Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease.

BACKGROUND: This manuscript reviews all patients who underwent orthotopic heart transplantations (OHT) at our program (116 patients underwent 119 OHT) to describe their diagnostic characteristics and to assess risk factors for mortality. METHODS: Median age at OHT was 179 days (mean, 1,446.6 ± 188.9 days [4.0 ± 0.5 years]; range, 5 days to 7,125 days [19.5 years]; 15 neonates, 68 infants). Median weight at OHT was 5.5 kg (mean, 17.2 ± 2.1 kg; range, 2.2 to 113 kg). Diagnoses were cardiomyopathy (n = 37), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 29), transplantation after prior cardiac surgery for HLHS or HLHS-related malformation (n = 9), non-HLHS congenital heart disease (n = 39), and retransplant (n = 5). RESULTS: Overall Kaplan-Meier 5-year survival was 72.7%. Operative mortality was 12.6% (15 patients). Late mortality was 13.4% (16 patients). Eighty-five patients survived, with a mean follow-up of 5.76 ± 0.48 years (median, 5.1 years; range, 0.12 to 14.0 years). Total follow-up was 507.0 years. No survival difference was seen among the five diagnostic subgroups (p = 0.20). Univariate association between risk factors and survival was assessed for the following variables: age (p = 0.91), weight (p = 0.86), sex (p = 0.47), race (p = 0.40), insurance classification (p = 0.42), high PRA (p = 0.20), pretransplant mechanical circulatory support (p < 0.001), posttransplant mechanical circulatory support (p < 0.001), redo sternotomy (p = 0.07), heterotaxy (p = 0.02), cardiopulmonary bypass time (p = 0.01), and donor heart cross-clamp time (p = 0.02). CONCLUSIONS: Excellent results are expected for children undergoing OHT regardless of diagnostic classification. Pretransplant mechanical circulatory support, posttransplant mechanical circulatory support, cardiopulmonary bypass time, donor heart cross-clamp time, and heterotaxy are risk factors for decreased survival.

Transposition of the great arteries: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons.

The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database contains data about 3258 patients with the diagnosis of transposition of the great arteries (TGA) who underwent surgery during the 4-year time interval from July 1, 2005 to June 30, 2009, inclusive. This cohort includes 2918 patients with concordant atrioventricular connections and discordant ventriculoarterial connections and 341 patients with congenitally corrected TGA (discordant atrioventricular connections and discordant ventriculoarterial connections). The 4 most common operations were the following: (1) arterial switch operation (ASO) for TGA with intact ventricular septum (n = 1196), (2) ASO with ventricular septal defect (VSD) repair for TGA with VSD (n = 420), (3) ASO with VSD repair and aortic arch repair for TGA with VSD and hypoplastic arch (n = 55), and (4) Rastelli operation for TGA with VSD and left ventricular outflow tract obstruction (n = 49). Detailed preoperative, intraoperative, and postoperative data were obtained about patients who underwent these 4 operations. Median age at surgery (days) was as follows: ASO: 6.0; ASO with VSD repair: 7.0; ASO with VSD repair and aortic arch repair: 7.0; and Rastelli: 309.0. Mean age at surgery (days) was as follows: ASO: 22.9; ASO with VSD repair: 24.8; ASO with VSD repair and aortic arch repair: 14.4; and Rastelli: 721.8. Discharge mortality was as follows: ASO: 2.2%; ASO with VSD repair: 5.5%; ASO with VSD repair and aortic arch repair: 7.3%; and Rastelli: 0%. Median length of stay (days) was as follows: ASO: 11.0; ASO with VSD repair: 11.0; ASO with VSD repair and aortic arch repair: 18.0; and Rastelli: 7.0. The sternum was left open in the following: ASO: 24.8%; ASO with VSD repair: 29.5%; ASO with VSD repair and aortic arch repair: 40.0%; and Rastelli: 6.1%. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that although surgery for TGA is often complex and may be associated with morbidity, most patients survive without major complications.

Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons.

According to The International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD), "Heterotaxy is synonymous with 'visceral heterotaxy' and 'heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as 'situs solitus', or patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus'." or patients with complete mirror-image arrangement of the internal organs along the left-right axis, also known as situs inversus. The purpose of this article is to review the data about heterotaxy in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. The investigators examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 to December 31, 2009, inclusive. This analysis resulted in a cohort of 77 153 total index operations. Of these, 1505 operations (1.95%) were performed in patients with heterotaxy. Of the 1505 index operations performed in patients with heterotaxy, 1144 were in patients with asplenia and 361 were in patients with polysplenia. In every STS -EACTS Congenital Heart Surgery Mortality Category, discharge mortality is higher in patients with heterotaxy compared with patients without heterotaxy (EACTS = European Association for Cardio-Thoracic Surgery). Discharge mortality after systemic to pulmonary artery shunt is 6.6% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 10.8% in single-ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 4.2% in single-ventricle patients with heterotaxy. The STS Congenital Heart Surgery Database is largest congenital heart surgery database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.

Congenital heart surgery databases around the world: do we need a global database?

The question posed in the title of this article is: "Congenital Heart Surgery Databases Around the World: Do We Need a Global Database?" The answer to this question is "Yes and No"! Yes--we need to create a global database to track the outcomes of patients with pediatric and congenital heart disease. No--we do not need to create a new "global database." Instead, we need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This review article will achieve the following objectives: (A) Consider the current state of analysis of outcomes of treatments for patients with congenitally malformed hearts. (B) Present some principles that might make it possible to achieve life-long longitudinal monitoring and follow-up. (C) Describe the rationale for the creation of a Global Federated Multispecialty Congenital Heart Disease Database. (D) Propose a methodology for the creation of a Global Federated Multispecialty Congenital Heart Disease Database that is based on linking together currently existing databases without creating a new database. To perform meaningful multi-institutional analyses, any database must incorporate the following six essential elements: (1) Use of a common language and nomenclature. (2) Use of a database with an established uniform core dataset for collection of information. (3) Incorporation of a mechanism to evaluate the complexity of cases. (4) Implementation of a mechanism to assure and verify the completeness and accuracy of the data collected. (5) Collaboration between medical and surgical subspecialties. (6) Standardization of protocols for life-long longitudinal follow-up. Analysis of outcomes must move beyond recording 30-day or hospital mortality, and encompass longer-term follow-up, including cardiac and non-cardiac morbidities, and importantly, those morbidities impacting health-related quality of life. Methodologies must be implemented in our databases to allow uniform, protocol-driven, and meaningful long-term follow-up. We need to create a platform that allows for the linkage of currently existing continental subspecialty databases (and continental subspecialty databases that might be created in the future) that will allow for the seamless sharing of multi-institutional longitudinal data across temporal, geographical, and subspecialty boundaries. This "Global Federated Multispecialty Congenital Heart Disease Database" will not be a new database, but will be a platform that effortlessly links multiple databases and maintains the integrity of these extant databases. Description of outcomes requires true multi-disciplinary involvement, and should include surgeons, cardiologists, anesthesiologists, intensivists, perfusionists, neurologists, educators, primary care physicians, nurses, and physical therapists. Outcomes should determine primary therapy, and as such must be monitored life-long. The relatively small numbers of patients with congenitally malformed hearts requires multi-institutional cooperation to accomplish these goals. The creation of a Global Federated Multispecialty Congenital Heart Disease Database that links extant databases from pediatric cardiology, pediatric cardiac surgery, pediatric cardiac anesthesia, and pediatric critical care will create a platform for improving patient care, research, and teaching related to patients with congenital and pediatric cardiac disease.

Polytetrafluoroethylene bicuspid pulmonary valve implantation: experience with 126 patients.

This article reports our initial experience in 126 consecutive patients treated with placement of a surgically created polytetrafluoroethylene (PTFE) bicuspid pulmonary valve at The Congenital Heart Institute of Florida (CHIF). A bicuspid pulmonary valve is created with PTFE and sutured into the right ventricular outflow tract. PTFE bicuspid pulmonary valves were placed in 126 patients (age: range, 3.1-64.7 years, mean, 17.9 years; weight: range, 14.2-113.6 kg, mean, 55.4 kg). All patients had pulmonary insufficiency, pulmonary stenosis, or both, most commonly after previous repair of tetralogy of Fallot (71 patients). Follow-up was up to 8.3 years (range, 0-8.3 years, mean, 3.34 years). Operative mortality was 1 patient (0.8%). Late mortality was non-valve-related in 3 patients (2.4%). The initial 84 patents in this series received valves constructed from PTFE with 0.6-mm thickness. The next 42 patients received valves constructed from PTFE with 0.1-mm thickness. Six patients of 126 (4.8%) required replacement of the PTFE bicuspid pulmonary valve because of immobile and calcified leaflets. All 6 who required replacement of the PTFE bicuspid pulmonary valve initially received a valve constructed from porous 0.6-mm PTFE material. We currently use nonporous 0.1-mm PTFE, which does not allow cellular in-growth and thickening. Early echocardiographic follow-up of these valve leaflets made with 0.1-mm PTFE has demonstrated improved leaflet mobility and pliability and lower transvalvar gradients. PTFE bicuspid pulmonary valve implantation is safe and effective and demonstrates acceptable performance for the intermediate term. It is anticipated that using thinner 0.1-mm PTFE will improve valve function and durability. Long-term follow-up is necessary to determine the true value of this technique.

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.

Successful reconstruction of traumatic carinal tissue loss using the esophagus in an infant.

We present a case report of an infant who underwent successful reconstruction of a traumatic tracheal and carinal chemically induced corrosive injury using an esophageal flap to reconstruct the trachea and subsequently re-establishing gastrointestinal continuity with a colon interposition.

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.