Outcomes of Treatment for Infective Endocarditis Following Transcatheter Pulmonary Valve Replacement.

BACKGROUND: Recipients of transcatheter pulmonary valve replacement (TPVR) have shown increased risk of infective endocarditis (IE). Little is known about the outcomes of different management strategies, particularly surgery, for IE after TPVR. METHODS: We queried the Pediatric Health Information System database for cases of IE after TPVR performed from 2010-2020. We described patient demographics, hospital courses, admission complications, and treatment outcomes based on therapy offered, surgical or medical only. We compared outcomes of initial therapy. Data are expressed as median or percent. RESULTS: Sixty-nine cases of IE were identified, accounting for 98 related hospital admissions; 29% of patients recorded IE-related readmissions. Of those readmitted after initial medical therapy only, 33% had relapse IE. Rates of surgery were 22% during initial admission and 36% overall. Likelihood of surgical intervention increased with each subsequent admission. Renal and respiratory failure were more common in those given initial surgery. Mortality rate was 4.3% overall and 8% in the surgical cohort. CONCLUSION: Initial medical therapy may result in relapses/readmissions and possible delay of surgical therapy, which appears to be most effective for treatment of IE. For those treated only medically, a more aggressive course of therapy may be more likely to prevent relapse. Mortality following surgical therapy for IE after TPVR appears higher than reported for surgical pulmonary valve replacement generally.

Slipping Rib Syndrome in Children: Natural History and Outcomes Following Costal Cartilage Excision.

INTRODUCTION: Slipping rib syndrome (SRS) or subluxation of the medial aspect of the lower rib costal cartilages is an underdiagnosed cause of debilitating pain in otherwise healthy children. Costal cartilage excision may provide definitive symptom relief. However, limited data exist on the natural history, difficulty in diagnosis, and patient-reported outcomes for SRS in children. METHODS: We performed a single-institution descriptive study using chart review and a patient-focused survey for patients who underwent surgery for SRS from 2012 to 2020. Data regarding demographics, symptoms, diagnostic workup, and patient-reported outcomes were collected. RESULTS: Surgical resection was performed in 13 children. The median age at symptom onset was 12.5 y [IQR 9.7, 13.9], with a preponderance of girls (10, 77%). Eight patients participated in competitive athletics at the time of symptom onset. Prior to diagnosis, patients were seen by a median 3 [IQR 2, 5] providers with a median of 4 [IQR 3, 6] non-diagnostic imaging exams performed. The children included in the study underwent surgery for left (8), bilateral (4), and right (1) SRS. Two were lost to follow-up. At median post-op follow-up of 3.5 mo [IQR 1.2, 9.6], 73% (8/11) had returned to full activity. One reported non-limiting persistent pain symptoms. CONCLUSIONS: Lack of knowledge regarding SRS may result in delayed diagnosis, excessive testing, and limitation of physical activity. Operative treatment appears to provide durable relief and should be considered for children with SRS. The challenge remains to decrease the number of non-diagnostic exams and time to diagnosis.

Midterm Outcomes of Heart Transplantation in Children With Genetic Disorders.

BACKGROUND: Many congenital heart diseases (CHD) are associated with genetic defects. Children with complex CHD often have heart failure requiring heart transplant. Given the broad spectrum of genetic pathologies and dearth of transplants performed in these children, little is known regarding their outcomes. METHODS: We conducted a retrospective review of heart transplants performed at a high-volume center from 2007 to 2021. Patients were separated into pathogenic molecular and copy number variants, aneuploidies, and variants of uncertain significance, and compared with patients without known genetic diagnoses. Variables included genetic diagnoses, bridge-to-transplant approach, preoperative comorbidities, operative characteristics, and postoperative complications. Outcomes included intensive care unit-free days to 28 days, hospital mortality, survival, rejection, retransplantation, and educational status at latest follow-up. RESULTS: In all, 223 patients received transplants over the study period: 9.9% (22 of 223) had pathogenic molecular variants; 4.5% (10 of 223) had copy number variants; 1.8% (4 of 223) had aneuploidies; and 9% (20 of 223) had variants of uncertain significance. The most common anomalies were Turner syndrome (n = 3) and 22q11.2 deletion syndrome (n = 2). Children with aneuploidies had higher rates of hepatic dysfunction and hypothyroidism, whereas children with pathogenic copy number variants had higher rates of preoperative gastrostomy and stroke. Children with aneuploidies were intubated longer after transplant, with greater need for reintubation, and had the fewest intensive care unit-free days. Mortality and mean survival did not differ. At median follow-up of 4.4 years (range, 1.9 to 8.8), 89.7% of survivors (26 of 29) with pathogenic anomalies were attending or had graduated school. CONCLUSIONS: Despite more preoperative comorbidities, midterm outcomes after heart transplant in children with genetic syndromes and disorders are promising.

