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.

Manejo del tracto de salida del ventrículo derecho con implante percutáneo de válvula Melody en pacientes pediátricos: experiencia en un centro de alta complejidad en Colombia / Management of right ventricular outflow tract with percutaneous Melody valve implantation in pediatric patients: experience in a high complexity center in Colombia

Resumen Antecedentes: Las cardiopatías congénitas plantean un desafío terapéutico, específicamente la estenosis de la válvula pulmonar. Esta ha sido tratada durante muchos años con procedimientos invasivos e inserción de bioprótesis, que con el tiempo se vuelven disfuncionales y pueden reestenosarse por acumulación de tejido fibroso y calcificación. Debido a las complicaciones generadas por la injuria quirúrgica, se han descrito medidas menos invasivas para el manejo de la estenosis residual e inicial por medios endovasculares en adultos y más recientemente en población pediátrica. Objetivo: El objetivo de este reporte es describir la misma en el manejo endovascular del tracto de salida del ventrículo derecho, como el inicio de un trabajo continuo para la mejoría de los resultados pediátricos en países en vía de desarrollo. Métodos: Se presentan siete casos pediátricos de manejo endovascular del tracto de salida derecho; tres de ellos sometidos a valvuloplastia quirúrgica con persistencia de la estenosis pulmonar, por lo cual se decidió inserción percutánea de una válvula pulmonar (IVPP) transcatéter con válvula Melody utilizando la técnica valve-in-valve, con lo que se consiguió una resolución del 100% de la estenosis y no se presentó ningún tipo de complicación asociada al procedimiento. Resultados: En cuatro pacientes se logró una implantación exitosa de la válvula por vía percutánea en diferentes cardiopatías congénitas, siendo uno de ellos en tracto nativo; además, destaca el caso de un paciente en quien se realizó fractura intencional de la válvula pulmonar, procedimiento innovador en el manejo endovascular pediátrico en Colombia. Conclusiones: En estos pacientes el procedimiento resultó ser poco invasivo, seguro y efectivo. La técnica IVPP podría ser considerada una opción viable en Colombia (y en otros países en desarrollo) para el manejo de implantes valvulares primarios fallidos o incluso en tractos nativos.

Abstract Background: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. Objective: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries. Methods: Seven pediatric patients with endovascular management of the right outflow tract are presented. Three of them underwent surgical valvuloplasty with persistent pulmonary stenosis. They decided to insert a percutaneous transcatheter pulmonary valve (PPVI) with a Melody valve using the valve-in-valve technique, with 100% stenosis and no complications associated with the procedure. Results: Four patients with successful percutaneous valve implantation had different congenital heart diseases. In addition, the case of a patient in whom an intentional pulmonary valve fracture was performed, an innovative procedure in pediatric endovascular management in the country, is highlighted. Conclusions: The procedure was minimally invasive, safe, and effective. The IVPP technique could be a viable option in our country for managing failed primary valve implantations or even in native tracts.

A Rare Tetralogy of Fallot patient with Absent Left Pulmonary Artery Syndrome Successfully Treated in BSMMU Cardiac Surgery- A Case Report

Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly. The absence of right pulmonary artery is usually not associated with cardiac diseases but the absence of left pulmonary artery (LPA) is associated with cardiac diseases. Absent LPA is usually associated with tetralogy of Fallot (TOF) and hence surgically treated. As such, the agenesis of the unilateral pulmonary artery without any cardiovascular malformation is rare. This malformation of the absence of left pulmonary artery is confirmed by cardiac catheterization and CT angiography, prior to this X-ray gives some clues regarding the diminished vascularity of the involved left lung. The incidence is approximately 1 in 2,00,000 individuals with diverse nonspecific clinical presentation. Chest X-ray and echocardiography are usually the initial supportive investigations. Chest X-ray may reveal a minimally small left pulmonary hilum and left lung. MDCT angiography of pulmonary arteries may reveal an absent left main pulmonary artery with systemic collaterals around the left hemithorax. An early diagnosis of a unilateral absence of pulmonary artery and appropriate intervention has a beneficial role in improving the outcome. Diagnosis can be hard due to the rarity of the circumstance and its nonspecific presentation. We are hereby reporting a case of absent left pulmonary artery associated with tetralogy of Fallot which was incidental finding during evaluation of cyanotic patient. We did successful intracardiac repair with good outcome and without any major adverse effect.

