Staged repair of borderline hypoplastic heart disease with early biventricular conversion.

Objective: In select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular conversion by 2 years of age. Methods: Between 2018 and 2023, 14 newborns with borderline hypoplastic heart disease deemed high risk for primary biventricular repair underwent palliative procedures as a neonate/infant, followed by staged or direct biventricular conversion. Results: Of the 14 patients, 6 had borderline left ventricles and 8 had borderline right ventricles. Index neonatal operations were performed in 12 patients and included the Norwood operation (n = 5), pulmonary artery band (n = 3), ductal stent (n = 3), and hybrid Norwood (n = 1). Five patients underwent direct biventricular conversion, and the remaining 9 patients underwent staged ventricular recruitment operations at a mean age of 6 months (range, 3-11 months). Ventricular recruitment operations included atrial septation with or without ventricular rehabilitation, atrioventricular valve repair, or outflow tract operations. At a mean duration of 8 months (range, 4-10 months) after ventricular recruitment, there was a significant increase in chamber volume, aortic valve, and mitral valve size in patients with borderline left ventricles, and a normalization of the right ventricle:left ventricle end-diastolic volume ratio in patients with borderline right ventricles. To date, 13 of 14 patients have undergone successful biventricular conversion at a mean age of 16 months (range, 4-31 months). Conclusions: In select newborns with borderline hypoplastic heart disease, single-ventricle palliation followed by staged or direct biventricular conversion may increase infant survival while allowing for early attainment of a biventricular circulation.

Survival after partial heart transplantation in a piglet model.

Partial heart transplantation (PHT) is a novel surgical approach that involves transplantation of only the part of the heart containing a valve. The rationale for this approach is to deliver growing heart valve implants that reduce the need for future re-operations in children. However, prior to clinical application of this approach, it was important to assess it in a preclinical model. To investigate PHT short-term outcomes and safety, we performed PHT in a piglet model. Yorkshire piglets (n = 14) were used for PHT of the pulmonary valve. Donor and recipient pairs were matched based on blood types. The piglets underwent PHT at an average age of 44 days (range 34-53). Post-operatively, the piglets were monitored for a period of two months. Of the 7 recipient piglets, one mortality occurred secondary to anesthesia complications while undergoing a routine echocardiogram on post-operative day 19. All piglets had appropriate weight gain and laboratory findings throughout the post-operative period indicating a general state of good health and rehabilitation after undergoing PHT. We conclude that PHT has good short-term survival in the swine model. PHT appears to be safe for clinical application.

Surgical Protocol for Partial Heart Transplantation in Growing Piglets.

Partial heart transplantation is a new approach to deliver growing heart valve implants. Partial heart transplants differ from heart transplants because only the part of the heart containing the necessary heart valve is transplanted. This allows partial heart transplants to grow, similar to the valves in heart transplants. However, the transplant biology of partial heart transplantation remains unexplored. This is a critical barrier to progress of the field. Without knowledge about the specific transplant biology of partial heart transplantation, children with partial heart transplants are empirically treated like children with heart transplants because the valves in heart transplants are known to grow. In order to progress the field, an animal model for partial heart transplantation is necessary. Here, we contribute our surgical protocol for partial heart transplantation in growing piglets. All aspects of partial heart transplantation, including the donor procedure, the recipient procedure, and recipient perioperative care are described in detail. There are important nuances in the conduct of virtually all aspects of open heart surgery that differs in piglets from humans. Our surgical protocol, which is based on our experience with 34 piglets, will allow other investigators to leverage our experience to seek fundamental knowledge about the nature of partial heart transplants. This is significant because the partial heart transplant model in piglets is complex and very resource intensive.

Partial Heart Transplantation - How to Change the System.

