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Children with single-ventricle (SV) heart disease possess a spectrum of heart malformations, yet progress through similar hemodynamic states, suggesting differences in outcomes are related to fundamental morphologic differences, patient characteristics, or procedural pathways. We sought to provide a holistic overview of survival after intervention for SV heart disease at our institution.SV heart disease was defined as patients born with a hypoplastic or dysfunctional ventricle with uncertain or unacceptable candidacy for a 2-ventricle circulation. Patients were stratified into 8 diagnostic groups and 11 procedural categories based on the initial interventional procedure.Between 2005 and 2020, 381 patients born with SV heart disease underwent intervention at our institution. Ten-year survival was highest for patients with double inlet left ventricle (89% ± 7%) and lowest for patients with hypoplastic left heart syndrome (55% ± 5%). Initial palliation with less invasive procedures, such as ductal stent (4-year: 100%) or pulmonary artery banding (10-year: 95% ± 5%), demonstrated superior survival compared with more invasive procedures such as the Norwood procedure (10-year: 59% ± 4%). Survival of patients who achieved a biventricular circulation was superior to patients who remained with SV physiology (10-year: 87% ± 5% vs 63% ± 3%, P = .04). In a multivariable analysis, chromosomal/syndromic abnormality, lower weight, hybrid Norwood procedure, nonleft ventricular dominance, and earlier year of operation were risk factors for death.Survival differences in patients with SV heart disease were related primarily to underlying cardiac anatomy, patient characteristics, and procedural complexity. Left ventricular dominance, more recent intervention, and attainment of a 2-ventricle circulation were associated with improved survival.

Background: The optimal timing of surgical repair for infants with complete atrioventricular canal defect remains controversial, as there are risks to both early and late repair. We address this debate by investigating the association of various risk factors, including age and weight at surgery, markers of failure to thrive, and pulmonary vascular disease, with postoperative length of stay following complete atrioventricular canal repair. Methods: Infants who underwent repair of complete atrioventricular canal were identified from our institutional Society of Thoracic Surgeons Congenital Heart Surgery Database. Additional clinical data were collected from the electronic medical record. Descriptive statistics were computed. Associations between postoperative length of stay and covariates of interest were evaluated using linear regression with bootstrap aggregation. Results: From 2001 to 2020, 150 infants underwent isolated complete atrioventricular canal repair at our institution. Pre-operative failure to thrive and evidence of pulmonary disease were common. Surgical mortality was 2%. In univariable analysis, neither weight nor age at surgery were associated with mortality, postoperative length of stay, duration of mechanical ventilation, or post-operative severe valvular regurgitation. In multivariable analysis of demographic and preoperative clinical factors using bootstrap aggregation, increased postoperative length of stay was only significantly associated with previous pulmonary artery banding (33.9 day increase, p = 0.03) and preoperative use of supplemental oxygen (19.9 day increase, p = 0.03). Conclusions: Our analysis shows that previous pulmonary artery banding and preoperative use of supplemental oxygen were associated with increased postoperative length of stay after complete atrioventricular canal repair, whereas age and weight were not. These findings suggest operation prior to the onset of pulmonary involvement may be more important than reaching age or weight thresholds.

The Single Ventricle Reconstruction Trial demonstrated increased risk of death or heart transplant 1 year after Norwood among subjects randomly assigned to modified Blalock-Taussig shunts compared with right ventricle to pulmonary artery (RV-PA) shunts. We used the Single Ventricle Reconstruction Trial public use database to evaluate incidence and risk factors for postoperative renal failure and relationships between renal failure, shunt type, and outcomes after Norwood.Postoperative renal failure was defined a priori as a threefold rise in creatinine from baseline, or dialysis use, within 7 days of Norwood. We used multivariate logistic regression to evaluate risk factors for postoperative renal failure and Cox hazard regression to determine the association between postoperative renal failure and 1 year after Norwood mortality.Overall, postoperative renal failure occurred in 8.4% (46 of 544) patients with risk factors including receipt of a modified Blalock-Taussig shunt (adjusted odds ratio [aOR] 3.3, P = .02), low center volume (aOR 2.7, P = .005), two or more preoperative complications (aOR 4.0, P.001), low birth weight (aOR 3.2, P = .002), postoperative heart block (aOR 8.5, P = .001), and delayed sternal closure (aOR 5.3, P = .026). Renal failure was an independent risk factor for 1-year mortality (aHR 1.9, P = .019). Assessing interaction by shunt type, mortality risk associated with renal failure was greatest in the RV-PA shunt group (aHR 3.3 vs RV-PA shunt without renal failure, P = .001), but was also increased in the modified Blalock-Taussig shunt group (aHR 1.9, P = .03).Postoperative renal failure is common after Norwood and is independently associated with mortality. Although renal failure is more common after modified Blalock-Taussig shunt, the highest mortality risk with renal failure occurs after RV-PA shunt.

