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1.
Circulation ; 149(21): 1650-1666, 2024 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-38344825

RESUMO

BACKGROUND: Much of our knowledge of organ rejection after transplantation is derived from rodent models. METHODS: We used single-nucleus RNA sequencing to investigate the inflammatory myocardial microenvironment in human pediatric cardiac allografts at different stages after transplantation. We distinguished donor- from recipient-derived cells using naturally occurring genetic variants embedded in single-nucleus RNA sequencing data. RESULTS: Donor-derived tissue resident macrophages, which accompany the allograft into the recipient, are lost over time after transplantation. In contrast, monocyte-derived macrophages from the recipient populate the heart within days after transplantation and form 2 macrophage populations: recipient MP1 and recipient MP2. Recipient MP2s have cell signatures similar to donor-derived resident macrophages; however, they lack signatures of pro-reparative phagocytic activity typical of donor-derived resident macrophages and instead express profibrotic genes. In contrast, recipient MP1s express genes consistent with hallmarks of cellular rejection. Our data suggest that recipient MP1s activate a subset of natural killer cells, turning them into a cytotoxic cell population through feed-forward signaling between recipient MP1s and natural killer cells. CONCLUSIONS: Our findings reveal an imbalance of donor-derived and recipient-derived macrophages in the pediatric cardiac allograft that contributes to allograft failure.


Assuntos
Aloenxertos , Rejeição de Enxerto , Transplante de Coração , Macrófagos , Humanos , Transplante de Coração/efeitos adversos , Macrófagos/metabolismo , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/genética , Masculino , Feminino , Criança , Pré-Escolar , Miocárdio/patologia , Sobrevivência de Enxerto , Lactente , Células Matadoras Naturais/imunologia , Células Matadoras Naturais/metabolismo , Adolescente
2.
JTCVS Open ; 17: 215-228, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38420530

RESUMO

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (-) of PVR and presence (+) versus absence (-) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication-). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR-/indication-). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). Conclusions: Although a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

3.
bioRxiv ; 2024 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-38196630

RESUMO

The right ventricle (RV) is one of the four pumping chambers of the heart, pumping blood to the lungs. In severe forms of congenital heart disease and pulmonary hypertension, the RV is made to pump into the systemic circulation. Such systemic RVs typically display early failure due to pressure overload. In rare cases a systemic RV persists into later decades of life - colloquially called a 'Super RV'. Here we present the single-nucleus transcriptome of a systemic RV from a 60-year-old with congenitally corrected transposition of great arteries (ccTGA). Our data shows two specific signaling pathways enriched in the ccTGA RV myocardium. First, we show increased insulin like growth factor (IGF1) signaling within the systemic RV myocardium: there is increased expression of the main receptor IGFR1 within the cardiomyocytes, and IGF1 ligands within the cardiofibroblasts and macrophages. Second, we find increased VEGF and Wnt9 ligand expression in cardiomyocytes and increased VEGF1R and Wnt9 receptors in endothelial cells, which are implicated in angiogenesis. We show that increased insulin and angiogenesis signaling are potentially beneficial RV adaptations to increased pressure overload. This study of an adult systemic RV provides an important framework for understanding RV remodeling to systemic pressures in congenital heart disease and pulmonary hypertension.