Clinical effectiveness of valve-sparing aortic root replacement in the treatment of patients with dilated aortic root after operation for tetralogy of Fallot / 中国胸心血管外科临床杂志

@#Objective     To evaluate the clinical effectiveness of valve-sparing aortic root replacement (VSARR) in the treatment of patients with dilated aortic root after operation for tetralogy of Fallot (TOF). Methods     A retrospective analysis was conducted on clinical data of TOF patients with aortic root dilation who underwent VSARR in our hospital from 2016 to 2022. Results     Finally 14 patients were collected, including 8 males and 6 females, with a median age of 22 years ranging from 12-48 years. Among them, 5 patients had severe aortic valve regurgitation, 4 moderate regurgitation, and 5 mild or no regurgitation. Six patients had sinus of valsalva dilation, and 8 significant dilation of the ascending aorta. One patient had residual shunt due to ventricular septal defect, and 9 severe pulmonary valve regurgitation. The David procedure was performed in 10 patients, Yacoub procedure in 2 patients, and Florida sleeve in 2 patients. There was no perioperative mortality in the group. The median follow-up time was 2.9 years (ranging from 0.4 to 6.0 years). One patient had mild aortic valve regurgitation, and the rest had minimal or no regurgitation. One patient had mild stenosis of the left ventricular outflow tract, and the rest patients had no obvious stenosis. Conclusion     VSARR is a satisfactory treatment for aortic root dilation in patients with TOF, with no significant increase in the incidence of left ventricular outflow tract stenosis or aortic regurgitation during mid-term follow-up.

Evaluation of pulmonary arterial vascular impedance in fetuses with tetralogy of Fallot by Doppler ultrasound / 中华超声影像学杂志

Objective:To evluate the vascular impedance of the pulmonary arteries in fetuses with tetralogy of Fallot (TOF) by Doppler echocardiography.Methods:A total of 42 fetuses with TOF (TOF group) and 84 gestational age-matched normal fetuses(control group)were prospectively collected from August 2022 to January 2023 in the Second Xiangya Hospital of Central South University. The severity of TOF was classified into mild TOF (Z score≥-2), moderate TOF (-4<Z score<-2) or severe TOF (Z score≤-4) according to the Z score values of the pulmonary annulus diameter. The pulsatility indexs (PI) of the main pulmonary artery (MPA), distal left pulmonary artery (DLPA) and distal right pulmonary artery (DRPA) were measured by pulsed-wave Doppler. The differences of clinical data and echocardiographic parameters between TOF group, control group and TOF subgroups were compared.Results:Compared with the control group, MPA-PI increased significantly, whereas DLPA-PI and DRPA-PI decreased in TOF group (all P<0.001). There were no significant differences in MPA-PI and DRPA-PI among mild TOF, moderate TOF and severe TOF (all P>0.05). However, DLPA-PI decreased significantly in severe TOF compared with mild TOF ( P<0.05). Conclusions:Fetuses with TOF present increas vascular impedance in pulmonary trunk and decreased impedance in distal pulmonary artery branches. Further large and follow-up studies are needed to demonstrate the associations between the changes in vascular impedance and the development of pulmonary artery in patients with TOF.

Tetralogía de Fallot: Revisión sistemática para un abordaje integral / Tetralogy of Fallot. Systematic review for a Comprehensive Approach

Resumen La tetralogía de Fallot es una cardiopatía congénita cianosante compleja, con prevalencia considerable a nivel mundial, la cual requiere intervención en el primer año de vida, cuyas características clínicas tendrán variación conforme al grado de obstrucción pulmonar y la edad de presentación. Objetivo: Presentar una revisión sistemática de cómo debe realizarse el abordaje integral de la tetralogía de Fallot de acuerdo con la literatura actual. Metodología: Se realizó una revisión sistemática utilizando términos del MeSH y DeCS como "Tetralogía de Fallot", "defectos del tabique interventricular", "hipertrofia ventricular derecha", "hipertensión pulmonar", "defectos cardíacos congénitos", "enfermedades y anomalías congénitas, hereditarias y neonatales". La fuente fueron Sciencedirect, PubMed, Scopus y WoS. Se escogieron artículos indexados originales y metaanálisis. Conclusión: El pronóstico y las secuelas a largo plazo dependerá del diagnóstico oportuno, manejo médico y quirúrgico óptimos y, por tanto, del conocimiento fisiopatológico de la misma.

