Tricuspid regurgitation (TR), back flow of blood from the right ventricle to the right atrium, has been reported in approximately 85% of the population, with 16% having mild or severe TR. Patients with untreated moderate to severe TR are likely to experience decreased exercise capacity and have increased morbidity and mortality, thus affecting the patient's quality of life. Current methods of repair offer limited rates of success, and many patients require further operations to correct returning levels of TR. Incomplete repair may be due to incomplete understanding of the functional anatomy and mechanics of the TV and the underlying causes of TR. It was hypothesized that alterations in the geometry of tricuspid valve annular and subvalvular apparatus induced by ventricular dilatation determine the severity of TR. In vivo measurements of papillary muscle (PM) position in patients with single or biventricular dilatation revealed PM displacement away from the center of the annulus as compared to control patients. Additionally, pulmonary arterial pressure, annulus area, ventricular size and apical displacement of the anterior PM were highly correlated with the severity of TR. An in vitro right-heart simulator was developed to investigate isolated mechanics of TR. Through these in vitro studies it was demonstrated that the tricuspid valve begins to leak at only 40% dilation, much lower than the mitral valve. Additionally, it was shown that isolated PM displacement resulted in significant TR. The highest levels of TR were achieved with a combination of annular dilatation and PM displacement. Alterations in leaflet coaptation, as quantified by measuring the amount of leaflet available for coaptation and leaflet mobility were observed with annular dilatation and PM displacement, both isolated and combined. The changes in leaflet coaptation resulted in redistribution of the forces on the chords originating from the anterior PM and inserting into the anterior and posterior leaflets. The findings herein provide the clinical and scientific community with a mechanistic understanding of the tricuspid valve to further improve intervention and repair of TV disease.

Pacemaker implantation can be associated with several complications, including myocardial perforation with or without pericardial effusion, venous thrombosis, vegetations of the tricuspid valve (TV) or pacing lead, and tricuspid regurgitation (TR). The TR is thought to be derived from deformity or perforation of the TV by the pacing lead or secondary to atrioventricular discordance with asynchronous ventricular pacing. Severe TR can be deleterious to the patient because it raises the central venous pressure by increasing the right sided preload. Chronically, the increase in right sided blood volume can result in an increase in the right atrial pressure leading to a decrease in venous return and low cardiac output. Severe TR from leaflet adhesion to the pacemaker lead has not been reported before. With the aging of the population and the expanding use of pacemakers and implantable cardioverter defibrillators (ICD) in clinical practice, this complication may be seen more frequently. We present a patient diagnosed with severe TR, years after his pacemaker implantation. His TR was thought to be caused by adhesion of the tricuspid valve to his pacemaker lead.

Objectives A substantial number of patients with severe tricuspid regurgitation (TR) and congestive heart failure (CHF) are medically managed without undergoing corrective surgery. We sought to assess the characteristics and outcomes of CHF patients who underwent tricuspid valve surgery (TVS), compared with those who did not. Methods Retrospective observational study involving 2556 consecutive patients with severe TR from the Cleveland Clinic Echocardiographic Database. Cardiac transplant patients or those without CHF were excluded. Survival difference between patients who were medically managed versus those who underwent TVS was compared using Kaplan-Meier survival curves. Multivariate analysis was performed to identify variables associated with poor outcomes. Results Among a total of 534 patients with severe TR and CHF, only 55 (10.3%) patients underwent TVS. Among the non-surgical patients (n=479), 30% (n=143) had an identifiable indication for TVS. At 38 months, patients who underwent TVS had better survival than those who were medically managed (62% vs 35%; p<0.001). On multivariate analysis, advancing age (HR: 1.23; 95% CI 1.12 to 1.35 per 10-year increase in age), moderate (HR: 1.39; 95% CI 1.01 to 1.90) and severe (HR: 2; 95% CI 1.40 to 2.80) right ventricular dysfunction were associated with higher mortality. TVS was associated with lower mortality (HR: 0.44; 95% CI 0.27 to 0.71). Conclusion Although corrective TVS is associated with better outcomes in patients with severe TR and CHF, a substantial number of them continue to be medically managed. However, since the reasons for patients not being referred to surgery could not be ascertained, further randomised studies are needed to validate our findings before clinicians can consider surgical referral for these patients. / Revisión por pares

