Right ventricular remodeling in response to volume overload in fetal sheep.

The fetal myocardium is known to be sensitive to hemodynamic load, responding to systolic overload with cellular hypertrophy, proliferation, and accelerated maturation. However, the fetal cardiac growth response to primary volume overload is unknown. We hypothesized that increased venous return would stimulate fetal cardiomyocyte proliferation and terminal differentiation, particularly in the right ventricle (RV). Vascular catheters and pulmonary artery flow probes were implanted in 16 late-gestation fetal sheep: a right carotid artery-jugular vein (AV) fistula was surgically created in nine fetuses, and sham operations were performed on seven fetuses. Instrumented fetuses were studied for 1 wk before hearts were dissected for component analysis or cardiomyocyte dispersion for cellular measurements. Within 1 day of AV fistula creation, RV output was 20% higher in experimental than sham fetuses ( P < 0.0001). Circulating atrial natriuretic peptide levels were elevated fivefold in fetuses with an AV fistula ( P < 0.002). On the terminal day, RV-to-body weight ratios were 35% higher in the AV fistula group ( P < 0.05). Both left ventricular and RV cardiomyocytes grew longer in fetuses with an AV fistula ( P < 0.02). Cell cycle activity was depressed by >50% [significant in left ventricle ( P < 0.02), but not RV ( P < 0.054)]. Rates of terminal differentiation were unchanged. Based on these studies, we speculate that atrial natriuretic peptide suppressed fetal cardiomyocyte cell cycle activity. Unlike systolic overload, fetal diastolic load appears to drive myocyte enlargement, but not cardiomyocyte proliferation or maturation. These changes could predispose to RV dysfunction later in life. NEW & NOTEWORTHY Adaptation of the fetal heart to changes in cardiac load allows the fetus to maintain adequate blood flow to its systemic and placental circulations, which is necessary for the well-being of the fetus. Addition of arterial-venous fistula flow to existing venous return increased right ventricular stroke volume and output. The fetal heart compensated by cardiomyocyte elongation without accelerated cellular maturation, while cardiomyocyte proliferation decreased. Even transient volume overload in utero alters myocardial structure and cardiomyocyte endowment.

Prenatal Diagnosis Influences Preoperative Status in Neonates with Congenital Heart Disease: An Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

The early postnatal course for a newborn with critical congenital heart disease (CHD) can be negatively impacted if diagnosis is delayed. Despite this, there continues to be inconsistent evidence regarding potential benefits associated with prenatal diagnosis (PND) in neonates who undergo cardiac surgery. The objective of this study was to better define the impact of a PND on pre-operative morbidity by utilizing a large clinical database. Neonates (< 30 days) undergoing heart surgery from 2010 to 2014 and entered in the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were included. Multivariable logistic regression was used to evaluate the association between PND and a composite measure including nine major pre-operative risk factors. Co-variates were included to adjust for important patient characteristics (e.g., weight-for-age z-score, genetic syndromes, prematurity), case complexity, and center effects. Centers and patients with excess missing data for relevant co-variates were excluded. Included were 12,899 neonates undergoing surgery at 112 centers. Major pre-operative risk factors were present in 34% overall. By univariate analysis, PND was associated with a lower overall prevalence of major pre-operative risk factors. After adjusting for potential confounders, major pre-operative risk factors were less prevalent among neonates with PND compared to neonates without PND (adjusted OR 0.62, 95% CI 0.57-0.68, p < 0.001). A sensitivity analysis excluding neonates with genetic syndromes, non-cardiac anatomic abnormalities, and prematurity demonstrated similar findings (adjusted OR 0.55, 95% CI 0.49-0.61, p < 0.0001). Among neonates with CHD, prenatal diagnosis is associated with significantly lower rates of pre-operative risk factors for cardiac surgery. Further studies are needed to define association of these pre-operative benefits of a PND with longer term clinical outcomes.

Timing of adding blood to prime affects inflammatory response to neonatal cardiopulmonary bypass.

