Aimee K. Armstrong

Usefulness of Heparin Therapy in Protein-Losing Enteropathy Associated With Single Ventricle Palliation

This retrospective study was designed to evaluate the effectiveness of subcutaneous heparin therapy for the treatment of protein-losing enteropathy (PLE) associated with single-ventricle palliation and to evaluate the side effects of long-term heparin use. PLE affects 4% to 13% of Fontan operative survivors. Five-year survival after onset of PLE is only 46% to 59%. We studied a cohort of patients with single-ventricle palliation who developed PLE and were treated with subcutaneous heparin. Seventeen patients were included in the study. Symptoms of PLE appeared on average 43 months after surgical palliation. At diagnosis of PLE, mean albumin level was 2.0 +/- 0.4 g/dl. At cardiac catheterization, mean systemic venous pressure was 11.6 mm Hg. Subjective symptomatic improvement on heparin therapy occurred in 13 patients (76%). Three patients (18%) went into clinical remission. Compared with the period before initiation of heparin, there was no significant difference in the number of hospital admissions (p = 0.99) or albumin infusions (p = 0.88) during the first year of heparin therapy. Five patients had x-rays of their thoracolumbar spine, and 9 patients had bone mineral analyses; all scans were grossly abnormal. In conclusion, subcutaneous heparin therapy leads to subjective improvement of PLE symptoms in most patients; however, it does not change the need for frequent albumin infusions and does not increase the rate of remission above that for standard medical therapy.

Transcatheter Pulmonary Valve Replacement Reduces Tricuspid Regurgitation in Patients With Right Ventricular Volume/Pressure Overload

BACKGROUND Tricuspid regurgitation (TR) is a common and important comorbidity in patients with postoperative right ventricular outflow tract (RVOT) obstruction or pulmonary regurgitation (PR). Transcatheter pulmonary valve replacement (TPVR) has become a useful tool in the management of postoperative RVOT obstruction and PR, but it is unknown whether relief of the right ventricular volume and/or pressure overload by TPVR will have a beneficial effect on TR, as is often seen with surgical pulmonary valve replacement. OBJECTIVES This study sought to assess the prevalence of and factors associated with significant TR in patients undergoing TPVR for RVOT obstruction or PR. METHODS Data were combined from 3 prospective multicenter trials of patients referred for TPVR. Follow-up data through 5 years post-implantation were analyzed. RESULTS Of 300 patients studied, 77 (25.6%) had moderate or severe TR at baseline. After TPVR, TR severity was improved in 65% of those patients, and more than one-half had mild TR or less TR at discharge. Of 13 patients with severe TR pre-implantation, only 1 had severe TR at 1-year follow-up and beyond. Moderate or severe baseline TR was associated with shorter freedom from RVOT reintervention after TPVR. CONCLUSIONS In this prospective multicenter study of post-operative patients with RVOT obstruction and/or PR, TR was common. In patients with significant baseline TR, TPVR resulted in clinically relevant acute reductions in TR that persisted over at least 5 years of follow-up. These observations support the application of TPVR therapy in patients with RVOT obstruction or PR who are anatomically suitable, even in the setting of significant concomitant TR.

Hybrid approach for pulmonary atresia with intact ventricular septum: Early single center results and comparison to the standard surgical approach

To examine acute and mid‐term patient outcomes following the hybrid approach to pulmonary atresia with intact ventricular septum (PA‐IVS) compared with the standard surgical approach.

Radiation Safety in Children with Congenital and Acquired Heart Disease: A Scientific Position Statement on Multimodality Dose Optimization from the Image Gently Alliance

There is a need for consensus recommendations for ionizing radiation dose optimization during multimodality medical imaging in children with congenital and acquired heart disease (CAHD). These children often have complex diseases and may be exposed to a relatively high cumulative burden of ionizing radiation from medical imaging procedures, including cardiac computed tomography, nuclear cardiology studies, and fluoroscopically guided diagnostic and interventional catheterization and electrophysiology procedures. Although these imaging procedures are all essential to the care of children with CAHD and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of these recommendations is to encourage informed imaging to achieve appropriate study quality at the lowest achievable dose. Other strategies to improve care include a patient-centered approach to imaging, emphasizing education and informed decision making and programmatic approaches to ensure appropriate dose monitoring. Looking ahead, there is a need for standardization of dose metrics across imaging modalities, so as to encourage comparative effectiveness studies across the spectrum of CAHD in children.

Multicenter Experience Evaluating Transcatheter Pulmonary Valve Replacement in Bovine Jugular Vein (Contegra) Right Ventricle to Pulmonary Artery Conduits

Background— Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit. Methods and Results— This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mm Hg; P<0.001). At most recent follow-up, the maximum pulmonary valve gradient by echocardiogram remained significantly reduced relative to prevalve implant measurements (65.9 versus 27.3 mm Hg; P<0.001). The incidence of Melody transcatheter pulmonary valve stent fracture (3.4%) and infectious endocarditis (4.3%) were both low. Serious adverse events occurred in 3 patients. Conclusions— Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis.

Superior vena cava thrombectomy with the X‐SIZER® catheter system in a child with Fontan palliation

A 4‐year‐old female with hypoplastic left heart syndrome and Fontan palliation presented with severe neurologic impairment from thrombosis of the superior vena cava (SVC). She underwent successful SVC thrombectomy with the X‐SIZER® Thrombectomy Catheter System, followed by balloon angioplasty. She demonstrated rapid improvement in her neurologic deficits after the procedure. This represents the first published use of the X‐SIZER in a child and its first published use for SVC thrombectomy.