4.
J Thorac Cardiovasc Surg ; 167(4): 1417-1426.e1, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37913838

RESUMO

BACKGROUND: In pediatrics, implantable continuous-flow ventricular assist devices (IC-VAD) are often used as a "temporary" support, bridging children to cardiac transplantation during the same hospital admission. METHODS: We conducted a retrospective review of our consecutive patients undergoing IC-VAD support at a tertiary pediatric heart center between 2008 and 2022. RESULTS: We identified 100 IC-VAD implant encounters: HeartWare HVAD (67; 67%), HeartMate II (17; 17%), and HeartMate 3 (16; 16%). The median (range) age, weight, and body surface area at implantation were 14.1 (3.0-56.5) years, 54.8 (13.3-140) kg, and 1.6 (0.6-2.6) m2, respectively. Cardiomyopathy (58; 58%) was the most common etiology, followed by congenital heart disease (37; 37%, including 13 single ventricle). At 6 months of IC-VAD support, 94 (94%) encounters achieved positive outcomes: ongoing support (59; 59%), transplant (33; 33%), and cardiac recovery (2; 2%). Eighty-two encounters (82%) resulted in home discharge with ongoing VAD support, including 38 (46%, out of 82) requiring readmission and 7 (9%, out of 82) resulting in death. There was a clinically significant decrease in morbidity rates before versus after home discharge: bleeding (1.55 vs 0.06), infection (0.84 vs 0.37), and stroke (0.84 vs 0.15 event per patient-year). Overall, 86 encounters (86%) reached positive end points at the latest follow-up (64 transplant, 15 ongoing support, and 7 recovery). Infection (29%; 4 of 14) was the most common cause of negative outcomes, followed by cerebrovascular accident (21%; 3), and unresolved frailty (21%; 3). The estimated overall survival at 1, 2, and 5 years was 90%, 86%, and 77%, respectively. CONCLUSIONS: This study suggests the feasibility of outpatient management of pediatric IC-VAD support. The ability to offer true long-term support maximizes the potential of IC-VAD support, not limited to a temporary bridging tool for heart transplantation.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Acidente Vascular Cerebral , Criança , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Coração Auxiliar/efeitos adversos , Resultado do Tratamento , Transplante de Coração/efeitos adversos , Estudos Retrospectivos
5.
World J Pediatr Congenit Heart Surg ; 14(5): 575-586, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37737596

RESUMO

Since its establishment in 2001, the Congenital Heart Surgeons' Society John W. Kirklin/David Ashburn Fellowship has contributed substantially to the field of congenital heart surgery research while simultaneously training the next generation of surgeon- scientists. To date, ten fellows (and counting) have successfully completed this rigorous training, producing over 40 published articles focused on longitudinal outcomes from the various Congenital Heart Surgeons' Society cohorts. As the Kirklin/Ashburn Fellowship expands and additional fellows matriculate, its legacy, the network of support, and the contribution to congenital heart surgery research will undoubtedly hold strong.


Assuntos
Cardiopatias Congênitas , Cirurgiões , Humanos , Bolsas de Estudo , Cardiopatias Congênitas/cirurgia
6.
JTCVS Open ; 14: 188-204, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-37425473

RESUMO

Objectives: A quality improvement initiative was introduced to the adult congenital cardiac surgery program at Toronto General Hospital in January 2016. A dedicated Adult Congenital Anesthesia and intensive care unit team was introduced within the cardiac group. The use of factor concentrates was introduced. The study compares perioperative mortality, adverse events, and transfusion burden before and after this process change. Methods: We performed a retrospective analysis of all adult congenital cardiac surgeries from January 2004 to July 2019. Two groups were analyzed: patients undergoing operation before and after 2016. The primary outcome was in-hospital mortality. One-year mortality and prevalence of key morbidities were analyzed as secondary outcomes. A separate analysis looked at patients who had and had not attended an anesthesia-led preassessment clinic. Results: In-hospital mortality was significantly reduced in patients undergoing operation after 2016 (1.1% vs 4.3%, P = .003) despite a higher risk profile. One-year mortality (1.3% vs 5.8%, P = .003) and ventilation times (5.5 hours [3.4-13.0] vs 6.3 hours [4.2-16.2], P = .001) were also reduced. The incidence of stroke and renal failure was similar between groups. Blood product exposure was comparable, but the incidence of chest reopening decreased (1.8% vs 4.8%, P = .022), despite more patients with multiple previous chest wall incisions, on anticoagulation, and with more complex cardiac anatomy. There were no significant outcome differences between those who did or did not attend the preassessment clinic. Conclusions: Both in-hospital and 1-year mortality were significantly reduced after the introduction of a quality improvement program, despite a higher risk profile. Blood product exposure remained unchanged, but there were less chest reopenings.