The long-term impact of ventricular dominance on Fontan outcomes is controversial. We examined this in a 25-year cohort.Patients from October 1998 to February 2022 were reviewed. Primary outcomes were transplant-free survival and Fontan failure (death, transplantation, takedown, protein-losing enteropathy, or plastic bronchitis). Secondary outcomes included hospital and intensive care length of stay. Kaplan-Meier methodology compared outcomes by ventricular dominance. Multiphase parametric risk hazard analysis identified risk factors for primary outcomes.There were 195 patients (104 right ventricular dominant). Baseline characteristics were comparable. Perioperative survival was similar (right ventricular dominant 98%, non-right ventricular dominant 100%, p=0.51). The proportion of patients experiencing death or transplantation was 8.7% and Fontan failure was 11.8% during a median follow-up of 4.5 [0.3 - 9.8] years. Right ventricular dominant patients had reduced transplant-free survival (10-year estimates: 80% (95% CI 70-91%) vs. 92% (83-100%), p=.04) and freedom from Fontan failure (73% (62-86%) vs. 92% (83-100%), p=.04). Multiphase hazard modeling resolved 2 risk phases. The early phase spanned from surgery to approximately 6 months after. The late phase spanned from approximately 6 months after surgery onwards. In multivariable analysis, right ventricular dominance was an independent risk factor for death or transplantation (parameter estimate 1.3±0.6, p=0.04) and Fontan failure (1.1±0.5, p=0.04) during the second phase, with no significant first phase risk factors.Right ventricular dominance was associated with long-term complications after Fontan, including mortality, transplantation, and Fontan failure. This cohort may benefit from heightened surveillance in a multi-disciplinary Fontan clinic after the perioperative period.

We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.

Cardiac hemangiomas are a rare tumor traditionally resected by median sternotomy. We performed a minimally invasive right ventricular cardiac hemangioma resection via a left anterior mini-incision (LAMI). The procedure was without complication, and the patient was discharged on postoperative day 2. The LAMI has been used broadly by our team for operations involving the right ventricular outflow tract, as an alternative to median sternotomy. Here we show that it can also be used for the resection of a cardiac tumor.

Abnormal atrioventricular valve present great challenges to the surgeon in achieving a successful repair, and thus present a great opportunity for enhanced 3D imaging to guide pre- and intra-operative management. Spatial and temporal resolution of 3D echocardiography enables 3D printing of valve morphology. However, non-linearity, angle dependence, speckle, blur, and resampling complicate segmentation compared to computed tomography (CT) and magnetic resonance imaging (MRI). A case of complex mitral valve disease in a pediatric patient is therefore presented to illustrate the technical challenges of segmentation and 3D printing from echocardiographic data.

Objective The benefits of minimally invasive adult cardiac surgery are well established. Nevertheless, minimally invasive congenital cardiac procedures, even for adult patients, are uncommon. In 2018, we started repairing anomalous aortic origin of a coronary artery (AAOCA) through a 5 cm anterior minithoracotomy when possible to improve cosmesis and avoid sternal precautions. We hypothesized this approach was safe and reliable. Methods A 5 cm incision was made in the right second intercostal space. The incision was carried down to the pericardium while preserving the internal mammary artery. With the pericardium in view, the second and third ribs were disarticulated. Central cardiopulmonary bypass was established, and the repair was carried out based on the patient’s anatomy. The technique was modified to a left anterior minithoracotomy for 1 patient who required pulmonary artery translocation. At any point, if the dissection or repair was not progressing appropriately, the minimally invasive exposure was converted to a partial or traditional median sternotomy. Results Between June 2018 and June 2019, 11 patients underwent minimally invasive anomalous coronary repair. Four patients (3 with body mass index >30) were converted to traditional sternotomy due to poor visualization. Postoperatively, 1 patient required coronary artery bypass after 335 days, due to extensive collaterals and stable angina. Otherwise, at a median follow-up of 437 days (IQR 340 to 480), patients had resumed baseline activity without recurrent symptoms. Conclusions Minimally invasive AAOCA repair may be appealing, although surgeons should be cautious given the high conversion rate.

