-IART in 5 of 9 patients (55%) terminated acutely (pu2009<0.05). All patients with normal heart (12/12, 100%) and 6 of the 10 patients (60%) with heart disease have their arrhythmia terminated acutely (pu2009<0.05). The only patients whose tachycardia did not terminate were 4 patients with IART and heart disease. The arrhythmia terminated in a median time of 60u2009minutes (range 30 to 120). There were no adverse events or proarrhythmia encountered. In conclusion, a single high dose of oral flecainide successfully terminated AF of less than 24 hours duration in all pediatric patients without side effects. This approach is less effective for AFL-IART in patients with heart disease.

Undiagnosed primary tracheal stenosis in tetralogy of Fallot: complete rings with a low carina.

Sir, The purpose of this letter is to highlight the occurrence of long-segment tracheal stenosis discovered only during problems with intubation in a child with tetralogy of Fallot (TOF). A 6-month-old boy with TOF presented for primary surgical repair. Preoperative imaging workup included frontal and lateral chest radiographs (Fig. 1) as well as echocardiography, which demonstrated a right-side aortic arch with mirror-image branching. Preoperative clinical evaluation did not reveal any obvious history of stridor. The infant did not have pulmonary artery sling. On arrival in the operating suite, the boy could not be intubated by the pediatric anesthesiologist. The emergent assistance of a pediatric otolaryngologist was required for eventual successful intubation with a smaller endotracheal tube, with detection of long-segment completering tracheal stenosis. The procedure was converted to slide tracheoplasty and TOF repair with good clinical outcome. Review of surgical literature suggests that our case is not an isolated occurrence. Scattered reports of increased morbidity caused by airway pathology document difficulty with intubation, prolonged ventilator time and emergency tracheal repair during cardiac surgery. There is limited quantitative evidence of the association of TOF with tracheal pathology. Kazim et al. [1] first reported the presence of concurrent tracheal anomaly in 11% (5/44) of patients with TOF. A large series of 12,000 cases of congenital heart disease revealed 43 cases of primary tracheal stenosis. Of the 8 patients ultimately repaired in a combined one-step operation, 4 were cases of TOF [2]. There is no consensus on preoperative imaging of the airway in TOF. Echocardiography alone cannot adequately assess the airway; however, radiation concerns limit indiscriminate use of radiography/CT as a screening tool in all patients. Furthermore, elucidation of a history of stridor is particularly difficult in this patient population, in which respiratory issues are often confused with infection or blamed on cyanotic heart disease. In our case, preoperative evidence of airway pathology proved elusive. In hindsight, close evaluation of the radiographs suggests long-segment tracheal narrowing. Only a very mild preoperative history of “noisy breathing” was ultimately uncovered. M. T. Starc Department of Radiology, St. Luke’s-Roosevelt Medical Center, Columbia University College of Physicians and Surgeons, New York, NY, USA