Better Late Than Never: Definitive Anatomic Repair of Dextro-Transposition of the Great Arteries.

We present a case of anatomic repair of dextro-transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) in a 55-year-old man who presented with acute heart failure. This case highlights the importance of multimodal imaging and multidisciplinary involvement in developing a comprehensive surgical and medical plan for adults with congenital heart disease. We think this is the oldest reported patient undergoing anatomic surgical repair of d-TGA with VSD.

Risk Factors for Perioperative Morbidity in Adults Undergoing Cardiac Surgery at Children's Hospitals.

BACKGROUND: Increasing numbers of adults with congenital heart disease (ACHD) undergo cardiac surgical procedures in children's hospitals, yet surgical outcomes data are limited. We sought to better understand the impact of preoperative risk factors on postoperative complications and cardiac intensive care unit (CICU) length of stay (LOS). METHODS: Surgical CICU admissions for patients aged 18 years and older in the Pediatric Cardiac Critical Care Consortium registry from August 2014 to January 2019 in 34 hospitals were included. Primary outcomes included prolonged LOS (defined as LOS ≥90th percentile) and major complications (cardiac arrest, extracorporeal membrane oxygenation, arrhythmia requiring intervention, stroke, renal replacement therapy, infection, and reoperation/reintervention). RESULTS: We analyzed 1764 surgical CICU admissions. Prolonged LOS was 7 days or longer. Eighteen patients (1.0%) died, of whom 9 (0.5%) died before the LOS cutoff and were excluded from analysis. Of 1755 CICU admissions, 8.8% (n = 156) had prolonged LOS, and 23.3% (n = 413) had 1 or more major complications. Several variables, including The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery 4/5 operation, 3 or more previous sternotomies, and preoperative renal dysfunction/dialysis were independent risk factors for both prolonged LOS and major complications (P < .05). Preoperative ventilation was associated with increased odds of prolonged LOS and preoperative arrhythmia with major complications. CONCLUSIONS: This analysis of postoperative ACHD care in pediatric CICUs found high complexity operations, 3 or more previous sternotomies, preoperative arrhythmias, renal dysfunction, and respiratory failure are associated with prolonged LOS and/or major complications. Future quality improvement initiatives focused on preoperative optimization and implementation of adult-specific perioperative protocols may mitigate morbidity in these patients undergoing cardiac surgical procedures at children's hospitals.

Improvement in Protein-Losing Enteropathy in a Patient With Fontan Circulation After Cardiac Rehabilitation and Prescriptive Exercise.

Fontan-associated protein-losing enteropathy is difficult to treat and associated with poor prognosis. Cardiac rehabilitation and exercise are thought to have beneficial effects for patients with Fontan circulation. We report the case of a young adult patient palliated to Fontan circulation, with a decade-long history of symptoms related to protein-losing enteropathy. At age 23 years, he appreciated an improvement in symptoms and laboratory values after cardiac rehabilitation and prescriptive exercise.

Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort.

BACKGROUND: The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. METHODS: All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). RESULTS: The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. CONCLUSIONS: Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.

The adult Fontan patient: update for 2011.

Congenital heart disease is the most common birth defect, with an estimated incidence of moderate to severe disease of 4-6 per 1,000 live births. Due to the dramatic advances in cardiac surgery and general pediatric cardiology care, approximately 85% of neonates with congenital heart disease (CHD) survive to adulthood. The most recent information, published in 2004, estimated 787,800 to 1.3 million adults with CHD living in the United States. This is expected to increase by 5% per year. It is estimated that within the next decade, 1 in every 150 young adults will have some form of CHD. DUe to the changing therapeutic options, a higher percentage of the adult CHD population will consist of more complicated cardiac disease during the coming years. It is estimated that 1-2% of children with CHD have single-ventricle physiology. Thus, as survival in this complex portion of the CHD population improves, the number of adult patients with previous Fontan palliation will dramatically increase. The goal of this article is to provide a brief background of the Fontan procedure and then discuss the late-term outcomes and complications in this unique patient type. The majority of the article will focus on information needed to adequately care for the adult Fontan patient.

Cardiothoracic surgery resident education: update on resident recruitment and job placement.

BACKGROUND: Applications to cardiothoracic surgery training programs have steadily declined. The application cycle for 2004 marked the first time the number of applicants was lower than the positions offered. This survey reflects on this trend in applications and the perspectives of current and graduating residents. METHODS: In June 2004, the Thoracic Surgery Residents Association, in conjunction with CTSNet, surveyed residents completing accredited cardiothoracic training or additional subspecialization. Participation was anonymous and voluntary. RESULTS: Of the 140 graduates, 88 responded. Most were male (92%) and married (72%). Their average age was 35.7 years, and 56% had children. The mean educational debt was less than $50,000. Of the 88 respondents, 69 (78%) had plans to seek jobs whereas 15 (17%) sought additional training. Among job-seeking residents, 12% received no offers. Also, 59% of graduates initially sought a position in academics and 41% in private practice. Nearly one quarter (23%) reported that they would not choose a career in cardiothoracic surgery again, and more than half (52%) would not strongly recommend cardiothoracic surgery to potential trainees. Almost all (90%) of the graduates believed that the number of cardiothoracic training spots should be decreased, and 92% believed that a reduction in training positions should be achieved by closing marginal training programs. Additionally, 91% believed reimbursement for cardiothoracic surgery was inadequate, and 88% thought low reimbursement resulted in restricted patient access and decreased quality of care. CONCLUSIONS: Cardiothoracic training programs are having difficulty in both applicant recruitment and in suitable job placement. This frustration in the job search coupled with reimbursement and lifestyle issues most likely contributes to the general dissatisfaction conveyed by the graduates. If these trends continue, the field will be faced with a crisis of unfilled residency programs and unemployed graduates.

Seven hundred fifty-three consecutive deaths in a level I trauma center: the argument for injury prevention.

BACKGROUND: The past century has seen improvement in trauma care, with a resulting decrease in therapeutically preventable deaths. We hypothesize that further major reduction in injury mortality will be obtained through injury prevention, rather than improvements in therapy. METHODS: Seven hundred fifty-three deaths in an American College of Surgeons-verified, Level I trauma center were reviewed as they occurred. Deaths were classified as therapeutically not preventable, possibly preventable, or preventable. These charts were also reviewed for factors that might have prevented or lessened the severity of the injury. RESULTS: Mean age was 43, mean Glasgow Coma Scale score was 5, mean Revised Trauma Score was 4, mean Injury Severity Score was 41, and mean probability of survival was 0.25 (according to TRISS). Forty-six percent underwent cardiopulmonary resuscitation in the field, 52% died within 12 hours, 74% died within 48 hours, and 86% died within 7 days. Primary causes of death included central nervous system injury in 51%, irreversible shock in 21%, multiple injuries (shock plus central nervous system injury) in 9%, multiple organ failure/sepsis and other causes in 3%, and pulmonary embolus in 0.1%. Seven hundred one (93%) were classified as not preventable with a change in therapy, 32 (4.2%) were classified as potentially preventable with a change in therapy, and 20 were classified as preventable with a change in therapy (2.6%). Forty-six percent had cardiopulmonary resuscitation performed before or immediately on arrival to the hospital. Another 23% had vital signs present on arrival, but had a Glasgow Coma Scale score of