Angira Patel

Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome.

BACKGROUNDnHypoplastic left heart syndrome may coexist with noncardiac congenital defects or genetic syndromes. We explored the impact of such lesions on outcomes after staged univentricular palliation.nnnMETHODSnSociety of Thoracic Surgeons database 2002 to 2006: Children diagnosed with hypoplastic left heart syndrome who underwent stage 1 Norwood (n = 1,236), stage 2 superior cavopulmonary anastamosis (n = 702) or stage 3 Fontan (n = 553) procedures were studied. In-hospital mortality, postoperative complications, and length of stay were compared at each stage between those with and without noncardiac-genetic defects. Congenital Heart Surgeons Society database 1994 to 2001: All 703 infants enrolled in the Congenital Heart Surgeons Society critical left ventricular outflow tract obstruction study who underwent primary stage 1 palliation were reviewed. The impact of noncardiac defects-syndromes on survival was explored using multivariable parametric models with bootstrap bagging.nnnRESULTSnSociety of Thoracic Surgeons database: Stage 1 in-hospital mortality (26% vs 20%, p = 0.04) and mean postoperative length of stay (42 versus 31 days, p < 0.0001) were greater, and postoperative complications significantly more prevalent in infants with noncardiac-genetic defects. Congenital Heart Surgeons Society database: Noncardiac-genetic defects were present in 55 (8%). Early hazard for death after Norwood was significantly worse in infants with noncardiac defects-syndromes (p = 0.008). Chromosomal defects (n = 14) were highly unfavorable: the early risk of death was doubled (10-year survival 25 +/- 9% vs 54 +/- 2%, p = 0.005). Turner syndrome accounted for the majority of chromosomal defects in this population (11 of 14, 79%). Mode of death was rarely attributable to the noncardiac-genetic defect.nnnCONCLUSIONSnSurvival in hypoplastic left heart syndrome is strongly influenced by the presence of noncardiac abnormalities. Strategies to improve mortality in infants with noncardiac abnormalities should be explored. Presence of chromosomal defects, especially Turner syndrome, should enter decision-management options for parents and physicians.

Intracardiac echocardiography to guide closure of atrial septal defects in children less than 15 kilograms.

Background: Intracardiac echocardiography (ICE) is increasingly replacing transesophageal echocardiography (TEE) as the primary imaging technique to guide device closure of atrial septal defects (ASD). Owing to the length of the procedure, the use of TEE requires general anesthesia. Investigators have reported the usefulness of ICE in adults and children. However, little is known about the use of ICE in children whose weight is <15 kg. Therefore, this study examines the use of ICE guided secundum ASD closure in children <15 kg. Methods: Nineteen patients with a median age of 3.1 years (range 1.8–4.8), and median weight of 13.2 kg (range 8.0–14.4) underwent transcatheter occlusion (Amplatzer occluder) of a secundum ASD using ICE guidance. ICE was performed using an Acunav® catheter. The ICE catheter (10 F shaft) was introduced into an 11 F sheath in a contralateral femoral vein. Diagnostic as well as periprocedure imaging was obtained. Results: Sixteen patients had single, and three had multiple defects. Median defect size as measured by ICE was 16 mm (range 2.5–25). The median balloon stretched diameter (obtained in eight patients) was 18 mm (range 10–21); the median size of the defect for these eight patients was 15 mm (range of 8–20). Both techniques for measuring the defect correlated well with r = 0.94. The ASD occluder size ranged from 7 to 26 mm with a median of 18 mm. The procedure was successful in 16 patients who had a device implanted and no residual shunt. ASD occlusion was not attempted in two patients due to deficient rims and in one patient, the attempt failed due to left atrial disk prolapse through the ASD. Four patients experienced transient complications during the catheter procedure, including supra ventricular tachycardia, sinus bradycardia, and two with complete heart block (resolving with device removal); all had subsequent successful device placement. No complications were attributed to the use of ICE and specifically, no vascular injury was noted. Conclusions: Comparable to results with larger patients, ICE provides adequate imaging (preprocedure diagnosis and periprocedure guidance) during device occlusion of secundum ASDs with no significant complications. Thus, ICE can successfully be used in the closure of ASD in smaller patients (<15 kg) and eliminate the need for endotracheal intubation.

Prevalence of Noncardiac and Genetic Abnormalities in Neonates Undergoing Cardiac Operations: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database