7.
Circ Cardiovasc Imaging ; 16(6): e015205, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37339175

RESUMO

BACKGROUND: Existing models for prediction of major adverse cardiovascular events (MACE) after repair of tetralogy of Fallot have been limited by modest predictive capacity and limited applicability to routine clinical practice. We hypothesized that an artificial intelligence model using an array of parameters would enhance 5-year MACE prediction in adults with repaired tetralogy of Fallot. METHODS: A machine learning algorithm was applied to 2 nonoverlapping, institutional databases of adults with repaired tetralogy of Fallot: (1) for model development, a prospectively constructed clinical and cardiovascular magnetic resonance registry; (2) for model validation, a retrospective database comprised of variables extracted from the electronic health record. The MACE composite outcome included mortality, resuscitated sudden death, sustained ventricular tachycardia and heart failure. Analysis was restricted to individuals with MACE or followed ≥5 years. A random forest model was trained using machine learning (n=57 variables). Repeated random sub-sampling validation was sequentially applied to the development dataset followed by application to the validation dataset. RESULTS: We identified 804 individuals (n=312 for development and n=492 for validation). Model prediction (area under the curve [95% CI]) for MACE in the validation dataset was strong (0.82 [0.74-0.89]) with superior performance to a conventional Cox multivariable model (0.63 [0.51-0.75]; P=0.003). Model performance did not change significantly with input restricted to the 10 strongest features (decreasing order of strength: right ventricular end-systolic volume indexed, right ventricular ejection fraction, age at cardiovascular magnetic resonance imaging, age at repair, absolute ventilatory anaerobic threshold, right ventricular end-diastolic volume indexed, ventilatory anaerobic threshold % predicted, peak aerobic capacity, left ventricular ejection fraction, and pulmonary regurgitation fraction; 0.81 [0.72-0.89]; P=0.232). Removing exercise parameters resulted in inferior model performance (0.75 [0.65-0.84]; P=0.002). CONCLUSIONS: In this single-center study, a machine learning-based prediction model comprised of readily available clinical and cardiovascular magnetic resonance imaging variables performed well in an independent validation cohort. Further study will determine the value of this model for risk stratification in adults with repared tetralogy of Fallot.


Assuntos
Tetralogia de Fallot , Disfunção Ventricular Direita , Humanos , Adulto , Tetralogia de Fallot/cirurgia , Volume Sistólico , Estudos Retrospectivos , Inteligência Artificial , Função Ventricular Esquerda , Função Ventricular Direita , Imageamento por Ressonância Magnética , Ventrículos do Coração , Aprendizado de Máquina , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/etiologia
8.
Artigo em Inglês | MEDLINE | ID: mdl-37290127
9.
J Am Coll Cardiol ; 81(19): 1937-1950, 2023 05 16.
Artigo em Inglês | MEDLINE | ID: mdl-37164527

RESUMO

BACKGROUND: Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). OBJECTIVES: This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. METHODS: This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. RESULTS: The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). CONCLUSIONS: Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Insuficiência da Valva Pulmonar , Tetralogia de Fallot , Adulto , Adolescente , Humanos , Feminino , Masculino , Tetralogia de Fallot/cirurgia , Qualidade de Vida , Estudos Transversais , Procedimentos Cirúrgicos Cardíacos/métodos
11.
ASAIO J ; 69(5): 429-437, 2023 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-36730653

RESUMO

Heart failure (HF) is common in adult congenital heart disease (ACHD) patients; however, use of continuous-flow ventricular assist devices (CF-VADs) remains rare. We reviewed outcomes of patients with congenital heart disease greater than or equal to 18 years of age at the time of CF-VAD implant at the affiliated pediatric and adult institutions between 2006 and 2020. In total, 18 ACHD patients (15 with great anatomical complexity) received 21 CF-VADs. Six patients (median age 34 years) received seven percutaneous CF-VADs with a median duration of support of 20 days (3-44 days) with all patients survived to hospital discharge and two patients were bridged to durable CF-VADs. Fourteen patients (median age 38 years) received durable CF-VADs. Thirteen patients (93%) survived to hospital discharge and the median duration of support was 25.8 months (6.4-52.1 months). Estimated survival on durable CF-VAD at 1, 3, and 5 years was 84%, 72%, and 36%, respectively. Three patients were successfully bridged to transplantation. Device-related complications include cerebrovascular accident (n = 5), driveline infection (n = 3), device infection requiring chronic antibiotic therapy (n = 4), gastrointestinal bleeding (n = 6), and presumed pump thrombosis (n = 5). These results show percutaneous and durable CF-VADs can support ACHD patients with advanced HF.