Complications from systemic inflammation are reported in neonates following exposure to cardiopulmonary bypass. Although the use of asanguinous primes can reduce these complications, in neonates, this can result in significant haemodilution, requiring addition of blood. This study investigates whether the addition of blood after institution of bypass alters the inflammatory response compared with a blood prime. Neonatal swine were randomised into four groups: blood prime, blood after bypass but before cooling, blood after cooling but before low flow, and blood after re-warming. All groups were placed on central bypass, cooled, underwent low flow, and then re-warmed for a total bypass time of 2 hours. Although haematocrit values between groups varied throughout bypass, all groups ended with a similar value. Although they spent time with a lower haematocrit, asanguinous prime groups did not have elevated lactate levels at the end of bypass compared with blood prime. Asanguinous primes released less tumour necrosis factor α than blood primes (p=0.023). Asanguinous primes with blood added on bypass produced less interleukin 10 and tumour necrosis factor α (p=0.006, 0.019). Animals receiving blood while cool also showed less interleukin 10 and tumour necrosis factor α production than those that received blood warm (p=0.026, 0.033). Asanguinous primes exhibited less oedema than blood primes, with the least body weight gain noted in the end cool group (p=0.011). This study suggests that using an asanguinous prime for neonates being cooled to deep hypothermia is practical, and the later addition of blood reduces inflammation.

The Impact of Aortic Valve Replacement on Left Ventricular Remodeling in Children.

There are scant data in pediatrics on the optimal timing for aortic valve repair (AVR). This study assesses the midterm response to AVR and possible predictors of poor outcome. From 2001 to 2006, 41 patients had greater than 3-month follow-up after AVR for aortic insufficiency, aortic stenosis, or both. Pre-, peri-, and post-operative data were collected, including demographics and clinical symptoms. Two reviewers measured echocardiographic parameters from the pre-operative and latest follow-up echocardiograms. Ventricular dimensions were indexed to body surface area (z-score). Median age at AVR was 13 years with 83 % having a Ross operation. The average left ventricular end-diastolic dimension pre-op, z-score of +1.3, significantly decreased at last follow-up to a mean z-score of -0.1 (p < 0.001). Similarly the indexed LV mass decreased from +3.9 to +0.5 (p < 0.001). There was no significant correlation between the presence of pre-op symptoms and the presence of post-op LV dilatation, hypertrophy, or dysfunction. In the subset of patients (7/41) with persistent LV dysfunction at last follow-up, there was a significant correlation with pre-op LV dilatation as assessed by both LVEDD (p = 0.02) and LVESD (p = 0.05). Children demonstrate significant reverse remodeling after AVR. Pre-op LV dilatation may predict patients with persistent LV dysfunction post-AVR. Symptoms are less useful in children, suggesting the need for more objective data for functional assessment.

Development of chylous ascites in an infant with ventricular septal defect: a case report.

This case report describes chylous ascites associated with a CHD in a 4-month-old infant. Although atraumatic chylous ascites are a rare clinical finding, the recognition and treatment of chylous ascites influence the timing of cardiac surgery.

A modification of the pulmonary autograft procedure to prevent late autograft dilatation.

Although the pulmonary autograft procedure for aortic valve replacement is a commonly utilized option for children, its use is diminishing in adult-aged patients. One commonly cited concern is the tendency for the pulmonary autograft to dilate in the aortic position. This article reviews a technique we have used in 36 patients since October, 2004 that stabilizes the autograft so that it cannot dilate. There have been no operative or late deaths and the autograft has continued to function in 34 patients. Two patients have undergone autograft replacement because of early failure, which we believe was likely related to technical considerations in our early technique (first reported in the 2005 STCVS Pediatric Cardiac Surgery Annual). The technical modifications described in this article have produced a more reliable and reproducible technique and have not resulted in any autograft failures in our experience. One patient with Marfan's syndrome and a bicuspid aortic valve is symptom- and dilation-free 8 years post op, with no autograft or pulmonary homograft insufficiency, normal activity and a stable aortic root by serial echocardiography. Our results suggest that this technique might be applicable for selected adult patients in whom autograft growth is not necessary and for whom the risk of autograft dilatation would provide a reason to avoid a pulmonary autograft procedure.

Personal Relationships and Well-being for Cardiothoracic Surgeons.

This article summarizes relevant research on relational psychology and interpersonal neurobiology and how it applies to cardiothoracic surgeons, their partners and their children. It also provides a synopsis of data retrieved from a well-being survey of cardiothoracic surgeons in the AATS, as well as a separate survey of the well-being experiences of their significant others. Additionally, the article makes recommendations for improving the well-being of cardiothoracic surgeons, as well as their relationships with their partners and children.

Assessment of the well-being of significant others of cardiothoracic surgeons.

