Long-Term Effects of Pulmonary Valve Implantation and Prosthesis Evolution in Patients with Repaired Tetralogy of Fallot. / Efeitos de Longo Prazo do Implante Valvar Pulmonar e Evolução da Prótese em Pacientes com Tetralogia de Fallot Corrigida.

BACKGROUND: Pulmonary valve regurgitation is a significant long-term complication in patients with tetralogy of Fallot (TOF). OBJECTIVE: This study aims to investigate the effects of pulmonary valve implantation (PVI) on the anatomy and function of the right ventricle (RV) and the long-term evolution of the implanted prosthesis in the pulmonary position. METHODS: A single-center retrospective cohort analysis was performed in 56 consecutive patients with TOF who underwent PVI. The study included patients of both sexes, aged ≥ 12 years, and involved assessing clinical and surgical data, pre- and post-operative cardiovascular magnetic resonance imaging, and echocardiogram data more than 1 year after PVI. RESULTS: After PVI, there was a significant decrease in RV end-systolic volume indexed by body surface area (BSA), from 89 mL/BSA to 69 mL/BSA (p < 0.001) and indexed RV end-diastolic volume, from 157 mL/BSA to 116 mL/BSA (p < 0.001). Moreover, there was an increase in corrected RV ejection fraction [ RVEFC = net pulmonary flow (pulmonary forward flow - regurgitant flow) / R V end-diastolic volume ] from 23% to 35% (p < 0.001) and left ventricular ejection fraction from 58% to 60% (p = 0.008). However, a progressive increase in the peak pulmonary valve gradient was observed over time, with 25% of patients experiencing a gradient exceeding 60 mmHg. Smaller prostheses (sizes 19 to 23) were associated with a 4.3-fold higher risk of a gradient > 60 mmHg compared to larger prostheses (sizes 25 to 27; p = 0.029; confidence interval: 1.18 to 17.8). CONCLUSION: As expected, PVI demonstrated improvements in RV volumes and function. Long-term follow-up and surveillance are crucial for assessing the durability of the prosthesis and detecting potential complications. Proper sizing of prostheses is essential for improved prosthesis longevity.

Efeitos de Longo Prazo do Implante Valvar Pulmonar e Evolução da Prótese em Pacientes com Tetralogia de Fallot Corrigida / Long-Term Effects of Pulmonary Valve Implantation and Prosthesis Evolution in Patients with Repaired Tetralogy of Fallot

Resumo Fundamento A regurgitação valvar pulmonar é uma importante complicação de longo prazo em pacientes com tetralogia de Fallot (TF). Objetivo O presente estudo tem como objetivo investigar os efeitos do implante valvar pulmonar (IVP) na anatomia e função do ventrículo direito (VD) e na evolução em longo prazo da prótese implantada em posição pulmonar. Métodos Uma análise de coorte retrospectiva e unicêntrica foi realizada em 56 pacientes consecutivos com TF submetidos a IVP. O estudo incluiu pacientes de ambos os gêneros, com idade ≥ 12 anos e compreendeu avaliação de dados clínicos e cirúrgicos, ressonância magnética cardiovascular pré e pós-operatória e dados ecocardiográficos obtidos mais de 1 ano após IVP. Resultados Após o IVP, houve uma diminuição significativa do volume sistólico final do VD indexado pela área de superfície corpórea (ASC), de 89 mL/ASC para 69 mL/ASC (p < 0,001) e do volume diastólico final indexado do VD, de 157 mL/ASC para 116 mL/ASC (p < 0,001). Além disso, houve aumento da fração de ejeção corrigida do VD [ FEVDc = fluxo pulmonar ajustado (fluxo pulmonar anterógrado − fluxo regurgitante) / volume diastólico final do VD ] de 23% para 35% (p < 0,001) e da fração de ejeção do ventrículo esquerdo de 58% para 60% (p = 0,008). No entanto, foi observado um aumento progressivo no gradiente de pico da válvula pulmonar ao longo do tempo, com 25% dos pacientes apresentando um gradiente superior a 60 mmHg. Próteses menores (tamanhos 19 a 23) foram associadas a um risco 4,3 vezes maior de gradiente > 60 mmHg em comparação com próteses maiores (tamanhos 25 a 27; p = 0,029; intervalo de confiança: 1,18 a 17,8). Conclusão Conforme esperado, o IVP demonstrou melhorias nos volumes e na função do VD. O acompanhamento e a vigilância a longo prazo são cruciais para avaliar a durabilidade da prótese e detectar potenciais complicações. O dimensionamento adequado das próteses é essencial para melhorar a longevidade da prótese.