Background Medically managed tricuspid regurgitation (TR) has detrimental outcomes. Transcatheter tricuspid valve interventions (TTVIs) represent an alternative to surgery in high-risk patients; however, only early experiences exist. Aim The aim of this study was to analyze the clinical and echocardiographic outcomes of TTVI. Methods MEDLINE, ISI Web of Science, and SCOPUS databases were searched for studies published up to June 2021. Studies reporting data on outcome post-TTVIs were included. This study was designed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) requirements. The primary endpoint was all-cause mortality at 30-day and 1-year post-TTVI. Results Out of 2,718 studies, 27 were included. Notably, 30-day and 1-year all-cause mortalities were 5% (95% confidence interval [CI]: 4–8%, p < 0.001) and 25% (95% CI: 12–45%, p = 0.016). Procedural success was associated with a 58% risk reduction in 1-year mortality vs. lack thereof (odds ratio 0.42, 95% CI: 0.27–0.66, p < 0.001). TTVI is associated with a significant reduction in TR severity (TR EROA, mean difference [MD] 0.31 cm2; 95% CI: 0.23–0.39 cm2, p < 0.001; regurgitant volume, MD 23.54 ml; 95% CI: 17.4–29.68 ml, p = 0.03) and increase in forward stroke volume (FSV, MD 3.98 ml; 95% CI: 0.11–7.86 ml, p = 0.04). Conclusion TTVI significantly reduces TR severity and increases FSV and is associated with improved survival at 1 year compared with patients without procedural success. Long-term outcomes compared with medical therapy await the results of ongoing pivotal trials; nonetheless, TTVIs appear to be a promising alternative to surgery for TR.

Aims To investigate whether there is evidence for distinct cardiac output (CO) based phenotypes in patients with chronic right heart failure associated with severe tricuspid regurgitation (TR) and to characterize their impact on TR treatment and outcome. Methods and results A total of 132 patients underwent isolated transcatheter tricuspid valve repair (TTVR) for functional TR at two centres. Patients were clustered according to k-means clustering into low [cardiac index (CI) < 1.7 L/min/m2], intermediate (CI 1.7–2.6 L/min/m2) and high CO (CI > 2.6 L/min/m2) clusters. All-cause mortality and clinical characteristics during follow-up were compared among different CO clusters. Mortality rates were highest for patients in a low (24%) and high CO state (42%, log-rank P < 0.001). High CO state patients were characterized by larger inferior vena cava diameters (P = 0.003), reduced liver function, higher incidence of ascites (P = 0.006) and markedly reduced systemic vascular resistance (P < 0.001) as compared to TTVR patients in other CO states. Despite comparable procedural success rates, the extent of changes in right atrial pressures (P = 0.01) and right ventricular dimensions (P < 0.001) per decrease in regurgitant volume following TTVR was less pronounced in high CO state patients as compared to other CO states. Successful TTVR was associated with the smallest prognostic benefit among low and high CO state patients. Conclusions Patients with chronic right heart failure and severe TR display distinct CO states. The high CO state is characterized by advanced congestive hepatopathy, a substantial decrease in peripheral vascular tone, a lack of response of central venous pressures to TR reduction, and worse prognosis. These data are relevant to the pathophysiological understanding and management of this important clinical syndrome. Graphical Abstract Proposed mechanism of hypercirculatory tricuspid regurgitation. Tricuspid regurgitation related backward failure causes liver congestion and dysfunction with portal hypertension and reduced washout of vasoactive substances. Consequent splanchnic and peripheral vasodilatation alongside with reduced renal blood flow results in renin–angiotensin–aldosterone system (RAAS) activation and sympathetic overactivation. The sympathetic drive and volume retention lead to further capacitance depletion and volume overload, eventually resulting in a high cardiac output state, with limited preload reduction and prognostic benefit following transcatheter tricuspid valve repair. The alterations in the graph should be interpreted as simultaneous interaction rather than a timeline. Continuous lines indicate findings in the present study. Dashed lines express currently accepted mechanistical considerations. AP, alkaline phosphatase; γGT, gamma-glutamyl-transferase; RA, right atrium; RV, right ventricle.