OBJECTIVES: We aimed to evaluate how the current working climate of cardiothoracic surgery and burnout experienced by cardiothoracic surgeons influences their spouses and significant others (SOs). METHODS: A 33-question well-being survey was developed by the American Association for Thoracic Surgery Wellness Committee and distributed by e-mail to the SOs of cardiothoracic surgeons and to all surgeon registrants of the 2020 and 2021 American Association for Thoracic Surgery Annual Meetings with a request to share it with their SO. The 5-item Likert-scale survey questions were dichotomized, and associations were determined by χ2 or independent samples t tests, as appropriate. RESULTS: Responses from 238 SOs were analyzed. Sixty-six percent reported that the stress on their cardiothoracic surgeon partner had a moderate to severe influence on their family, and 63% reported that their partner's work demands didn't leave enough time for family. Fifty-one percent reported that their partner rarely had time for intimacy, 27% reported poor work-life balance, and 23% reported that interactions at home were usually or always not good-natured. SOs were most affected when their partner was <5 years out from training, worked in private vs academic practice, and worked longer hours. Having children, particularly younger than age 19 years, and a lack of workplace support resources further diminished well-being. CONCLUSIONS: The current work culture of cardiothoracic surgeons adversely affects their SOs, and the risk for families is concerning. These data present a major area for exploration as we strive to understand and mitigate the factors that lead to burnout among cardiothoracic surgeons.

Anomalous left coronary artery from the pulmonary arteryin an adult.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that usually presents before the age of 1 year. Several surgical options exist for the correction of ALCAPA; however, debate continues regarding the optimal repair technique in adult populations. We report the case of successful surgical repair of ALCAPA with a direct aortic implantation technique in a 44-year-old mother of 4 children.

Short- and Mid-Term Autograft Outcomes Following the Reinforced Ross Operation.

BACKGROUND: The Ross operation for aortic valve replacement continues to be a controversial option because of concerns related to late autograft dilation and progressive neo-aortic insufficiency. In 2005, the reinforced Ross procedure was described at our institution to address this problem. We aim to analyze the short and mid-term outcomes following this procedure. METHODS: This is a retrospective study of patients who underwent the reinforced Ross operation between 2004 and 2019. A comprehensive chart review was performed. Echocardiograms were independently reviewed by an adult congenital cardiologist. The time to reintervention was evaluated with a Kaplan-Meier curve. Analysis was conducted in JMP 15.1 (SAS Inc., Cary, NC). RESULTS: Twenty-five patients underwent the reinforced Ross operation. Twenty-three patients (92%) had bicuspid aortic valve and the most common indication for surgery was a combination of aortic insufficiency and stenosis (n = 18, 72%). The mean follow-up was 6.1 ± 5.0 years. All patients were alive at the time of follow-up. Six patients (24%), from early in our experience, required subsequent aortic reintervention. Median time to reintervention was 41.8 months (0-81.5 months). Sixteen (64%) patients had less than moderate aortic insufficiency at last follow-up. Additionally, average aortic root measurements remained unchanged. CONCLUSIONS: The reinforced Ross technique was initially proposed as a way to mitigate aortic root dilation seen in the traditional Ross procedure. Our experience suggests an associated learning curve with the majority of aortic reinterventions occurring within the first few years following surgery. Continued follow-up is warranted to assess its long-term durability and functionality.

Well-being of Cardiothoracic Surgeons in the Time of COVID-19: A Survey by the Wellness Committee of the American Association for Thoracic Surgery.

The prevalence of burnout among physicians has been increasing over the last decade, but data on burnout in the specialty of cardiothoracic surgery are lacking. We aimed to study this topic through a well-being survey. A 54-question well-being survey was developed by the Wellness Committee of the American Association for Thoracic Surgery (AATS) and sent by email from January through March of 2021 to AATS members and participants of the 2021 annual meeting. The 5-item Likert-scale survey questions were dichotomized, and associations were determined by Chi-square tests or independent samples t-tests, as appropriate. The results from 871 respondents (17% women) were analyzed. Many respondents reported at least moderately experiencing: 1) a sense of dread coming to work (50%), 2) physical exhaustion at work (58%), 3) a lack of enthusiasm at work (46%), and 4) emotional exhaustion at work (50%). Most respondents (70%) felt that burnout affected their personal relationships at least "some of the time," and many (43%) experienced a great deal of work-related stress. Importantly, most respondents (62%) reported little to no access to workplace resources for emotional support, but those who reported access reported less burnout. Most respondents (57%) felt that the COVID-19 pandemic has negatively affected their well-being. On a positive note, 80% felt their career was fulfilling and enjoyed their day-to-day job at least "most of the time." Cardiothoracic surgeons experience high levels of burnout, similar to that of other medical professionals. Interventions aimed at mitigating burnout in this profession are discussed.

