Two-Stage Liver Transplantation and Tricuspid Valve Replacement After Blunt Trauma in a Pediatric Patient.

In cases of uncontrollable hepatic hemorrhage or acute hepatic failure after trauma, liver transplantation can be a lifesaving procedure. Traumatic tricuspid valve injuries are rare, and symptoms can range from indolent to acute right heart failure. When concomitant, traumatic liver transplant and tricuspid injuries have significant physiologic interplay and management implications. We present a 14-year-old male injured in an all-terrain vehicle accident, who sustained a devastating disruption of the common bile duct and celiac artery injury, leading to acute hepatic failure, necessitating a two-stage liver transplantation. He was subsequently found to have a severe traumatic tricuspid injury, which required tricuspid valve replacement. At 4 years post-injury, he is without major complications. This is the first case presentation of the cooccurrence of these complex pathologies. Importantly, we demonstrate the complex decision-making surrounding traumatic liver transplantation and timing of subsequent tricuspid valve repair, weighing the complex interplay of these 2 pathologies.

Left Ventricular Outflow Tract Obstruction in Congenital Heart Disease: The Role of Cardiovascular Computed Tomography in Surgical Decision Making.

Patients with many forms of congenital heart disease (CHD) and hypertrophic cardiomyopathy undergo surgical intervention to relieve left ventricular outflow tract obstruction (LVOTO). Cardiovascular Computed Tomography (CCT) defines the complex pathway from the ventricle to the outflow tract and can be visualized in 2D, 3D, and 4D (3D in motion) to help define the mechanism and physiologic significance of obstruction. Advanced cardiac visualization may aid in surgical planning to relieve obstruction in the left ventricular outflow tract, aortic or neo-aortic valve and the supravalvular space. CCT scanner technology has advanced to achieve submillimeter, isotropic spatial resolution, temporal resolution as low as 66 msec allowing high-resolution imaging even at the fast heart rates and small cardiac structures of pediatric patients ECG gating techniques allow radiation exposure to be targeted to a minimal portion of the cardiac cycle for anatomic imaging, and pulse modulation allows cine imaging with a fraction of radiation given during most of the cardiac cycle, thus reducing radiation dose. Scanning is performed in a single heartbeat or breath hold, minimizing the need for anesthesia or sedation, for which CHD patents are highest risk for an adverse event. Examples of visualization of complex left ventricular outflow tract obstruction in the subaortic, valvar and supravalvular space will be highlighted, illustrating the novel applications of CCT in this patient subset.

Interdigitating Technique for Repair of Aortic Arch Obstruction to Reduce Reintervention Rates.

BACKGROUND: The incidence of reintervention for aortic arch obstruction is 5% to 14% after coarctation or hypoplastic aortic arch repair and 25% after the Norwood procedure. Institutional practice review indicated higher than reported reintervention rates. Our aim was to assess the impact of an interdigitating reconstruction technique on reintervention rates for recurrent aortic arch obstruction. METHODS: Children (<18 years) were included if they had undergone aortic arch reconstruction by sternotomy or the Norwood procedure. Three surgeons participated in the intervention with staggered rollout dates between June 2017 and January 2019, with the study ending December 2020 and review for reinterventions ending February 2022. Preintervention cohorts represented patients who underwent aortic arch reconstructions with patch augmentation, and postintervention cohorts represented patients who underwent an interdigitating reconstruction technique. Reinterventions by cardiac catheterization or operation were measured within 1 year of initial operation. Wilcoxon rank sum and χ2 tests were used to compare preintervention and postintervention cohorts. RESULTS: Overall, 237 patients were included for participation in this study, with 84 patients in the preintervention cohort and 153 in the postintervention cohort. Patients undergoing the Norwood procedure represented 30% (n = 25) of the retrospective cohort and 35% (n = 53) of the intervention cohort. Overall reinterventions were significantly decreased after the study intervention from 31% (n = 26/84) to 13% (n = 20/153; P < .001). Reintervention rates were decreased for each intervention cohort: aortic arch hypoplasia (24% [n = 14/59] vs 10% [n = 10/100]; P = .019) and Norwood procedure (48% [n = 12/25] vs 19% [n = 10/53]; P = .008). CONCLUSIONS: The interdigitating reconstruction technique for obstructive aortic arch lesions was successfully implemented and is associated with a decrease in reinterventions.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients.

