Management of pediatric sternal wounds following congenital heart surgery: The role of the plastic surgeon in debridement and closure.

BACKGROUND: Management of sternal wound infections (SWIs) in pediatric patients following congenital heart surgery can be extremely difficult. Patients with congenital cardiac conditions are at risk for complications such as sternal dehiscence, infection, and cardiopulmonary compromise. In this study, we report a single-institution experience with pediatric SWIs. METHODS: Fourteen pediatric patients requiring plastic surgery consultation for complex sternal wound closure were included. A retrospective chart review was performed with the following variables of interest: demographic data, congenital cardiac condition, respective surgical palliations, development of mediastinitis, causative organism, number of debridements, presence of sternal wires, and choice of flap coverage. Primary endpoints included achieved chest wall closure and overall survival. RESULTS: Of the 14 patients, 8 (57%) were diagnosed with culture-positive mediastinitis. The sternum remained wired at the time of final flap closure in eight (57%) patients. All patients were reconstructed with pectoralis major flaps, except one (7%) who also received an omental flap and two (14%) who received superior rectus abdominis flaps. One patient (7%) was treated definitively with negative pressure wound therapy, and one (7%) was too unstable for closure. Six patients developed complications, including one (7%) with persistent mediastinitis, two (14%) with hematoma formation, one (7%) with abscess, and one (7%) with skin necrosis requiring subsequent surgical debridement. There were three (21%) mortalities. CONCLUSIONS: The management of SWI in congenital cardiac patients is challenging. The standard tenets for management of SWI in adults are loosely applicable, but additional considerations must be addressed in this unique subset population.

Late primary transplantation in complex congenital heart disease.

Heart transplantation is a viable option for patients with congenital heart disease who manifest heart failure, predominantly post-surgical palliation. We present a case of complex congenital heart disease, where surgical palliation was considered not to be an option. Considerate medical management with close follow-up allowed for a nine-yr delay until eventual successful heart transplantation. The case also highlights the natural history over nine yr of the effects of right ventricular outflow tract obstruction on the left ventricular remodeling which is analogous to a PA banding in patients with dilated cardiomyopathy.

Superior Mesenteric Arterial Flow Pattern is Associated with Major Adverse Events in Adults with Fontan Circulation.

Factors contributing to the failure of Fontan circulation in adults are poorly understood. Reduced superior mesenteric arterial (SMA) flow has been identified in pediatric Fontan patients with protein-losing enteropathy. SMA flow has not been profiled in an adult Fontan population and its association with adverse events is unknown. We aimed to examine associations between SMA flow patterns and adverse events in adult Fontan patients. We performed a retrospective review of adult Fontan patients who underwent echocardiograms between 2008 and 2014. SMA Doppler data included peak systolic and end-diastolic velocity and velocity time integral (VTI). Systolic/diastolic (S/D) ratio and resistive index were calculated. The relationship between SMA flow parameters and major adverse events (death or transplantation) was examined using proportional hazard Cox regression analyses. Kaplan-Meyer analysis was conducted to construct survival curve of patients with and without adverse events. 91 post-Fontan adult patients (76 % systemic left ventricle, 20 % atriopulmonary Fontan, mean age 27.9 years) were analyzed. Adverse events occurred in nine patients (death = 4, transplant = 5). When compared with the non-event group, the event group had increased end-diastolic velocity [hazard ratio (HR) 1.5, 95 % confidence interval (CI) 1.1-1.8; p = 0.002], increased systolic VTI (HR 1.5, 95 % CI 1.1-2.2, p = 0.02), increased diastolic VTI (HR 1.7, 95 % CI 1.2-2.4, p = 0.004), decreased S/D velocity ratio (HR 0.32, 95 % CI 0.14-0.71, p = 0.006), decreased S/D VTI ratio (HR 0.76, 95 % CI 0.61-0.97, p = 0.02), and decreased resistive index (HR 0.29, 95 % CI 0.14-0.60, p = 0.0007). Increased end-diastolic velocity and VTI in mesenteric arterial flow, with lower systolic/diastolic ratio and resistive index, were associated with death and need for heart transplant in adult Fontan patients. The mesenteric hyperemic flow was also associated with clinical signs of portal venous outflow obstruction, suggesting the presence of vasodilatory state in end-stage adult Fontan circulation.

Outcomes and hospital costs associated with the Norwood operation: beyond morbidity and mortality--CORRIGENDUM.