Hyperthyroidism may present with signs and symptoms related to dysfunction of a variety of organs. Cardiovascular pathology in hyperthyroidism is common. A few case reports describe isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension as the prominent cardiovascular manifestations of hyperthyroidism. Although most textbooks do not mention hyperthyroidism as a cause of pulmonary hypertension and isolated right heart failure, the literature suggests that some hyperthyroid patients may develop reversible pulmonary hypertension and isolated right heart failure. We report a case of hyperthyroidism presenting with signs and symptoms of isolated right heart failure, tricuspid regurgitation, and pulmonary hypertension, which resolved with treatment of hyperthyroidism.

Introdução: As principais operações para correção da anomalia de Ebstein baseiam-se na reconstrução da valva atrioventricular direita (AVD) em formato monovalvular, tendo resultados comprometidos pela necessidade de substituição ou alta reincidência de insuficiência valvar. Uma nova técnica foi desenvolvida, diferenciado-se das anteriores, pela correção anatômica da valva AVD, resultando na coaptação plena das válvulas no fechamento valvar. O objetivo deste estudo é avaliar a aplicabilidade dessa técnica, estudando os seus efeitos na evolução clínica, função da valva, restauração do ventrículo direito funcional e remodelamento reverso do coração no pós-operatório imediato (POI) e no pós-operatório em longo prazo (POL). Métodos: Estudo retrospectivo de 52 pacientes consecutivos, com idade média de 18,5±13,8anos, submetidos técnica do cone para correção da anomalia de Ebstein, entre novembro 1993 e dezembro de 2006, cujos principais detalhes cirúrgicos são: as válvulas anterior e posterior da valva AV direita são mobilizadas das suas implantações anômalas no ventrículo direito (VD), a borda livre desse conjunto é rodada no sentido horário para ser suturada à, previamente mobilizada, borda septal da válvula anterior, formando um cone cujo vértice permanece fixo ao ápice do VD e a base é suturada no nível do anel atrioventricular verdadeiro, reduzido ao mesmo tamanho da base do cone. A válvula septal, sempre que possível, é incorporada à parede do cone. A porção atrializada do VD é reduzida por plicatura longitudinal. Os dados clínicos, ecocardiográficos e os índices cardiotorácicos, obtidos nos períodos pré-operatório (PREOP) e pós-operatório, foram analisados. Resultados: Houve dois óbitos hospitalares (3,8 %) e dois óbitos no seguimento em longo prazo A melhora clínica foi significante, sendo a distribuição dos pacientes em classes funcionais de insuficiência cardíaca (NYHA), IV = 4, III = 27, II = 11 e I = 5 no PREOP, modificada para IV = 0, III = 1, II = 2 e I = 44 no pós-operatório em longo prazo (POL) (p<0,0001), com seguimento médio de 57 meses. Quatro pacientes foram reoperados, sendo realizada nova plastia valvar. O índice cardiotorácico de 0,66±0,09 no PREOP diminuiu para 0,54±0,06 no POL (p<0,0001). Os ecocardiogramas mostraram redução dos graus de insuficiência da valva AV direita (p<0,001), sendo a distribuição dos pacientes no pré-operatório, grau 1 = 0, grau 2 = 1, grau 3 = 15, grau 4 = 24, modificada para grau 1 = 19, grau 2 = 17, grau 3 = 4, grau 4 = 0 no POI, com pequena alteração no POL (grau 1 = 11, grau 2 = 22, grau 3 = 7, grau 4 = 0). A cavidade funcional do VD foi restaurada pela operação, ocorrendo aumento da área do VD funcional indexada de 8,53± 7,02 cm2/m2 no PREOP para 21,01±6,87 cm2/m2 no POI (p<0,001), e ficando inalterada em 20,28±5,26 cm2/m2 no POL (p>0,05). Conclusões: Esta técnica foi aplicável com baixa mortalidade hospitalar e sem necessidade de substituição valvar. Houve melhora clínica pós-operatória e baixa incidência de reoperações em longo prazo. A correção da insuficiência valvar foi eficaz e duradoura na maioria dos pacientes. Houve restauração da área funcional do VD e remodelamento reverso do coração. / Background: The main operations for Ebsteins anomaly repair are conceived to reconstruct the tricuspid valve (TV) in a monocusp format, but their results are restricted either by the need for valve replacement or by high incidence of postoperative valve regurgitation. A new surgical technique was developed, that performs an anatomical reconstruction of the tricuspid valve, realizing a leaflet-to-leaflet coaptation at the TV closure. The objective of this study is to access the feasibility of this