ABSTRACT Tetralogy of Fallot is a complex cyanotic congenital heart disease, with a considerable prevalence worldwide, which requires preferently intervention during the first year, and whose clinical characteristics will vary according to the degree of pulmonary obstruction and the age of onset. Clinical evaluation must arrange from anamnesis with a prenatal and postnatal history, comprehensive physical examination to imaging evaluation. Diagnosis is multivariate. Knowledge of hypoxemic crises is essential for its management in severe cases. Therefore, surgical management is the only adequate management for this entity, unless patient has a hypoxic crisis where volume replacement is the fundamental for its management. Although at the present, the most performed surgical management is the placement of a transannular patch with enlargement/release of the outflow tract, when the obstruction is severe in the neonatal stage, stent placement has been successfully performed at the level of the outflow tract that even exceeds the pulmonary valve in order to ensure effective pulmonary flow. Prognosis and long-term sequelae will depend on timely diagnosis, optimal medical and surgical management and pathophysiological understanding.

Correlation of Venous to Arterial Carbon Dioxide Partial Pressure Difference with other Cardiac Output Indices in Patients Undergoing Intracardiac Repair for Tetralogy of Fallot

Background: Clearance of tissue carbon dioxide by circulation is measured by venous to arterial carbon dioxide partial pressure difference (AVCO2 ) and is correlated with cardiac output (CO) in critically ill adult patients. This study aimed to correlate AVCO2 with other CO indices like arteriovenous oxygen saturation difference (AVO2 ), central venous oxygen saturation (ScVO2 ), and serum lactate in pediatric patients undergoing intracardiac repair (ICR) for tetralogy of Fallot (TOF). Methods: We conducted a prospective observational study in 50 patients, of age 5months to 5 years, undergoing ICR for TOF and analyzed AVO2 , AVCO2 , ScVO2 , and lactate from arterial and venous blood gas pairs obtained at different time intervals from admission to pediatric intensive care unit(PICU)(T0 ), at 6 h (T1 ), 12 h (T2 ), 24 h (T3 ), and 48 h (T4 ) postoperatively. Bivariate correlations were analyzed using Pearson for parametric variables. Results: Admission AVCO2 was not correlated with AVO2 (R2 = 0.166, P = 0.246), ScVO2 (R2 = ?2.2, P = 0.124), and lactate (R2 = ?0.07, P = 0.624). At T1 , AVCO2 was correlated with AVO2 (R2 = 0.283, P = 0.0464) but not with ScVO2 (R2 = ? 0.25, P = 0.079) and lactate (R2 = ?0.07, P = 0.623). At T2 , T3 and T4 , AVCO2 was correlated with AVO2 (R2 = 0.338,0.440 & 0.318, P = 0.0162, 0.0013, and 0.024), ScVO2 (R2 = ? 0.344, ? 0.488, and ?0.366; P = 0.0143, <0.0001, and 0.017), and lactate (R2 = 0.305, 0.467 and 0.607; P = 0.0314, 0.00062 and <0.0001). AVCO2 was negatively correlated with ScVO2 . No correlation observed between admission AVCO2 and mechanical ventilation duration. Two nonsurvivors had higher value of admission AVCO2 compared to survivors. Conclusion: AVCO2 is correlated with other CO surrogates like AVO2 , ScVO2 , and lactate in pediatric patients undergoing ICR for TOF.

A rare finding of left innominate artery arising from the left pulmonary artery in a case of tetralogy of fallot

Right-sided aortic arch with an aberrant left innominate artery arising from the left pulmonary artery is a very rare finding in the tetralogy of fallot (TOF). A 2-year-old boy presented for routine intracardiac repair of TOF. A routine angiogram raised suspicion of the left innominate artery with abnormal origin. 640-slice CT was done to delineate the anatomy. The left innominate artery was re-implanted in the descending aorta and the intracardiac repair was performed.

A case report on pregnancy with uncorrected tetralogy of Fallot with pulmonary hypertension managed uneventfully at a tertiary health care in India

Tetralogy of Fallot (ToF) is the most frequent kind of cyanotic congenital heart disease beyond the age of one year, accounting for around 10% of all congenital heart disease cases. Natural (non-corrective) survival into the fourth decade is quite unusual (3 %). There are various physiological and heamodynamic changes that occur in pregnancy, which makes causes complications in pregnancy and delivery in already compromised women, including women with uncorrected ToF. ToF is still a major source of maternal morbidity (62%) and even fatality (10%) in ToF patients, and it has a considerable impact on foetal outcome. Discussed below a case of pregnancy in a 26-year-old woman with uncorrected ToF, was diagnosed to have pulmonary hypertension, was admitted for safe confinement. She was taken up for elective LSCS with due risk and was later shifted to ICU for further management.