Aims The HeartMate 3 (HM3) ventricular assist device (VAD) is gaining popularity in adults due to a favorable risk profile. However, reports of HM3 use in children are limited, potentially due to concerns with device size. Materials and methods Here we report the successful use of an HM3-VAD as a bridge to transplantation in a 21 kg (BSA 0.84), an 8-year-old male child with Fontan failure on home inotropes. Results Urgent VAD implantation was performed. The standard adult sewing ring was used. The tricuspid valve and papillary muscles were completely excised from the ventricular cavity to prevent inflow obstruction. The pump was placed in the left pleural space. Outflow graft and driveline implantation were routine. VAD function appeared excellent with a reduction in Fontan pressures and improved kidney and liver function. Reoperation was required once to rule out tamponade and twice to evacuate a recurrent right hemothorax. The patient was discharged 3 months later in good condition and underwent successful heart transplantation 10 months after VAD placement. Discussion This report demonstrates the feasibility of HM3-VAD implantation in a 21-kg Fontan patient, suggesting HM3 size is not a prohibitive limitation at this weight.

Objective Marfan syndrome (MFS) represents a genetic disorder with a range of clinical features, including proximal aortic aneurysms. Extensive research has revealed an abundance of transforming growth factor beta from a mutation in fibrillin-1 to be the key biochemical mechanism of aneurysm formation. Many important signaling pathways downstream of transforming growth factor beta have been further characterized. Our laboratory has previously demonstrated a unique murine model of MFS resulting in the accelerated formation of ascending aortic aneurysms and dilated cardiomyopathies. This study aims to characterize the relevance of this model to known signaling mechanisms in MFS. Methods Fibrillin 1C1039G/+ heterozygous mice (ie, MFS), with a mutation in fibrillin-1, were supplemented with 4.5 mg/kg/d angiotensin II to accelerate aneurysm formation. Four mouse groups were analyzed: wild type with or without angiotensin II and MFS with or without angiotensin II. Aortic tissue from these samples were subjected to western blotting and phosphoimaging to query various signaling pathways. Results Mice with MFS displayed downstream regulation in both the canonical (Smad2) and noncononical (extracellular signal-regulated kinases and P38) pathways characteristic of MFS. However, these downstream signals were exaggerated in the MFS mice supplemented with angiotensin II (accelerated model), matching the observed phenotypic severity of this model. Conclusions The murine MFS model depicted here accelerates ascending aortic aneurysm formation and cardiomyopathies via well-characterized MFS signaling cascades. The mechanistic relevance of the accelerated murine MFS model suggests that it could be an important tool in future studies hoping to characterize MFS signaling in an expedited experimental design.

The majority of congenital and adult cardiac surgery is performed through a median sternotomy. For surgeons, this incision provides excellent exposure; however, for patients, a median sternotomy confers a poorer cosmetic outcome and the possibility of postoperative respiratory dysfunction, chronic pain, and deep sternal wound infections. Despite the advances in adult cardiac surgery, the use of minimally invasive techniques in pediatric patients is largely limited to small case series and less complex repairs. In this article, we review the risks, benefits, and limitations of the minimally invasive congenital cardiac approaches being performed today. The interest in these approaches continues to grow as more data supporting reduced morbidity, decreased length of stay, and faster recovery are published. In the future, as the technology and surgical familiarity improve, these alternative approaches will become more common, and may someday become the standard of care.