Abstract Background Pulmonary valve regurgitation is a significant long-term complication in patients with tetralogy of Fallot (TOF). Objective This study aims to investigate the effects of pulmonary valve implantation (PVI) on the anatomy and function of the right ventricle (RV) and the long-term evolution of the implanted prosthesis in the pulmonary position. Methods A single-center retrospective cohort analysis was performed in 56 consecutive patients with TOF who underwent PVI. The study included patients of both sexes, aged ≥ 12 years, and involved assessing clinical and surgical data, pre- and post-operative cardiovascular magnetic resonance imaging, and echocardiogram data more than 1 year after PVI. Results After PVI, there was a significant decrease in RV end-systolic volume indexed by body surface area (BSA), from 89 mL/BSA to 69 mL/BSA (p < 0.001) and indexed RV end-diastolic volume, from 157 mL/BSA to 116 mL/BSA (p < 0.001). Moreover, there was an increase in corrected RV ejection fraction [ RVEFC = net pulmonary flow (pulmonary forward flow − regurgitant flow) / R V end-diastolic volume] from 23% to 35% (p < 0.001) and left ventricular ejection fraction from 58% to 60% (p = 0.008). However, a progressive increase in the peak pulmonary valve gradient was observed over time, with 25% of patients experiencing a gradient exceeding 60 mmHg. Smaller prostheses (sizes 19 to 23) were associated with a 4.3-fold higher risk of a gradient > 60 mmHg compared to larger prostheses (sizes 25 to 27; p = 0.029; confidence interval: 1.18 to 17.8). Conclusion As expected, PVI demonstrated improvements in RV volumes and function. Long-term follow-up and surveillance are crucial for assessing the durability of the prosthesis and detecting potential complications. Proper sizing of prostheses is essential for improved prosthesis longevity.

Heart Transplantation in Children and Adults With Congenital Heart Disease: 3 Decades of Evolution.

Heart transplantation is the treatment of choice for children and adults with congenital heart disease. We report the heart transplant single-center experience. The number of transplantations has increased over the last 3 decades. The Kaplan-Meier survival curves in the first, second, and third decades at 5 and 10 years were 69% and 59%, 62% and 52%, and 66% and 60%, respectively.

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child

Abstract Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

Initial experience with del Nido cardioplegia solution at a Pediatric and Congenital Cardiac Surgery Program in Brazil.

OBJECTIVE: The aim of this study was to evaluate outcome measures between our standard multidose cardioplegia protocol and a del Nido cardioplegia protocol in congenital heart surgery patients. METHODS: Retrospective single-center study including 250 consecutive patients that received del Nido cardioplegia (DN group) with a mandatory reperfusion period of 30% of cross clamp time and 250 patients that received a modified St. Thomas' solution (ST group). Groups were matched by age, weight, gender, and Risk Adjustment for Congenital Heart Surgery (RACHS-1) scores. Preoperative hematocrit and oxygen saturation were also recorded. Outcomes analyzed were the vasoactive inotropic score (VIS), lactate, ventilation time, ventricular dysfunction with low cardiac output syndrome (LCOS), intensive care unit (ICU) length of stay (LOS), hospital LOS, bypass and aortic cross-clamp times, and in-hospital mortality. RESULTS: Both groups were comparable demographically. Statistically significant differences (p ⩽ 0.05) were noted for cardiac dysfunction with LCOS, hematocrit at end of surgery (p = 0.0038), VIS on ICU admission and at end of surgery (p = 0.0111), and ICU LOS (p = 0.00118) with patients in the DN group having more desirable values for those parameters. Other outcome measures did not reach statistical significance. CONCLUSION: In our congenital cardiac surgery population, del Nido cardioplegia strategy was associated with less ventricular dysfunction with LCOS, a lower VIS and decreased ICU LOS compared with patients that received our standard myocardial protection using a modified St. Thomas' solution. Despite the limitation of this study, including its retrospective nature and cohort size, these data supported our transition to incorporate del Nido cardioplegia solution with a mandatory reperfusion period as the preferred myocardial protection method in our program.

