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There has been a paradigm shift in the management of patients with congenital heart disease with a move away from conventional surgical treatment in favor of a percutaneous catheter-based approach across the spectrum of valvular heart diseases. The Sapien S3 valve implantation in the pulmonary position has been previously reported using a conventional transcatheter approach in patients with pulmonary insufficiency due to an enlarged right ventricular outflow tract. In this report, we present 2 unique cases of intraoperative hybrid implantation of Sapien S3 valves in patients with complex pulmonic and tricuspid valvular disease.

Transcatheter closure of ventricular septal defects is considered first-line therapy when anatomically appropriate but is often challenged by proximity to the conduction system in perimembranous defects, or irregular defect shape, especially residual defects that may remain post-operatively. Advancements in device design, however, have allowed for significant improvements in deployment techniques and overall safety. Here we describe the first use of the Lifetech Konar-multifunction™ occluder device in North America, and our specific use of this device to close complex post-operative muscular and a perimembranous-ventricular septal defects in the same patient.

Background: Numerous studies have detailed outcomes of Transcatheter pulmonary valve placement (TPV) in young patients, but there is a paucity of data in older patients. We sought to evaluate short and mid-term outcomes of TPV in patients older than 40 years of age. Methods: Retrospective multicenter study of patients older than 40 years at the time of TPV from January 2010 to February 2018. Demographics, procedure details, and follow up data were collected. Results: A total 75 subjects presented for TPV with median age of 49.8 (IQ 45–60). Half of the patients 39 (52%) had evidence of NYHA class III or IV at time of presentation. A total of 65 (87%) had successful TPV. Patients with severe PR had immediate resolution of PI (except for one). Patients with PS had significant improvement in gradient from median of 25.5 to 5.5 mmHg (p

Objectives Arch branching has never been shown to influence recoarctation after extended end-to-end anastomosis via thoracotomy, yet in each study bovine arch identification is grossly underreported. This study aims to (1) assess chart review reliability in bovine arch identification; (2) determine recoarctation risk with a bovine arch; and (3) explore an anatomic explanation for recurrent arch obstruction based on arch anatomy. Patients A total of 49 consecutive patients underwent thoracotomy with extended end-to-end aortic coarctation repair at a single institution (2007-2012). Methods Echocardiograms from these patients were reviewed for arch anatomy and compared with the echocardiographic reports. Recurrent arch obstruction was defined as an echocardiographic gradient across the repair of 20 mm Hg or greater. For cases with angiographic images (n = 17), a scaled clamping distance between the left subclavian artery and the maximal proximal clamp location on orthogonal projections was then calculated across arch anatomies. Results Chart review identified 6.1% (3/49) of patients with a bovine arch compared with 28.6% (14/49) on targeted image review. A total of 28.6% (4/14) of patients with a bovine arch had a follow-up gradient of 20 mm Hg or greater. Only 5.7% (2/35) of patients with normal arch branching had a follow-up gradient of 20 mm Hg or greater. The mean clamping index was significantly diminished in patients with bovine arch anatomy. Conclusions Arch anatomy often goes undocumented on preoperative imaging, yet children undergoing extended end-to-end repair with bovine arch anatomy are at a significantly increased risk of recoarctation. This may be due to a reduced clampable distance to facilitate repair. These results should be considered in the preoperative assessment, parental counseling, and surgical approach for children with discrete aortic coarctation.

In cases where femoral access is untenable for secundum atrial septal defect closure, the internal jugular vein can be used instead. We report a case of atrial septal defect closure in a patient with significant vascular thrombosis, requiring the use of the left internal jugular vein for access. To the best of our knowledge, this is the first report that documents the use of the left internal jugular vein for atrial septal defect closure in a patient with situs solitus anatomy.