Possible Association of Pulmonary Atresia with In-Utero Coxsackievirus B Exposure.

Gestational viral infection has been associated with congenital heart disease (CHD). Few studies, however, have studied the potential role of gestational Coxsackievirus B (CVB) exposure in the pathogenesis of CHD. We prospectively enrolled women with pregnancies affected by CHD to explore possible associations with in utero CVB exposure. Serum samples were obtained from 122 women referred for fetal echocardiography between 2006 and 2018. We quantified CVB IgG and IgM levels, with titers ≥ 15.0 U/mL considered positive and measured neutralizing antibodies for three CVB serotypes: CVB1, CVB3, and CVB4. Using data from the national enterovirus surveillance system, we compared the annual exposure rates for each serotype in our cohort to infections reported across the United States. 98 pregnancies with no genetic defects were included. Overall, 29.6% (29/98) had positive IgG and 4.1% (4/98) of women had positive CVB IgM titers. To explore first-trimester CVB exposure, we focused exclusively on the 26 women with positive IgG and negative IgM titers. 61.5% (16/26) had neutralizing antibodies against a single serotype and 38.5% (10/26) against multiple CVB serotypes. CVB4 neutralizing antibodies were the most common (65.4%, 17/26), followed by CVB3 (53.9%, 14/26) and CVB1 (30.8%, 8/26). Among these, 30.8% of babies presented pulmonary valve anomalies: 19.2% (5/26) pulmonary atresia, and 11.5% (3/26) pulmonary stenosis. 23.1% (6/26) of babies had coronary sinusoids. CVB exposure in our cohort mirrored that of reported infections in the United States. Our results suggest a possible association between gestational CVB exposure and specific CHD, particularly pulmonary valve anomalies and coronary sinusoids.

Improved Outcomes of Infant Lung Transplantation Over 3 Decades.

BACKGROUND: Lung transplantation is the definitive surgical treatment for end-stage lung disease. However, infants comprise less than 5% of pediatric cases. This study sought to provide an overview of infant lung transplantation outcomes over the past 3 decades by using linked United Network for Organ Sharing (UNOS) and Pediatric Health Information System (PHIS) data. METHODS: Infants undergoing lung transplantation from 1989 to 2020 in UNOS were reviewed. UNOS and PHIS records for patients who underwent lung transplantation from 1995 to 2020 were linked using date of birth, sex, and date of surgery ± 3 days. The study assessed underlying diagnoses, pretransplant and posttransplant extracorporeal membrane oxygenation support, retransplant-free survival to discharge, hospital experience (≥1 annual transplant for ≥4 years in a 5-year period), operative decade, bronchiolitis obliterans syndrome, long-term survival, and functional status at latest follow-up. RESULTS: A total of 112 lung transplants were performed in 109 infants over 31 years. Of these, 21 patients died before discharge, and 2 underwent repeat transplantation during the same admission. The study linked 80.6% (83 of 103) of UNOS and PHIS records. Hospital survival was lower for infants with idiopathic pulmonary hypertension and those who underwent transplant procedures at less experienced centers. All 7 infants requiring postoperative extracorporeal membrane oxygenation support died. Median freedom from bronchiolitis obliterans syndrome was 8.1 years (interquartile range, 4.6 to 11.6 years). After discharge, median survival was 10.3 years (interquartile range, 6.3 to 14.4 years), with improved 10-year survival for those patients who underwent transplantation from 2010 to 2020 (87.3%) vs 2000 to 2009 (52.4%; P = .098) and 1989 to 1999 (34.1%; P = .004). A total of 84.6% (33 of 39) of survivors had minor or no restrictions at latest follow-up. CONCLUSIONS: Carefully selected infants experience promising short- and long-term outcomes after lung transplantation.