Preliminary study of cardiac morphology and function in fetuses with tetralogy of Fallot using fetal heart quantification / 中华超声影像学杂志

Objective:To evaluate the fetal heart shape and function in tetralogy of Fallot (TOF) by fetal heart quantitative analysis (fetal HQ).Methods:A total of 52 fetuses with TOF diagnosed by fetal echocardiography and 200 normal fetuses matched with their gestational weeks from March 2020 to March 2022 at Sir Run Run Shaw Hospital, Zhejiang University were retrospectively evaluated. The basic parameters of fetal cardiac blood vessels in the two groups were measured by fetal HQ technology and conventional M-mode ultrasound technology: aortic valve diameter(AV), pulmonary artery valve diameter(PV), main pulmonary artery diameter (MPA) and Z-score. The overall morphometric measurements including end-diastolic length diameter, transverse diameter, area, and global spherical index (GSI) of the fetal heart in the 4-chamber view(4CV), area and length of the right and left ventricles and their ratios. Measurements of left and right ventricular function included ejection fraction (EF), fraction area change rate (FAC), tricuspid annular plane systolic excursion (TAPSE), left and right ventricular global longitudinal strain (GLS), and left and right ventricular end-diastolic diameter (ED), spherical index (SI), and fractional shortening rate (FS) of 24-segments. The differences of above parameters between TOF group and control group were compared. In addition, the relationships between the absolute value of left and right ventricular GLS of TOF fetus and PV/AV, PV Z-score and MPA Z-score were analyzed. The optimal critical values of GSI, left ventricular EF and left ventricular FAC of TOF fetus were determined by ROC curve, and their corresponding sensitivity and specificity were obtained.Results:Compared with control group, there were significant differences in 4CV end-diastolic length, area, GSI, left ventricular area, left ventricular length, left ventricular EF, left ventricular FAC and left ventricular GLS in TOF group (all P<0.05). There were significant differences in ED between left ventricular 15-24 segments and right ventricular 1-21 segments (all P<0.05). There were significant differences in SI between left ventricular 1-16 segments, 21-24 segments and all segments of right ventricle (all P<0.05). The differences in FS were statistically significant (all P<0.05) when comparing all segments of the left ventricle and 1-2 segments of the right ventricular, and the remaining parameters were not statistically significant (all P>0.05). The left ventricular GLS absolute value of TOF fetuses was positively correlated with PV/AV, PV Z-score and MPA Z-score( rs=0.338, 0.441 and 0.458, all P<0.05), the right ventricular GLS absolute value of TOF fetuses was positively correlated with PV AV, PV Z-score and MPA Z-score( rs=0.418, 0.368 and 0.338, all P<0.05). The optimal critical values of GSI, left ventricular EF, and left ventricular FAC in the diagnosis of fetal TOF were 1.19, 59.05%, and 44.4%, respectively. At this time, the sensitivities of diagnosis of TOF were 0.78, 0.75, and 0.80, respectively. The specificities were 0.88, 0.88 and 0.83, respectively. The areas under ROC curve were 0.89, 0.88 and 0.89, respectively. Conclusions:Fetal HQ technology can provide a simple and reliable quantitative evaluation of fetal heart shape and function, and provide certain theoretical parameters for the study of fetal heart shape and function.

Value of cardiac MRI in evaluation of left atrial function in the postoperative tetralogy of Fallot and pulmonary stenosis patients / 中华放射学杂志