Background The arterial switch operation is standard of care for infants born with dextrotransposition of the great arteries. Supravalvar pulmonary stenosis is a common complication that may require reintervention—balloon angioplasty, stenting, or surgical augmentation. A subset of patients requires more than one reintervention. Methods We performed a retrospective review of patients who underwent the arterial switch operation for dextrotransposition of the great arteries at a single institution between August 1990 and January 2014. Anatomic, perioperative and follow-up data were collected. Reinterventions were stratified in a site-specific manner. Statistical analysis was performed using IBM SPSS version 21 (IBM Corp, Armonk, NY). Results Of the 103 patients who met inclusion criteria, 28% (29) required reintervention for supravalvar pulmonary stenosis; 41% of those receiving primary reintervention required an additional 21 reinterventions. Balloon angioplasty of the main pulmonary artery and left pulmonary artery was associated with the need for multiple reinterventions (odds ratio 4.9, p = 0.051, and odds ratio 5.1, p = 0.029, respectively). Freedom from future reintervention at the main pulmonary artery and left pulmonary artery was significantly shorter after balloon angioplasty relative to alternative reintervention options (hazard ratio 10, p = 0.005, and hazard ratio 3.2, p = 0.02, respectively). Balloon angioplasty of the right pulmonary artery was not associated with an increased risk of reintervention ( p = 0.42). Conclusions Supravalvar pulmonary stenosis after the arterial switch operation for dextrotransposition of the great arteries is common and more than one reintervention are required in a subset of patients. The benefit of balloon angioplasty of the main pulmonary artery and left pulmonary artery was shown to be temporary. Attempting balloon angioplasties at these locations remain reasonable, although families should be counseled about the increased incidence of, and decreased time to, subsequent reintervention that is associated with this treatment option.

Supravalvular aortic stenosis as an early complication of transposition of the great artery repair is rare with few cases reported. Furthermore, transcatheter intervention is uncommon as surgical re-intervention has been traditionally done. We describe two cases of supravalvular aortic stenosis at the anastomotic site as an early complication of the arterial switch operation. Both patients underwent balloon angioplasty of the supravalvular aorta with improvement in postangioplasty gradients and angiographic appearance. Both patients at short-term follow-up had persistent improved gradient without need for further intervention.

A 6-year-old male child born with hypoplastic left heart syndrome (HLHS) was palliated with an extracardiac nonfenestrated Fontan procedure (18-mm Gore-Tex tube graft). He developed low-pressure (mean Fontan pressure 10 mmHg) protein-losing enteropathy 6 months after Fontan palliation. After initially responding to medical therapy and transcatheter pulmonary artery stent implantation, he developed medically refractory protein-losing enteropathy. At this time, his transthoracic echocardiogram showed new restriction across his native atrial septum with an 8 mmHg mean gradient. Cardiac catheterization now showed high-pressure (mean Fontan pressure 18-20 mmHg) protein-losing enteropathy and a new 6 mmHg mean gradient across the atrial septum. To avoid cardiopulmonary bypass, he underwent successful transcatheter relief of atrial septal restriction and creation of a fenestration with rapid clinical and biochemical improvement of his protein-losing enteropathy.

Introduction A 24-year-old female presented for percutaneous pulmonary valve placement. She was born with tetralogy of Fallot and had initial palliation with a Blalock–Taussig shunt followed by complete repair at age of 4 years including placement of a homograft conduit in the right ventricle to pulmonary artery position. She had developed severe obstruction in the conduit. Procedure Angiography showed a heavily calcified conduit with moderate insufficiency. During pre-dilation, a total of six balloons ruptured due to heavy conduit calcification prior to reaching desired inflation diameter. Subsequently, double balloon technique was attempted using two 9-mm Conquest balloons. One of the conquest balloons was then replaced by 16-mm Atlas balloon and conduit dilation was performed. At full inflation, the Conquest balloon ruptured. The deflated ruptured Conquest balloon was kept in the conduit and the Atlas balloon was exchanged for 18 and then 20-mm Atlas balloons and both were used to dilate the conduit. The deflated ruptured Conquest balloon protected the Atlas balloons and conduit angioplasty proceeded successfully without further rupture of any balloon. Subsequently, four stents were placed in the conduit followed by delivery of Melody Valve using a 20-mm Ensemble system with excellent results. Conclusion This case illustrates a novel method of using the body of a ruptured balloon to protect subsequent balloons from rupture due to heavy conduit calcification. This method requires the presence of two venous access lines but might save time, effort, and cost from repeated balloon ruptures. © 2015 Wiley Periodicals, Inc.

Background— Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes. Methods and Results— Across 10 US centers, 27 patients with a dysfunctional LVOT conduit were evaluated in the catheterization laboratory between December 2008 and August 2015 with the intent to perform TPVR. TPVR was successful in 23 patients (85%). Five serious adverse events occurred in 4 cases (15%), including pulmonary hemorrhage, hypotension requiring vasoactive support, conduit disruption requiring covered stent (n=2), and acute RV dysfunction with flash pulmonary edema. After TPVR, the LVOT peak systolic ejection gradient decreased from median of 35 to 17 mm Hg ( P P =0.02) and higher post-TPVR RV sphericity index (median 0.88 versus 0.52; P =0.004). Post-TPVR, there were 2 late deaths because of RV failure and 1 cardiac transplantation because of progressive RV dysfunction and tricuspid regurgitation. Conclusions— TPVR in dysfunctional LVOT conduits is feasible but associated with an important rate of TPV nonimplantation and procedural serious adverse events. Worsening systemic RV function and tricuspid regurgitation may develop after LVOT TPVR.