This paper contains an error in Table 4. The authors apologise for this error. In the last row of the Table, it states: Reimbursement (charges/collections)=46% It should actually be: Reimbursement (collections/ charges)=46%

Outcomes and hospital costs associated with the Norwood operation: beyond morbidity and mortality.

BACKGROUND: Although much is known about morbidity and mortality, there are limited data focussing on the financial aspect of the Norwood operation. Our objective is to characterise the hospitalisation and detail the hospital costs. METHODS: We retrospectively studied 86 newborns with hypoplastic left heart syndrome who underwent Norwood palliation between 2008 and 2012. Clinical and financial data were collected. Financial data have been reported for 2011-2012. RESULTS: At surgery, median age and weight of the patients were 4 days (range 1-13) and 3 kg (range 2-4.8), respectively. The median time from admission to surgery was 4 days (range 1-10), with the postoperative ICU stay and total length of stay at the hospital being 10 days (range 4-135) and 16 days (range 5-136), respectively. Discharge mortality was 14/86 (16%) patients. For patients operated on between 2011 and 2012 (n=40), median hospital costs, charges, and collections per patient were $117,021, $433,054, and $198,453, respectively, and mean hospital costs, charges, and collections per patient were $322,765, $1,109,500, and $511,271, respectively. A breakdown of total hospital costs (direct and indirect) by department showed that the top four areas of resource utilisation (excluding physician fees) were as follows: the cardiac ICU (35%), laboratory (12%), pharmacy (12%), and operating room (7%). Interestingly, point-of-care laboratory evaluations accounted for almost half of the laboratory total (5%). Extracorporeal membrane oxygenation, although only utilised in eight patients between 2011 and 2012, accounted for 7% of utilisation.General radiology only accounted for 2%, despite numerous radiographs. CONCLUSIONS: Limited data are available that detail the hospitalisation and costs associated with the Norwood operation. We hope that this analysis will identify areas for quality and value improvement from both system and patient perspectives.

"Double whammy": anomalous pulmonary and systemic venous drainage in a patient with scimitar syndrome.

Anomalously draining right pulmonary veins are expected with scimitar syndrome, but systemic venous abnormalities are rare. We present an unusual case of a female patient with scimitar and an interrupted inferior vena cava.

Risk factors associated with morbidity and mortality after pulmonary valve replacement in adult patients with previously corrected tetralogy of Fallot.

Patients with palliated tetralogy of Fallot (TOF) often require pulmonary valve replacement in adulthood, yet the data regarding their outcomes are scarce. This study aimed to identify risk factors associated with postoperative complications in these patients and to establish long-term survival data for this patient group. A retrospective cohort study investigated 153 consecutive patients with a history of TOF repair who underwent pulmonary valve replacement at a single large academic center between March 1996 and March 2010. In part 1 of the study, logistic models were constructed to assess demographic, medical, and surgical risk factors for operative mortality; occurrence of a major adverse event (stroke, renal failure, prolonged ventilation, deep sternal infection, reoperation, or operative mortality); and prolonged hospital stay (>7 days). Risk factors with a p value less than 0.10 by univariate analysis were included in the subsequent multivariate analysis. In part 2 of the study, long-term, all-cause mortality was determined by construction of a Kaplan-Meier curve for the cohort. Seven patients died (4.5%). Significant risk factors for mortality in the multivariable analysis included age older than 40 years (odds ratio (OR) 9.89) and concomitant surgery (OR 6.65). A major adverse event occurred for 22 patients (14.4%). The only significant risk factor in the multivariable analysis for an adverse event was concomitant surgery (OR 6.42). The hospital stay was longer than 7 days for 31 patients (20.3%). The significant risk factors for a prolonged hospital stay included the presence of preoperative arrhythmias (OR 4.17), New York Heart Association class 3 (OR 4.35), and again, concomitant surgery (OR 4.2). Among the 146 hospital survivors, only 5 patients died in the intervening period. The predicted survival rates were 98.5% at 1 year, 96.7% at 5 years, and 93.5% at 10 years. Pulmonary valve replacement in adults with palliated TOF is a safe procedure with excellent long-term survival, but there remain important risk factors for postoperative mortality, prolonged hospital stay, and major adverse events. Awareness and modification of important risk factors may help to improve outcomes.

Torrential Mitral Regurgitation After Transcatheter Edge-to-Edge Mitral Valve Repair.

A patient with severe mitral regurgitation and chronic systolic heart failure taking inotropic support at home presents for transcatheter edge-to-edge mitral valve repair, complicated by torrential mitral regurgitation from damaged mitral leaflets requiring escalating mechanical circulatory support and ultimately expedited orthotopic heart transplantation. (Level of Difficulty: Intermediate.).