Objective:To explore the value of cardiac MRI (CMRI) in evaluating left atrial function in patients with postoperative tetralogy of Fallot (rTOF) and postoperative pulmonary stenosis (rPS).Methods:Totally 67 pediatric patients (49 with rTOF, 18 with rPS) with preserved left ventricular ejection fraction (EF) were recruited between January 2019 and October 2021 in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiao Tong University. Thirty-three healthy volunteers, matched in gender and age, were included as controls from July 2017 to August 2018. Left atrial EF, strain and strain rate of three phases (reservoir, conduit and pump), left atrial volume (maximum volume index, minimum volume index and pre-atrial contraction volume index) were measured with corresponding cardiac function analysis software. Then, the differences in these parameters were analyzed between the three groups by ANOVA or Kruskal-Wallis test with post hoc comparison and Bonferroni correction.Results:Compared with controls, patients with rTOF had lower reservoir function parameters (EF, strain and strain rate), conduit EF, conduit strain, and left atrial maximum volume index ( P<0.05), but higher pump EF ( P<0.05). In patients with rPS, only the reservoir strain rate decreased compared with controls ( P<0.05), and the remaining data showed no significant difference ( P>0.05). The reservoir and conduit EF and strain in patients with rPS were higher than those in patients with rTOF ( P<0.05). Conclusions:In patients with rTOF and rPS, left atrial function has changed despite the preservation of left ventricular EF, which may be an early marker of left ventricular diastolic dysfunction. In children with rTOF, left atrial reservoir and conduit functions decreased while the pump function increased. The reservoir and pump functions in rPS were better than those in rTOF. In addition, CMRI can detect left atrial dysfunction early before it enlarged.

A Case of Surgical Treatment of a Low-Birth-Weight Infant with Tetralogy of Fallot/Pulmonary Valve Absence, Cervical Arch, and Single Coronary Artery / 日本心臓血管外科学会雑誌

The case is an infant referred to the mother after a fetal ultrasound at 22 gestational weeks revealed a left renal cyst and congenital heart disease. He was born at 36 weeks and five days, weighing 2,282 g, with Apgar 8/8 points, and diagnosed with tetralogy of Fallot, absent pulmonary valve, pulmonary regurgitation, persistent left superior vena cava, right aortic arch, and cervical arch. He was placed on ventilatory management immediately after birth, but was extubated at age nine days. On approximately day 26, since the SpO2 markedly decreased and bronchoscopy showed only mild stenosis, we concluded that decreased pulmonary blood flow mainly caused cyanosis. Because of the low body weight and presence of a single coronary artery, we decided to proceed with a palliative surgery. However, since the patient had a cervical arch and the usual Blalock-Taussig shunt was anatomically difficult, we performed a central shunt＋main pulmonary artery ligation on day 49. Cyanosis improved and his respiratory condition was stable, but contrast-enhanced computed tomography showed a tendency for left pulmonary artery enlargement and left main bronchus compression. On day 87, the Rastelli procedure＋bilateral pulmonary artery plication was performed because worsening airway symptoms were anticipated. The patient’s respiratory condition stabilized postoperatively, and he was extubated 10 days postoperatively. On day 136, the patient was discharged with a home high-flow nasal cannula.

Tetralogy of Fallot with an Anomalous Course of the Brachiocephalic Vein

ABSTRACT Clinical data: Infant, 11-month-old, male, diagnosis of Tetralogy of Fallot with retrotracheoesophageal course of the brachiocephalic vein. Usual findings of Tetralogy of Fallot on physical examination. Technical description: Chest radiography showed slightly reduced pulmonary vascular markings and no cardiomegaly. Normal preoperative electrocardiogram with postoperative right bundle branch block. Usual findings of Tetralogy of Fallot on echocardiogram. Postoperative computed tomography angiography confirmed left brachiocephalic vein with anomalous retrotracheoesophageal course, configuring a U-shaped garland vein, in addition to postoperative findings of total correction of Tetralogy of Fallot. Operation: Complete surgical repair was performed with pulmonary valve commissurotomy and placement of bovine pericardial patch to solve right ventricular outflow tract obstruction, pulmonary trunk enlargement, and ventricular septal defect closure. Comments: Systemic venous drainage may show variations in patients with Tetralogy of Fallot. These abnormalities are usually of little clinical relevance, as they are asymptomatic. We presented a rare case of retrotracheoesophageal course of an anomalous left brachiocephalic vein with intraoperative diagnosis, confirmed by imaging during postoperative follow-up, without compromising clinical management or surgical approach.

Predicting Factors of Surgical Mortality in Children and Adolescents Undergoing Correction of Tetralogy of Fallot

