Reducing Unnecessary Diagnostic Testing in Pediatric Syncope: A Quality Improvement Initiative.

Our aim was to reduce the frequency of unnecessary testing used by pediatric cardiologists in the evaluation of pediatric patients with syncope or pre-syncopal symptoms without negatively affecting patient outcomes. Guidelines for cardiac testing in pediatric patients with syncope were developed and disseminated to members of our pediatric cardiology division. Educational brochures and water bottles labeled with tips on preventing syncope were made available to families and providers in our clinics. Compliance to the guidelines was tracked and shared with providers. Segmented regression analysis was used to model cardiac testing utilization and guideline compliance by provider over time before and after the implementation of the guidelines. A pre-intervention cohort of 237 patients (June 2014-May 2015) was compared to 880 post-intervention patients (August 2015-June 2019). There was a significant decrease in the utilization of unnecessary tests [odds ratio (OR) 0.3; 95% confidence interval (CI) 0.14, 0.65; p = 0.002] after the intervention. Charges associated with patient evaluation were significantly lower in the post-intervention cohort (interquartile range $0, $1378 vs $0, $213; p = 0.005). Post-intervention visits to emergency departments within our system were significantly decreased, with no change in the incidence of cardiac arrest, hospitalization for syncope, or referral to pediatric electrophysiologists. We demonstrated a significant reduction in the use of unnecessary testing and associated charges by developing guidelines related to the evaluation of pediatric patients with syncope or pre-syncopal symptoms. There was no demonstrable negative impact on patient outcomes.

A prospective observational multicenter study of balloon angioplasty for the treatment of native and recurrent coarctation of the aorta.

OBJECTIVES: Balloon angioplasty (BA) is an important treatment option for coarctation of the aorta. The congenital cardiovascular interventional study consortium (CCISC) represents a multi-institutional and multi-national effort to prospectively investigate congenital cardiac interventions. A prospective observational analysis of the efficacy and safety of balloon aortic angioplasty was conducted. METHODS: Data were collected prospectively from 36 CCISC sites from 2004 to 2012. One hundred and thirty patients underwent BA for native (n = 76) and recurrent (n = 54) coarctation. Acute, short-term, and intermediate outcomes are described for BA performed in the setting of native and recurrent coarctation of the aorta. Outcome measures included residual upper to lower extremity blood pressure gradient (ULG), use of antihypertensive medications, aortic wall injury, reobstruction, and need for reintervention. RESULTS: There was no procedural mortality. Acutely in native and recurrent coarctation, BA achieved an ULG less than 15 mm Hg in 73-80% and to less than 10 mm Hg in 54-68% of patients, respectively. At intermediate follow-up, ULG further improved, particularly for those who underwent initial reintervention for recurrent coarctation. No significant differences in aortic wall complications were seen and intervention free survival was similar for both groups. Following angioplasty, there was no significant difference in aortic wall complications; however follow up integrated imaging decreased over time. CONCLUSIONS: BA is a safe and effective treatment for coarctation of the aorta acutely and at intermediate term. Although aortic injury occurred in patients with both native and recurrent coarctation, at intermediate follow-up, aneurysm was noted more often in those with initial intervention for native coarctation.

Clinical practice, resource utilization, and outcomes of device closure of patent foramen ovale in pediatrics.

BACKGROUND: There are few data on patent foramen ovale closure and its outcome in children. In this study, we evaluated the current clinical practice, resource utilization, and outcome of device closure of patent foramen ovale in children. We hypothesized that patent foramen ovale closure would not result in a demonstrated benefit in children. METHODS: We undertook a prospective survey of all consecutive patients (<20 years) who underwent patent foramen ovale closure in our metropolitan area between 1995 and 2010. Differences in proportions were tested using the chi-square test or Fisher's exact test where appropriate. Differences in group medians were tested using Wilcoxon signed-rank test. RESULTS: A total of 153 patients (104 girls), median age 16 years (range 7-19) were studied. Indications for patent foramen ovale closure included: (1) migraine headache (104; 68%), (2) nonmigraine headache (24; 16%), (3) visual symptoms (110; 72%), (4) transient ischemic attack symptoms (42; 28%), and (5) stroke-like symptom (24; 16%). Patent foramen ovale was closed with an Amplatzer septal occluder in 115 (75%) and a Helex septal occluder in 47 (30%). The mean length of hospital stay was 18 ± 11 hours; the mean hospital charge was $24,126 ± $5808. The median duration of follow-up was 12 months, and 80 patients responded to the study survey. On follow-up, symptoms improved in 143 (93%), of which 29 (19%) had a residual shunt. None of the patient or treatment parameters predicted lack of improvement on follow-up. CONCLUSIONS: Despite the lack of proven benefit, children undergo closure of the patent foramen ovale for a variety of reasons, with the vast majority (92%) of patients reporting significant improvement in their symptoms. However, patent foramen ovale closure is an expensive procedure with serious potential complications. Symptomatic improvement even in the presence of a residual shunt suggests a strong placebo effect.

Effect of endovascular stenting of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome on stage II outcomes.