A novel technique of coronary reconstruction during complex aortic root replacement.

Many modifications of the Bentall technique have been described since its introduction in 1968. The crucial phases of the operation include the re-establishment of coronary flow and control of intraoperative hemorrhage. Based on their experience with Blalock-Taussig shunts, the proximal take-off of which is at the innominate/right subclavian artery junction, the present authors have developed a novel technique of coronary reconstruction. The details of a new technique of coronary reconstruction to minimize the duration of cardiopulmonary bypass, myocardial ischemia and bleeding, are reported.

Care of the adult congenital heart disease patient in the United States: a summary of the current system.

With improvements in care, there has been exponential growth in the population of adult congenital heart disease (ACHD) patients. We sought to assess the availability of specialized ACHD care in the United States. We analyzed the Adult Congenital Heart Association's ACHD clinic directory for information on patient volume, provider training, and other characteristics. The information is self-reported and unverified. The ACHD directory included 72 programs in the United States. Across programs, the majority of patients (33%) seen were between 21 and 30 years old. Program directors had between 2 and 50 years (median 15) of ACHD experience and had dedicated between 10 and 100% (median 30%) of their clinical time to ACHD. There were 2,800 ACHD operations performed per year, ranging from 0 to 230 (median 28) per program. There were between 0 and 5 cardiac surgeons (median 2) involved per program. Each surgeon averaged 20 ACHD operations per year. The growing ACHD population is a largely underserved group. Few programs in the United States provide specialized care, and this care is variably conducted within pediatric and/or adult facilities. These data should serve as a stimulus to improving accessibility of services for this vulnerable population.

Late pulmonary valve replacement in congenital heart disease patients without original congenital pulmonary valve pathology.

Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect (n = 3), complete atrioventricular septal defect (n = 1), and anomalous left coronary artery from the pulmonary artery (PA) (n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 +/- 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease.

Concomitant Aortic Root Replacement With Anterior Konno Annular Enlargement.

The need for concomitant aortic annular enlargement and aortic root replacement may be necessary in situations of multilevel left ventricular outflow tract obstruction and/or prior aortic or aortic valve surgery, particularly in the setting of congenital heart disease. It can be extremely tedious and fraught with numerous complications. Major challenges include bleeding in difficult-to-reach areas and the potential need for subsequent operations. This article describes a technique of concomitant aortic annular enlargement and aortic root replacement that minimizes the risk of significant blood loss and facilitates future surgery should it be necessary.

Impact of Accredited Training Programs in Congenital Heart Surgery.

Historically, training in congenital heart surgery was variable and followed 1 of 3 pathways: residents identified during the primary training process were given the opportunity for a career training position within their own or other institution, residents would travel abroad for training and return to look for a faculty appointment based on their training experience, or a resident would enter a 1-year fellowship position after completing Thoracic Surgery training. These training opportunities and fellowships lacked uniformity and quality control. The purpose of an Accreditation Council of Graduate Medical Education (ACGME)-accredited residency program and an American Board of Thoracic Surgery subspecialty certificate in congenital heart surgery was to recognize and document a standard of education, operative experience, and cognitive knowledge for surgeons practicing in the field. There have been several important papers related to congenital cardiac training published over the years: (1) a manuscript by Kogon published in 2006 outlining congenital cardiac training prior to ACGME program accreditation, (2) a manuscript by Kogon et al published in 2016 outlining congenital cardiac training subsequent to ACGME accreditation with a focus on job transition and early work experience, and (3) a manuscript published in 2017 focusing primarily on job transition and early work experience. The purpose of this review is to provide an update with respect to congenital cardiac training. More importantly, in comparing survey results from these previous papers with the current survey, some interesting trends have been revealed, good and bad.

Force frequency relationship of the human ventricle increases during early postnatal development.