Abstract Background In tetralogy of Fallot, correction surgery is a priority choice, seeing that it is desirable to minimize pulmonary hypoflow and severe hypoxemia, which result in hypoxemic crises, with sudden worsening of cyanosis, tachypnea, and, in some cases, loss of consciousness, seizures, and even death. Objective To evaluate the predicting factors of surgical mortality in children and adolescents undergoing correction of tetralogy of Fallot. Methods Retrospective cross-sectional study carried out by consulting all surgical records of the Child and Adolescent Cardiology Service of the Brazilian National Institute of Cardiology, during the period from 2007 to 2010. Results with p values < 0.05 were considered significant. Results The study evaluated 93 medical records. In relation to the characteristics of the population at the time of surgery, the median age was 3.69 (2.13 to 5.79) years, and 58.06% (n = 54) were male. White was the most common skin color, accounting for 55.43% (n = 51) of cases. In relation to assessment of nutritional status, median weight was 13.25 (10.10 to 17.60) kg, and body mass index was 14.49 (13.44 to 16.28) kg/m2. Down syndrome was present in 11.83% (n = 11) of the patients. All patients underwent correction surgery (n = 93, 100%). Prior cyanotic crisis was found in 53.85% (n = 49) with p = 0.013; surgical procedure duration was 218.83 ± 60.63 minutes, with p = 0.003, and lactate was 1.88 ± 1.33 mg/dL during the immediate postoperative period, with p = 0.009. Regarding the outcome of surgical death, it was found in 15.05% (n = 14) of patients. Mean follow-up lasted 5.68 ± 3.76 years. Conclusions According to the factors analyzed, the duration of the surgical procedure, prior cyanotic crisis, and blood lactate level may be relevant to surgical mortality.

Adult Congenital Heart Disease: Report from a Public Reference Hospital in Northeastern Brazil

ABSTRACT Introduction: The increasing worldwide number of adults with congenital heart disease (CHD) demands greater attention from health professionals. The purpose of this report is to describe the clinical demographic profile, frequency, and invasive treatment status of adults with CHD in a public reference hospital in northeastern Brazil. Methods: This is a retrospective cross-sectional study including 704 patients attended between August 2016 and August 2020. Data were collected from virtual database. Results: Patients' age varied from 17 to 81 years (mean 32±14; median 27 years); 294 (41.8%) patients were male, and 410 (58,2%) were female; 230 (32,7%) had diagnosis from age 18 and up. Cardiac complexity categories were "simple defects" (134 [19%] patients), "moderate complexity" (503 [71.5%]), and "great complexity" (67 [9.5%]). Atrial septal defect (ASD) was diagnosed in 216 (30.7%) patients, ventricular septal defect (VSD) in 101 (14.3%), tetralogy of Fallot in 93 (13.2%), and other CHD in 294 (41.8%). New York Heart Association (NYHA) functional classes were I (401 [57%]), II (203 [28.8%]), III (76 [10.8%]), and IV (24 [3.4%]). Complications were arrhythmias (173 [24%]) and severe pulmonary hypertension (69 [9.8%]). Invasive treatments were corrective surgery (364 (51.6%]), reoperation (28 [4.0%]), palliation (11 [1.6%]), interventional catheterization (12 [1.7%]), surgery plus interventional catheterization (5 [0.7%]), and preoperation (91 [12.9%]). Treatment was not required in 102 (14,5%) patients, and 91 (12.9%) were inoperable. Conclusion: The leading diagnosis was ASD. Frequency of unrepaired patients was high, mainly ASD, due to late diagnosis, which favored complications and denotes a matter of great concern.

Frequency of chromosome 22q11.2 deletion among newborns with non-syndromic congenital heart defects from western Mexico / Frecuencia de la deleción del cromosoma 22q11.2 en recién nacidos con cardiopatías congénitas no sindrómicas del occidente de México

Abstract Background: Congenital heart defects (CHD) are among the most frequent manifestations of 22q11.2 deletion syndrome. Although we found relatively few studies aimed at specifically detecting 22q11.2 deletion in newborns (NB) with CHD, none of them has been performed in Mexico. Methods: We conducted a prospective hospital-based study from January 2017 to March 2021 in the Genetics and Pediatric Cardiology Services of the Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, Mexico). All consecutive NBs identified with any non-syndromic major CHD confirmed by echocardiography were eligible to participate. A total of 98 NBs were included, 51 males and 47 females. Fluorescence in situ hybridization (FISH) analysis was conducted to search for deletion of chromosome 22q11.2 in interphase nuclei of standard lymphocyte cultures. Results: We found eight patients (8.2%) with CHD and the 22q11.2 deletion, all of them with conotruncal defects, particularly of the truncus arteriosus (p = 0.013), tetralogy of Fallot (p = 0.024), and pulmonary atresia with ventricular septal defect (p = 0.031) subtypes. With de exception of one infant with hypocalcemia and another with hypocalcemia and thymic aplasia, the diagnosis of 22q11.2 deletion was not clinically suspected in the other patients. Conclusions: Our results confirm the importance of excluding the presence of the 22q11.2 deletion in every NB with CHDs, particularly of the conotruncal subtype, even in the absence of other manifestations.