There is growing awareness that the Norwood procedure with the Sano modification is prone to early right ventricular to pulmonary artery (RV-PA) conduit stenosis resulting in systemic oxygen desaturation, increased interstage morbidity, and death. We report our experience with endovascular stent placement for conduit stenosis and compare the outcomes at stage II surgery between stented and nonstented infants. The medical records of all patients with hypoplastic left heart syndrome who received an RV-PA conduit at Norwood palliation from May 2005 to January 2010 were reviewed. The preoperative anatomy, demographics, operative variables, and outcomes pertaining to the Norwood and subsequent stage II surgeries were obtained and compared between stented and nonstented infants. The pre- and post-stent oxygen saturation, stenosis location, type and number of stents implanted, concomitant interventions, procedure-related complications, and reinterventions were collected. Of the 66 infants who underwent the Norwood procedure with RV-PA conduit modification, 16 (24%) received stents. The anatomy, demographics, and outcome variables after the Norwood procedure were similar between the stented and nonstented infants. The age at catheterization was 93 ± 48 days, and the weight was 4.9 ± 1.2 kg. The oxygen saturation increased from 66 ± 9% before intervention to 82 ± 6% immediately after stenting (p <0.0001). No interstage surgical shunt revisions were performed in either group. Age, weight, pre-stage II echocardiographic variables, oxygen saturation, and operative and outcome variables, including mortality, were similar between the 2 groups. In conclusion, endovascular stent placement for RV-PA conduit stenosis after the Norwood procedure leads to improved systemic oxygen levels and prevents early performance of stage II surgery without compromising stage II outcomes.

Practice variability and outcomes of coil embolization of aortopulmonary collaterals before Fontan completion: a report from the Pediatric Heart Network Fontan Cross-Sectional Study.

BACKGROUND: The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation. METHODS: We compared hospital length of stay after Fontan in 80 subjects who underwent APC coiling with 459 subjects who did not. Secondary outcomes included post-Fontan complications and assessment of health status and ventricular performance at cross-sectional evaluation (mean 8.6 ± 3.4 years after Fontan). RESULTS: Centers varied markedly in frequency of pre-Fontan APC coiling (range 0%-30% of subjects, P < .001). The coil group was older at Fontan (P = .004) and more likely to have single right ventricular morphology (P = .054) and pre-Fontan atrioventricular valve regurgitation (P = .03). The coil group underwent Fontan surgery more recently (P < .001), was more likely to have a prior superior cavopulmonary anastomosis (P < .001), and more likely to undergo extracardiac Fontan connection (P < .001) and surgical fenestration (P < .001). In multivariable analyses, APC coiling was not associated with length of stay (hazard ratio for remaining in-hospital 0.91, 95% CI 0.70-1.18, P = .48) or postoperative complications, except more post-Fontan catheter interventions (hazard ratio 1.74, 95% CI 1.04-2.91, P = .03), primarily additional APC coils. The groups had similar outcomes at cross-sectional evaluation. CONCLUSION: Management of APCs before Fontan shows marked practice variation. We did not find an association between pre-Fontan coiling of APCs and shorter postoperative hospital stay or with better late outcomes. Prospective studies of this practice are needed.

Coarctation repair in neonates and young infants: is small size or low weight still a risk factor?

OBJECTIVE: Previous reports of neonatal coarctation repair demonstrate a high rate of recurrent arch obstruction in small neonates. This study assesses the effect of patient size on reintervention and survival in neonates and infants undergoing repair of simple aortic coarctation. METHODS: From 1996 to 2006, 167 neonates and infants younger than 90 days with simple coarctation underwent repair. Median patient age was 16 days (range, 1-85 days). Median patient weight was 3.4 kg (range, 0.8-6.0 kg), with 29 patients weighing less than 2.5 kg. All 167 patients included in the study underwent repair through a left thoracotomy. RESULTS: There was 1 early death (1/167, 0.6%). Median follow-up of 4.8 years (range, 0-11.8 years) demonstrated 2 late deaths unrelated to recurrent coarctation. Eighteen patients underwent intervention for recurrent arch obstruction a median of 0.48 years postoperatively (range, 0.14-9.8 years). All were treated with balloon angioplasty and have required no additional intervention. Actuarial freedom from reintervention was 90% at 1 year and 89% at 5 years for infants weighing more than 2.5 kg and 89% at 1 year and 86% at 5 years (P = .31) for infants weighing less than 2.5 kg. There was no difference between survival or reintervention for neonates 30 days of age or younger compared with infants 31 to 90 days of age. Use of polypropylene sutures and female sex did correlate with increased reintervention. CONCLUSIONS: Low weight does not affect survival or reintervention rates after coarctation repair in neonates and infants less than 3 months of age. Balloon angioplasty is an effective treatment for recurrent obstruction after coarctation repair in infancy. In the current era, timing of the operation should be based on clinical status.

Amplatzer occlusion of accessory ventriculopulmonary connections.

BACKGROUND: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. OBJECTIVE: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. METHODS: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. RESULTS: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. CONCLUSIONS: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease.

In-hospital mortality for children with hypoplastic left heart syndrome after stage I surgical palliation: teaching versus nonteaching hospitals.

