Jeffrey P. Jacobs

Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease: A multi-institutional study

BACKGROUNDnAlthough heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined.nnnMETHODSnWe used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras.nnnRESULTSnThe cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.15-2.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.59-4.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups.nnnCONCLUSIONnUse of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.

Development and implementation of a new data registry in congenital cardiac anesthesia.

A new multisite registry for congenital cardiac anesthesia patients has now been incorporated into The Society of Thoracic Surgeons Congenital Heart Surgery Database. This new registry, The Joint Congenital Cardiac Anesthesia Society-Society of Thoracic Surgeons Congenital Cardiac Anesthesia Database, is part of the Congenital Cardiac Anesthesia Societys commitment to patient care and research on outcomes improvement. This report will review the planning and funding of the initial start-up as well as the data elements being used in the registry. Patients in the registry include not only cardiac surgical patients but also those with congenital heart disease undergoing procedures in locations other than the operating room, including in the cardiac catheterization laboratory, intensive care unit, general operating room, and radiology suite. Initial results from the first data harvests are reported, including site participation, patient population submitted, and adverse outcomes observed. These initial results validate the concept and serve as a benchmark for further development and implementation of the registry. Because of the relative infrequency of anesthesia-related events in this low-volume procedure, a multisite data harvest is the most reasonable approach to capture a sufficient number of patient encounters in a timely manner to support outcomes analysis, quality assessment, and quality improvement.

Major Infection After Pediatric Cardiac Surgery: External Validation of Risk Estimation Model

BACKGROUNDnA multivariable risk estimation model, in which the primary outcome was major infection, was recently developed and published using The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. We have applied this risk estimation model to our congenital heart surgery program over a 16-year time interval to validate this risk estimation model and verify its specific risk factors for major infection.nnnMETHODSnUsing complete and verified data, we selected patients in whom major procedures had been classified using both Aristotle Basic Score and Risk Adjustment for Congenital Heart Surgery (RACHS-1) and created a multivariable model in which primary outcome was major infection (septicemia, mediastinitis, or endocarditis). We checked the STS risk estimation model for major infection. We also assessed the significance of the STS risk factors in our program.nnnRESULTSnA total of 6,314 patients were analyzed. We identified 197 (3.1%) major infections (septicemia 3%, endocarditis 0.015%, mediastinitis 0.09%). Hospital mortality, ventilation time, and length of stay were greater in patients with major infections. The following preoperative risk factors identified by the STS risk estimation model were significant in multivariate analysis in our patients: young age, high complexity, medium complexity, previous operation, and preoperative ventilation (p<0.0001). Estimated infection risk ranged from 0.32% to 11.58%. The model discrimination was good (c index, 0.808). Risks of infections after most common congenital heart surgery procedures were similar in both studies (rs=0.952, p=0.0003).nnnCONCLUSIONSnOur external validation study confirmed that the STS model can be used as a preoperative risk stratification tool for major infection risk at the single institutional level.

Fontan's operation: evolution from a procedure to a process.

It was in 1971, Fontan and Baudet 1 published their experience using a surgical procedure to restore a physiologic circulation in three patients with tricuspid atresia. Of the three patients, two survived, thus ushering a new era in the treatment of children with complex forms of congenital cardiac disease. This operation itself, however, was the culmination of a series of experimental and clinical observations that underlined the clinical possibility totally to bypass the right side of the heart.

Acute myocardial infarction in a 15-year old secondary to myxomatous embolisation

We present a 15-year-old white male who presented with an acute myocardial infarction secondary to embolisation of tissue from a left atrial myxoma. He successfully underwent resection of the myxoma, and embolectomy of the fragments of tumour lodged in the coronary artery.

Electronic Supplement of The Inaugural Meeting of The World Society for Pediatric and Congenital Heart Surgery

THE WORLD SOCIETY FOR PEDIATRIC AND Congenital Heart Surgery will hold its inaugural meeting on Thursday, May 3, and Friday, May 4, 2007 in Washington, District of Columbia, hosted by Richard A. Jonas. Christo I. Tchervenkov, of Montreal, Canada, is the founding President of this newly formed society. The remaining officers are shown in Table 1. As of April 19, 2007, the World Society has 423 Founding Members from 66 countries (Appendix 1). The Founding Members include 392 members who have joined the World Society and 31 special Honorary Founding Members (Appendix 2). Registration for the inaugural meeting stands at 302 registrants representing 61 countries. The meeting features a postgraduate course over the first day, devoted to tetralogy of Fallot, with the scientific session for the second day including 18 oral presentations. The presentations were selected from 90 abstracts submitted for consideration for presentation at the scientific session. In this electronic Supplement to Cardiology in the Young, we include the text of the 18 abstracts selected for oral presentation, as well as the 22 most outstanding presentations selected to be displayed as posters. The World Society takes its origin from the highly successful first joint meeting of the Congenital Heart Surgeons’ Society and the European Congenital Heart Surgeons Association, held in Montréal, Canada, through October 2 and 4 2004.1 This meeting was organized by Christo I. Tchervenkov during the Centennial celebrations of the Montreal Children’s Hospital, and was dedicated to the memory of John W. Kirklin, a remarkable pioneer of cardiac surgery who had died earlier in 2004. During this 3-day event, two genuine Canadian pioneers of cardiac surgery were also honoured, namely Anthony R.C. Dobell from Montréal, and George A. Trusler from Toronto, both long-time friends of John Kirklin. The joint meeting was attended by a total of 127 physicians, made up of 57 members of the Congenital Heart Electronic Supplement of The Inaugural Meeting of The World Society for Pediatric and Congenital Heart Surgery