OBJECTIVES We sought to evaluate outcomes of stent implantation (SI) for recurrent coarctation of the aorta (RC) following the Norwood operation. BACKGROUND RC is common following the Norwood operation. Balloon angioplasty (BA) is standard treatment but may result in unsatisfactory relief of RC. SI may improve RC, but outcome data are limited. METHODS We performed a multi-center retrospective study of patients who underwent SI for RC between the Norwood operation and Fontan completion. Outcomes were examined, including procedural success, serious adverse events (SAE), and freedom from re-intervention. A core laboratory was utilized to review angiograms. Coarctation Index (CI) was calculated before and after SI. Paired t-test and Wilcoxon signed-rank test were used to compare pre- and post-SI variables. RESULTS Thirty-three patients at 8 centers underwent SI for RC at a median age of 5 months (IQR 4.1, 13.3) and weight of 5.9 kg (5.2, 8.6). Aortic arch gradient improved from 20 (15, 24) to 0 (0, 2) mmHg following SI (P

Objectives We sought to review our current philosophy that all primary invasive electrophysiologic (EP) studies in patients with atrial switch procedures (ASPs) should undergo hemodynamic evaluation and have interventional expertise available. Background Patients who have undergone an ASP for dextro-transposition of the great arteries have a high incidence of both hemodynamic and EP sequelae. We present our data to support the combined assessment approach for these patients. Methods Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed. Results A total of 18 patients underwent concurrent EP invasive procedure and cardiac catheterization. The median age was 31 (14–43 years) with the majority being male (67%). Patients underwent a total of 30 concurrent primary invasive EP procedure and cardiac catheterization; 14 (47%) of the catheterization procedure were interventional. Some of the catheterization procedures required more than one intervention with total of 19 separate interventions. There were nine (47%) unexpected interventions. The majority of patients (n = 14, 77.8%) had one or more abnormal hemodynamic finding including baffle obstruction (n = 13, 72%), elevated filling pressures (n = 3, 17%), and secondary pulmonary hypertension (n = 3, 17%). Non-EP–related interventional procedures included systemic or pulmonary venous baffle stenting for significant obstruction (n = 7). EP-related interventions included transbaffle puncture for ablation of left-sided reentry circuits (n = 5), closure of previously undiagnosed baffle leaks prior to pacemaker/implantable cardioverter defibrillator (ICD) placement to prevent paradoxical embolism (n = 3), superior baffle stenting to facilitate pacemaker/ICD lead placement (n = 2), and retrieval of retained transvenous pacemaker/ICD lead (n = 2). Conclusion Due to the frequency of abnormal hemodynamics or interventional needs, strong consideration for routine concurrent hemodynamic assessment and availability of interventional expertise is warranted during primary invasive EP procedures in patients post ASP.

An infant diagnosed by fetal echocardiography with hypoplastic left heart syndrome and suspected severe restriction of the foramen ovale developed postdelivery peripheral oxygen desaturation as low as 30% soon after transfer from the neonatal nursery to the cardiac intensive care unit. Supplemental oxygen was administered via nasal cannula and the infant underwent emergent cardiac catheterization soon after birth. The catheterization revealed cor triatriatum and an obstructed vertical vein with aberrant pulmonary venous drainage. This anatomy provided significant challenges for the anesthesia and cardiology teams. The difficulties in anesthetic, diagnostic and interventional management and decision-making in this complex anatomical diagnosis are discussed and the literature is reviewed.

Objective We sought to review and analyze the hemodynamic derangements during prograde transcatheter aortic intervention (PTAI) in single ventricle patients. Background Although PTAI for postsurgical recurrent coarctation in single ventricle patients has been described; hemodynamic instability during the intervention is variably reported. Methods Pre-, intra-, and postprocedural records and outcomes of patients with SVP undergoing PTAI for post-Norwood aortic coarctation were retrospectively reviewed. The full disclosure waveform review was used to further categorize hemodynamic derangements during the intervention. Results A total of 26 PTAIs were performed in 11 patients between October 2007 and December 2013. The median age and weight was 4.2 (2.3–43) months and 5.3 (3.2–15.7) kg. PTAI included balloon angioplasty (BA) in 73% of procedures (n = 19) and stent implantation (SI) in 27% (n = 7). Hemodynamic derangement was more severe in the SI group compared with the BA group. Two of seven (29%) of the SI group required cardiopulmonary resuscitation. Conclusions Hemodynamic instability during PTAI is common in patients with SVP and more profound during SI. These findings have important implications for informed consent, anesthetic considerations, inotropic support, additional central venous access, and extracorporeal support/surgical backup.