INTRODUCTION: Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. MATERIALS AND METHODS: Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher's exact test and overlapping 83.7% confidence intervals. RESULTS: Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1-14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. DISCUSSION: In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

Transcatheter and Surgical Aortic Valve Implantation in Children, Adolescents, and Young Adults With Congenital Heart Disease.

Transcatheter aortic valve implantation (TAVI) is common in adults but rare in children and adolescents. Since 2014, our institution has incorporated a transcatheter approach as an option for aortic valve replacement in this population. The purpose of this study was to compare short-term outcomes of TAVI with surgical aortic valve replacement (SAVR). This single-center, retrospective study included patients aged 10 to 21 years who had a native SAVR or TAVI between January 2010 to April 2020. Comparative analysis of baseline characteristics and a composite outcome (stroke within 6 months, readmission within 30 days, death) between SAVR and TAVI were made using chi-square test or Wilcoxon rank sum test, as appropriate. Of the 77 patients who underwent native aortic valve implantation during the study period (60 SAVR, 17 TAVI), 46 were aged 10 to 21 years (30 SAVR, 16 TAVI). Median follow-up was 3.8 years (interquartile range 1.5 to 4.9) for the SAVR group and 1.5 years (interquartile range 1.1 to 1.2) for the TAVI group. There was no difference in the composite outcome between groups. Patients in the SAVR group were more likely to have undergone concomitant surgical intervention and have longer intensive care unit and hospital stays. In conclusion, our study suggests similar short-term outcomes between SAVR and TAVI in children and young adults aged 10 to 21 years. Longer-term studies are essential to understand the utility of TAVI and to better consider the option of a transcatheter approach as an alternative to SAVR in the pediatric population.

Fetal growth and gestational age improve outcome predictions in neonatal heart surgery.

OBJECTIVE: Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies. METHODS: Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value. RESULTS: The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant at <32 weeks with a BW z score of -2 to -1. CONCLUSIONS: This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

Short-Term Left Ventricular Reverse Remodeling after Transcatheter Aortic Valve Replacement in Children.

BACKGROUND: There are no published data on left ventricular (LV) reverse remodeling after transcatheter aortic valve replacement (TAVR) in children. The aim of this study was to assess changes in LV echocardiographic parameters 6 months after TAVR in children. METHODS: This single-center, retrospective study included all 22 patients (age < 21 years) who underwent TAVR. The median age was 14.7 years (interquartile range, 13.3-15.9 years), median weight was 57 kg (interquartile range, 46.0-66.3 kg), and 59% of patients were male. Demographics, type and duration of aortic valve dysfunction, symptom and treatment data, and preprocedural and 6-month follow-up echocardiographic data (LV volume, mass, end-diastolic dimension, end-systolic dimension, ejection fraction [EF], sphericity, and longitudinal strain) were collected. Failure to reverse remodel at 6 months was defined as meeting at least two of the following: Z score ≥ 2 that was unchanged or increased from baseline for LV volume, mass, end-diastolic dimension, or end-systolic dimension; abnormally high sphericity index that was unchanged or increased; and abnormally low EF or longitudinal strain. Median, interquartile range, and range are reported for continuous variables, and pre- and post-TAVR data were compared using the Wilcoxon signed rank test. RESULTS: Eight patients (36%) had isolated aortic stenosis, four (18%) had isolated regurgitation, and 10 had (46%) mixed disease. Twelve (55%) had symptoms and 20 (91%) had prior surgical or catheter valve interventions. The primary complication was left bundle branch block, occurring in four children (18%). At 6 months, LV volume, mass, end-diastolic dimension, end-systolic dimension, and sphericity index improved. EF and strain were normal at baseline and at follow-up. Of three patients who failed to reverse remodel, two had left bundle branch block. Of three patients with persistent symptoms, one had failure of reverse remodeling. CONCLUSIONS: Most pediatric patients had evidence of reverse LV remodeling 6 months after TAVR, suggesting a possible alternative to surgical aortic valve replacement in this population. Functional parameters (EF and strain) were normal at baseline and follow-up. Future studies are needed to determine optimal timing of TAVR and to explore the association of postprocedural left bundle branch block on failed reverse remodeling and outcomes in this population.

Time-Related Risk of Pulmonary Conduit Re-replacement: A Congenital Heart Surgeons' Society Study.