technique, evaluating its effects in clinical outcome, tricuspid valve function, right ventricle (RV) morphology and reverse remodeling of the heart.Methods: Retrospective study on 52 consecutive patients, mean age of 18,5+- 13,8 years, treated with a new surgical technique for Ebsteins anomaly repair (the cone technique), between November 1993 and December 2006, which principal details are: a) the anterior and posterior tricuspid valve leaflets re mobilizedfrom their anomalous attachments in the RV, the free edge of this complex is rotated clockwise to be sutured to the septal border of anterior leaflet, creatind a cone which vertex remains fixed at RV apex and whose base is the sutured to a true tricuspid annulus, plicated to match it to base of said cone. The septal leaflet is incorporated into the cone wall ewhenever possible. The atrialized chamber is reduced by longitudinal placation. The clinical and echocardiographic data and the patients cardiothoracic ratios, collected at the preoperative, early and late postoperative periods, were analyzed. Results: There were two hospital deaths (3.8 %) and two more deaths in the long term followup. The significant clinical improvement was evident by the change of patients functional class of heart failure (NYHA) from IV=4, III=27, II=11 and I=5, in the preoperative to IV =0, III = 1, II = 2 e I = 44 at 57 months mean long term follow-up (p<0,0001). Four patients required late TV re-repair. Atrioventricular block did not occur and there was no need for tricuspid valve replacement at any time. The cardiothoracic ratio decreased from 0,66+-0,09, preoperatively, to 0,54+-0,06 in long term follow-up (p<0,001). Echocardiographic studies showed significant TV insufficiency reduction from the preoperative patient distribution of: grade 1 = 0, grade 2 = 1, grade 3 = 15, grade 4 = 24, modified to: grade 1 = 19, grade 2 = 17, grade 3 = 4, grade 4 = 0 on early postoperative period (p<0.001), with little change afterwards (grade 1 = 11, grade 2 = 22, grade 3 = 7, grade 4 = 0). The normal RV morphology was surgically restored, indicated by the enlargement of RV indexed area from 8.53+-7.02 cm2/m2, preoperatively to 21.01+-6.87 cm2/m2 in the early perioperative period (p<0.001), remaining unchanged, 20.28+-5.26 cm2/m2 in long term echocardiogram (p>0,05). Conclusions: This operative technique was feasible with low hospital mortality and no need for TV replacement. There was improvement in the patients clinical status and low incidence of reoperations in long term follow-up. The TV repair was efficacious and durable for the great marjority of patients and there was immediate RV morphology restoration and reverse remodeling of heart in long term follow-up

Introdução: As principais operações para correção da anomalia de Ebstein baseiam-se na reconstrução da valva atrioventricular direita (AVD) em formato monovalvular, tendo resultados comprometidos pela necessidade de substituição ou alta reincidência de insuficiência valvar. Uma nova técnica foi desenvolvida, diferenciado-se das anteriores, pela correção anatômica da valva AVD, resultando na coaptação plena das válvulas no fechamento valvar. O objetivo deste estudo é avaliar a aplicabilidade dessa técnica, estudando os seus efeitos na evolução clínica, função da valva, restauração do ventrículo direito funcional e remodelamento reverso do coração no pós-operatório imediato (POI) e no pós-operatório em longo prazo (POL). Métodos: Estudo retrospectivo de 52 pacientes consecutivos, com idade média de 18,5±13,8anos, submetidos técnica do cone para correção da anomalia de Ebstein, entre novembro 1993 e dezembro de 2006, cujos principais detalhes cirúrgicos são: as válvulas anterior e posterior da valva AV direita são mobilizadas das suas implantações anômalas no ventrículo direito (VD), a borda livre desse conjunto é rodada no sentido horário para ser suturada à, previamente mobilizada, borda septal da válvula anterior, formando um cone cujo vértice permanece fixo ao ápice do VD e a base é suturada no nível do anel atrioventricular verdadeiro, reduzido ao mesmo tamanho da base do cone. A válvula septal, sempre que possível, é incorporada à parede do cone. A porção atrializada do VD é reduzida por plicatura longitudinal. Os dados clínicos, ecocardiográficos e os índices cardiotorácicos, obtidos nos períodos pré-operatório (PREOP) e pós-operatório, foram analisados. Resultados: Houve dois óbitos hospitalares (3,8 %) e dois óbitos