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In a recent trial, tricuspid annuloplasty (TA) during mitral valve surgery (MVS) for degenerative mitral regurgitation and moderate or less tricuspid regurgitation (TR) reduced the composite rate of death, reoperation for TR, or TR progression at 2 years. However, this benefit was counterbalanced by an increase in implantation of permanent pacemakers (PPMs). In this study, we analyzed the timing, indications, and risk factors for these implantations. We randomized 401 patients (MVS alone = 203; MVS + TA = 198). Potential risk factors for PPMs were assessed using multivariable time-to-event models with death and PPM implantation for heart failure indications as competing risks. A PPM was implanted in 36 patients (9.6; 95% CI, 6.8-13.0) within 2 years of randomization, with 30/187 (16.0%) in the MVS + TA and 6/188 (3.2%) in the MVS groups (rate ratio, 5.08; 95% CI, 2.16-11.94; P <.001). Most (29/36; 80.6%) implantations occurred within 30 days postoperatively. Independent risk factors for PPM implantation within 2 years were TA (hazard ratio [HR], 5.94; 95% CI, 2.27-15.53; P <.001), increasing age (5 years, HR, 1.23; 95% CI, 1.01-1.52; P =.04), and left ventricular ejection fraction (LVEF; HR, 0.96; 95% CI, 0.92-0.99; P =.02). In the subset of TA recipients (n = 197), age (5 years, HR, 1.05; 95% CI, 1.00-1.10; P =.04) and LVEF (HR, 0.95; 95% CI, 0.91-0.99; P =.01) were associated with PPM within 2 years. Concomitant TA, age, and baseline LVEF were risk factors for PPM implantation in patients who underwent MVS for degenerative mitral regurgitation. Although TA was effective in preventing progression of TR, innovation is needed to identify ways to decrease PPM implantation rates. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: In an effort to optimize neurologic outcome, cerebral oxygen saturation (Sco 2) is often measured intraoperatively and postoperatively. We hypothesized that Sco 2 would be related to stage of palliation in children with single ventricle congenital heart disease. Methods: Cerebral oxygen saturation was continuously recorded intraoperatively in 34 infants and children undergoing palliative surgery on cardiopulmonary bypass for single ventricle congenital heart defects and in a control group of 12 neonates with ductus-dependent circulation undergoing complete repair. Saturations were correlated with the patient’s stage and outcome. Results: Baseline Sco 2 was 61% in single ventricle neonates (group P1, n = 10), 55% in neonates undergoing repair (group R), 42% in infants undergoing stage 2 palliation (group P2, n = 6), and 70% in children undergoing Fontan (group P3, n = 14). Baseline was lowest (41%) in infants undergoing interstage operations (group I, n = 4). After bypass, there was a significant improvement in Sco 2 to 53% in group P2 infants (p = 0.04); there were no significant changes in the other groups. By the end of the operation, there was a significant decrease in Sco 2 to 48% in group P1 (p = 0.001), with other groups unchanged from baseline. There were five perioperative deaths. Cerebral oxygen saturation at the conclusion of surgery was lower in children who died (38% versus 61%, p = 0.01). Conclusions: In children with single ventricle physiology, Sco 2 decreases after initial palliation, remains low before second-stage palliation, but is normal before and after the Fontan. This has implications for perioperative mortality, neurologic injury, and potentially for interim mortality. Low postoperative Sco 2 predicts perioperative mortality. [Copyright &y& Elsevier]

Background: Recent work has focused attention on interim mortality (death after hospital discharge and before second-stage surgery) in hypoplastic left heart syndrome. This study investigates interim mortality in infants undergoing systemic-to-pulmonary artery shunts for pulmonary atresia with intact ventricular septum. Methods: At two centers in 11 years (January 1991 through December 2001), 35 infants underwent placement of shunts for palliation of pulmonary atresia with intact septum. Patients were identified from the cardiology database at each institution, and data were collected retrospectively. The infants were classified into two groups, with and without severe right ventricular hypoplasia, based on the initial surgical plan (Fontan or two-ventricle repair). Results: The mean age and weight of the infants were 9 days and 3.1 kg. The right ventricle was severely hypoplastic in 22 of 35 infants. Hospital death occurred in 2 patients (9.1%), 1 with severe right ventricular hypoplasia. The remaining 33 patients form the study population. There were a total of 5 deaths (15%) after discharge and before second-stage operation, all in patients with severe right ventricular hypoplasia. Two patients, 1 with hypoplastic right ventricle, died after second-stage operation. Conclusions: These data confirm a significant incidence of interim death in infants with pulmonary atresia and hypoplastic right ventricle. The interim mortality rate in the current two-institution study of infants with pulmonary atresia with intact ventricular septum is similar to that in hypoplastic left heart syndrome if all patients are considered (15%), and is somewhat higher (24%) than that for hypoplastic left heart syndrome if only patients with severe right ventricular hypoplasia are considered. This rate of interim death must be considered when different treatment options (such as shunt or transplant) are contemplated. [Copyright &y& Elsevier]

