Use of the Gore Viabahn VBX balloon‐expandable endoprosthesis in the congenital heart disease population

The Gore Viabahn VBX balloon‐expandable endoprosthesis (W. L. Gore & Associates, Flagstaff, AZ) is a flexible covered stent that is FDA‐approved for the treatment of iliac artery stenosis, including lesions at the aortic bifurcation. In this case series, we report the first use of the VBX covered stent in congenital heart disease, highlighting several of its unique advantages.     

Prenatal Diagnosis of a Persistent Fifth Aortic Arch,Pulmonary‐to‐Systemic type: An Unusual Association with Evolving Aortic Coarctation

Persistent fifth arch (PFA) is a rare anomaly that is often underdiagnosed and missed. Different PFA types that have been reported in the literature are systemic‐to‐systemic type (most common), systemic‐to‐pulmonary artery (PA), and PA‐to‐systemic types. The designations of systemic‐to‐PA or PA‐to‐systemic are based on if the PFA is a source of PA or systemic blood flow, respectively, in the setting of critical proximal obstruction (pulmonary atresia or aortic atresia). This case describes an unusual PFA, which connects the distal PA to distal ascending aorta; however, it is not associated with critical proximal obstruction, and while it appeared to be an incidental finding in early gestation, progressive serial distal obstruction of the left fourth arch was seen to develop. This case highlights that prenatal diagnosis of PFA is possible and that once a diagnosis is made, serial fetal echocardiograms should be performed to evaluate for evolving lesions.     

Interventional re‐opening of a PDA for reverse potts shunt circulation after ADO I implantation in a child

We report interventional re‐opening of a PDA for reverse Potts shunt circulation 12 months after closure in a 3.8 year old child, suffering from right ventricular (RV) failure due to suprasystemic pulmonary artery hypertension (PAH) after ADO I implantation. After ex vivo simulation, perforation through the mesh of the ADO I with the use of transseptal needle, wire looping (AO‐PA), balloon dilatation, stent implantation (Palmaz 6 mm) and post dilatation, a reverse Pott‐shunt circulation was established. A follow‐up period of 11 months was achieved with preserved RV function and reverse Pott shunt circulation maintained a post ductal saturation of 94–88%. © 2016 Wiley Periodicals, Inc.     

First reported use of drug‐eluting bioabsorbable vascular scaffold in congenital heart disease

The aim of catheter intervention for vascular stenosis is the restoration of lumen area and optimization of distal blood flow. In pediatric practice, this has traditionally been a compromise between less effective balloon angioplasty and bare metal stent insertion with its attendant limitations of size. Bioabsorbable stents offer short‐term relief of stenosis, radial support of the healing lesion, return of endothelial function and crucially, in children, the potential for long‐term growth. Initial experience, in pediatric practice, with metal‐based bioabsorbable stents was relatively disappointing with frequent restenosis secondary to early reabsorption. Design modifications resulting in polymer‐based, drug eluting, bioabsorbable vascular scaffolds (BVS) have reportedly overcome some of these faults. We describe the first reported use of a drug eluting BVS in three patients with: (1) A newborn with severe right pulmonary artery (RPA) stensosis post repair of type two common arterial trunk. (2) A child with pulmonary atresia/ventricular septal defect (VSD) and major aorto‐pulmonary collateral arteries (MAPCAs), and (3) An infant with severe left pulmonary artery (LPA) stenosis in the setting of an LPA sling. © 2015 Wiley Periodicals, Inc.     

Pulmonary artery stents: Long‐term follow‐up

Objectives: Determine the long‐term outcomes of branch pulmonary artery (PA) stents. Background: PA stents in congenital heart disease effectively relieve stenoses in the short‐term. Published long‐term data are limited. Methods : Patients enrolled in an FDA IDE protocol from 1989–92 were included. Clinical follow‐up and catheterization data were evaluated. Patients were included if >5 year follow‐up data was available or if mortality occurred following the initial procedure. Results : There were five deaths: four due to progression of their underlying heart disease, and one from a complication during a follow‐up catheterization. Clinical data for 43 surviving patients demonstrated 39 patients (91%) are in NYHA class I or II. Seven patients underwent surgical intervention during the follow‐up period (five RV‐PA conduit, two Fontan revisions), but none addressed PA stenosis. Final repeat catheterizations were performed in 36 patients (55 stents) 7.2 ± 4.3 years post stent insertion with 1.2 ± 0.9 further procedures with stent dilations. In this subgroup, the minimum vessel diameter increased from 4.7 ± 1.8 to 13.4 ± 2.4 mm (P < 0.001), and the pressure gradient improved from 41 ± 25 to 9 ± 11 mm Hg (P < 0.001). Higher initial gradient and smaller balloons were associated with a final stent diameter of <14 mm (P = 0.030 and 0.046). Jailed vessels occurred in 49% of stents with abnormal angiographic flow in 18/55. Six repeat catheterizations resulted in complications, including the one procedural death. Conclusion : Stents implants for PA stenoses provide effective improvement in vessel caliber in the long‐term. Although repeat interventions are necessary, this procedure reduces RV pressure and provides an important alternative to surgery for residual PA obstruction. © 2009 Wiley‐Liss, Inc.     

Use of 3D rotational angiography to perform computational fluid dynamics and virtual interventions in aortic coarctation

Computational fluid dynamics (CFD) can be used to analyze blood flow and to predict hemodynamic outcomes after interventions for coarctation of the aorta and other cardiovascular diseases. We report the first use of cardiac 3‐dimensional rotational angiography for CFD and show not only feasibility but also validation of its hemodynamic computations with catheter‐based measurements in three patients.     

Working backward: Retrograde balloon angioplasty of atretic arteries in chronic thromboembolic pulmonary hypertension

We describe a patient with chronic thromboembolic pulmonary hypertension who presented to the pediatric cardiac catheterization laboratory for peripheral pulmonary artery recanalization and angioplasty. This case report outlines serial pulmonary arterial rehabilitation via a retrograde approach through intrapulmonary collateral arterial connections.     

The 745.5 issue in code‐based,adult congenital heart disease population studies: Relevance to current and future ICD‐9‐CM and ICD‐10‐CM studies

Objective

Although the ICD‐9‐CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for “rule‐out” congenital heart disease (CHD). The ICD‐10‐CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age.

Design

Echocardiograms of patients with an ICD‐9‐CM code of 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 were reviewed to validate the true incidence of an ASD. This observational, cross‐sectional record review included patients between 11 and 64 years of age.

Results

Medical charts and echocardiograms of 190 patients (47.9% males) were reviewed. The number of falsely coded patients with 745.5 (no ASD) was high (76.3%). Forty‐five (23.7%) patients had a true ASD. Among the 145 patients without an ASD, 100 (52.6%) were classified as having a PFO, 37 (19.5%) had a normal non‐CHD echocardiogram, and 8 (4.2%) had some other CHD anomaly. The likelihood that 745.5 coded for a true ASD was higher in children aged 11‐20 (64.3%) than adults aged 21‐64 years (20.6%).

Conclusions

This validation study demonstrates that 745.5 performed poorly across all ages. As 745.5 is widely used in population‐level investigations and ICD‐10‐CM perpetuates the problem, future analyses utilizing CHD codes should consider separate analysis of those identified only through code 745.5.