Pentalogy of Cantrell with double-outlet right ventricle: a case of surgical correction.

Pentalogy of Cantrell is characterised by a combination of severe defects in the middle of the chest including the sternum, diaphragm, heart, and abdominal wall. Mortality rate after cardiac surgery is usually high. We report a successful total correction of the cardiac defects in a case of Pentalogy of Cantrell with a double-outlet right ventricle prior to abdominal wall defect repair.

Human innate Mycobacterium tuberculosis-reactive alphabetaTCR+ thymocytes.

The control of Mycobacterium tuberculosis (Mtb) infection is heavily dependent on the adaptive Th1 cellular immune response. Paradoxically, optimal priming of the Th1 response requires activation of priming dendritic cells with Th1 cytokine IFN-gamma. At present, the innate cellular mechanisms required for the generation of an optimal Th1 T cell response remain poorly characterized. We hypothesized that innate Mtb-reactive T cells provide an early source of IFN-gamma to fully activate Mtb-exposed dendritic cells. Here, we report the identification of a novel population of Mtb-reactive CD4(-) alphabetaTCR(+) innate thymocytes. These cells are present at high frequencies, respond to Mtb-infected cells by producing IFN-gamma directly ex vivo, and display characteristics of effector memory T cells. This novel innate population of Mtb-reactive T cells will drive further investigation into the role of these cells in the containment of Mtb following infectious exposure. Furthermore, this is the first demonstration of a human innate pathogen-specific alphabetaTCR(+) T cell and is likely to inspire further investigation into innate T cells recognizing other important human pathogens.

Valve-sparing replacement of the aortic root for a 2-year-old child with Loeys-Dietz syndrome.

Loeys-Dietz syndrome is a newly recognized constellation that presents with aortic aneurysm or dissection similar to Marfan's syndrome. We describe successful surgical treatment in a 2-year-old with the syndrome in whom we performed a valve-sparing replacement of the aortic root because of significant dilation of the aortic root and the ascending aorta.

National practice patterns for management of adult congenital heart disease: operation by pediatric heart surgeons decreases in-hospital death.

BACKGROUND: Surgery for grown-up (age > or = 18 years) patients with congenital heart disease (GUCH) is frequently performed by surgeons without specialization in pediatric heart surgery. We sought to define national practice patterns and to determine whether outcomes for GUCH patients are improved if they are treated by specialized pediatric heart surgeons (PHSs) compared with non-PHSs. METHODS AND RESULTS: We identified index cardiac procedures in patients with 12 congenital heart disease diagnostic groups using the Nationwide Inpatient Sample 1988 to 2003. PHSs were defined as surgeons whose annual practice volumes were made of >75% annual pediatric heart cases. GUCH operations were defined as operations within these 12 diagnoses occurring in patients > or =18 years of age. We identified 30,250 operations, yielding a national estimate of 152,277 +/- 7,875 operations. Of these, 111,816 +/- 7,456 (73%) were pediatric operations, and 40,461 +/- 1,365 (27%) were GUCH operations. PHSs performed 68% of pediatric operations in all diagnostic groups, whereas non-PHSs performed 95% of GUCH operations within the same diagnostic groups (P<0.0001). In-hospital death rates for GUCH patients operated on by PHSs were lower than death rates for GUCH patients operated on by non-PHSs (1.87% [95% CI, 0.62 to 3.13] versus 4.84% [95% CI, 4.30 to 5.38%]; P<0.0001). Survival advantage increased with increasing surgeon annual pediatric volume (P=0.0031). CONCLUSIONS: Pediatric patients within specific diagnostic groups are more likely to undergo operation by PHSs, whereas GUCH patients within the same diagnostic groups are more likely to undergo operation by non-PHSs. In-hospital death rates are lower for GUCH patients operated on by PHSs. GUCH patients should be encouraged to obtain surgical operation by PHS.

Whole Brain Leadership for Creating Resonant Multidisciplinary Health Care Teams.

The culture of health care creates important challenges for health care professionals. In particular, we work in a culture that is (1) hierarchical, (2) competitive, and (3) perfectionistic. Unfortunately, the consequence of acquiescing to those demands is contrary to promoting Resonant teamwork, and it is important for leaders of multidisciplinary teams to understand how to create environments that flatten the hierarchy (by encouraging all members of the team to contribute; and to genuinely seek the wisdom and knowledge of their colleagues), that encourage collaboration and cooperation (emphasizing collective wins and losses both for the immediate team as well as for all of us, as a profession), and that invites excellence (which is a process) versus expectation of perfection (which is an unrealistic outcome).