BACKGROUND: Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2). METHODS: From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI. RESULTS: Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P < .001), concomitant aortic valve intervention (HR 7.6, P = .009), aortic allograft (HR 2.2, P = .008), younger age (HR 1.4, P < .001), and larger Z score of PC1 (HR 1.2, P = .04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P = .006), porcine unstented conduit (HR 4.7, P < .001), and older age (HR 2.3, P = .01). CONCLUSIONS: Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible.

Successfully Reducing Fat-modified Diet Duration for Treating Postoperative Chylothorax in Children.

BACKGROUND: Medical management, primarily a fat-modified diet (FMD), is the mainstay of treatment for most patients with chylothorax. Duration of FMD is traditionally reported as 6 weeks, but no studies have demonstrated the shortest effective duration that prevents recurrence of chylothorax. The aim of this study was to decrease FMD duration to 2 weeks in children with postoperative chylothorax without a significant increase in recurrence. METHODS: This single-center study included pediatric (aged <18 years) patients in whom chylothorax developed within 30 days of cardiac surgery. Patients with cavopulmonary anastomoses were excluded. The preintervention cohort consisted of 19 patients with a diagnosis of chylothorax between February 2014 and June 2015, and the postintervention cohort comprised 98 patients from July 2015 to December 2019. FMD duration was decreased from 6 weeks to 4 weeks in May 2016 and to 2 weeks in June 2018. Recurrence was defined as a return of a chylous effusion requiring chest tube placement or hospital readmission within 30 days of resuming a regular diet. RESULTS: The median duration of FMD decreased from 42 days (interquartile range, 30, 43 days) in the preintervention cohort to 26 days (interquartile range, 14, 29 days) in the postintervention cohort, with no recurrence of chylothorax in any group. Compliance with the FMD duration instruction in the 6-week, 4-week, and 2-week groups was 100%, 84%, and 67% respectively. Compared with the first 6 months, compliance with the 2-week FMD instruction during the final 12 months increased from 40% (6/15) to 79% (26/33). CONCLUSIONS: At the study center, FMD duration decreased from 6 weeks to 2 weeks without any recurrence of chylothorax.

Usefulness of Left Ventricular Myocardial Deformation in Children Hospitalized for Acute Myocarditis who Develop Arrhythmias.

Cardiac arrhythmias occur in 3-40% of patients with acute myocarditis and cause significant morbidity and mortality. Myocardial injury also results in abnormal myocardial deformation. The relationship between left ventricular (LV) deformation, measured by two-dimensional speckle tracking echocardiography (2D-STE), and arrhythmia in pediatric myocarditis is unknown. We evaluated the association between 2D-STE and arrhythmias in children hospitalized with acute myocarditis. We reviewed patients ≤ 18 years hospitalized for acute myocarditis from 2008 to 2018. Arrhythmias were defined as 1) non-sustained or sustained ventricular tachycardia or ventricular fibrillation, 2) sustained supraventricular tachycardia (SVT), 3) high-grade or complete heart block, and 4) any arrhythmia treated with an antiarrhythmic medication. Systolic LV strain values (including LV global longitudinal strain (GLS), global circumferential strain (GCS), and six segments of LV regional long axis strain) were obtained from initial echocardiograms during hospitalization. Of 66 patients hospitalized, 23 (35%) had arrhythmias. SVT was the predominant arrhythmia (74%). Global and regional strain indices were reduced in the arrhythmia group: LV GLS [-8.9 (IQR -13.6, -6.1) vs. -13.7 (IQR -16.9, -9.7), p = 0.038]; basal inferior/septal [-10.7 (IQR -15.5, -7.8) vs. -16.4 (IQR -18, -11.8), p = 0.009]; basal anterior/lateral [-7.1 (IQR -12.8, -4.7) vs. -9.4 (IQR -16.7, -7.4), p = 0.025]; and mid inferior/septal segments [-9 (IQR -13, -7.7) vs. -14.1 (IQR -22.5, -10.7), p = 0.007]. After controlling for age, reductions in GLS and segmental strain in the two basal and two mid-segments were associated with increased arrhythmia occurrence (p <0.05). Our findings suggest that echocardiographic LV deformation by 2D-STE may be useful in identifying pediatric patients with acute myocarditis at risk for arrhythmias.