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.     

Technique of temporary subcutaneous “Figure‐of‐eight” sutures to achieve hemostasis after removal of large‐caliber femoral venous sheaths

Over the last decade, significant advances have been made in percutaneous treatment of structural heart diseases. Many of these interventions require insertion of large caliber sheaths in the femoral veins. Manual compression, compression devices, and various closure devices have been used for removal of large‐sized venous sheaths. Here, we describe the use of a temporary subcutaneous “Figure‐of‐Eight” suture technique for venous access site closure after removal of large‐caliber sheaths. © 2011 Wiley‐Liss, Inc.     

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.     

Insufficiency of a Damus‐Kaye‐Stansel anastomosis in a Fontan patient: Transfemoral implantation of an Edwards Sapien 3 valve

We present a 22‐year‐old patient with a univentricular heart who had already undergone five open heart surgeries including a Damus‐Kaye‐Stansel procedure, Fontan completion and tricuspid valve replacement. In addition, epimyocardial pacemaker implantation and repeated revisions had been necessary. He developed symptomatic free regurgitation of the pulmonary portion of his DKS anastomosis. To avoid additional high‐risk open‐heart surgery, we successfully implanted an Edwards Sapien 3 valve transfemorally in the pulmonary portion of the DKS anastomosis relieving insufficiency.     

Catheter‐associated bloodstream infection incidence and outcomes in congenital cardiac surgery

Objective: Catheter‐associated bloodstream infections complicate and prolong hos‐ pitalizations. The incidence of catheter‐associated bloodstream infections in children undergoing congenital cardiac surgery has not been reported. This study sought to define the incidence of catheter‐associated bloodstream infections after congenital cardiac surgery in neonates and infants ≤12 months old and compare hospital out‐ comes and costs to those who underwent surgery and did not have a catheter‐associ‐ ated bloodstream infections.
Design: Retrospective review of hospital admissions between October 2013 and November 2015 for neonates and infants ≤12 months old at admission with ICD‐9 codes for congenital cardiac surgery from discharge data from Vizient Clinical Data Base/Resource Manager (formerly University HealthSystem Consortium), an ana‐ lytic platform for performance improvement. Hospitals were included if they had >100 congenital cardiac surgery admissions during the study period. Admissions were stratified by age at admission: Neonates (<1 month) and Infants (1‐12 months). Established database flags for catheter‐associated bloodstream infections were uti‐ lized. Length of stay, mortality, and direct costs were compared between admissions with and without catheter‐associated bloodstream infections using t test or χ², as appropriate.
Results: Catheter‐associated bloodstream infections incidence after congenital car‐ diac surgery was higher in Neonates than Infants (1.5 vs 0.8%, P = .024). Length of stay and direct costs were significantly higher for patients with catheter‐associated bloodstream infections in both groups. Mortality was higher in the Infant group with catheter‐associated bloodstream infections compared to those without catheter‐as‐ sociated bloodstream infections.
Conclusion: Neonates develop catheter‐associated bloodstream infections at nearly twice the rate of older infants. For those who develop infection, mortality is 2‐8‐fold greater and hospital costs are 4‐6‐fold higher, which further highlight the importance of catheter‐associated bloodstream infections prevention in this population.     

Transcatheter treatment for systemic‐to‐pulmonary artery shunt obstruction in infants and children

Systemic‐to‐pulmonary artery shunt occlusion is a life‐threatening complication in patients with shunt‐dependent pulmonary blood flow. In the current era, the definitive diagnosis of shunt obstruction, and interventions to re‐establish shunt patency are performed in the catheterization laboratory, rather than the operating room. This review summarizes the various transcatheter techniques used by the interventionalist to re‐establish flow through occluded shunts. © 2008 Wiley‐Liss, Inc.     

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.     

Robotic PCI: Evolving from novel toward non‐inferior

• Robotic‐assisted PCI appears to be safe and feasible in both simple and complex lesions.
• In this small cohort study, analysis of manual versus robotic PCI suggests comparable clinical outcomes.
• Further adequately powered, randomized, multicenter studies are needed to definitively evaluate outcomes in manual versus robotic‐assisted PCI.

     

Hybrid transcatheter tricuspid valve‐in‐valve placement of an Edwards Sapien XT valve in palliated hypoplastic left heart syndrome

Tricuspid regurgitation in the context of hypoplastic left heart syndrome (HLHS) carries a significant burden of both mortality and morbidity. We report successful hybrid trans‐atrial implantation of an Edwards Sapien XT valve into a dysfunctional bioprosthetic valve in the tricuspid position of a 9‐year‐old patient with HLHS. There was an immediate fall in the Fontan pressures combined with significant clinical improvement and the valve continues to function well nearly 3 years post implant. To the best of our knowledge, this is the first described implantation of a transcatheter valve in this specific position and anatomy.     

