Paul J. Chai

Management of 239 Patients with Hypoplastic Left Heart Syndrome and Related Malformations from 1993 to 2007

BACKGROUNDnWe reviewed our entire programmatic operative experience with children with hypoplastic left heart syndrome (HLHS) and related malformations.nnnMETHODSnAs of October 1, 2007, 239 patients with HLHS and related malformations underwent surgical treatment at the Congenital Heart Institute of Florida. This manuscript focuses on the 199 initially treated with Norwood stage 1.nnnRESULTSnOne hundred and ninety-nine patients were initially treated with Norwood stage 1. Univariate analysis demonstrated the following significant predictors of mortality: right ventricular dominance (p = 0.0023), mechanical circulatory support before stage 1 (p = 0.0192), and significant noncardiac abnormality or syndrome, including Down syndrome, Turner syndrome, heterotaxy, asplenia, polysplenia, biliary atresia, or other chromosomal abnormality (p < 0.0001). Multivariable logistic regression analysis revealed the presence of a significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support before stage 1 to be a significant predictor of mortality (p < 0.0001). Over the 14 years of this patient series, survival for the 157 low-risk patients managed with Norwood staged palliation (those patients without significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support prior to Stage 1) was 86%, 80%, and 69% at 30 days after Stage 1, hospital discharge after Stage 1, and 1 year of after Stage 1, respectively.nnnCONCLUSIONSnSeveral treatment options are available for HLHS and related malformations. The appropriate treatment strategy must be matched to the individual patient, taking into consideration anatomic variables as well as other patient-specific characteristics. The majority of patients with HLHS and related malformations can undergo successful staged palliation with risk that varies according to several documented anatomic and patient-specific variables.

Mentorship, learning curves, and balance

Professionals working in the arena of health care face a variety of challenges as their careers evolve and develop. In this review, we analyze the role of mentorship, learning curves, and balance in overcoming challenges that all such professionals are likely to encounter. These challenges can exist both in professional and personal life. As any professional involved in health care matures, complex professional skills must be mastered, and new professional skills must be acquired. These skills are both technical and judgmental. In most circumstances, these skills must be learned. In 2007, despite the continued need for obtaining new knowledge and learning new skills, the professional and public tolerance for a learning curve is much less than in previous decades. Mentorship is the key to success in these endeavours. The success of mentorship is two-sided, with responsibilities for both the mentor and the mentee. The benefits of this relationship must be bidirectional. It is the responsibility of both the student and the mentor to assure this bidirectional exchange of benefit. This relationship requires time, patience, dedication, and to some degree selflessness. This mentorship will ultimately be the best tool for mastering complex professional skills and maturing through various learning curves. Professional mentorship also requires that mentors identify and explicitly teach their mentees the relational skills and abilities inherent in learning the management of the triad of self, relationships with others, and professional responsibilities.Up to two decades ago, a learning curve was tolerated, and even expected, while professionals involved in healthcare developed the techniques that allowed for the treatment of previously untreatable diseases. Outcomes have now improved to the point that this type of learning curve is no longer acceptable to the public. Still, professionals must learn to perform and develop independence and confidence. The responsibility to meet this challenge without a painful learning curve belongs to both the younger professionals, who must progress through the learning curve, and the more mature professionals who must create an appropriate environment for learning. In addition to mentorship, the detailed tracking of outcomes is an essential tool for mastering any learning curve. It is crucial to utilize a detailed database to track outcomes, to learn, and to protect both yourself and your patients. It is our professional responsibility to engage in self-evaluation, in part employing voluntary sharing of data. For cardiac surgical subspecialties, the databases now existing for The European Association for CardioThoracic Surgery and The Society of Thoracic Surgeons represent the ideal tool for monitoring outcomes. Evolving initiatives in the fields of paediatric cardiology, paediatric critical care, and paediatric cardiac anaesthesia will play similar roles.A variety of professional and personal challenges must be met by all those working in health care. The acquisition of learned skills, and the use of special tools, will facilitate the process of conquering these challenges. Choosing appropriate role models and mentors can help progression through any learning curve in a controlled and protected fashion. Professional and personal satisfaction are both necessities. Finding the satisfactory balance between work and home life is difficult, but possible with the right tools, organization skills, and support system at work and at home. The concepts of mentorship, learning curves and balance cannot be underappreciated.