no seguimento em longo prazo A melhora clínica foi significante, sendo a distribuição dos pacientes em classes funcionais de insuficiência cardíaca (NYHA), IV = 4, III = 27, II = 11 e I = 5 no PREOP, modificada para IV = 0, III = 1, II = 2 e I = 44 no pós-operatório em longo prazo (POL) (p<0,0001), com seguimento médio de 57 meses. Quatro pacientes foram reoperados, sendo realizada nova plastia valvar. O índice cardiotorácico de 0,66±0,09 no PREOP diminuiu para 0,54±0,06 no POL (p<0,0001). Os ecocardiogramas mostraram redução dos graus de insuficiência da valva AV direita (p<0,001), sendo a distribuição dos pacientes no pré-operatório, grau 1 = 0, grau 2 = 1, grau 3 = 15, grau 4 = 24, modificada para grau 1 = 19, grau 2 = 17, grau 3 = 4, grau 4 = 0 no POI, com pequena alteração no POL (grau 1 = 11, grau 2 = 22, grau 3 = 7, grau 4 = 0). A cavidade funcional do VD foi restaurada pela operação, ocorrendo aumento da área do VD funcional indexada de 8,53± 7,02 cm2/m2 no PREOP para 21,01±6,87 cm2/m2 no POI (p<0,001), e ficando inalterada em 20,28±5,26 cm2/m2 no POL (p>0,05). Conclusões: Esta técnica foi aplicável com baixa mortalidade hospitalar e sem necessidade de substituição valvar. Houve melhora clínica pós-operatória e baixa incidência de reoperações em longo prazo. A correção da insuficiência valvar foi eficaz e duradoura na maioria dos pacientes. Houve restauração da área funcional do VD e remodelamento reverso do coração. / Background: The main operations for Ebsteins anomaly repair are conceived to reconstruct the tricuspid valve (TV) in a monocusp format, but their results are restricted either by the need for valve replacement or by high incidence of postoperative valve regurgitation. A new surgical technique was developed, that performs an anatomical reconstruction of the tricuspid valve, realizing a leaflet-to-leaflet coaptation at the TV closure. The objective of this study is to access the feasibility of this technique, evaluating its effects in clinical outcome, tricuspid valve function, right ventricle (RV) morphology and reverse remodeling of the heart.Methods: Retrospective study on 52 consecutive patients, mean age of 18,5+- 13,8 years, treated with a new surgical technique for Ebsteins anomaly repair (the cone technique), between November 1993 and December 2006, which principal details are: a) the anterior and posterior tricuspid valve leaflets re mobilizedfrom their anomalous attachments in the RV, the free edge of this complex is rotated clockwise to be sutured to the septal border of anterior leaflet, creatind a cone which vertex remains fixed at RV apex and whose base is the sutured to a true tricuspid annulus, plicated to match it to base of said cone. The septal leaflet is incorporated into the cone wall ewhenever possible. The atrialized chamber is reduced by longitudinal placation. The clinical and echocardiographic data and the patients cardiothoracic ratios, collected at the preoperative, early and late postoperative periods, were analyzed. Results: There were two hospital deaths (3.8 %) and two more deaths in the long term followup. The significant clinical improvement was evident by the change of patients functional class of heart failure (NYHA) from IV=4, III=27, II=11 and I=5, in the preoperative to IV =0, III = 1, II = 2 e I = 44 at 57 months mean long term follow-up (p<0,0001). Four patients required late TV re-repair. Atrioventricular block did not occur and there was no need for tricuspid valve replacement at any time. The cardiothoracic ratio decreased from 0,66+-0,09, preoperatively, to 0,54+-0,06 in long term follow-up (p<0,001). Echocardiographic studies showed significant TV insufficiency reduction from the preoperative patient distribution of: grade 1 = 0, grade 2 = 1, grade 3 = 15, grade 4 = 24, modified to: grade 1 = 19, grade 2 = 17, grade 3 = 4, grade 4 = 0 on early postoperative period (p<0.001), with little change afterwards (grade 1 = 11, grade 2 = 22, grade 3 = 7, grade 4 = 0). The normal RV morphology was surgically restored, indicated by the enlargement of RV indexed area from 8.53+-7.02 cm2/m2, preoperatively to 21.01+-6.87 cm2/m2 in the early perioperative period (p<0.001), remaining unchanged, 20.28+-5.26 cm2/m2 in long term echocardiogram (p>0,05). Conclusions: This operative technique was feasible with low hospital mortality and no need for TV replacement. There was improvement in the patients clinical status and low incidence of reoperations in long term follow-up. The TV repair was efficacious and durable for the great marjority of patients and there was immediate RV morphology restoration and reverse remodeling of heart in long term follow-up

