Sharon L. Hill

Hybrid Approach for Hypoplastic Left Heart Syndrome: Intermediate Results After the Learning Curve

BACKGROUND Lessons learned during the development of a novel hybrid approach have resulted in a reliable, reproducible alternative treatment for hypoplastic left heart syndrome (HLHS). Herein we report our results using this hybrid approach in a uniform risk cohort. METHODS This is a review of prospectively collected data on patients treated for HLHS using a hybrid approach (n = 40) between July 2002 and June 2007. The hybrid approach includes pulmonary artery bands, a ductal stent, and atrial septostomy as a neonate, comprehensive stage 2 procedure resulting in Glenn shunt physiology at six months and Fontan completion at two years. RESULTS Forty patients had a hybrid stage 1 with 36 undergoing a comprehensive stage 2 procedure. Fifteen patients have completed the Fontan procedure with 17 pending. Overall survival was 82.5% (33 of 40). The seven deaths included one at stage 1, two between stages 1 and 2, three at stage 2, and one between stages 2 and 3. One patient had successful heart transplantation during the interstage period. CONCLUSIONS The hybrid approach can yield acceptable intermediate results that are comparable with a traditional Norwood strategy. Potential advantages of the hybrid approach include the avoidance of circulatory arrest and shifting the major surgical stage to later in life. These data provide the platform for a prospective trial comparing these two surgical options to assess whether there is less cumulative impact with the hybrid approach, thereby improving end organ function, quality, and quantity of life.

Percutaneous Tricuspid Valve Replacement in Congenital and Acquired Heart Disease

OBJECTIVES This study sought to describe the first human series of percutaneous tricuspid valve replacements in patients with congenital or acquired tricuspid valve (TV) disease. BACKGROUND Percutaneous transcatheter heart valve replacement of the ventriculoarterial (aortic, pulmonary) valves is established. Although there are isolated reports of transcatheter atrioventricular heart valve replacement (hybrid and percutaneous), this procedure has been less frequently described; we are aware of no series describing this procedure for TV disease. METHODS We approached institutions with significant experience with the Melody percutaneous pulmonary valve (Medtronic, Inc., Minneapolis, Minnesota) to collect data where this valve had been implanted in the tricuspid position. Clinical and procedural data were gathered for 15 patients. Indications for intervention included severe hemodynamic compromise and perceived high surgical risk; all had prior TV surgery and significant stenosis and/or regurgitation of a bioprosthetic TV or a right atrium-to-right ventricle conduit. RESULTS Procedural success was achieved in all 15 patients. In patients with predominantly stenosis, mean tricuspid gradient was reduced from 12.9 to 3.9 mm Hg (p < 0.01). In all patients, tricuspid regurgitation was reduced to mild or none. New York Heart Association functional class improved in 12 patients. The only major procedural complication was of third-degree heart block requiring pacemaker insertion in 1 patient. One patient developed endocarditis 2 months after implant, and 1 patient with pre-procedural multiorgan failure did not improve and died 20 days after the procedure. The remaining patients have well-functioning Melody valves in the TV position a median of 4 months after implantation. CONCLUSIONS In selected cases, patients with prior TV surgery may be candidates for percutaneous TV replacement.

Multicenter Experience with Perventricular Device Closure of Muscular Ventricular Septal Defects

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure.Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

The Retrograde Aortic Arch in the Hybrid Approach to Hypoplastic Left Heart Syndrome