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child.

Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

Heart Retransplantation for Coronary Allograft Vasculopathy in Children: 25 Years of Single-Center Experience.

BACKGROUND: Pediatric end-stage heart disease is surgically managed by heart transplantation. A major complication of primary transplantation (PTx) is coronary allograft vasculopathy (CAV), a form of accelerated atherosclerosis. Retransplantation (RTx) has been the management of CAV; however, there is limited comprehensive literature on this subject. Here we report 25 years of single-center experience in managing CAV with RTx and place it in the context of recent studies. METHODS: A retrospective cohort study was undertaken on patients who underwent PTx <18 years old and subsequent RTx due to CAV at the Heart Institute (InCor) University of São Paulo Medical School between 1992 and 2018. The maintenance immunosuppression protocol was double immunosuppression. For both PTx and RTx, quantitative and qualitative analyses were conducted for transplantation indication, donor/recipient demographics, post-transplant survival, rejection, infection, and immunosuppression. RESULTS: Between 1992 and 2018, 200 children underwent heart transplantation. Ten re-transplantations were performed, for which 7 (70%) were for CAV. Ages at RTx ranged from 11.5 to 29.3 years (19.1 ± 5.68 years; median 18.2 years). The mean time between PTx and RTx was 12.9 ± 3.4 years (median 13.4 years). The Kaplan-Meier survival rate at 1 month, 3 years, and 5 years was 85.7%, 71.5%, and 47.6%, respectively. CONCLUSION: Cardiac RTx can be a management option for CAV in patients who have undergone PTx in childhood with double immunosuppression therapy.

Rational Use of Mechanical Circulatory Support as a Bridge to Pediatric and Congenital Heart Transplantation.

INTRODUCTION: Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. METHODS: All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. RESULTS: Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). CONCLUSION: Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

Rational use of mechanical circulatory support as a bridge to pediatric and congenital heart transplantation

Abstract Introduction: Donor shortage and organ allocation is the main problem in pediatric heart transplant. Mechanical circulatory support is known to increase waiting list survival, but it is not routinely used in pediatric programs in Latin America. Methods: All patients listed for heart transplant and supported by a mechanical circulatory support between January 2012 and March 2016 were included in this retrospective single-center study. The endpoints were mechanical circulatory support time, complications, heart transplant survival and discharge from the hospital. Results: Twenty-nine patients from our waiting list were assessed. Twelve (45%) patients were initially supported by extracorporeal membrane oxygenation (ECMO) and a centrifugal pump was implanted in 17 (55%) patients. Five patients initially supported by ECMO were bridged to another device. One was bridged to a centrifugal pump and four were bridged to Berlin Heart Excor®. Among the 29 supported patients, 18 (62%) managed to have a heart transplant. Thirty-day survival period after heart transplant was 56% (10 patients). Median support duration was 12 days (interquartile range [IQR] 4 - 26 days) per run and the waiting time for heart transplant was 9.5 days (IQR 2.5-25 days). Acute kidney injury was identified as a mortality predictor (OR=22.6 [CI=1.04-494.6]; P=0.04). Conclusion: Mechanical circulatory support was able to bridge most INTERMACS 1 and 2 pediatric patients to transplant with an acceptable complication rate. Acute renal failure increased mortality after mechanical circulatory support in our experience.

EuroSCORE II and the importance of a local model, InsCor and the future SP-SCORE.