OBJECTIVES: Teaching hospitals are perceived to provide a higher quality of care for the treatment of rare disease and complex patients. A substantial proportion of stage I palliation for hypoplastic left heart syndrome (HLHS) may be performed in nonteaching hospitals. This study compares the in-hospital mortality of stage I palliation between teaching and nonteaching hospitals. METHODS: The authors conducted a retrospective cohort study using the Kids' Inpatient Database 1997 and 2000. Patients with HLHS undergoing stage I palliation were identified using International Classification of Diseases, Ninth Revision, Clinical Modification diagnostic and procedural codes. RESULTS: Seven hundred fifty-four and 880 discharges of children with HLHS undergoing stage I palliation in 1997 and 2000, respectively, were identified. The in-hospital mortality for the study population was 28% in 1997 and 24% in 2000. Twenty percent of stage I palliation operations were performed in nonteaching hospitals in 1997. Two percent of operations were performed in nonteaching hospitals in 2000. In 1997 only, in-hospital mortality remained higher in nonteaching hospitals after controlling for stage I palliation hospital volume and condition-severity diagnoses. Low-volume hospitals performing stage I palliation were associated with increased in-hospital mortality in 1997 and 2000. CONCLUSIONS: Patients with HLHS undergoing stage I palliation in nonteaching hospitals experienced increased in-hospital mortality in 1997. A significant reduction in the number of stage I palliation procedures performed in nonteaching hospitals occurred between 1997 and 2000. This centralization of stage I palliation into teaching hospitals, along with advances in postoperative medical and surgical care for these children, was associated with a decrease in mortality. Patients in low-volume hospitals performing stage I palliation continued to experience increased mortality in 2000.

Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood.

BACKGROUND: The purpose of this study was to compare the long-term outcomes of children randomized to surgery or balloon angioplasty (BA) for native coarctation (CoA). A prior randomized, short-term comparison of BA and surgery for native CoA in 36 children demonstrated equivalent relief of obstruction. The risk of aneurysm formation and possibly restenosis was higher among patients treated with BA. METHODS AND RESULTS: Blood pressure, residual aortic obstruction, and exercise performance were evaluated. Need for repeat intervention was reviewed. Aortic arch anatomy was assessed with magnetic resonance angiography. For subjects who were not available to return for evaluation, the most recent clinical record was utilized. Among the 36 subjects initially randomized, 21 returned for evaluation (11 BA, 10 surgery). The average time since initial intervention to evaluation for all subjects was 10.6+/-4.7 years for BA subjects and 11.3+/-3.7 years for surgical subjects. Resting blood pressure, CoA gradient, exercise performance, MRI analysis of the aortic arch, and need for repeat interventions were not different for the 2 treatment strategies. There was a higher incidence of aneurysm formation (35% versus 0%) and a greater difference in blood pressure between the right and left legs with exercise among BA subjects. Some aneurysms developed late, first being detected more than 5 years after the initial intervention. Only 50% of BA subjects remained free of both aneurysm formation and repeat intervention compared with 87.5% of surgical subjects (P=0.03). CONCLUSIONS: BA for the treatment of childhood CoA is associated with a higher incidence of aneurysm formation and iliofemoral artery injury than surgery. These differences should be considered when undertaking treatment for native CoA during childhood.

Reactivity of the ductus arteriosus: Implications for transcatheter therapy.

We report our experience in a 13-month-old boy undergoing transcatheter coil occlusion of a patent ductus arteriosus. Constriction of the ductus arteriosus with subsequent relaxation resulted in inadvertent coil embolization. This case report and review of the literature have implications for transcatheter treatment of persistent ductus arteriosus.

Transcatheter treatment of a large traumatic ventricular septal defect.

We report the first successful transcatheter closure of a large traumatic ventricular septal defect in a 16-year-old adolescent. The risks of standard open heart repair using cardiopulmonary bypass were felt to be prohibitive due to coexistent injuries. This case demonstrates the feasibility of transcatheter therapy for traumatic ventricular septal defects.

Safety of endomyocardial biopsy in children.

Endomyocardial biopsy in children remains important in the evaluation of cardiomyopathy, myocarditis, and rejection following cardiac transplantation. We sought to evaluate the morbidity associated with endomyocardial biopsy on the basis of a large experience from a single institution. We reviewed records of all endomyocardial biopsies performed at our institution. Complications were defined as adverse events resulting from the biopsy requiring intervention or additional observation. We also reviewed the most recent echocardiogram from all the patients for the presence and severity of tricuspid valvar regurgitation. Between November 1986 and April 2002, we performed 1051 endomyocardial biopsies in 135 patients ranging in age from 9 days to 18 years. The internal jugular vein was the site of vascular access in 68% of the procedures. There were 10 acute procedural complications requiring intervention or additional observation. Severe tricuspid regurgitation developed in two patients who had undergone multiple biopsies after cardiac transplantation, one of whom underwent subsequent replacement of the tricuspid valve. There were no deaths or cardiac perforations. The total incidence of morbidity was 1.1%. No demographic or procedural factors were identified to be predictive of complications. In experienced hands, therefore, endomyocardial biopsy can be safely performed in children with very low morbidity.