BACKGROUND Before palliative stage 2 for hypoplastic left heart syndrome, the coronary and cerebral circulations are often dependent on retrograde perfusion by means of the aortic arch. Results of hybrid palliation with a focus on patients exhibiting retrograde aortic arch obstruction (RAAO) were analyzed. METHODS From July 2002 to March 2008 66 consecutive hybrid procedures for hypoplastic left heart syndrome were performed. Patients requiring RAAO intervention based on cardiology-surgery consensus were defined as group 1 (n = 16), whereas all other hypoplastic left heart syndrome patients formed group 2 (n = 50). RESULTS At birth there were no differences between groups in terms of demographics or cardiac function. Group 1 had more patients with aortic atresia (94% versus 58%; p = 0.01), and 69% of patients had initial echocardiographic comments regarding incipient RAAO versus 26% in group 2 (p = 0.007). The type of ductal stent, balloon versus self-expandable, did not influence the subsequent development of RAAO. Before RAAO intervention (mean age, 74 days), group 1 patients had significantly more tricuspid regurgitation. The main treatment for RAAO in group 1 was coronary stent insertion, with 3 patients having a reverse central shunt. At a mean follow-up of 611 days, group 1 had reduced survival interstage (56.3% versus 88%; p = 0.005) and overall (43.7% versus 70%; p = 0.03). CONCLUSIONS Clinically important RAAO occurred in 24% of the hypoplastic left heart syndrome patients in this series. If RAAO is detected at birth or early interstage, a Norwood operation is now favored. Palliative interventional catheterization remains very important mid and late interstage for continuing the hybrid strategy toward comprehensive stage 2.

Atrial septal interventions in patients with hypoplastic left heart syndrome

Objectives: To report an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS). Background: The success of the Hybrid approach in palliating patients with HLHS is crucially dependant on relieving any significant interatrial restriction. Data on transcatheter interventions to relieve atrial septal restrictions in patients with HLHS are limited. Methods: We retrospectively reviewed 67 transcatheter atrial septal interventions that were performed between July 2002 and September 2007 in 56 patients with HLHS. The median weight was 3.35 kg. About 10.7% of patients had an intact atrial septum. Balloon atrial septostomy (BAS) was used in 77.6% of procedures, additional techniques in 35.8% of procedures. Patients were divided into those with standard atrial septal anatomy (group A, n = 33) and those with complex atrial septal anatomy (group B, n = 23). Results: The mean trans‐septal gradient was reduced significantly from 7 mm Hg to 1 mm Hg with the median time to discharge being 3.5 days. Major adverse events were seen in 8.9% of procedures, whereas minor adverse events occurred in 26.8% of procedures. Adverse events were significantly less common in patients with standard atrial septal anatomy, compared to those with complex atrial septal anatomy (25.6% versus 50.0%). About 19.6% patients required repeated atrial septal interventions. Survival up to and including Comprehensive stage II palliation was 73% group A, and 57% in group B. Conclusions: With utilization of appropriate techniques and equipment, atrial septal interventions in HLHS can be performed successfully in virtually all patients. Complex atrial septal anatomy is technically challenging and has a higher incidence of procedural adverse events. In the majority of patients, standard BAS can be performed safely, and is usually the only intervention required to achieve adequate relief of atrial septal restriction until Comprehensive stage II palliation.

Stenting complex aortic arch obstructions

To present our institutional experience of endovascular stent therapy in patients with complex aortic arch lesions.

Radiofrequency perforation and cutting balloon septoplasty of intact atrial septum in a newborn with hypoplastic left heart syndrome using transesophageal ICE probe guidance

Newborns with hypoplastic left heart syndrome and intact atrial septum present an emergent and unique challenge to a childrens heart center. This case report describes new transcatheter techniques (use of radiofrequency energy to perforate the atrial septum followed by cutting balloon and static balloon septoplasty) and novel use of a transesophageal ICE probe. Catheter Cardiovasc Interv 2005;64:214–217.

Interstage Echocardiographic Changes in Patients Undergoing Hybrid Stage I Palliation for Hypoplastic Left Heart Syndrome

OBJECTIVE The hybrid procedure is an alternative for initial palliation for patients with hypoplastic left heart syndrome. No echocardiographic data exist for the interstage (IS) period. The goal of this study was to describe the echocardiographic changes during this period. METHODS A chart review was performed on patients discharged from the hospital with the diagnosis of hypoplastic left heart syndrome who underwent hybrid palliation. Echocardiograms at hospital discharge (post-hybrid), before and after any IS interventions, and before comprehensive stage II procedure were reviewed. Distal right pulmonary artery (RPA) and left pulmonary artery (LPA) velocity, slope, velocity time integral (VTI), pressure halftime (p1/2), pulsatility index (PI), and systolic/diastolic (S/D) ratio of the waveforms were recorded. Atrial septal defect (ASD) mean gradient, ductus arteriosus peak velocity, retro-aortic arch peak velocity, tricuspid regurgitation (TR), and right ventricular function were documented. Exploratory hypotheses were tested with chi-square and t tests. Stepwise logistic regression was used to identify any multiple sets of relatively independent variables. RESULTS Thirty patients met inclusion criteria. Fourteen patients underwent 22 different interventions at the atrial septum, ductus arteriosus, or retro-aortic arch in the IS period. Baseline ASD gradient (P = .012) and ductus arteriosus velocity (P = .002) predicted an IS intervention. There were significant differences in LPA and RPA VTI (P = .011, .03), p1/2 (P = .038, .008), and S/D (P = .012, .033); RPA slope (P = .013); ASD gradient (P = .003); ductus arteriosus velocity (P = .021); and TR (P = .031) before and after an intervention. There were significant differences in post-hybrid versus pre-comprehensive stage II LPA and RPA VTI (P = .009, .022), PI (P = .031, .022), and peak velocity (P = .004, .037); RPA S/D (P = .025) and p1/2 (P = .029); ductus arteriosus velocity (P < .001); retro-aortic arch peak velocity (P = .035); and ASD mean gradient (P < .001). Pre-comprehensive stage II function tended to predict death (P = .085). CONCLUSION Echocardiographic parameters help predict IS course and guide clinical therapy for this patient population.

Hybrid procedures: adverse events and procedural characteristics--results of a multi-institutional registry.

INTRODUCTION Procedural cooperation between cardiac surgeon and interventional cardiologist to facilitate interventions such as device delivery or angioplasty (hybrid procedure) has become increasingly common in the management of patients with congenital heart disease. DESIGN Data were prospectively collected using a multicenter registry (C3PO). Between February 2007 and December 2008, seven institutions submitted data regarding 7019 cardiac catheterization procedures. Procedural data and adverse events (AEs) of 128 hybrid procedures were evaluated. RESULTS There was significant variability in the number of hybrid procedures per center, ranging from one to 89 with a median of eight. A total of 60% of interventional (vs. strictly diagnostic) hybrid procedures were performed by one center. The median weight was 3.7 kg (0.7-86 kg). Single-ventricle circulation was present in 60% of the procedures. Hybrid procedures included: patent ductus arteriosus (PDA) stent placement (n = 55), vascular rehabilitation (n = 25), ventricular septal defect (VSD) device closure (n = 7), valvotomy (n = 3), and diagnostic hybrid procedures (n = 38). Sixteen AEs occurred in 15/128 (12%) procedures. These included minor or trivial AEs (n = 9), moderate AEs (n = 5), major AEs (n = 1), and catastrophic AEs (n = 1). The type of AE documented included arrhythmias (n = 6), hypoxia or hypotension (n = 3), vessel or cardiac trauma (n = 2), and other events (n = 5). Of documented AEs, 9/16 (56%) were classified as not preventable, 6/16 (38%) as possibly preventable, and 1/16 (6%) as preventable. The incidence of AE related to PDA stent placement with surgical exposure (5/50, 10%) was significantly lower when compared with PDA stent placement performed percutaneously (4/5, 80%, P= .002). CONCLUSION Hybrid procedures appear to have a low incidence of associated major AEs. PDA stent placement performed as a palliation of hypoplastic left heart syndrome (HLHS) or complex single/two ventricle patients may have a lower incidence of AEs if performed using a direct approach with surgical exposure rather than a percutaneous approach. Accurate definitions of these innovative procedures are required to facilitate prospective data collection.

Treatment of aortic arch aneurysm with a NuMED-covered stent and restoration of flow to excluded left subclavian artery: Perforation and dilation of e-PTFE can be done!†

We describe a case using a NuMED‐covered Cheatham‐Platinum (CP) stent (NuMED, Hopkinton, NY) to treat an aneurysm after previous balloon angioplasty and bare stent implantation for coarctation of the aorta (CoA). Exclusion of the left subclavian artery (LSCA) was anticipated. After wire perforation of the covered CP stent, balloon angioplasty was performed through a stent cell to recannulize the LSCA.