James A. Quintessenza

Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect

The extant nomenclature for ventricular septal defect (VSD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Tracheal allograft reconstruction: the total North American and worldwide pediatric experiences

BACKGROUND We reviewed both the North American and the total worldwide pediatric experience with tracheal allograft reconstruction as treatment for patients with long segment and recurrent tracheal stenosis. METHODS The stenosed tracheal segment is opened to widely patent segments. The anterior cartilage is resected and the posterior trachealis muscle or tracheal wall remains. A temporary silastic intraluminal stent is placed and absorbable sutures secure the chemically preserved cadaveric trachea. After initial success with this technique in Europe, several North American centers have now performed the procedure. The cumulative North American experience includes 6 patients (3 adults and 3 children). Worldwide, more than 100 adults and 31 children, aged 5 months to 18 years, with severe long segment tracheal stenosis have undergone tracheal allograft reconstruction. RESULTS In North America, 5 of 6 patients have survived, with one early death due to bleeding from a tracheal-innominate artery fistula in a previously irradiated neck. Worldwide, 26 children survived (26 of 31 = 84%) with follow-up from 5 months to 14 years. Only 1 of 26 pediatric survivors (1 of 26 = 3.8%) had a tracheostomy. CONCLUSIONS Tracheal allograft reconstruction demonstrates encouraging short- to medium-term results for patients with complex tracheal stenosis. Allograft luminal epithelialization supports the expectation of good long-term results.

Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window

The extant nomenclature for aortopulmonary window (AP window) and pulmonary artery origin from ascending aorta (hemitruncus) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

The role of airway stents in the management of pediatric tracheal, carinal, and bronchial disease

OBJECTIVE A variety of stents are available to aid in the management of complex tracheal, carinal and bronchial stenoses. We reviewed our multi-institutional experience with airway stents in children. METHODS Thirty-three children (age, 13 days-18 years) from four institutions have had a total of 40 stents placed to aid in the management of complex airway stenoses. Three stent types were utilized: 29 silastic stents, five expandable metal stents and six customized carinal stents (four patients had two stents and one patient had four stents). Thirty children had tracheal stents, six children had bronchial stents, and two infants had carinal stents (three children had stenting of more than one area and two had stenting of all three locations). Twenty-eight patients (age, 5 months-18 years; mean, 8.06 years; SEM, 1.13 years) had stents placed after a variety of airway reconstructive procedures. Four underwent stenting in a non-operative setting and one as preoperative stabilization. RESULTS Twenty-seven patients survived. One patient died early due to bleeding. Five patients died late: two due to bleeding, one from mediastinitis, and two patients with functional airways died late from unrelated problems. Complications are related to stent type and location. Carinal stents can migrate; several techniques are available to help manage this problem. Wire stents are essentially non-removable requiring periodic dilation. Silastic stents stimulate granulation tissue formation requiring periodic bronchoscopic removal. CONCLUSION Tracheal stenting can aid in the management of pediatric airway problems. Complications are common, but can be managed with appropriate intervention.

Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect

The extant nomenclature for atrioventricular (AV) canal/atrioventricular septal defect is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The three general categories are: partial AV canal (ostium primum defect), transitional (intermediate) AV canal, and complete AV canal. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model: Part 2—Clinical Application

BACKGROUND The empirically derived 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model incorporates adjustment for procedure type and patient-specific factors. The purpose of this report is to describe this model and its application in the assessment of variation in outcomes across centers. METHODS All index cardiac operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (January 1, 2010, to December 31, 2013) were eligible for inclusion. Isolated patent ductus arteriosus closures in patients weighing less than or equal to 2.5 kg were excluded, as were centers with more than 10% missing data and patients with missing data for key variables. The model includes the following covariates: primary procedure, age, any prior cardiovascular operation, any noncardiac abnormality, any chromosomal abnormality or syndrome, important preoperative factors (mechanical circulatory support, shock persisting at time of operation, mechanical ventilation, renal failure requiring dialysis or renal dysfunction (or both), and neurological deficit), any other preoperative factor, prematurity (neonates and infants), and weight (neonates and infants). Variation across centers was assessed. Centers for which the 95% confidence interval for the observed-to-expected mortality ratio does not include unity are identified as lower-performing or higher-performing programs with respect to operative mortality. RESULTS Included were 52,224 operations from 86 centers. Overall discharge mortality was 3.7% (1,931 of 52,224). Discharge mortality by age category was neonates, 10.1% (1,129 of 11,144); infants, 3.0% (564 of 18,554), children, 0.9% (167 of 18,407), and adults, 1.7% (71 of 4,119). For all patients, 12 of 86 centers (14%) were lower-performing programs, 67 (78%) were not outliers, and 7 (8%) were higher-performing programs. CONCLUSIONS The 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model facilitates description of outcomes (mortality) adjusted for procedural and for patient-level factors. Identification of low-performing and high-performing programs may be useful in facilitating quality improvement efforts.

Atrioventricular Septal Defects: Lessons Learned About Patterns of Practice and Outcomes From the Congenital Heart Surgery Database of the Society of Thoracic Surgeons

During the 4-year time interval of 2005 through 2008, the Society of Thoracic Surgeons Congenital Heart Surgery Database documented data about 2882 operations to repair atrioventricular (AV) canal defects: partial, 623 (21.5%); intermediate, 342 (11.8%);. complete, 1917 (66.3%). Mean age at complete repair (years) was as follows: partial, 6.1; intermediate, 2.9; complete, 0.6. Median age at complete repair (years) was as follows: partial, 2.6; intermediate, 0.9; complete, 0.4. Down syndrome was present in 1767 patients (61.1%). Debanding of the pulmonary artery was rarely performed: partial, 1 (0.2%); intermediate, 0 (0.0%); complete, 66 (3.4%). Deep hypothermic circulatory arrest was rarely used: partial, 6 (1.0%); intermediate, 5 (1.5%); complete, 52 (2.7%). Discharge mortality was low: partial, 2 (0.3%); intermediate, 3 (0.9%); complete, 38 (2.0%). Atrioventricular block requiring permanent pacemaker occurred but was uncommon: partial, 6 (1.0%); intermediate, 2 (0.6%); complete, 29 (1.5%). Unplanned reoperation prior to hospital discharge occurred in 3.9% of complete AV canal repairs. The sternum was left open in 3.0% of complete AV canal repairs. Postoperative cardiac arrest occurred in 1.9% of complete AV canal repairs. Mean postoperative length of stay (days) was as follows: partial, 5.2; intermediate, 7; complete, 13.1. Median postoperative length of stay (days) was as follows: partial, 4; intermediate, 4; complete, 7. This review of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that 98% to 99% of patients survive complete repair of AV canal and 96% to 97% survive complete repair of AV canal with no major complications.

Surgical advances in the treatment of adults with congenital heart disease

Purpose of review This article reviews three common abnormalities requiring surgical treatment in adults with congenital heart disease (CHD): Ebsteins anomaly, the failing Fontan, and pulmonary valve disease. Recent findings As of 2000, more adults than children are alive with CHD. Each year, more of these adults with CHD undergo surgery. Three common operations performed on adults with CHD are surgery for Ebsteins anomaly, Fontan revision, and pulmonary valve replacement (PVR). Recent advances have been made in each of these three operations. Summary Surgery for Ebsteins anomaly can now be offered to older patients at low risk and with good late outcome. The operation includes tricuspid valve repair or replacement and frequent concomitant procedures such as atrial septal defect closure, arrhythmia surgery (the maze procedure), and coronary artery bypass grafting. Fontan conversion involves: conversion of the previously created atriopulmonary connection to a total cavopulmonary artery, extracardiac Fontan; arrhythmia surgery, typically with a modified biatrial maze procedure along with placement of an antitachycardia, dual-chamber pacemaker with steroid-eluting epicardial leads in all patients; and concomitant procedures to treat associated lesions. The need for PVR is increasing for many adults with CHD. In the past, residual defects such as chronic pulmonary regurgitation following repair of tetralogy of Fallot were considered benign. Recent evidence suggests that pulmonary regurgitation causes significant morbidity, producing right ventricular dilatation and dysfunction, exercise intolerance, arrhythmias, and sudden death. Multiple options exist for PVR including several recent developments such as PVR with a man-made polytetrafluoroethylene bicuspid valve and percutaneous PVR.

The modern approach to patent ductus arteriosus treatment: complementary roles of video-assisted thoracoscopic surgery and interventional cardiology coil occlusion

BACKGROUND In an effort to analyze our experience and develop treatment guidelines, we reviewed all our patients with patent ductus arteriosus (PDA) treated with video-assisted thoracoscopic surgery (VATS) or interventional cardiology coil occlusion. METHODS One hundred patients underwent 102 cardiac catheterizations. Forty-five children underwent VATS. The entire cohort of patients is 141 because 4 patients underwent both catheterization and VATS. RESULTS Successful PDA coil occlusion occurred in 91 patients (91 of 100; 91%); 8 had unsuccessful attempts at coil occlusion and 1 was referred for surgical ligation after catheterization without any attempt at coil placement. Thirty-nine children had successful VATS PDA closure. Six children required conversion to thoracotomy because of inadequate exposure during VATS. Hospital stay for children more than 45 days of age was as follows: VATS median stay, 1 day, mean, 1.4 days; thoracotomy median stay, 4 days, mean, 4.6 days. One patient treated with PDA coil occlusion developed a recurrent PDA and required reembolization. Three children underwent initial catheterization without successful coil placement with subsequent successful VATS. All VATS patients left the operating theater with echocardiography documenting no residual PDA. Two children who underwent successful VATS with no residual PDA at hospital discharge were found on outpatient follow-up to have developed tiny recurrent PDAs and both were successfully coil occluded; 1 of these 2 children is 1 of the 3 children initially evaluated by catheterization and then referred for VATS. CONCLUSIONS Video-assisted thoracoscopic surgery and coil occlusion represent complementary techniques for PDA treatment. A rationale for selection of the appropriate treatment modality can be based upon the size and age of the patient and the size and morphology of the PDA.

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

OBJECTIVE Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.