Use of a dose‐dependent follow‐up protocol and mechanisms to reduce patients and staff radiation exposure in congenital and structural interventions

Background: Increasingly complex structural/congenital cardiac interventions require efforts at reducing patient/staff radiation exposure. Standard follow‐up protocols are often inadequate in detecting all patients that may have sustained radiation burns. Methods: Single‐center retrospective chart review divided into four intervals. Phase 1 (07/07–06/08, 413 procedures (proc)): follow‐up based on fluoroscopy time only; frame rate for digital acquisition (DA) 30 fps, and fluoroscopy (FL) 30 fps. Dose‐based follow‐up was used for phase 2–4. Phase 2 (07/08–08/09, 458 proc): DA: 30 fps, FL: 15 fps. Phase 3 (09/09–06/10, 350 proc): DA: 15–30 fps, FL: 15 fps, use of added radiation protection drape. Phase 4 (07/10–10/10, 89 proc): DA: 15–30 fps, FL: 15 fps, superior noise reduction filter (SNRF) with high‐quality fluoro‐record capabilities. Results: There was a significant reduction in the median cumulative air kerma between the four study periods (710 mGy vs. 566 mGy vs. 498 mGy vs. 241 mGy, P < 0.001), even though the overall fluoroscopy times remained very similar (25 min vs. 26 min vs. 26 min vs. 23 min, P = 0.957). There was a trend towards lower physician radiation exposure over the four study periods (137 mrem vs. 126 mrem vs. 108 mrem vs. 59 mrem, P = 0.15). Fifteen patients with radiation burns were identified during the study period. When changing to a dose‐based follow‐up protocol (phase 1 vs. phase 2), there was a significant increase in the incidence of detected radiation burns (0.5% vs. 2%, P = 0.04). Conclusions: Dose‐based follow‐up protocols are superior in detecting radiation burns when compared to fluoroscopy time‐based protocols. Frame rate reduction of fluoroscopy and cine acquisition and use of modified imaging equipment can achieve a significant reduction to patient/staff exposure. © 2011 Wiley‐Liss, Inc.     

Left‐side pericardial agenesis: Putting the pieces together

A 56‐year‐old woman complained of atypical chest pain. At initial diagnostic workup, chest radiograph and transthoracic echocardiogram depict specific findings that raised the suspicion of pericardial agenesis. Cardiac computed tomography showed extreme levoposition of the heart and interposition of lung parenchyma between the aorta and the pulmonary artery. Those findings were consistent with the diagnosis of left‐side pericardial agenesis, which was subsequently confirmed in cardiac magnetic resonance study. Left‐side pericardial agenesis is rare and can result in nonspecific symptoms. An integrated multimodality imaging approach may provide incremental value on diagnosis approach. Its benign prognosis allows a conservative approach.     

Hand‐held echocardiography in children with hypoplastic left heart syndrome

Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4‐point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement.
Results: Thirty‐two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days‐4.2 years). Median weight was 5.9 kg (2.6‐15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds.
Conclusions: HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS.     

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.     

Transcatheter closure of perimembranous ventricular septal defects using a novel wire‐maintaining technique

Objectives : We aimed to evaluate the clinical feasibility and safety of a novel wire‐maintaining technique (WMT) for transcatheter closure of perimembranous ventricular septal defects (PmVSDs). Background : Transcatheter device closure of PmVSDs has been increasingly performed and this procedure is now offered as primary therapy at many institutions. Methods : A total of 103 patients with complex PmVSDs were randomized to either the conventional technique (CT) group (n = 51) or the WMT group (n = 52). In the CT group, the track wire was withdrawn before the occluder insertion and deployment. If inappropriate, the initial occluder was withdrawn and the “arteriovenous wire loop” was re‐established. In the WMT group, the track wire was maintained in the delivery sheath during the procedure. If the initial occluder was inappropriate, the delivery sheath could be reintroduced over the maintained wire. Results : For those patients who could not achieve optimal results with the initially selected occluders and required further device replacement, the procedure and fluoroscope time was reduced significantly in the WMT group compared with the CT group ([50.8 ± 13.2] min vs. [61.5 ± 15.4] min, P < 0.01 and [21.6 ± 8.6] min vs. [27.3 ± 7.4] min, P < 0.05; respectively). There was no difference in the incidence of complications of two groups. Conclusions : The WMT was feasible and safe for the transcatheter treatment of PmVSDs, especially for those complex defects with great challenge. Using this novel technique, the reconstruction of “arteriovenous wire loop” could be avoided in patients requiring device replacement. © 2009 Wiley‐Liss, Inc.     

Impact of durable ventricular assist devices on post‐transplant outcomes in adults with congenital heart disease

Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy.