Late Pulmonary Autograft Dilation: Can We Make a Good Operation Great? The Tailored Approach.

While it is the main viable option in the growing child and young adult, the Ross procedure has expanded its applicability to older patients, for whom long-term results are equivalent, if not superior, to prosthetic aortic valve replacement. Strategies aiming at mitigating long-term autograft failure from root enlargement and valve regurgitation have led some to advocate for root reinforcement with prosthetic graft material. On the contrary, we will discuss herein the rationale for a tailored approach to the Ross procedure; this strategy is aimed at maintaining the natural physiology and interplay between the various autograft components. Several technical maneuvers, including careful matching of aortic and autograft annuli and sino-tubular junction as well as external support by autologous aortic tissue maintain these physiologic relationships and the viability of the autograft, and could translate in a lower need for late reintervention because of dilation and/or valve regurgitation.

Valve-Sparing Aortic Root Replacement State of the Art Review Part II: Surgical Techniques.

Aortic valve disease is common and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement (VSRR) has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long term results vary based on underlying anatomy, pathology, and patient selection, and surgeon expertise. The purpose of Part II of this VSRR State of the Art Review article is to provide technical pearls related to VSRR.

Valve-Sparing Aortic Root Replacement State-of-the-Art Review, Part I: Anatomy and Physiology.

Aortic valve disease is common, and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging, but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long-term results vary based on the underlying anatomy, pathology, and patient selection, as well as surgeon expertise. The first installment of this Valve-Sparing Aortic Root Replacement State-of-the-Art Review article addresses patient anatomy and physiology as it relates to candidacy for VSRR.

Aortic Thrombosis and Subsequent Myocardial Infarction in a Previously Healthy 12-Year-Old Male.

Aortic thrombus formation in children is uncommon, particularly in an otherwise healthy pediatric patient. Thromboembolism of such thrombi resulting in subsequent ST-segment elevation myocardial infarction is, thus, exceedingly rare. (Level of Difficulty: Intermediate.).

Historical Accounts of Congenital Heart Surgery.

We present historical accounts of congenital heart surgery since the early 1900s, as our specialty evolved from individual heroic efforts into an established and sophisticated surgical specialty with consistent and excellent results. We highlight colleagues and intrepid pioneers who have strived to solve seemingly insurmountable problems during this remarkable journey and celebrate continued success into the 21st century with surgical advances that have resulted in innovative operations, database inquiries, quality measures, new techniques of medical illustration, and the establishment of the Congenital Heart Surgeons' Society, which has become the leading organization dedicated to congenital heart surgery in North America.

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children.

BACKGROUND: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. METHODS: Sixty-nine patients (median age, 12 years; range, 0.25-17.9) underwent the RP between January 1996 and December 2018. A concomitant Konno procedure was performed on 20 of 69 patients (29%). Prior interventions included balloon valvuloplasty in 30 (44%) and/or surgical valvuloplasty in 39 (57%). Technical modifications included using the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction, and wrapping the native root remnant around the PVA. RESULTS: Operative mortality was 1 of 69 patients (1.5%), with no late deaths. No patient had neoaortic valvar stenosis and 7 of 68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median, 9.4 years; range, 0.4-21.3) there was no significant change in Z scores of annulus, sinus, or sinotubular junction diameters when compared with those at discharge. Three patients (4.4%) required late autograft replacement, 2 PVA repair, and 2 resection of a pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 years. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80%, respectively. CONCLUSIONS: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients.

Heart Transplantation for Pediatric and Congenital Cardiac Disease: A Comparison of Two Eras over 23 Years and 188 Transplants at a Single Institution.

BACKGROUND: To assess changes in patterns of practice and outcomes over time, we reviewed all patients who underwent heart transplantation (HTx) at our institution and compared two consecutive eras with significantly different immunosuppressive protocols (cohort 1 [80 HTx, June 1995-June 2006]; cohort 2 [108 HTx, July 2006-September 2018]). METHODS: Retrospective study of 180 patients undergoing 188 HTx (June 1995-September 2018; 176 first time HTx, 10 second HTx, and 2 third HTx). In 2006, we commenced pre-HTx desensitization for highly sensitized patients and started using tacrolimus as our primary postoperative immunosuppressive agent. The primary outcome was mortality. Survival was modeled by the Kaplan-Meier method. Univariable and multivariable Cox proportional hazard models were created to identify prognostic factors for survival. RESULTS: Our 188 HTx included 18 neonates, 85 infants, 83 children, and 2 adults (>18 years). Median age was 260.0 days (range: 5 days-23.8 years). Median weight was 7.5 kg (range: 2.2-113 kg). Patients in cohort 1 were less likely to have been immunosensitized preoperatively (12.5% vs 28.7%, P = .017). Nevertheless, Kaplan-Meier analysis suggested superior survival in cohort 2 (P = .0045). Patients in cohort 2 were more likely to be alive one year, five years, and ten years after HTx. Multivariable analysis identified the earlier era (hazard ratio [HR] [95% confidence interval] for recent era = 0.32 [0.14-0.73]), transplantation after prior Norwood operation (HR = 4.44 [1.46-13.46]), and number of prior cardiac operations (HR = 1.33 [1.03-1.71]) as risk factors for mortality. CONCLUSIONS: Our analysis of 23 years of pediatric and congenital HTx reveals superior survival in the most recent 12-year era, despite the higher proportion of patients with elevated panel reactive antibody in the most recent era. This improvement was temporally associated with changes in our immunosuppressive strategy.

Recovery from Total Acute Lung Failure After 20 Months of Extracorporeal Life Support.

Since the first successful case report in 1972, extracorporeal life support or extracorporeal membrane oxygenation (ECMO) has become a standard approach for severe respiratory failure unresponsive to other therapy. In the past, if there was no recovery by approximately 30 days or if right ventricular heart failure occurred, ECMO was discontinued and the patient died. More recently patients with severe lung disease have been maintained for months, as opposed to days, with eventual decannulation and recovery. We report the case of a child, 7 years old, with severe inhalational burn injury and rapid progression to multisystem organ failure. She was supported by ECMO with no lung function for almost 2 years. Central nervous system function remained normal and lung function recovered. This is the longest successful case of ECMO to date and prompts further discussion regarding "irreversible" lung injury.

Management of the aortic arch in patients with Loeys-Dietz syndrome.

OBJECTIVES: We sought to develop strategies for management of the aortic arch in patients with Loeys-Dietz syndrome (LDS) through a review of our clinical experience with these patients and a comparison with our experience in patients with Marfan syndrome (MFS). METHODS: We reviewed hospital and follow-up records of 79 patients with LDS and compared them with 256 patients with MFS who served as reference controls. RESULTS: In the LDS group, 16% of patients presented initially with acute aortic dissection (AAD) (67% type A, 33% type B) or developed AAD during follow-up, compared with 10% of patients with MFS (95% type A, 5% type B). There was no difference between patients with LDS or MFS in need for subsequent arch interventions after aortic root surgery (46% vs 50%, P = 1.0). Among the patients who never had AAD, the need for arch repair at initial root surgery was greater in patients with LDS (5% vs 0.4%, P = .04), as was the need for any subsequent aortic surgery (12% vs 1.3%, P = .0004). Late mortality in patients with LDS after arch repair was greater than in those patients who had no arch intervention (33% vs 6%, P = .007). CONCLUSIONS: In the absence of dissection, patients with LDS have a greater rate of arch intervention after root surgery than patients with MFS. After a dissection, arch reintervention rates are similar in the 2 groups. Arch intervention portends greater late mortality in LDS.

Children's Heart Assessment Tool for Transplantation (CHAT) Score: A Novel Risk Score Predicts Survival After Pediatric Heart Transplantation.