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.

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

OBJECTIVEnOrthotopic 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.nnnMETHODSnWe 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.nnnRESULTSnThe 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.nnnCONCLUSIONnCardiac 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.

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.

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 77u2009153 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.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 77u2009153 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.

Surgical pulmonary valve insertion--when, how, and why.

Relief of right ventricular outflow tract obstruction in tetralogy of Fallot or similar physiology often results in pulmonary regurgitation. The resultant chronic volume overload can lead to right ventricular dilatation, biventricular dysfunction, heart failure symptoms, arrhythmias, and sudden death. Although pulmonary valve replacement can lead to improvement in functional class and a substantial decrease or normalisation of right ventricular volumes, the optimal timing of pulmonary valve replacement is not well defined. Benefits of pulmonary valve replacement have to be weighed against the risks of this procedure, including subsequent reoperation. This article will review the benefits and risks of pulmonary valve replacement, options for pulmonary valve substitute, and timing of pulmonary valve replacement in patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction.

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.

Late replacement of the pulmonary valve: when and what type of valve?

patients present in need of reoperative surgical reconstruction of the right ventricular outflow tract. The predominant physiologic lesion is pulmonary insufficiency, but there may also be varying degrees of obstruction of the right ventricular outflow tract. In the past, it has been felt that patients tolerate pulmonary insufficiency reasonably well. In some patients, however, the long-term effects of pulmonary insufficiency and subsequent right ventricular dilation and dysfunction are associated with poor exercise tolerance and increased incidence of arrhythmias and sudden death.1,2 Numerous studies support replacement of the pulmonary valve as treatment for pulmonary insufficiency in order to improve performance, optimize hemodynamics, and better control arrhythmias.3–10 The indications for reconstruction of the right ventricular outflow tract in this setting, nonetheless, as well as the operative strategy, continue to evolve. There are multiple surgical options for replacement of the pulmonary valve for these patients, including aortic and pulmonary homografts, stented and stentless porcine valves, porcine valved conduits, bovine jugular venous conduits, and even mechanical valves and mechanical valved conduits.11–32 It was a less than ideal experience with these currently available options that stimulated our interest into employing alternative materials and techniques. Favorable experimental and clinical experience with valves made of a polytetrafluoroethylene monoleaflet33–36 encouraged us to consider a new method of reconstruction with this material, using a bifoliate polytetrafluoroethylene valve. In this work, we review our indications for replacement of the pulmonary valve after repair of tetralogy of Fallot, the surgical options available, and our experience reconstructing the right ventricular outflow tract with a new surgically created bifoliate polytetrafluoroethylene valve.

Heart Cells with Regenerative Potential from Pediatric Patients with End Stage Heart Failure: A Translatable Method to Enrich and Propagate

Background. Human cardiac-derived progenitor cells (hCPCs) have shown promise in treating heart failure (HF) in adults. The purpose of this study was to describe derivation of hCPCs from pediatric patients with end-stage HF. Methods. At surgery, discarded right atrial tissues (hAA) were obtained from HF patients (n = 25; hAA-CHF). Minced tissues were suspended in complete (serum-containing) DMEM. Cells were selected for their tissue migration and expression of stem cell factor receptor (hc-kit). Characterization of hc-kitpositive cells included immunohistochemical screening with a panel of monoclonal antibodies. Results. Cells, including phase-bright cells identified as hc-kitpositive, spontaneously emigrated from hAA-CHF in suspended explant cultures (SEC) after Day 7. When cocultured with tissue, emigrated hc-kitpositive cells proliferated, first as loosely attached clones and later as multicellular clusters. At Day 21~5% of cells were hc-kitpositive. Between Days 14 and 28 hc-kitpositive cells exhibited mesodermal commitment (GATA-4positive and NKX2.5positive); then after Day 28 cardiac lineages (flk-1positive, smooth muscle actinpositive, troponin-Ipositive, and myosin light chainpositive). Conclusions. C-kitpositive hCPCs can be derived from atrial tissue of pediatric patients with end-stage HF. SEC is a novel culture method for derivation of migratory hc-kitpositive cells that favors clinical translation by reducing the need for exogenously added factors to expand hCPCs in vitro.