: BackgroundGraft failure after cardiac transplantation in children can be managed acutely with mechanical support, most commonly extracorporeal membrane oxygenation (ECMO). The purpose of this study was to evaluation the long-term outcome of ECMO support early and late after pediatric cardiac transplantation.: MethodsFrom February 1982 through October 2002, 168 patients underwent isolated cardiac transplantation. Twenty patients (11.9%) required mechanical support early or late after transplantation. Inpatient and outpatient records of these were reviewed.: ResultsIndication for transplantation was complex congenital heart disease in 12, cardiomyopathy in 7, and graft failure (retransplant) in 1. One patient was also on ECMO preoperatively. Fifteen patients required circulatory support immediately or shortly (less than 6 weeks) after transplantation. The remaining 5 patients were placed on ECMO for ventricular dysfunction late (3 months to 7 years) after transplantation. In the perioperative ECMO group, 8 (53%) were successfully decannulated and subsequently discharged. Three of 5 (60%) patients placed on ECMO late were successfully decannulated, 1 of whom died in hospital 10 days later and 2 of whom are still alive.: ConclusionsMechanical circulatory support using ECMO can be a useful strategy is the management of graft dysfunction after pediatric cardiac transplantation both early and late postoperatively. The mortality rate is acceptable in this very high risk group of patients and long-term outcome is good. [Copyright &y& Elsevier]

: BackgroundThis retrospective review examines the risks and causes of death in infants with aortopulmonary shunts between the time of hospital discharge and planned reintervention.: MethodsFrom January 1991 through December 2000, a total of 146 infants aged 60 days or less underwent placement of systemic-to–pulmonary artery shunts and were discharged from the hospital alive. Inpatient, outpatient, and autopsy records were reviewed.: ResultsIndications for surgery were single-ventricle anatomy in 90 cases and complex double-ventricle anatomy in 56. Of the patients, 21 (14%) died after discharge and before further planned surgery. Of these 21 infants, 17 (81%) were clinically doing well before sudden death. Autopsies were obtained in 15 cases and attributed the cause of death to shunt thrombosis in 5 infants (33%), myocardial infarction in 2 (13%), and pneumonia or lung disease in 3. Five autopsies were nondiagnostic. The mortality of patients discharged on aspirin (11.1%) was almost identical to that of patients discharged on no anticoagulation (12.3%). Four infants with sudden death had been notably irritable for 24 to 48 hours before death.: ConclusionsThere is a significant incidence of sudden death among infants who have undergone shunting. Death may be preceded by unexplained irritability, and such symptoms should therefore be carefully evaluated. Autopsy-proven shunt thrombosis is one of the leading causes of interim sudden death, and aspirin therapy may not be helpful. Options to reduce interim mortality include alternative regimens of anticoagulation (such as low–molecular weight heparin), alternative conduit material, and earlier reoperation. [Copyright &y& Elsevier]

Tricuspid valve annuloplasty (TA) during mitral valve repair (MVr) is associated with increased risk of permanent pacemaker (PPM) implantation, but the magnitude of risk and long-term clinical consequences have not been firmly established. This study assesses the incidence rates of PPM implantation after isolated MVr and following MVr with TA as well as the associated long-term clinical consequences of PPM implantation. State-mandated hospital discharge databases of New York and California were queried for patients undergoing MVr (isolated or with concomitant TA) between 2004 and 2019. Patients were stratified by whether or not they received a PPM within 90 days of index surgery. After weighting by propensity score, survival, heart failure hospitalizations (HFHs), endocarditis, stroke, and reoperation were compared between patients with or without PPM. A total of 32,736 patients underwent isolated MVr (n = 28,003) or MVr + TA (n = 4,733). Annual MVr + TA volumes increased throughout the study period (P < 0.001, trend), and PPM rates decreased (P < 0.001, trend). The incidence of PPM implantation <90 days after surgery was 7.7% for MVr and 14.0% for MVr + TA. In 90-day conditional landmark-weighted analyses, PPMs were associated with reduced long-term survival among MVr (HR: 1.96; 95% CI: 1.75-2.19; P < 0.001) and MVr + TA recipients (HR: 1.65; 95% CI: 1.28-2.14; P < 0.001). In both surgical groups, PPMs were also associated with an increased risk of HFH (HR: 1.56; 95% CI: 1.27-1.90; P < 0.001) and endocarditis (HR: 1.95; 95% CI: 1.52-2.51; P < 0.001), but not with stroke or reoperation. Compared to isolated MVr, adding TA to MVr was associated with a higher risk of 90-day PPM implantation. In both surgical groups, PPM implantation was associated with an increase in mortality, HFH, and endocarditis. [Display omitted] [ABSTRACT FROM AUTHOR]

The National Heart, Lung, and Blood Institute convened a workshop in August 2021 to identify opportunities in pediatric and congenital cardiovascular research that would improve outcomes for individuals with congenital heart disease across the lifespan. A subsidiary goal was to provide feedback on and visions for the Pediatric Heart Network. This paper summarizes several key research opportunities identified in the areas of: data quality, access, and sharing; aligning cardiovascular research with patient priorities (eg, neurodevelopmental and psychological impacts); integrating research within clinical care and supporting implementation into practice; leveraging creative study designs; and proactively enriching diversity of investigators, participants, and perspectives throughout the research process. [ABSTRACT FROM AUTHOR]