BACKGROUND: Given the shortage of donor organs in pediatric heart transplantation (HTx), pretransplant risk stratification may assist in organ allocation and recipient optimization. We sought to construct a scoring system to preoperatively stratify a patient's risk of one-year mortality after HTx. METHODS: The United Network for Organ Sharing database was queried for pediatric (<18 years) patients undergoing HTx between 2000 and 2016. The population was randomly divided in a 4:1 fashion into derivation and validation cohorts. A multivariable logistic regression model for one-year mortality was constructed within the derivation cohort. Points were then assigned to independent predictors ( P < .05) based on relative odds ratios (ORs). Risk groups were established based on easily applicable, whole-integer score cutoffs. RESULTS: A total of 5,700 patients underwent HTx; one-year mortality was 10.7%. There was a similar distribution of variables between derivation (n = 4,560) and validation (n = 1,140) cohorts. Of the 12 covariates included in the final model, nine were allotted point values. The low-risk (score 0-9), intermediate-risk (10-20), and high-risk (>20) groups had a 5.18%, 10%, and 28% risk of one-year mortality ( P < .001), respectively. Both intermediate-risk (OR = 2.46, 95% confidence interval [95% CI]: 1.93-3.15; P < .001) and high-risk (OR = 9.24, 95% CI: 6.92-12.35; P < .001) scores were associated with an increased risk of one-year mortality when compared to the low-risk group. CONCLUSIONS: The Children's Heart Assessment Tool for Transplantation score represents a pediatric-specific, recipient-based system to predict one-year mortality after HTx. Its use could assist providers in identification of patients at highest risk of poor outcomes and may aid in pretransplant optimization of these children.

Valve-sparing aortic root replacement in children: Outcomes from 100 consecutive cases.

OBJECTIVE: Valve-sparing root replacement is an attractive alternative to composite mechanical or biologic prostheses for aortic root aneurysms in children. Data on outcomes in pediatric patients are limited. We present our institutional experience with 100 consecutive pediatric valve-sparing aortic root procedures. METHODS: All children who underwent valve-sparing root replacement at our institution from May 1997 to August 2017 were identified, and echocardiographic and clinical data were reviewed. The primary end point was mortality, and secondary end points included complications, further interventions, and subsequent valvular dysfunction. RESULTS: Median age at operation was 13.6 years (interquartile range, 9.42-15.9); 51 patients (51%) had Marfan syndrome, and 39 patients (39%) had Loeys-Dietz syndrome. Mean preoperative maximum sinus diameter was 4.4 ± 0.71 cm (z score 7.3 [5.7-9.3]). Most patients (n = 80, 80%) underwent reimplantation procedures with a Valsalva graft. Four patients (4%) underwent David I reimplantation with a straight-tube graft, 13 patients (13%) underwent a Yacoub remodeling procedure, and 3 patients (3%) underwent a Florida sleeve procedure. Perioperative valve-sparing root replacement mortality was 2% (n = 2). Six patients required late reintervention for development of pseudoaneurysms. Eight patients underwent additional aortic surgery. Average time to reoperation was 7.23 ± 4.56 years. Of the 84 patients undergoing a reimplantation procedure, 5 (5.9%) underwent late valve replacement versus 5 (33.3%) of the 15 patients who received a remodeling procedure (P = .001). CONCLUSIONS: Valve-sparing root replacement is a safe and effective option for children with aortic root aneurysms in children. The reimplantation procedure is preferred. Late aortic insufficiency and pseudoaneurysm formation remain late concerns.

Size Mismatching Increases Mortality After Lung Transplantation in Preadolescent Patients.

BACKGROUND: The effect of size mismatch between donor and recipient in pediatric lung transplantation (PLTx) is currently unknown. Previous studies in adults have suggested that oversized allografts are associated with improved outcomes after lung transplantation. We investigated this relationship to quantify its effect on posttransplant outcomes in children. METHODS: The United Network of Organ Sharing database was queried for preadolescent (age <13 years) patients undergoing PLTx. Donor-to-recipient height, weight, and predictive total lung capacity (pTLC; ages 4 to 13; pTLC = 0.160 x exp[0.021 x height]) ratios were calculated. Exploratory analysis was performed to identify disjoint intervals at which survival was statistically different. Patients were categorized as well-matched, undersized, or oversized. Multivariate Cox proportional hazard regression modeling assessed the adjusted effect of mismatching on mortality. Survival analysis was performed using the Kaplan-Meier method. RESULTS: The analysis included 540 children. One-year mortality was higher with a height mismatch of 5% or less (hazard ratio [HR], 2.97; p = 0.001) and above 5% (HR, 2.22; p = 0.009). Similarly, 1-year mortality was worse with weight mismatch of 10% or less (HR, 1.99; p = 0.035) and above 10% (HR, 2.04; p = 0.028). On unadjusted analysis, a pTLC ratio of less than 0.9 was associated with worse survival (p = 0.017). This finding persisted after multivariate risk adjustment (HR, 2.93; p = 0.02). Contrary to findings in adults, an oversized allograft (pTLC ratio > 1.1) was not associated with improved survival (HR, 1.95; p = 0.147). CONCLUSIONS: In preadolescent children undergoing PLTx, size mismatching is associated with increased death. Our findings differ from studies in adults, which demonstrated improved survival associated with oversized allografts. Accordingly, well-matched allografts should be prioritized when assessing donor-recipient pairs for transplantation.

HeartWare Ventricular Assist Device Implantation for Pediatric Heart Failure-A Single Center Approach.

While pediatric HeartWare HVAD application has increased, determining candidacy and timing for initiation of pediatric VAD support has remained a challenge. We present our experience with a systematic approach to HVAD implantation as a bridge to pediatric heart transplantation. We performed a retrospective, single center review of pediatric patients (n = 11) who underwent HVAD implantation between September 2014 and January 2018. Primary endpoints evaluated were survival to heart transplantation, need for right ventricular assist device (RVAD) at any point, ongoing HVAD support, or death. Median patient age was 11 years (range: 3-16). Median BSA was 1.25 m2 (range: 0.56-2.1). Heart failure etiologies requiring support were dilated cardiomyopathy (n = 8), myocarditis (n = 1), congenital mitral valve disease (n = 1), and single ventricle heart failure (n = 1). Median time from cardiac ICU admission for heart failure to HVAD placement was 15 days (range 3-55), based on standardized VAD implantation criteria involving imaging assessment and noncardiac organ evaluation. The majority of patients (91%) were INTERMACS Level 2 at time of implant. Three patients (27%) had CentriMag RVAD placement at time of HVAD implantation. Two of these three patients had successful RVAD explanation within 2 weeks. Median length of HVAD support was 60 days (range 6-405 days). Among the 11 patients, survival during HVAD therapy to date is 91% (10/11) with 9 (82%) bridged to heart transplantation and one (9%) continuing to receive support. Posttransplant survival has been 100%, with median follow-up of 573 days (range 152-1126). A systematic approach to HVAD implantation can provide excellent results in pediatric heart failure management for a variety of etiologies and broad BSA range.

The Prevalence and Impact of Congenital Diaphragmatic Hernia Among Patients Undergoing Surgery for Congenital Heart Disease.

There has not been a multicenter investigation to elucidate the prevalence and impact of congenital diaphragmatic hernia (CDH) on children undergoing cardiac surgery. We investigated the prevalence of CDH across congenital cardiac diagnostic and procedural groups and its impact on outcomes. The STS Congenital Heart Surgery Database was queried to identify children undergoing cardiac surgery who also had CDH. Baseline perioperative characteristics and postoperative outcomes were compared between groups. Subgroup analyses were performed based on case complexity as determined by STAT Categories. Overall 426 of 157,419 (0.27%) pediatric patients undergoing an index cardiac operation had a diagnosis of CDH including 89 neonates (0.25% of all neonatal index operations), 217 infants (0.39%), and 120 children (0.18%). The frequency of concomitant CDH varied across diagnostic groups and, in neonates and infants, was highest for tetralogy of Fallot (0.45%, n = 41), coarctation (0.39%, n = 27) and complete atrioventricular septal defects (0.31%, n = 19). For neonates and infants outcomes were generally worse in CDH vs no-CDH patients across the various procedural strata. For example, STAT ≥3 operative mortality was 34.4% vs 10.3% ( P < 0.001) for neonates, and 24.2% vs 5.3% ( P < 0.001) for infants with vs without CDH, respectively. In older children, outcomes disparities were less clear cut with no significant differences in mortality, and variable differences for complication and length of stay outcomes. CDH occurs in a small percentage of patients born with congenital heart disease. Concomitant diagnosis of CDH portends increased morbidity and mortality in infants and neonates undergoing cardiac surgery.

Risk Factors for Survival After Heart Transplantation in Children and Young Adults: A 22-Year Study of 179 Transplants.

BACKGROUND: This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality. METHODS: Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis. RESULTS: Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups ( P = .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P = .422). CONCLUSIONS: Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.