Assuntos
Cardiopatias Congênitas , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Criança , Humanos , Adulto , Resultado do Tratamento , Estudos Retrospectivos , Estudos Multicêntricos como Assunto
12.
J Thorac Cardiovasc Surg ; 166(6): 1756-1763.e2, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-36681561

RESUMO

OBJECTIVE: We report the largest pediatric single-center experience with an Impella (Abiomed Inc) catheter-based axial pump support. METHODS: We conducted a retrospective cohort study of all patients with acute decompensated heart failure or cardiogenic shock requiring catheter-based axial pump support between October 2014 and February 2022. The primary outcome per individual encounter (hospital admission) was defined as bridge-to-recovery, bridge-to-durable ventricular assist device support, bridge-to-cardiac transplantation, or death at 6 months after catheter-based axial pump explantation. Adverse events were defined according to the Pediatric Interagency Registry for Mechanical Circulatory Support criteria. RESULTS: Our final study cohort included 37 encounters with 43 catheter-based axial pump implantations. A single catheter-based axial pump device was used for support in 33 encounters (89%), with 2 catheter-based axial pump devices used in 3 (8%) separate encounters and 3 catheter-based axial pump devices used in 1 (3%) encounter. The median [range] age, weight, and body surface area at implantation were 16.8 [6.9-42.8] years, 61.1 [23.1-123.8] kg, and 1.7 [0.8-2.5] m2, respectively. The predominant causes of circulatory failure were graft failure/rejection in 16 patients (43%), followed by cardiomyopathy in 7 patients (19%), arrhythmia refractory to medical therapies in 6 patients (16%), myocarditis/endocarditis in 4 patients (11%), and heart failure due to congenital heart disease in 4 patients (11%). Competing outcomes analysis showed a positive outcome with bridge-to-recovery in 58%, bridge-to-durable VAD support in 14%, and bridge-to-cardiac transplantation in 14% at 6 months. Fourteen percent of encounters resulted in death at 6 months. CONCLUSIONS: We demonstrate that catheter-based axial pump support in children results in excellent 1- and 6-month survival with an acceptable adverse event profile.


Assuntos
Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Humanos , Criança , Adolescente , Adulto Jovem , Adulto , Estudos Retrospectivos , Resultado do Tratamento , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/terapia , Choque Cardiogênico , Catéteres
13.
Perfusion ; 38(8): 1565-1567, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-36154503

RESUMO

We report the case of a 5-year-old boy who suffered from an intracardiac air influx with suspected cerebral air embolism during the Fontan procedure. We immediately transformed the cardiopulmonary bypass circuit to perform a retrograde cerebral perfusion, which resulted in successful neuroprotection. He was extubated in the operating room without any neurological defects.


Assuntos
Embolia Aérea , Técnica de Fontan , Pré-Escolar , Humanos , Masculino , Circulação Cerebrovascular , Embolia Aérea/etiologia , Técnica de Fontan/efeitos adversos , Coração , Perfusão/métodos
14.
Pediatr Cardiol ; 44(3): 530-539, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36322202

RESUMO

Although enormous effort has focussed on how to build an effective culture in the business community, relatively little effort has addressed how to achieve this in the hospital environment, specifically related to the field of congenital heart disease teams. The examination of culture in pediatric cardiac care is particularly important for several key reasons: first, it represents high-stakes medicine, second, there are multiple stakeholders requiring collaboration between cardiologists, surgeons, anaesthesiologists, perfusionists, nursing staff, and allied health care professionals, and finally, both the patient and the family are intimately involved in the care pathway. This review article investigates some of the critical components to building an effective culture, drawing upon similarities in other disciplines, thereby fostering high performance multidisciplinary teams in congenital cardiology care. Strategies to change culture such as Kotter's model of change are also discussed. High performance teams share one common vital characteristic: psychological safety for team members to speak their minds, thereby fostering an open culture, in which creativity can flourish to facilitate major breakthroughs. Adoption of the "Flight Plan" review promotes patient centric care and champions a psychologically safe culture.