OBJECTIVE: To examine the association between digoxin use and cardiac function assessed by echocardiographic indices in infants with single ventricle congenital heart disease during the interstage period. DESIGN: Retrospective cohort study. SETTING: Fifteen North American hospitals. PATIENTS: Infants discharged home following stage 1 palliation (S1P) and prior to stage 2 palliation (S2P). Infants with no post-S1P and pre-S2P echocardiograms were excluded. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 373 eligible infants who met inclusion criteria, 140 (37.5%) were discharged home on digoxin. In multivariable linear and logistic regression, we found that compared to infants discharged home without digoxin, those discharged with digoxin had a smaller increase in end-systolic volume (β=−8.17 [95% CI: −15.59, −0.74], p=0.03) and area (β=−1.27 [−2.45, −0.09], p=0.04), as well as a smaller decrease in ejection fraction (β=3.38 [0.47, 6.29], p=0.02) and fractional area change (β=2.27 [0.14, 4.41], p=0.04) during the interstage period. CONCLUSIONS: Digoxin may partially mitigate the expected decrease in cardiac function during the interstage period through its positive inotropic effects. Prospective clinical trials are needed to establish the pharmacokinetics, safety, and efficacy of digoxin use in SV CHD.

Value-based bundles require surgeons to understand their costs. Current approaches to cost reporting are confusing and difficult to reproduce. Using the Epic surgical receipt function, we describe an intuitive and systematic approach for evaluating financial data within the operating room.We conducted a retrospective review of all congenital cardiac procedures performed at a single academic medical center between January 1, 2020, and January 1, 2021. Direct operating room supply costs were obtained using the Epic surgical receipt function. Costs were analyzed on the basis of contribution to total annual cost and variability in case cost. Implications for strategies identified within congenital cardiac surgery were then evaluated in adult cardiac surgery.Five procedures representing 71 patients accounted for more than 50% of the total direct operating room supply costs (left ventricular assist device, Norwood procedure, pulmonary valve replacement, right ventricle-to-pulmonary artery shunt, and aortic arch augmentation). Disposable vascular clips, suture brand preference, and surgical patch materials accounted for 3.7%, 6.6%, and 26.5% of annual direct operating room supply costs, respectively. Improvements to these categories would represent 12% to 14% ($250 000) in annual savings without an anticipated effect on outcomes. Across adult and congenital cardiac surgery, 95% of all name-brand suture use was tied to preference cards. An opt-in vs default approach to name-brand polypropylene suture could save more than $250 000 annually.The surgical receipt represents a reliable and intuitive way for reporting surgical costs. Systematically analyzing costs and their impact on outcomes will help surgeons improve the value of care they provide.

We developed a technique for the Norwood operation utilizing continuous perfusion of the head, heart, and lower body at mild hypothermia named Sustained Total All-Region (STAR) perfusion. We hypothesized that STAR perfusion would be associated with shorter operative times, decreased coagulopathy, and expedited post-operative recovery compared to standard perfusion techniques. Between 2012 and 2020, 80 infants underwent primary Norwood reconstruction at our institution. Outcomes for patients who received successful STAR perfusion (STAR, n = 37) were compared to those who received standard Norwood reconstruction utilizing regional cerebral perfusion only (SNR, n = 33), as well as to Norwood patients reported in the PC

Objective:Pulmonary insufficiency requiring reintervention frequently occurs after primary tetralogy of Fallot repair. Repeat interventions present a challenge for both the surgeon and patient. We compare a minimally invasive, 5 cm left anterior mini-incision to redo median sternotomy for pulmonary valve replacement in tetralogy of Fallot patients.Methods:Following Internal Review Board approval, we conducted a single institution retrospective review of patients with tetralogy of Fallot who underwent pulmonary valve replacement via redo median sternotomy or left anterior mini-incision between 13 July, 2016 and 6 March, 2020.Results:Twenty-three patients underwent pulmonary valve replacement following primary tetralogy of Fallot repair between March 2016 and March 2020. Twelve patients received a redo-median sternotomy from March 2016 to August 2018. Left anterior mini-incision was first offered in August of 2018 and was chosen by all eleven patients thereafter. The two groups had similar baseline characteristics including preoperative pulmonary valve dysfunction. Early trends suggest a longer cardiopulmonary bypass time for patients who received left anterior mini-incisions. Other outcomes were comparable, including operative times, blood product requirements, residual pulmonary valve dysfunction, postoperative pain, narcotic requirements, ICU length of stay, total length of stay, and postoperative complications.Conclusions:In patients who have previously undergone primary repairs of tetralogy of Fallot, outcomes for pulmonary valve replacement via left anterior mini-incision are comparable to those via redo median sternotomy.