A comparison of cardiopulmonary bypass versus extracorporeal membrane oxygenation: Does intraoperative circulatory support strategy affect outcomes in pediatric lung transplantation?

OBJECTIVE: Data on adult lung transplantation suggest perioperative benefits of intraoperative extracorporeal membrane oxygenation (ECMO) compared to cardiopulmonary bypass (CPB). Information regarding their pediatric counterparts, however, is limited. This study compares outcomes of intraoperative ECMO versus CPB in pediatric lung transplantation. METHODS: We reviewed all pediatric lung transplants at our institution from 2014 to 2019 and compared those supported intraoperatively on ECMO (n = 13) versus CPB (n = 22), plus a conditional analysis excluding re-transplantations (ECMO [n = 13] versus CPB [n = 20]). We evaluated survival, surgical times, intraoperative transfusions, postoperative support, complications, and duration of hospitalization. RESULTS: Total time on ECMO support was significantly less than that of CPB support (P = .018). Intraoperatively, the ECMO group required fewer transfusions of fresh-frozen plasma (8.9 [5.8-22.3] vs 16.6 [11.4-39.0] mL/kg, P = .049) and platelets (4.2 [0.0-6.7] vs 8.0 [3.5-14.0] mL/kg, P = .049). When excluding re-transplantations, patients on ECMO required fewer packed red blood cells intraoperatively (12.6 [2.1-30.7] vs 28.2 [14.0-54.0] mL/kg, P = .048). There were no differences in postoperative support requirements, complications, or mortality at one, six, and twelve months. CONCLUSIONS: Intraoperative ECMO support during pediatric lung transplantation appears to decrease intraoperative transfusion requirements when compared to CPB. Data from additional institutions may strengthen these observations.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

BACKGROUND: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention. OBJECTIVES: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies. METHODS: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment. RESULTS: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002). CONCLUSIONS: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

Symptom persistence after vascular ring repair in children.

PURPOSE: Vascular rings are often diagnosed after evaluation for swallowing and breathing difficulties. Data regarding symptoms following vascular ring repair is sparse. We sought to determine whether symptoms persist using chart review and a survey. METHODS: Sixty-three patients underwent open vascular ring repair from July 2007 to May 2018. Data regarding vascular anatomy, demographics, pre- and postoperative symptoms, and chromosomal abnormalities were collected. Freedom from reoperation, 30-day mortality, and complications were assessed. Patient families were contacted for a symptom focused survey. RESULTS: The median age of surgical intervention was 14.4 months (IQR 5.8-34.7 months) for single aortic arches with an aberrant subclavian artery (SAA), and 5.3 months (IQR 1.3-10.1 months) for double aortic arches (DAA) (Table). Prior to surgery, all but two SAA were symptomatic. There was no operative mortality. Three patients required re-exploration for chylothorax, and three required late aortopexy. At last follow-up, 45% (18/40) SAA and 65% (15/23) DAA had post-operative symptoms. Fourteen patient families completed the symptom survey (10 SAA, 4 DAA). Five SAA had breathing and swallowing symptoms, and 3 SAA and 3 DAA had breathing difficulties. CONCLUSIONS: Open vascular ring repair remains a safe repair. However, further investigation of the persistent symptoms in these patients is merited. STUDY TYPE / LEVEL OF EVIDENCE: Retrospective Comparative Study, Level III.