Objectives We investigated the durability of tricuspid regurgitation (TR) reduction and the clinical outcomes through 12 months after transcatheter tricuspid valve repair (TTVr) with the PASCAL Transcatheter Valve Repair System. Background TTVr has rapidly developed and demonstrated favorable acute outcomes, but longer follow-up data are needed. Methods Overall, 30 patients (age 77 ± 6 years; 57% female) received PASCAL implantation from September 2017 to May 2019 and completed a clinical follow-up at 12 months. Results The TR etiology was functional in 25 patients (83%), degenerative in three (10%), and mixed in two (7%). All patients had TR severe or greater (massive or torrential in 80%) and heart failure symptoms (90% in NYHA III or IV) under optimal medical treatment. Single-leaflet device attachment occurred in two patients. Moderate or less TR was achieved in 23/28 patients (82%) at 30 days, which was sustained at 12 months (86%). Two patients underwent repeat TTVr due to residual torrential TR (day 173) and recurrence of severe TR (day 280), respectively. One-year survival rate was 93%; 6 patients required rehospitalization due to acute heart failure. NYHA functional class I or II was achieved in 90% and 6-minute walk distance improved from 275 ± 122 m at baseline to 347 ± 112 m at 12-month (+72 ± 82 m, p < .01). There was no stroke, endocarditis, or device embolization during the follow-up. Conclusions Twelve-month outcomes from this multicenter compassionate use experience with the PASCAL System demonstrated high procedural success, acceptable safety, and significant clinical improvement.

Background: The etiology of congenital heart valve defects is not well understood; little is known about the risk factors that contribute to the survival and mortality outcomes of children with these defects. Methods: Using data from the Texas Birth Defects Registry (TBDR) we conducted a retrospective cohort study of 2070 singleton infants with congenital aortic, pulmonary, or tricuspid valve atresia or stenosis born in Texas between January 1, 1996 and December 31, 2007 to Hispanic, Non-Hispanic (NH) black, and NH white women. TBDR data were death-to-birth matched by the Texas Vital Statistics Unit for deaths between January 1, 1996 and December 31, 2008. Using Kaplan-Meier survival estimates with log rank tests and Cox proportional hazards regression model hazard ratios (HR) with 95% confidence intervals (CI), we examined whether infant sex and maternal race/ethnicity affected early childhood survival or risk of mortality for children with congenital heart valve defects. Covariates included birth weight and gestational age, maternal age, maternal education, and number of co-occurring birth defects. Results: In children with aortic valve atresia and aortic valve stenosis, we found males had higher early childhood survival than females (55.0% vs. 41.5%, P=0.0451 and 91.6% vs. 82.5%, P=0.0492, respectively). Early childhood survival for males (94.9%) with pulmonary valve stenosis was slightly lower than females (97.1%, P=0.0116), and was also lower for NH black (94.1%) and Hispanic (95.3%) children than NH white children (97.8%, P=0.0340). After adjusting for covariates, early childhood mortality in children with pulmonary valve atresia with hypoplastic right ventricle was greater in NH black than NH white children (HR=2.93, CI 1.09-7.85, P=0.0329) and greater in NH black males than NH white males (HR=4.63, CI 1.12-19.19, P=0.0349). For children with tricuspid valve atresia, early childhood survival was lower in NH black males (35.7%) and Hispanic males (64.0%) than NH white males (81.0%, P=0.0269); after adjusting for covariates, risk for early childhood mortality was higher in NH black than NH white children (HR=3.39, CI 1.41-8.13, P=0.0062), and higher in NH black males than NH white males (HR=5.23, CI 133-20.58, P=0.0179). Conclusions: Our findings demonstrate there are disparities in early childhood survival and risk of mortality by infant sex and maternal race/ethnicity for children with congenital heart valve defects. These findings provide a foundation for further investigation to better understand why these disparities exist and what can be done to improve the outcomes for children with these defects.