INTRODUCTION: The most widely used model for predicting mortality in cardiac surgery was recently remodeled, but the doubts regarding its methodology and development have been reported. OBJECTIVE: The aim of this study was to assess the performance of the EuroSCORE II to predict mortality in patients undergoing coronary artery bypass grafts or valve surgery at our institution. METHODS: One thousand consecutive patients operated on coronary artery bypass grafts or valve surgery, between October 2008 and July 2009, were analyzed. The outcome of interest was in-hospital mortality. Calibration was performed by correlation between observed and expected mortality by Hosmer Lemeshow. Discrimination was calculated by the area under the ROC curve. The performance of the EuroSCORE II was compared with the EuroSCORE and InsCor (local model). RESULTS: In calibration, the Hosmer Lemeshow test was inappropriate for the EuroSCORE II (P=0.0003) and good for the EuroSCORE (P=0.593) and InsCor (P=0.184). However, the discrimination, the area under the ROC curve for EuroSCORE II was 0.81 [95% CI (0.76 to 0.85), P<0.001], for the EuroSCORE was 0.81 [95% CI (0.77 to 0.86), P<0.001] and for InsCor was 0.79 [95% CI (0.74-0.83), P<0.001] showing up properly for all. CONCLUSION: The EuroSCORE II became more complex and resemblance to the international literature poorly calibrated to predict mortality in patients undergoing coronary artery bypass grafts or valve surgery at our institution. These data emphasize the importance of the local model.

EuroSCORE II e a importância de um modelo local, InsCor e o futuro SP-SCORE / EuroSCORE II and the importance of a local model, InsCor and the future SP-SCORE

Introdução: O modelo mais utilizado para predição de mortalidade em cirurgia cardíaca foi recentemente remodelado, mas dúvidas referentes à sua metodologia e desenvolvimento têm sido relatadas. Objetivo: O objetivo deste estudo foi avaliar o desempenho do EuroSCORE II na predição de mortalidade em pacientes submetidos a cirurgia de coronária e/ou valva na instituição. Métodos: Mil pacientes, operados consecutivamente de coronária e/ou valva, entre outubro de 2008 e julho de 2009, foram analisados. O desfecho de interesse foi mortalidade intra-hospitalar. A calibração foi realizada pela correlação entre mortalidade esperada e observada por meio do teste de Hosmer Lemeshow. A discriminação foi calculada pela área abaixo da curva ROC. O desempenho do EuroSCORE II foi comparado com os modelos EuroSCORE e InsCor (modelo local). Resultados: Na calibração, o teste de Hosmer Lemeshow foi inadequado para o EuroSCORE II (P=0,0003) e bom para os modelos EuroSCORE (P=0,593) e InsCor (P=0,184). No entanto, na discriminação, a área abaixo da curva ROC para o EuroSCORE II foi de 0,81 [IC 95% (0,76-0,85), P<0,001]; para o EuroSCORE foi de 0,81 [IC 95% (0,77-0,86), P<0,001] e para o InsCor foi de 0,79 [IC 95% (0,74-0,83), P<0,001], revelando-se adequada para todos. Conclusão: O EuroSCORE II se tornou mais complexo e, à semelhança com a literatura internacional, mal calibrado para predizer mortalidade nos pacientes operados de coronária e/ou valva em nosso meio. Esses dados reforçam a importância do modelo local. .

Introduction: The most widely used model for predicting mortality in cardiac surgery was recently remodeled, but the doubts regarding its methodology and development have been reported. Objective: The aim of this study was to assess the performance of the EuroSCORE II to predict mortality in patients undergoing coronary artery bypass grafts or valve surgery at our institution. Methods: One thousand consecutive patients operated on coronary artery bypass grafts or valve surgery, between October 2008 and July 2009, were analyzed. The outcome of interest was in-hospital mortality. Calibration was performed by correlation between observed and expected mortality by Hosmer Lemeshow. Discrimination was calculated by the area under the ROC curve. The performance of the EuroSCORE II was compared with the EuroSCORE and InsCor (local model). Results: In calibration, the Hosmer Lemeshow test was inappropriate for the EuroSCORE II (P=0.0003) and good for the EuroSCORE (P=0.593) and InsCor (P=0.184). However, the discrimination, the area under the ROC curve for EuroSCORE II was 0.81 [95% CI (0.76 to 0.85), P<0.001], for the EuroSCORE was 0.81 [95% CI (0.77 to 0.86), P<0.001] and for InsCor was 0.79 [95% CI (0.74-0.83), P<0.001] showing up properly for all. Conclusion: The EuroSCORE II became more complex and resemblance to the international literature poorly calibrated to predict mortality in patients undergoing coronary artery bypass grafts or valve surgery at our institution. These data emphasize the importance of the local model. .