Understanding developmental changes in contractility is critical to improving therapies for young cardiac patients. Isometric developed force was measured in human ventricular muscle strips from two age groups: newborns (<2 wk) and infants (3-14 mo) undergoing repair for congenital heart defects. Muscle strips were paced at several cycle lengths (CLs) to determine the force frequency response (FFR). Changes in Na/Ca exchanger (NCX), sarcoplasmic reticulum Ca-ATPase (SERCA), and phospholamban (PLB) were characterized. At CL 2000 ms, developed force was similar in the two groups. Decreasing CL increased developed force in the infant group to 131 +/- 8% (CL 1000 ms) and 157 +/- 18% (CL 500 ms) demonstrating a positive FFR. The FFR in the newborn group was flat. NCX mRNA and protein levels were significantly larger in the newborn than infant group whereas SERCA levels were unchanged. PLB mRNA levels and PLB/SERCA ratio increased with age. Immunostaining for NCX in isolated newborn cells showed peripheral staining. In infant cells, NCX was also found in T-tubules. SERCA staining was regular and striated in both groups. This study shows for the first time that the newborn human ventricle has a flat FFR, which increases with age and may be caused by developmental changes in calcium handling.

Readmission After Pediatric Cardiothoracic Surgery: An Analysis of The Society of Thoracic Surgeons Database.

BACKGROUND: Hospital readmission after pediatric cardiac surgery is incompletely understood. This study analyzed data from The Society of Thoracic Surgeons Congenital Heart Surgery Database to determine prevalence, to describe patient characteristics, and to evaluate risk factors for readmission. METHODS: Readmission was defined by the "readmission within 30 days after discharge" field. Routine variables were summarized. Regression analysis was used to identify factors associated with readmission. RESULTS: The study cohort included 56,429 patient records from 100 centers. Overall, 6,208 (11%) patients were readmitted. The most common reasons for readmission were respiratory or airway complications (14.2%), septic or infectious complications (11.4%), and reasons not related to the preceding surgical procedure (20.2%). Primary reason for readmission varied across benchmark operation groups. In multivariable analysis, factors associated with increased odds of readmission included the presence of noncardiac abnormalities (odds ratio [OR], 1.24), chromosomal abnormalities or genetic syndromes (OR, 1.24), preoperative mechanical circulatory support (OR, 1.36), other preoperative factors (OR, 1.21), prior cardiac surgery (OR, 1.31), Hispanic ethnicity (OR, 1.13), higher STAT procedural complexity (Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery) (STAT level 3 vs 1, OR, 1.22; STAT 4 vs 1, OR, 1.48; STAT 5 vs 1, OR, 2.62), prolonged postoperative length of stay (OR, 1.07 per day from 0 to 14 days; OR, 1.01 per week >14 days), any major complication (OR, 1.27), any other postoperative complications (OR, 2.00), and discharge on a weekday (OR, 1.07). CONCLUSIONS: Readmission is common after congenital heart surgery, mostly for noncardiovascular reasons. Process improvement initiatives targeted at high-risk patients could minimize its impact.

Unusual Repair of Aortic Coarctation: Transcatheter Intervention Implications.

A 56-year old male was evaluated for exertional dyspnea. He had previously undergone mechanical aortic valve replacement and aortic coarctation repair. Following uncomplicated coronary angiography using Judkins left and right catheters, aortography revealed that the loud murmur was related to an unusual, extra-anatomic surgical repair: transverse aorta to descending aorta bypass. Recognition of an extra-anatomic surgical bypass of coarctation is important, as this repair would leave a continuous murmur on exam.

Adult Congenital Cardiac Care.

BACKGROUND: The Adult Congenital Heart Association (ACHA) is dedicated to supporting patients with congenital heart disease. To guide patients to qualified providers and programs, it maintains a publicly accessible directory of dedicated adult congenital cardiac programs. We analyzed the directory in 2006 and 2015, aiming to evaluate the growth of the directory as a whole and to evaluate the growth of individual programs within the directory. We also hope this raises awareness of the growing opportunities that exist in adult congenital cardiology and cardiac surgery. METHODS: Data in the directory are self-reported. Only data from US programs were collected and analyzed. RESULTS: By the end of 2015, compared to 2006, there were more programs reporting to the directory in more states (107 programs across 42 states vs 57 programs across 33 states), with higher overall clinical volume (591 vs 164 half-day clinics per week, 96,611 vs 34,446 patient visits). On average, each program was busier (5 vs 2 half-day clinics per week per program). Over the time period, the number of reported annual operations performed nearly doubled (4,346 operations by 210 surgeons vs 2,461 operations by 125 surgeons). Access to ancillary services including specific clinical diagnostic and therapeutic services also expanded. CONCLUSION: Between 2006 and 2015, the clinical directory and the individual programs have grown. Current directory data may provide benchmarks for staffing and services for newly emerging and existing programs. Verifying the accuracy of the information and inclusion of all programs will be important in the future.