Assuntos
Cardiologia , Cardiopatias Congênitas , Humanos , Criança , Cultura Organizacional , Equipe de Assistência ao Paciente , Liderança , Cardiopatias Congênitas/terapia
16.
J Thorac Cardiovasc Surg ; 163(2): 387-395.e3, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33966882

RESUMO

BACKGROUND: Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients. METHODS: A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates. RESULTS: A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P < .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P < .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029). CONCLUSIONS: Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.


Assuntos
Síndrome de Heterotaxia , Veias Pulmonares/cirurgia , Pneumopatia Veno-Oclusiva/cirurgia , Síndrome de Cimitarra/cirurgia , Procedimentos Cirúrgicos Vasculares , Feminino , Síndrome de Heterotaxia/diagnóstico por imagem , Síndrome de Heterotaxia/mortalidade , Síndrome de Heterotaxia/fisiopatologia , Humanos , Masculino , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/fisiopatologia , Pneumopatia Veno-Oclusiva/diagnóstico por imagem , Pneumopatia Veno-Oclusiva/mortalidade , Pneumopatia Veno-Oclusiva/fisiopatologia , Recidiva , Reoperação , Estudos Retrospectivos , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/mortalidade , Síndrome de Cimitarra/fisiopatologia , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade
18.
J Heart Lung Transplant ; 41(3): 283-286, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-34953719

RESUMO

The Fontan circulation has inherent long-term vulnerabilities such that adult Fontan patients now comprise the largest, most rapidly growing subgroup of adult congenital heart disease referred for transplant assessment. Almost all have Fontan Associated Liver Disease (FALD). There is an absence of mid to late hepatic outcome data after heart transplant alone. Therefore, we analyzed outcomes of survivors of heart only transplant in patients with failing Fontan circulation. Including all 10 of our adult Fontan patients surviving >1 year after isolated heart transplant, we report evolution of their clinical features, bloodwork, hemodynamic data, and liver ultrasound findings over a median of 4.7 years. Nonprogression of FALD, resolution of ascites and freedom from hepatocellular carcinoma in the mid-term highlight the outcomes in this selected group once normal cardiac output and venous pressures are established by heart transplant.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Transplante de Coração , Hepatopatias/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Falha de Tratamento , Resultado do Tratamento
19.
Artigo em Inglês | MEDLINE | ID: mdl-34116778

RESUMO

Patients with ccTGA or d-TGA managed via atrial switch (Mustard or Senning operations) have biventricular circulations with a sub-aortic right ventricle (2V-RV). Other than in a tiny percentage of ccTGA patients, premature heart failure (HF) is common, driven by chronic RV dilatation and dysfunction and/or tricuspid regurgitation. These patients are different from the general HF population in that they are younger, more heterogeneous, are predisposed to pulmonary hypertension and present unique and complex surgical challenges. Despite their young age, they experience disproportionately poor access to advanced therapies and are often disqualified for transplant by pulmonary hypertension, HLA sensitization, program risk-tolerance and psychosocial issues. Mechanical support of the subaortic RV with ventricular assist device (subaortic RVAD, also known as SVAD), although technically challenging, can be an effective alternative to palliative care and offers high likelihood of bridging patients to heart transplant candidacy. In addition, temporary trans-catheter SVAD Impella support has been advantageous for stabilization of decompensated 2V-RV patients or as bridge to durable SVAD support. Improved awareness of and access to specialist ACHD-HF teams offering mechanical support (and transplantation) for 2V-RV patients is increasingly urgent for this aging population, and will improve options and outcomes for these patients as HF emerges.