Partial heart transplantation is the first clinically successful approach to deliver growing heart valve implants. To date, 13 clinical partial heart transplants have been performed. However, turning partial heart transplantation into a routine procedure that is available to all children who would benefit from growing heart valve implants poses formidable logistical challenges. Firstly, a supply for partial heart transplant donor grafts needs to be developed. This challenge is complicated by the scarcity of donor organs. Importantly, the donor pools for orthotopic heart transplants, partial heart transplants and cadaver homografts overlap. Secondly, partial heart transplants need to be allocated. Factors relevant for equitable allocation include the indication, anatomical fit, recipient clinical status and time on the wait list. Finally, partial heart transplantation will require regulation and oversight, which only recently has been undertaken by the Food and Drug Administration, which regulates human cellular and tissue-based products. Overcoming these challenges will require a change in the system. Once this is achieved, partial heart transplantation could open new horizons for children who require growing tissue implants.

Days alive and out of hospital for children born with single-ventricle heart disease.

BACKGROUND: This study describes the illness burden in the first year of life for children with single-ventricle heart disease, using the metric of days alive and out of hospital to characterize morbidity and mortality. METHODS: This is a retrospective single-centre study of single-ventricle patients born between 2005 and 2021 who had their initial operation performed at our institution. Patient demographics, anatomical details, and hospitalizations were extracted from our institutional single-ventricle database. Days alive and out of hospital were calculated by subtracting the number of days hospitalized from number of days alive during the first year of life. A multivariable linear regression with stepwise variable selection was used to determine independent risk factors associated with fewer days alive and out of hospital. RESULTS: In total, 437 patients were included. Overall median number of days alive and out of hospital in the first year of life for single-ventricle patients was 278 days (interquartile range 157-319 days). In a multivariable analysis, low birth weight (<2.5kg) (b = -37.55, p = 0.01), presence of a dominant right ventricle (b = -31.05, p = 0.01), moderate-severe dominant atrioventricular valve regurgitation at birth (b = -37.65, p < 0.05), index hybrid Norwood operation (b = -138.73, p < 0.01), or index heart transplant (b = -158.41, p < 0.01) were all independently associated with fewer days alive and out of hospital. CONCLUSIONS: Children with single-ventricle heart defects have significant illness burden in the first year of life. Identifying risk factors associated with fewer days alive and out of hospital may aid in counselling families regarding expectations and patient prognosis.

Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery.

Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d). Multivariable logistic regression and Cox proportional hazards modeling were used. Results: Fifty-one percent (137/269) of patients met TO. Common reasons for TO failure were prolonged LOS (78/132, 59%) and ventilator duration (67/132, 51%). In multivariable analysis, higher weight [odds ratio, OR: 1.44 (95% confidence interval, CI: 1.15-1.84), P = .002] was a positive predictor of TO achievement while right ventricular dominance [OR 0.47 (0.27-0.81), P = .007] and higher preoperative pulmonary vascular resistance [OR 0.58 (0.40-0.82), P = .003] were negative predictors. After controlling for preoperative factors and excluding operative mortalities, TO achievement was independently associated with a decreased risk of death over long-term follow-up [hazard ratio: 0.50 (0.25-0.99), P = .049]. Textbook outcome achievement was also associated with lower direct cost of care [$137,626 (59,333-167,523) vs $262,299 (114,200-358,844), P < .0001]. Conclusion: Achievement of the Glenn TO is associated with long-term survival and lower costs and can be predicted by certain risk factors. As outcomes continue to improve within congenital heart surgery, operative mortality will become a less informative metric. Textbook outcome analysis may represent a more balanced measure of a successful outcome.

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction.

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. Objective: To deliver the first heart valve implant that grows. Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury. Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves. Main Outcomes and Measures: Transplanted valve growth and hemodynamic function. Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves. Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

Use of microaxial flow pumps in adolescents.