Effect of age on lipid profiles in pediatric heart transplant recipients

Abstract:  This studys objectives were to determine if pediatric orthotopic heart transplant (OHT) recipients over all ages develop hyperlipidemia and, secondarily, to identify the effects of immunosuppressive agents and statins on lipid profiles in these patients. Retrospective chart review was performed for pediatric patients transplanted between January 1987 and June 2002. Of the 100 OHTs performed, 50 patients satisfied the inclusion criteria and were grouped by age at OHT as follows: group 1 (n = 16): 0–4 yr; group 2 (n = 10): 5–9 yr; group 3 (n = 15): 10–14 yr; group 4 (n = 9): 15–18 yr. There were 2789 lipid levels recorded, and each patient had an average of 14 post‐OHT lipoprotein panels measured. Post‐OHT total cholesterol and low‐density lipoprotein (LDL) levels were significantly greater than those of the general population for the entire follow‐up period in all age groups, except for LDL levels in group 2. Cyclosporin level and prednisone dose were positively associated with total cholesterol and LDL levels (p < 0.03). Statins significantly decreased total cholesterol and LDL levels (p < 0.001). Hyperlipidemia affects OHT patients of all ages. Even the youngest patients may benefit from immunosuppression using an alternative to cyclosporin, such as tacrolimus, and steroid‐free regimens, which may improve lipid profiles. Once safety and efficacy data are available, all age groups may benefit from statins.

Use of a Pressure Guidewire to Assess Pulmonary Artery Band Adequacy in the Hybrid Stage I Procedure for High‐risk Neonates with Hypoplastic Left Heart Syndrome and Variants

OBJECTIVE The hybrid stage I procedure is an alternative palliative strategy for patients with hypoplastic left heart syndrome who traditionally have undergone the Norwood operation. At our institution, the hybrid stage I procedure is employed only for patients with high operative risk. Our objective was to describe our use of a pressure guidewire during the hybrid stage I procedure to assess quantitatively pulmonary artery band adequacy. DESIGN After reviewing the charts on all high-risk patients who underwent a hybrid stage I procedure at our institution, we compared two groups of patients: those who underwent the standard hybrid stage I palliation (standard cohort) and those with pressure wire-facilitated assessment of distal branch pulmonary artery pressure (pressure wire cohort) to evaluate the impact of pressure guidewire use on procedural risk, radiation time, patient outcomes, and need for reoperation for pulmonary artery band adjustment. RESULTS The pressure guidewire was used in 8 of 14 patients at the time of hybrid stage I procedure and was successful and without complication in all attempts. In the standard cohort, 67% of patients needed reoperation for pulmonary artery band adjustment, compared to 12.5% of patients in the pressure wire cohort (P =.09). Procedure time, radiation exposure, and survival to hospital discharge were not different between groups. CONCLUSIONS This novel use of a pressure guidewire to assess quantitatively pulmonary artery band adequacy at the time of placement is feasible, safe and may decrease the need for reoperation for pulmonary artery band adjustment.

Percutaneous Patent Ductus Arteriosus (PDA) Closure During Infancy: A Meta-analysis

CONTEXT: Patent ductus arteriosus (PDA) is a precursor to morbidity and mortality. Percutaneous (catheter-based) closure is the procedure of choice for adults and older children with a PDA, but use during infancy (<1 year) is not well characterized. OBJECTIVE: Investigate the technical success and safety of percutaneous PDA closure during infancy. DATA SOURCES: Scopus, Web of Science, Embase, PubMed, and Ovid (Medline) were searched through December 2015 with no language restrictions. STUDY SELECTION: Publications needed to clearly define the intervention as percutaneous PDA closure during infancy (<1 year of age at intervention) and must have reported adverse events (AEs). DATA EXTRACTION: The study was performed according to the Systematic Reviews and Meta-Analysis checklist and registered prospectively. The quality of the selected studies was critically examined. Data extraction and assignment of AE attributability and severity were independently performed by multiple observers. Outcomes were agreed on a priori. Data were pooled by using a random-effects model. RESULTS: Thirty-eight studies were included; no randomized controlled trials were found. Technical success of percutaneous PDA closure was 92.2% (95% confidence interval [CI] 88.8–95.0). Overall AE and clinically significant AE incidence was 23.3% (95% CI 16.5–30.8) and 10.1% (95% CI 7.8–12.5), respectively. Significant heterogeneity and publication bias were observed. LIMITATIONS: Limitations include lack of comparative studies, lack of standardized AE reporting strategy, and significant heterogeneity in reporting. CONCLUSIONS: Percutaneous PDA closure during infancy is feasible and associated with few catastrophic AEs; however, the limitations constrain the interpretability and generalizability of the current findings.

Hypoplastic Left Heart Syndrome With Intact or Restrictive Atrial Septum: A Report From the International Fetal Cardiac Intervention Registry

Infants with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum (HLHS-IAS) represent a high-risk subset. Fetal cardiac intervention (FCI) for fetuses with HLHS-IAS has been reported in single-institution series as a therapy that may improve outcomes. This study uses the International Fetal Cardiac Intervention Registry (IFCIR) to examine fetal and maternal characteristics and pregnancy and neonatal outcome data for FCI in this population. For this descriptive analysis, the IFCIR was queried for fetuses with HLHS-IAS evaluated between 2001 and March 2015 for possible FCI and felt to be candidates at the referral center by a variety of published criteria including foramen ovale (FO) size, shunt flow direction across the FO, and pulmonary venous Doppler flow patterns. It includes those who were determined not to be candidates because of other fetal noncardiac conditions, maternal conditions, or maternal preferences, or because FCI was not performed at the participating institution. Further details on the methodology of the IFCIR registry are found in the initial report.1 Participating IFCIR member sites obtained local institutional review board and ethics board approval or a waiver, as governed …