Resumen Introducción: Las cardiopatías congénitas (CC) son una de las manifestaciones más frecuentes del síndrome de deleción 22q11.2. A pesar de que existen relativamente pocos estudios dirigidos a detectar específicamente la deleción 22q11.2 en recién nacidos (RN) con CC, ninguno de ellos ha sido realizado en México. Métodos: Se realizó un estudio prospectivo de base hospitalaria desde enero de 2017 hasta marzo de 2021 en los Servicios de Genética y Cardiología Pediátrica del Hospital Civil de Guadalajara Dr. Juan I. Menchaca (Guadalajara, México). Todos los RN consecutivos identificados con cualquier tipo de CC mayor no sindrómica confirmada por ecocardiografía fueron elegibles para participar. Se incluyeron 98 recién nacidos, 51 de sexo masculino y 47 de sexo femenino. Mediante el análisis de hibridación fluorescente in situ (FISH, por sus siglas en inglés) se realizó la búsqueda de la deleción del cromosoma 22q11.2 en núcleos en interfase de cultivos de linfocitos estándar. Resultados: Se encontraron ocho pacientes (8.2%) con CC y la deleción 22q11.2, todos ellos con defectos conotruncales, particularmente de los subtipos tronco arterioso (p = 0.013), tetralogía de Fallot (p = 0.024) y atresia pulmonar con comunicación interventricular (p = 0.031). Con excepción de un lactante con hipocalcemia y otro con hipocalcemia y aplasia tímica, el diagnóstico de deleción 22q11.2 no se sospechó clínicamente en los demás pacientes. Conclusiones: Los resultados de este trabajo confirman la importancia de excluir la presencia de la deleción 22q11.2 en todos los RN con CC, particularmente del subtipo conotruncal, incluso en ausencia de otras manifestaciones.

Fatores associados ao acidente vascular cerebral isquêmico em crianças e adolescentes com tetralogia de Fallot / Factores asociados con accidente vascular cerebral isquémico en niños y adolescentes con tetralogía Fallot / Factors associated with ischemic stroke in children and adolescents with tetralogy of Fallot

Objetivo: verificar os fatores associados ao acidente vascular cerebral isquêmico em crianças e adolescentes com tetralogia de Fallot. Método: estudo transversal, realizado com análise dos prontuários eletrônicos de crianças e adolescentes com tetralogia de Fallot acompanhados em um ambulatório especializado em cardiologia pediátrica no nordeste brasileiro. Os dados foram processados no SPSS 21.0. Aplicou-se Teste Qui-quadrado e/ou Exato de Fisher para medidas de associação (p≤0,05). Resultados: dos 104 prontuários eletrônicos analisados, o acidente vascular cerebral isquêmico na análise bivariada foi associado com hipotireoidismo, trombofilia, policitemia, endocardite, parada cardiorrespiratória, insuficiência renal aguda e insuficiência cardíaca. No modelo final da análise multivariada, foram preditores de acidente vascular cerebral isquêmico a trombofilia (p=0,011), policitemia (p<0,001) e parada cardiorrespiratória (p=0,005). Conclusão: trombofilia, policitemia e a parada cardiorrespiratória foram associados ao acidente vascular cerebral isquêmico em crianças e adolescentes com tetralogia de Fallot.

Objective: to verify the factors associated with ischemic stroke in children and adolescents with tetralogy of Fallot. Method: a cross-sectional study, carried out with analysis of electronic medical records of children and adolescents with tetralogy of Fallot followed up in an outpatient clinic specialized in pediatric cardiology in northeastern Brazil. The data was processed in SPSS 21.0. Chi-square and/or Fisher's exact test were used for association measures (p≤0.05). Results: of the 104 electronic medical records analyzed, ischemic stroke in the bivariate analysis was associated with hypothyroidism, thrombophilia, polycythemia, endocarditis, cardiac arrest, acute renal failure and heart failure. In the final multivariate analysis model, thrombophilia (p=0.011), polycythemia (p<0.001) and cardiac arrest (p=0.005) were predictors of ischemic stroke. Conclusion: thrombophilia, polycythemia, and cardiac arrest were associated with ischemic stroke in children and adolescents with tetralogy of Fallot.