BACKGROUNDnAmong patients with congenital heart disease (CHD), the coexistence of noncardiac congenital anatomic abnormalities (NC), genetic abnormalities (GA), and syndromes (S) may influence therapeutic strategies and outcomes. The appreciated prevalence of these abnormalities has risen because increased screening and improved diagnostic precision enable identification of these comorbidities in a larger fraction of neonates with CHD. We examined the contemporary prevalence and distribution of NC/GA/S across diagnostic groups among neonates undergoing cardiac operations using a large nationally representative clinical registry.nnnMETHODSnThe Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) was queried to identify neonates (≤30 days) who underwent index cardiac operations from 2010 to 2013. The fundamental cardiac diagnosis was used to identify 10 diagnostic groups. The prevalence of NC/GA/S was reported across each group.nnnRESULTSnThe cohort included 15,376 index neonatal operations from 112 centers. Overall, 18.8% (2,894 of 15,376) of operations were performed in neonates with NC/GA/S. Patients with atrioventricular septal defect (212 of 357 [59.4%]), interrupted aortic arch (248 of 567 [43.7%]), truncus arteriosus (204 of 554 [36.8%]), and tetralogy of Fallot (417 of 1,383 [30.2%]) had the highest prevalence of NC/GA/S abnormalities, whereas those with transposition of the great arteries (111 of 2,778 [4.0%]) had the lowest prevalence. The most commonly identified NC/GA/S included heterotaxy (597 of 15,376 [3.9%]), DiGeorge syndrome or 22q11 deletion (550 of 15,376 [3.6%]), Down syndrome or trisomy 21 (318 of 15,xa0376 [2.1%]), intestinal malrotation (220 of 15,376 [1.4%]), and Turner syndrome or 45XO (189 of 15,376 [1.2%]).nnnCONCLUSIONSnThe prevalence of NC/GA/S varies widely across CHD diagnostic groups. This information may be useful for patient counseling, recommendations for screening for anomalies and genetic disorders, and perioperative management.

Can we prevent sudden cardiac death in young athletes: the debate about preparticipation sports screening

All high school athletes in the United States require a preparticipation screening examination. The American Heart Association recommends a focused history and physical examination. The European Society of Cardiology recommends that all examinations include an electrocardiogram (ECG). We review the risks and costs of screening, discuss legal ramifications and analyse the ethical implications of these considerations. There are too many unknown about ECG screening to require it as routine testing for all high school athletes.

Physicians’ Lack of Adherence to National Heart, Lung, and Blood Institute Guidelines for Pediatric Lipid Screening

Objectives. To determine adherence to the 2011 National Heart, Lung, and Blood Institute lipid screening guidelines and identify patient factors promoting screening. Methods. Records of children who received well-child care at age 11 years and turned 12 in 2013 were reviewed. Subjects were stratified by guideline-defined dyslipidemia risk based on documented medical or family history risk factors. We defined adherence as the order of a lipid profile when age 11 years or completed lipid screening at 9 to 10 years. Results Of 298 subjects, 42% were assigned to the dyslipidemia high-risk subgroup. Records of 27.2% demonstrated adherence. Fifty-six percent of high-risk subjects versus 6% of their non-high-risk counterparts received lipid screening by age 12 (P < .001). Among screened subjects, history of obesity and parental history of dyslipidemia were significantly associated with lipid testing. Conclusions. Lipid screening rates were low. Strategies to increase lipid screening in the primary care setting are needed.

Cardiac Teratoma of the Interventricular Septum with Congenital Aortic Stenosis in a Newborn

A 10-day-old infant presented with tachypnea, harsh murmur, and cardiomegaly. Echocardiography revealed a multicystic mass invading the entire interventricular septum and protruding into the left ventricular cavity without left ventricular tract outflow tract obstruction and aortic stenosis. The mass was subsequently resected and biopsy revealed it to be a cardiac teratoma. We present an unusually rare case of an intramyocardial teratoma with associated congenital aortic stenosis.

Doubly Committed and Juxtaarterial Ventricular Septal Defect: Outcomes of the Aortic and Pulmonary Valves

BACKGROUNDnThe morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review wasxa0to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves.nnnMETHODSnBetween 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets.nnnRESULTSnOperative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI.nnnCONCLUSIONSnThe incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI.

Fulminant Clostridium difficile toxic megacolon in a pediatric heart transplant recipient

Patel A, Gossett JJ, Benton T, Rowell E, Russell H, Cham E, Pahl E. Fulminant Clostridium difficile toxic megacolon in a pediatric heart transplant recipient. u2028Pediatr Transplantation 2010: : E30–E34.

Parental refusal of surgery in an infant with tricuspid atresia

We present a case of a fetal diagnosis of tricuspid atresia (TA). The pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born. The neonatologist argued that outcomes for TA are similar to those for hypoplastic left heart syndrome, and the standard practice at the institution was to allow parents to choose surgery or end-of-life care for those infants. The team requested an ethics consultation to assist in determining whether forgoing life-prolonging interventions in this case would be ethically supportable. In this article, we ask a pediatric intensivist, a pediatric cardiologist, and a neonatologist to discuss the ethics of withholding life-sustaining treatment of a baby with TA.

ECHOCARDIOGRAPHY TO ASSESS NATURAL HISTORY AND REGRESSION OF CLASS III-V CORONARY ARTERY ANEURYSMS IN KAWASAKI DISEASE

Background: Limited data exist on regression or progression of coronary artery aneurysms (CAA) in higher risk patients with Kawasaki disease (KD) in the current era of early treatment with immunomodulatory and anticoagulation therapy. Coronary risk classification in KD is defined by: Class I with no coronary artery involvement, Class II with transient coronary ectasia that disappears within 6-8 weeks, Class III with 1 small-medium CAA, Class IV with >=1 large or giant CAA, and Class V with coronary artery occlusion. We assessed the hypothesis that Class III-V CAA may regress, but coronary artery dimensions often do not normalize.