Assuntos
Insuficiência Cardíaca , Coração Auxiliar , Adulto , Idoso , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Ventrículos do Coração/cirurgia , Humanos , Estudos Retrospectivos , Resultado do Tratamento
20.
CJC Open ; 3(5): 619-626, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-34027366

RESUMO

BACKGROUND: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy. However, the efficacy and safety of this invasive therapy in the prevention of recurrent ICD shocks in patients with TOF has not been well evaluated. METHODS: Records of a total of 47 consecutive TOF patients (mean age: 43.1 ± 13.2 years, male sex: n = 34 [72.3%]) who underwent ICD implantation for secondary prevention between 2000 and 2018 were reviewed. RESULTS: Twenty (42.6%) patients underwent invasive therapy (radiofrequency catheter ablation, n = 8; surgical ablation with pulmonary valve replacement, n = 12) before ICD implantation. Twenty-seven patients (57.4%) were managed noninvasively. During follow-up (median 80.5 [interquartile range, 28.5-131.0] months), 2 (10.0%) patients in the invasive group and 10 (37.0%) patients in the noninvasive group received appropriate ICD shocks (P = 0.036). Logistic regression analysis showed that invasive therapy was associated with a decreased risk of ICD shocks by 81.1% (odds ratio, 0.189; 95% confidence interval, 0.036-0.990; P = 0.049). Furthermore, invasive therapy was associated with decreased risk of the composite outcomes of ICD shock, death, cardiac transplantation, and hospital admission (odds ratio, 0.090; 95% confidence interval, 0.025-0.365; P = 0.013) compared with noninvasive therapy. CONCLUSIONS: Invasive substrate modification therapy was associated with a lower likelihood of ICD shocks and improvement of long-term outcomes in TOF patients.


CONTEXTE: Les défibrillateurs cardioverteurs implantables (DCI) sont efficaces pour prévenir la mort cardiaque subite provoquée par une arythmie chez les patients présentant une tétralogie de Fallot (TF). Bien que le traitement des arythmies ventriculaires malignes par DCI puisse sauver des vies, les chocs administrés peuvent avoir des conséquences délétères. L'ablation du substrat est devenue le traitement de référence pour prévenir l'administration à répétition de chocs par DCI chez les patients atteints d'une cardiomyopathie ischémique. L'efficacité et l'innocuité de ce traitement invasif pour prévenir l'administration de chocs répétés chez les patients présentant une TF n'ont toutefois pas été bien évaluées. MÉTHODOLOGIE: Nous avons examiné les cas consécutifs de 47 patients présentant une TF (âge moyen : 43,1 ± 13,2 ans; hommes : n = 34 [72,3 %]) ayant reçu un DCI en prévention secondaire entre 2000 et 2018. RÉSULTATS: Au total, 20 (42,6 %) patients ont subi un traitement invasif (ablation par cathéter par radiofréquence, n = 8; ablation chirurgicale et remplacement de la valve pulmonaire, n = 12) avant l'implantation d'un DCI. Vingt-sept patients (57,4 %) ont été pris en charge de façon non invasive. Au cours de la période de suivi (durée médiane de 80,5 [intervalle interquartile : 28,5 à 131,0] mois), 2 (10,0 %) patients du groupe ayant subi une intervention invasive et 10 (37,0 %) patients du groupe ayant subi une intervention non invasive ont reçu un choc approprié par DCI (p = 0,036). Les résultats de l'analyse par régression logistique montrent que le traitement invasif est associé à une réduction du risque de choc par DCI de 81,1 % (rapport des cotes : 0,189; intervalle de confiance à 95 % : de 0,036 à 0,990; p = 0,049). En outre, le traitement invasif est associé à une réduction du risque de survenue d'un des événements du paramètre d'évaluation composé, soit un choc administré par DCI, le décès, une transplantation cardiaque ou une hospitalisation (rapport des cotes : 0,090; intervalle de confiance à 95 % : de 0,025 à 0,365; p = 0,013) par rapport au traitement non invasif. CONCLUSIONS: La modification invasive du substrat a été associée à une probabilité plus faible de choc administré par DCI et à une amélioration des résultats à long terme chez les patients présentant une TF.

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