Temporal Trends in Medical Student Burnout.

BACKGROUND AND OBJECTIVES: There is a paucity of longitudinal data documenting the temporal development of distress and burnout during medical school. The aim of this study was to examine trends and identify stressors associated with medical student distress over 4 years of medical education. METHODS: Medical students from the class of 2016 at a Liaison Committee on Medical Education-accredited medical school completed surveys nine times from orientation through after the residency match. Surveys included demographic variables and measured distress domains using the Medical Student Well-Being Index. The authors used Microsoft Excel to calculate the proportion of students screening positive for individual distress domains at each of the nine acquisition periods for descriptive analysis. RESULTS: Students completed 886 total surveys for an 85% response rate, which was relatively consistent across collection periods. Medical student distress and burnout increased from two (2%) to 12 (12%) respondents and from 19 (17%) to 37 (38%) respondents, respectively, from matriculation through after the residency match (P<0.01). Depersonalization increased from 15 (13%) to 34 (35%) respondents and emotional exhaustion increased from six (5%) to 22 (22%) respondents across 4 years of medical education (P<0.01). Emotional exhaustion peaked after medical school year 1, at 37 (45%), and year 3, at 45 (44%) respondents, with improvement after summer break and residency match. CONCLUSIONS: The results supported the literature demonstrating the development of burnout during medical school. Depersonalization increased early in the education process with minimal regression after development. Emotional exhaustion demonstrated a surprising increase after exposure to clinical clerkships. Further studies could support or refute the universality of these trends and evaluate prevention and intervention efforts targeting these key inflection points.

The improvement of care for paediatric and congenital cardiac disease across the World: a challenge for the World Society for Pediatric and Congenital Heart Surgery.

The diagnosis and treatment for paediatric and congenital cardiac disease has undergone remarkable progress over the last 60 years. Unfortunately, this progress has been largely limited to the developed world. Yet every year approximately 90% of the more than 1,000,000 children who are born with congenital cardiac disease across the world receive either suboptimal care or are totally denied care.While in the developed world the focus has changed from an effort to decrease post-operative mortality to now improving quality of life and decreasing morbidity, which is the focus of this Supplement, the rest of the world still needs to develop basic access to congenital cardiac care. The World Society for Pediatric and Congenital Heart Surgery [http://www.wspchs.org/] was established in 2006. The Vision of the World Society is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The Mission of the World Society is to promote the highest quality comprehensive care to all patients with pediatric and/or congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with emphasis on excellence in education, research and community service.We present in this article an overview of the epidemiology of congenital cardiac disease, the current and future challenges to improve care in the developed and developing world, the impact of the globalization of cardiac surgery, and the role that the World Society should play. The World Society for Pediatric and Congenital Heart Surgery is in a unique position to influence and truly improve the global care of children and adults with congenital cardiac disease throughout the world [http://www.wspchs.org/].

Medium-Term Outcomes After Implantation of Expanded Polytetrafluoroethylene Valved Conduit.

BACKGROUND: Pulmonary valve replacement is becoming the most frequent congenital heart surgery performed on adolescents and young adults. Numerous surgical options are available, including autologous pericardium, mechanical valves, allografts, and bioprosthetic valves. Each option has limitations with durability and freedom from reintervention for stenosis or insufficiency, particularly in the pediatric population. The purpose of this study was to analyze our uniquely designed, expanded polytetrafluoroethylene (ePTFE) valved conduit (VC) for right ventricular outflow tract reconstruction. METHODS: Beginning in 2012, ePTFE VC were implanted in 26 patients with a median age of 9.8 years (range, 1.0 to 15.9). Bicuspid VC were used in the first 3 patients, and tricuspid VC were used in 23 patients. The ePTFE VC is fashioned from commercially available ePTFE tube graft (16 mm in 1 patient, 20 mm in 7 patients, 24 mm in 18 patients) and 0.1 mm thick ePTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. RESULTS: There were no postoperative hospital deaths. Mean follow-up was 2.6 years (range, 4 months to 4.7 years), and there have been no reoperations for the ePTFE VC. There was one reintervention for stenosis at the distal anastomosis. Pulmonary insufficiency was mild or less in 24 patients (92%). The average peak instantaneous pressure gradient between the right ventricle and the pulmonary artery was 22.4 ± 15.1 mm Hg at latest follow-up. CONCLUSIONS: Compared with historical data for other pulmonary valve replacement options, our ePTFE VC shows superior medium-term performance, with less reintervention or significant valve dysfunction.