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

BACKGROUND: In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. METHODS: A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse events (death or heart transplant) were examined using the Cox proportional hazard analysis. Correlations between the hemodynamic measurements and signs of end-organ dysfunction (MELD-XI, Child-Pugh, VAST score, estimated glomerular filtration rate [eGFR]) were analyzed. RESULTS: Sixty post-Fontan patients (85% systemic left ventricle, 40% atriopulmonary Fontan, mean age of 28 years, and mean time since Fontan operation of 21.9 years) were included. At baseline, those with an event were statistically younger, had lower transcutaneous oxygen saturations, were more likely to have an atriopulmonary Fontan, and were more likely to have a pacemaker. Eighteen experienced a cardiovascularly significant event. Using univariate analysis to compare the event and nonevent groups, mean CI was 2.8 ± 0.9 vs. 2.4 ± 0.5 L/min/m2 (P = .004), and CVP was 18.6 ± 6.5 vs. 16.1 ± 4.3 mmHg (P = .03). However, the statistical significances did not persist in the multivariate model. Higher CVP and pulmonary capillary wedge pressure (PCWP) were associated with higher MELD-XI and Child-Pugh scores, and the VAST score was only associated with PCWP. CONCLUSIONS: Symptomatic adult Fontan patients who experienced an event manifested with a higher CI and CVP, although the multivariate Cox proportional hazard analysis did not yield any significant associations. The presences of hepatic dysfunction and portal venous outflow obstruction were associated with a higher CVP and PCWP. Renal dysfunction was prevalent but no statistically significant association between the hemodynamic measurements was identified, although trends toward a higher CVP and transpulmonary gradient were identified.

Age-Dependent Effect of Pediatric Cardiac Progenitor Cells After Juvenile Heart Failure.

UNLABELLED: Children with congenital heart diseases have increased morbidity and mortality, despite various surgical treatments, therefore warranting better treatment strategies. Here we investigate the role of age of human pediatric cardiac progenitor cells (hCPCs) on ventricular remodeling in a model of juvenile heart failure. hCPCs isolated from children undergoing reconstructive surgeries were divided into 3 groups based on age: neonate (1 day to 1 month), infant (1 month to 1 year), and child (1 to 5 years). Adolescent athymic rats were subjected to sham or pulmonary artery banding surgery to generate a model of right ventricular (RV) heart failure. Two weeks after surgery, hCPCs were injected in RV musculature noninvasively. Analysis of cardiac function 4 weeks post-transplantation demonstrated significantly increased tricuspid annular plane systolic excursion and RV ejection fraction and significantly decreased wall thickness and fibrosis in rats transplanted with neonatal hCPCs compared with saline-injected rats. Computational modeling and systems biology analysis were performed on arrays and gave insights into potential mechanisms at the microRNA and gene level. Mechanisms including migration and proliferation assays, as suggested by computational modeling, showed improved chemotactic and proliferative capacity of neonatal hCPCs compared with infant/child hCPCs. In vivo immunostaining further suggested increased recruitment of stem cell antigen 1-positive cells in the right ventricle. This is the first study to assess the role of hCPC age in juvenile RV heart failure. Interestingly, the reparative potential of hCPCs is age-dependent, with neonatal hCPCs exerting the maximum beneficial effect compared with infant and child hCPCs. SIGNIFICANCE: Stem cell therapy for children with congenital heart defects is moving forward, with several completed and ongoing clinical trials. Although there are studies showing how children differ from adults, few focus on the differences among children. This study using human cardiac progenitor cells shows age-related changes in the reparative ability of cells in a model of pediatric heart failure and uses computational and systems biology to elucidate potential mechanisms.

Current Readings: Issues Surrounding Pulmonary Valve Replacement in Repaired Tetralogy of Fallot.

An increasing number of patients with congenital heart disease are surviving into adulthood, and many will require repeat surgical intervention. Those patients with tetralogy of Fallot likely represent the largest group. Of the clinically important complications that affect adults with repaired tetralogy of Fallot, pulmonary regurgitation is the most common. Without intervention, pulmonary regurgitation results in dilation of the right ventricle and may lead to additional complications, such as biventricular dysfunction, arrhythmias, heart failure, and death. Pulmonary valve replacement (PVR) is a means to ameliorate these adverse sequelae and is the most common reoperation performed in adults with congenital heart disease. Although still controversial, much has been written on the indications for and timing of PVR. This article examines some of the less-reported issues surrounding PVR in repaired tetralogy of Fallot, including contemporary surgical outcomes, the role of the percutaneous and mechanical valve, and the role of concomitant aortic and tricuspid operations during PVR.