Purpose: Healthcare spending within the United States increased 4.6% in 2019 and represented over $3.8 trillion, or 17.7% of our GDP. The operating margin for most hospitals is 1-3%. As costs continue to rise, insurers will continue to shift these financial risks and responsibilities onto hospitals and hospitals eventually onto surgeons. Methods: Using a recently introduced surgical receipt function within Epic, we set out to develop cost containment strategies within congenital cardiac surgery at Duke University Hospital. Surgical receipt data was obtained between January 1, 2020 and January 1, 2021. Variability in case costs and product utilization were identified. Findings were discussed with surgeons to identify high impact changes with low barriers to implementation. Results: Between January 1, 2020 and January 1, 2021, there were 340 index cases and 449 unique surgical receipts. Four procedures representing 54 patients accounted for more than 50% of the total annual direct operating room supply costs (LVAD, 5 patients, 22.7% of annual costs; Norwood, 20 patients, 12.7%; RV-PA shunt, 16 patients, 9.9%; PVR, 13 patients, 9.4%). Exploration chest for postoperative hemorrhage was associated with the highest variability in case cost. In addition to mechanical devices and valves, cost drivers included the use of disposable vascular clips, suture brand preference between surgeons, and surgical patch material utilization which accounted for 3.7%, 6.6%, and 26.5% of overall annual direct operating room supply costs. Conclusions: We project 6-6.5% in annual savings due to changes in suture and surgical clip utilization. Changes to surgical patch utilization should carry an additional 4-4.5% in savings. The surgical receipt represents a reliable and uniform way of reporting surgical costs with broader implications for all surgical subspecialties.

We present the case of a newborn with complex congenital heart disease who was treated with a neonatal Norwood operation and total anomalous pulmonary venous return repair. During the Norwood reconstruction, a novel technique was utilized to perfuse the head, lower body, and heart continuously to minimize ischemic injury.

The acute phase of recovery following the Norwood procedure can be complicated by considerable instability due in part to bleeding, acute kidney injury, systemic inflammatory response, and low cardiac function. The traditional conduct of cardiopulmonary bypass with cooling, re-warming, myocardial ischemia, and upper or lower body ischemia likely exacerbates these problems. Total body perfusion during Norwood reconstruction, without significant cooling or ischemic time to any organs, may improve postoperative recovery by reducing coagulopathy and end-organ injury. Here, we introduce a novel technique for sustained total all-region perfusion of the heart, upper body, and lower body throughout the Norwood operation.

Background: The use of systemic-to-pulmonary shunts (SPS) in neonates with single ventricle heart defects and ductal-dependent pulmonary blood flow (ddPBF) was historically associated with high morbidity and mortality at our center. As a result, we transitioned to the preferential use of ductus arteriosus stents (DS) when feasible. This report describes our initial results with this strategy. Methods: A single-center study of single ventricle patients that received DS or SPS from 2015 to 2019 was performed to assess whether DS was associated with decreased in-hospital morbidity and increased survival to stage II palliation. Results: A total of 34 patients were included (DS = 11; SPS = 23). Underlying cardiac anomalies were similar between groups and included pulmonary atresia, unbalanced atrioventricular septal defect, and tricuspid atresia. Procedure success was similar between groups (82% vs 83%). Two DS patients were converted to SPS, due to ductal vasospasm or pulmonary artery obstruction, and four SPS patients required surgical shunt revision. In DS patients, postprocedure mechanical ventilation duration was shorter (one vs three days, P = .009) and fewer required postprocedure extracorporeal membrane oxygenation (9% vs 39%, P = .11). A higher proportion of DS patients survived to stage II palliation (100% vs 64%, P = .035), and the probability of one-year survival was higher in DS patients (100% vs 61%, P = .02). Conclusions: At our center, patients with single ventricle heart defects and ddPBF that received DS experienced reduced in-hospital morbidity and increased survival to stage II palliation compared to SPS.

