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.

Repair with a pulmonary neovalve in tetralogy of Fallot: does this avoid ventricular dysfunction?

OBJECTIVES: Given the anatomical variations of tetralogy of Fallot (TOF), different surgical techniques can be used to achieve correction. Transannular patches (TAPs) are the most commonly used technique; they are associated with right ventricular dysfunction, the incidence of which can be reduced through pulmonary valve preservation. METHODS: Between January 2010 and July 2019, we performed 274 surgical corrections of tetralogy of Fallot at Fundación Cardioinfantil; 63 patients (23%) underwent repair with a TAP in addition to a pulmonary neovalve (Group I), 66 patients (24.1%) received a TAP without a pulmonary valve (Group II) and 145 patients (52.9%) had a repair with valve preservation (Group III). We analysed patient's characteristics before, during and after surgery at a 30-day follow-up. RESULTS: We found that patients in Group III were older (P = 0.04). Group II had the lowest level of O2 saturation before surgery (82%, P = 0.001). Cardiopulmonary bypass and aortic cross-clamp times were longer in Group I (P < 0.001). Right ventricular dysfunction was less frequent in Group III (15.9%, P = 0.011). Severe residual pulmonary regurgitation was more common in Group II (21.9%, P = 0.001). CONCLUSIONS: Preservation of the pulmonary valve is an important factor for immediate postoperative management of tetralogy of Fallot. Patients who were repaired with a TAP with or without a pulmonary neovalve had a higher incidence of right ventricular dysfunction than those with pulmonary valve preservation.

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

To investigate the role of right ventricular free wall strain (RVFWSL) to predict low functional capacity in repaired tetralogy of Fallot (rTOF). We prospectively enrolled 33 patients with rTOF with moderate to severe PR who underwent rest and peak exercise echocardiography on a semisupine cycloergometer. Conventional function and strain imaging parameters of both ventricles were measured. Patients performing < 7 METS were defined to have low functional capacity. Logistic regression was used to identify parameters associated with low functional capacity. Eleven patients (33.3%) had low functional capacity. These patients were shorter (height 155 ± 7 vs 163 ± 9 cm, p = 0.023), more frequently female (27.3 vs 72.7%, p = 0.024) and had history of Blalock-Taussig shunt (45.5 vs 9.1%, p = 0.027). On multivariate analysis RVFWSL was the only predictor of low functional capacity OR 1.39 (CI 95%, 1.06-1.83., p = 0.018) per % change. A RVFWSL < 17% (absolute value) had an AUC of 0.785, sensitivity of 81.8% and specificity of 77.3% to predict low functional capacity. Right ventricular free wall strain is an independent predictor of low functional capacity in repaired tetralogy of Fallot with moderate to severe PR. A value < 17% might be useful in deciding when to perform pulmonary valve replacement, when functional capacity cannot be objectively measured.

[Treatment of Fallot tetralogy with a transannular patch. Six years follow-up]. / Tratamiento de la tetralogía de Fallot con parche transanular. Seguimiento a 6 años.

BACKGROUND: Primary repair of Fallot tetralogy has been performed successfully for the last 45 years. It has low surgical mortality (< 5%), with excellent long-term results. However, there are delayed adverse effects: progressive right ventricular dilation and dysfunction, arrhythmia, and sudden death. In our centre, Fallot tetralogy is the most common form of cyanotic congenital heart disease (including transannular patch) and accounts for 7.5% of all cardiovascular surgical procedures. The mid-term follow-up results are reported. MATERIAL AND METHODS: Case series. The study included patients who had complete repair of Fallot tetralogy with transannular patch from January 2000 to December 2009. An analysis was performed on the clinical variables, morbidity and mortality. RESULTS: There were 52 patients in the study, with mean age 4 ± 2 years. Perioperative mortality in 6 patients, with 5 associated with residual right ventricular obstruction and, 1 associated with further surgery. The survival rate was 88% (46) patients, with a follow-up 75 ± 26 months. Late morbidity occurred in 14, due to right ventricular dysfunction in 11, recurrent distal obstruction in 2, and residual ventricular septal defect in 1. Associated risk factors were severe pulmonary insufficiency (p=0.001); QRS > 160 ms, p=0.001); cardiothoracic > 0.60 index, (p=0.048), and tricuspid regurgitation (p=0.001). CONCLUSIONS: There was reasonable long-term survival and excellent quality of life after total correction of Fallot tetralogy; however, progressive right ventricular dysfunction requires continuous monitoring, as well as the choice of optimal timing of pulmonary valve replacement.