Objective To determine whether superior vena cava (SVC) stent implantation is superior to balloon angioplasty for relieving SVC stenosis. Background SVC stent and balloon dilation have been used as treatment for SVC stenosis. Although safe and effective, outcome data comparing the two methods are limited. Methods A Pediatric Cardiac Care Consortium review identified SVC stenosis. Patients who required SVC intervention were divided into two subgroups—balloon dilation (Group A) and stent implantation (Group B). Logistic regression and the log-rank test were used to test the need for re-intervention within 6 months after the initial procedure. Results SVC intervention was performed on 210/637 patients with SVC stenosis (33%). There were 108/210 (51%) patients with balloon dilation (Group A) and 102/210 (49%) with stent implantation (Group B). Re-intervention within 6 months of the initial intervention was more common in Group A compared to Group B [Group A = 31/40 (77.5%); Group B = 5/22 (22.7%)]. The odds-ratio for re-intervention within 6 months of the initial procedure for balloon vs. stent, is 7.3 [95% CI: (2.91, 22.3), P < 0.0001]. In addition, during the first 6 months after an intervention for SVC stenosis the proportion of patients with stent implantation that remained free of re-intervention was significantly higher than after balloon angioplasty (log-rank test, P < 0.0001). Neither age nor weight was significantly associated with the need for re-intervention. Conclusions SVC stent implantation is more effective than angioplasty in relief of SVC obstruction. Weight and age are not risk factors for early re-intervention. © 2012 Wiley Periodicals, Inc.

PE can occur following HCT. However, the incidence, etiology, risk factors, and treatment remain unclear. We performed a retrospective study evaluating 355 pediatric recipients of HCT treated at a single institution between January 2005 and August 2010. No cases of PE were identified in the autologous HCT (auto-HCT) recipients (0/43), while 19% (57/296) of allogeneic HCT (allo-HCT) developed PE. Among the 57 PE patients, 40 (70%) were males; the median age at transplantation was 6.6 yr (0.1-17.3 yr). Thirty-six patients (63%) had significant PE with 23 patients (40%) treated by pericardiocentesis, and 19 (33%) experiencing recurrent PE. OS rates for patients who developed PE were 84% at 100 days and 65% at three yr after HCT. Risk factors associated with PE on multivariate analysis included myeloablative conditioning (p = 0.01), delayed neutrophil engraftment (p < 0.01), and CMV + serostatus of the recipient (p = 0.03). Recipients with non-malignant diseases were significantly less likely to die after development of PE (p = 0.02 and 0.004 when comparing with standard and high-risk diseases, respectively). In summary, PE is a common and significant complication of pediatric allo-HCT. Prospective studies are needed to better determine the etiology and optimal method of PE treatment after HCT.

The risk factors for superior vena cava (SVC) obstruction after pediatric orthotopic heart transplantation (OHT) have not been identified. This study tested the hypothesis that pretransplant superior cavopulmonary anastomosis (CPA) predisposes patients to SVC obstruction. A retrospective review of file Pediatric Cardiac Care Consortium registry from 1982 through 2007 was performed. Previous CPA, other cardiac surgeries, gender, age at transplantation, and weight at transplantation were assessed for the risk of developing SVC obstruction. Death, subsequent OHT, or reoperation involving the SVC were treated as competing risks. Of the 894 pediatric OHT patients identified, 3.1% (n = 28) developed SVC obstruction during median follow-up of 1.0 year (range: 0 to 19.5 years). Among patients who developed SVC obstruction, 32% (n = 9) had pretransplant CPA. SVC surgery before OHT was associated with posttransplant development of SVC obstruction (p

Congenital pulmonary valve stenosis is a common congenital heart disease. Isolated pulmonary valve stenosis comprises 8-10% of all congenital heart disease. While surgical pulmonary valvotomy has been available as a treatment since 1956, it requires a median sternotomy, use of cardiopulmonary bypass, and post-surgical ICU admission with multi-day hospitalization. Recognizing the potential advantages of a less invasive approach, the first attempts at percutaneous catheter-based dilation of stenotic pulmonary valves were performed in the 1950s. As the technique was refined and catheter and balloon technology have advanced, the results of balloon pulmonary valvuloplasty have improved and the approach has become the standard of care for treating pulmonary valve stenosis. The purpose of this article is to discuss the anatomy and physiology of pulmonary valve stenosis, describe the techniques and current technology, and review the outcomes of balloon pulmonary valvuloplasty.