Introduction: We developed a technique for Stage 1 “Norwood” reconstruction with continuous perfusion of the head, heart, and lower body at mild hypothermia, deemed Sustained Total All-Region (STAR) perfusion. Hypothesis: We hypothesized that STAR perfusion would shorten cardiopulmonary bypass and procedural times and expedite post-operative recovery, due to lack of cooling and re-warming and decreased total body ischemia. Methods: Between 2012-2019, 65 infants underwent Norwood reconstruction at our institution. Those who underwent prior pulmonary artery banding were excluded, yielding a cohort of 51 infants who underwent primary Norwood reconstruction. Outcomes for patients who underwent Norwood reconstruction using STAR perfusion (n=21) were compared to those who underwent Norwood reconstruction with conventional techniques using regional cerebral perfusion only (n=30). STAR perfusion was performed with innominate artery cannulation as well as olive tip cannulas inserted into the opened descending aorta and native aortic root to provide continuous perfusion of the head, heart, and lower body throughout the procedure (Figure), under mild hypothermia (32-34°C). Results: Norwood reconstruction with STAR perfusion was associated with shorter median CPB times vs. conventional perfusion techniques (161 vs. 226 mins,p Conclusions: Norwood reconstruction with STAR perfusion is a safe technique that eliminates ischemic time to the heart and lower body and allows for the procedure to be performed at warmer temperatures. We found evidence of reduced CPB and operative times, as well as improved hemostasis, end-organ function, and post-operative recovery with this technique.

Introduction: The Single Ventricle Reconstruction (SVR) trial randomized neonates with a single morphologic right ventricle undergoing Norwood to a modified Blalock-Taussig shunt (mBTS) or a right ventricle-to-pulmonary artery shunt at 15 centers. The trial demonstrated increased risk of death or heart transplant at one-year post-Norwood in subjects randomized to a mBTS. Using SVR public data, we evaluated incidence and risk factors for post-op renal failure after Norwood, and evaluated the relationship between renal failure, shunt type and one-year post-Norwood mortality. Methods: Post-op renal failure was defined a-priori as a 3-fold rise in creatinine from baseline, or dialysis use within 7 days of Norwood. We used multivariate logistic regression to evaluate risk factors for post-op renal failure and Cox hazard regression to determine the association between post-op renal failure and one-year post-Norwood mortality. Results: Overall 46/544 (8.4%) SVR trial subjects developed post-op renal failure, the majority (32/46, 69.6%) by post-op day 3. In multivariate analysis, risk factors for post-op renal failure included receipt of a mBTS (aOR 3.3, p=0.02), center volume Conclusions: In the SVR trial, post-op renal failure was relatively common but with modifiable risk factors including receipt of a mBTS. Our findings suggest that post-op renal failure accounts for some of the increased mortality risk associated with the mBTS.

The development of biventricular repair and conversion pathways for patients with borderline hypoplastic heart disease represents an area of recent inquiry and innovation. This review summarizes emerging techniques and novel treatment algorithms for borderline hypoplastic heart disease with a focus on surgical advances within the last 10 years. Many patients with borderline hypoplastic heart disease are amenable to primary biventricular repair, or biventricular conversion following single-ventricle palliation coupled with ventricular rehabilitation strategies. New insights into the potential for growth and recovery of borderline ventricles have been uncovered. However, questions remain regarding optimal patient selection and the long-term outcomes of select patient groups treated with single-ventricle palliation versus biventricular repair/conversion or transplantation. Efforts to direct a greater proportion of borderline hypoplastic heart patients towards a biventricular circulation are accelerating and represent important avenues for progress and future research in the field of congenital heart disease.

Pulmonary insufficiency is a known complication following Tetralogy of Fallot repair. With over 90% of patients now surviving to adulthood, surgeons are once again faced with the question of when, and more importantly, how to reintervene. We developed a novel approach to pulmonary valve replacement in this population through a 5-cm left anterior mini-incision. The incision is optimized for exposing and operating on the right ventricular outflow tract and the main pulmonary artery in patients with a history of median sternotomy. Early outcomes are reassuring, and we believe our approach is a safe and reliable alternative to median sternotomy within this patient population, with the ability to quickly convert intraoperatively when needed.