Objectives: The Impella 5.5 has been successfully used in the adult population; however, safety and efficacy data in patients aged less than 18 years are limited. Methods: Six pediatric patients, aged 13 to 16 years and weighing 45 to 113 kg, underwent axillary artery graft placement and attempted placement of the Impella 5.5 device at our institution between August 2020 and March 2023. Results: Indications for implantation were heart failure secondary to myocarditis (2), rejection of prior orthotopic heart transplant, idiopathic dilated cardiomyopathy (2), and heart failure after transposition of the great arteries repair. Placement was unsuccessful in a 13.8-year-old female patient due to prohibitively acute angulation of the right subclavian artery, and venoarterial extracorporeal membrane oxygenation cannulation was performed via the axillary graft. In 5 patients with successful Impella 5.5 placement, median duration of support was 13.5 days (range, 7-42 days). One experienced cardiac arrest secondary to coagulation-associated device failure, requiring temporary HeartMate3 implantation. Four patients were bridged to transplant; 3 patients received a transplant directly from Impella 5.5, and 1 patient received a transplant after HeartMate3. The final patient received the HeartMate3 on Impella day 42 and is awaiting transplant. Conclusions: Although exact size cutoffs and anatomy are still being determined, our experience provides a framework for use of the Impella 5.5 in adolescents.

Center-level factors associated with shorter length of stay following stage 1 palliation: An analysis of the national pediatric cardiology quality improvement collaborative registry.

BACKGROUND: Stage 1 single ventricle palliation (S1P) has the longest length of stay (LOS) of all benchmark congenital heart operations. Center-level factors contributing to prolonged hospitalization are poorly defined. METHODS: We analyzed data from infants status post S1P included in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry. Our primary outcome was patient-level LOS with days alive and out of hospital before stage 2 palliation (S2P) used as a balancing measure. We compared patient and center-level characteristics across quartiles for median center LOS, and used multivariable regression to calculate center-level factors associated with LOS after adjusting for case mix. RESULTS: Of 2,510 infants (65 sites), 2037 (47 sites) met study criteria (61% male, 61% white, 72% hypoplastic left heart syndrome). There was wide intercenter variation in LOS (first quartile centers: median 28 days [IQR 19, 46]; fourth quartile: 62 days [35, 95], P < .001). Mortality prior to S2P did not differ across quartiles. Shorter LOS correlated with more pre-S2P days alive and out of hospital, after accounting for readmissions (correlation coefficient -0.48, P < .001). In multivariable analysis, increased use of Norwood with a right ventricle to pulmonary artery conduit (aOR 2.65 [1.1, 6.37]), shorter bypass time (aOR 0.99 per minute [0.98,1.0]), fewer additional cardiac operations (aOR 0.46 [0.22, 0.93]), and increased use of NG tubes rather than G tubes (aOR 7.03 [1.95, 25.42]) were all associated with shorter LOS centers. CONCLUSIONS: Modifiable center-level practices may be targets to standardize practice and reduce overall LOS across centers.

Understanding and Managing Direct Operating Room Supply Costs in Cardiac Surgery.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Minimally Invasive Approach for Cardiac Hemangioma Resection in a Teenager.

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.

Decreases in daily ambulation forecast post-surgical re-admission.

BACKGROUND: Surgical readmissions are clinically and financially problematic. Our purpose is to determine if a decrease in postoperative ambulation (steps/day) is associated with hospital readmission. METHODS: In this prospective cohort study, patients undergoing elective operations wore an accelerometer activity tracker to measure steps/day for 28 consecutive postoperative days. The primary outcome was hospital readmission. The change in steps/day over two consecutive days prior to the day of the readmission were examined. Predetermined thresholds for decreases of consecutive daily ambulation levels were used to calculate sensitivity and specificity for the outcome of hospital readmission. RESULTS: 215 patients (aged 63 ± 12 years) were included. Readmission occurred in 10% (n = 21). For each of the first 28-postoperative days, the entire cohort had an average daily step increase of 136 ± 146 steps/day (Spearman correlation rho = 0.990; p < 0.001). A decrease in steps for two consecutive days of >50% from the prior day had a 79% sensitivity and 90% specificity for hospital readmission. CONCLUSIONS: A decrease of >50% daily ambulation (steps/day) over two consecutive post-discharge days accurately forecasts hospital readmission. The implications of these findings are that monitoring daily ambulation could serve as a form of outpatient telemetry aiding to forecast post-surgical readmissions.