Insuficiencia valvular pulmonar en el posoperatorio alejado de tetralogía de Fallot. Aporte del ecocardiograma transtorácico convencional para la toma de decisiones / Pulmonary valve insufficiency in the postoperative tetralogy of Fallot away. Contribution of conventional transthoracic echocardiography for decision making

La insuficiencia valvular pulmonar constituye uno de los principales problemas en el posoperatorio alejado de tetralogíade Fallot, siendo su incidencia muy frecuente y dependiente de múltiples aspectos. En este trabajo se efectúa una revisiónrelacionada con la utilidad de la ecocardiografía transtorácica convencional como instrumento para la evaluación delos pacientes con insuficiencia valvular pulmonar en el posoperatorio alejado de la tetralogía de Fallot. Se exponen losprincipales conceptos fisiopatológicos de esta entidad y, en función de ellos, se presentan los parámetros ecocardiográficosmás relevantes a tener en cuenta en la valoración de estos pacientes. Finalmente se consideran las pautas y laoportunidad para el reemplazo valvular pulmonar.

Insuficiencia valvular pulmonar en el posoperatorio alejado de tetralogía de Fallot. Aporte del ecocardiograma transtorácico convencional para la toma de decisiones / Pulmonary valve insufficiency in the postoperative tetralogy of Fallot away. Contribution of conventional transthoracic echocardiography for decision making

La insuficiencia valvular pulmonar constituye uno de los principales problemas en el posoperatorio alejado de tetralogía de Fallot, siendo su incidencia muy frecuente y dependiente de múltiples aspectos. En este trabajo se efectúa una revisión relacionada con la utilidad de la ecocardiografía transtorácica convencional como instrumento para la evaluación delos pacientes con insuficiencia valvular pulmonar en el posoperatorio alejado de la tetralogía de Fallot. Se exponen los principales conceptos fisiopatológicos de esta entidad y, en función de ellos, se presentan los parámetros ecocardiográficos más relevantes a tener en cuenta en la valoración de estos pacientes. Finalmente se consideran las pautas y la oportunidad para el reemplazo valvular pulmonar.

Pulmonary valve regurgitation is one of the main problems in the late post-op of Tetrallogy of Fallot, having a great incidence and depending on many aspects . In these paper a review is done considering the usefulness of conventional transtoracic ecocardiography as a tool to evaluate patients with pulmonary regurgitation.in the late post-op of Tetralogy of Fallot. Main physiopathologic concepts about these entity are presented, and as a function of that, the most important ecocardiographic parameters to be considered evaluating these patients are also described. Finally guidelines on the opportunity for pulmonary valve replacement are considered.

Use of right ventricular remodeling surgery with a porcine pulmonary prosthesis for congenital heart disease.

BACKGROUND: The reconstruction of the right ventricular outflow tract (RVOT) in congenital heart disease has attracted the interest of cardiac surgeons determined to alleviate the anatomic obstruction and restore RV function. METHODS: From June 1991 to September 2008, 203 consecutive patients (mean, 3.0 years; range, 2 months to 35 years) underwent operations. These patients were classified into 5 groups: group 1, tetralogy of Fallot with pulmonary hypoplasia (144 cases, 70.9%); group 2, pulmonary atresia (PA) with ventricular septal defect (VSD) (32 cases, 15.7%); group 3, truncus arteriosus (12 cases, 5.9%); group 4, transposition of the great arteries with left ventricular outflow tract obstructions (8 cases, 3.9%); and group 5, PA with intact ventricular septum (7 cases, 3.4%). Remodeling surgery of the RV consisted of patch closure of the VSD (n = 176), tricuspid valvoplasty repair (n = 25), infundibulum muscle resection, and reconstruction of the RVOT (all patients). The Lecompte procedure was performed in 8 patients in group 4, and the one and a half ventricle technique was performed in 7 patients in group 5. RESULTS: There were 21 hospital deaths (10.3%); 180 patients (88.6%) survived. Patients were followed up from 4 to 206 months (mean, 98.0 months). Sixteen patients (8.8%) underwent reoperation for prosthesis dysfunction, with 2 in-hospital deaths (12.5%). The rest of the patients (164, 80.7%) remain free of reoperation. CONCLUSION: Earlier reconstruction of the pulmonary valve and the RVOT may preserve ventricular performance for a long period. Nevertheless, the porcine pulmonary prosthesis has shown satisfactory results when it has been used for the reconstruction of different types of RV obstructions.

Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect.

A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR) and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH) was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular / Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP) e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC) com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.

A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR) and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH) was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.