Objetivo: verificar los factores asociados al ictus isquémico en niños y adolescentes con tetralogía de Fallot. Método: estudio transversal, realizado con análisis de registros médicos electrónicos de niños y adolescentes con tetralogía de Fallot seguidos en una consulta externa especializada en cardiología pediátrica en el noreste de Brasil. Los datos se procesaron en SPSS 21.0. Para las medidas de asociación se utilizó la prueba de Chi-cuadrado y / o Exacta de Fisher (p≤0.05). Resultados: de las 104 historias clínicas electrónicas analizadas, el ictus isquémico en el análisis bivariado se asoció con hipotiroidismo, trombofilia, policitemia y las complicaciones fueron endocarditis, parada cardiorrespiratoria, insuficiencia renal aguda e insuficiencia cardíaca. En el modelo final del análisis multivariado, los predictores de ictus isquémico fueron trombofilia (p = 0,011), policitemia (p <0,001) y paro cardíaco (p = 0,005). Conclusión: la trombofilia, la policitemia y el paro cardiorrespiratorio se asociaron al ictus isquémico en niños y adolescentes con tetralogía de Fallot.

Tetralogia de Fallot Associada a Artéria Subclávia Direita Aberrante. Implicações Clínicas / Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications

Resumo Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).

Abstract Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).

Tetralogia de Fallot em crianças e adolescentes do Nordeste brasileiro: um estudo descritivo / Tetralogía de Fallot en niños y adolescentes del nordeste de Brasil: un estudio descriptivo / Tetralogy of Fallot in children and adolescents in northeastern Brazil: A descriptive study

Objetivo: caracterizar o perfil demográfico e clínico de crianças e adolescentes com tetralogia de Fallot e suas complicações clínicas. Materiais e método: estudo exploratório, de caráter descritivo, quantitativo, de corte transversal, com base na iniciativa Strengthening the Reporting of Observational Studies in Epidemiology (Strobe), a partir da análise dos prontuários eletrônicos de crianças e adolescentes acompanhadas em um ambulatório de referência em cardiologia e cirurgia cardiovascular pediátrica no Nordeste do Brasil. Os dados foram coletados de 2017 a 2019. Foram avaliadas variáveis demográficas, clínicas e complicações ocorridas, e calculadas a mediana, intervalo interquartil, frequências absolutas e relativas. Resultados: das 670 crianças e adolescentes atendidos com cardiopatia congênita, 104 (15,5%) apresentam diagnóstico de tetralogia de Fallot; a maioria era do sexo masculino (59,6%) e com idade entre 10 e 19 anos (49%). Foram evidenciadas complicações como acidente vascular cerebral isquêmico (6,7%), endocardite (2,9%) e insuficiência cardíaca (1,9%). Conclusões: faz-se necessário que os serviços e os profissionais de saúde, em especial o enfermeiro, estejam preparados para identificar os sinais e sintomas da tetralogia de Fallot e reconheçam as possíveis complicações relacionadas a essa cardiopatia congênita a fim de promover um cuidado de saúde com qualidade.

Objetivo: caracterizar el perfil demográfico y clínico de niños y adolescentes con tetralogía de Fallot y sus complicaciones clínicas. Materiales y método: estudio exploratorio, descriptivo, cuantitativo, transversal, basado en la iniciativa Fortalecimiento del Reporte de Estudios Observacionales en Epidemiología (STROBE), que incorpora el análisis de las historias clínicas electrónicas de niños y adolescentes en seguimiento por consulta externa en cardiología y cirugía cardiovascular pediátrica en el nordeste de Brasil. Se recolectaron datos de 2017 a 2019, analizando variables demográficas, clínicas y complicaciones. Además, se realizó el cálculo de la mediana, el rango intercuartílico y las frecuencias absolutas y relativas. Resultados: de los 670 niños y adolescentes tratados con cardiopatía congénita, 104 (15,5%) fueron diagnosticados con tetralogía de Fallot, la mayoría eran de sexo masculino (59,6%) entre 10 y 19 años (49%). Se evidenciaron complicaciones como ictus isquémico (6,7%), endocarditis (2,9%) e insuficiencia cardíaca (1,9%). Conclusiones: es necesario que los servicios y los profesionales de la salud, especialmente en enfermería, estén preparados para identificar los signos y síntomas de la tetralogía de Fallot y así reconocer posibles complicaciones relacionadas con esta cardiopatía congénita, con el fin de promover una atención de la salud con calidad.

Objective: To characterize the demographic and clinical profile of children and adolescents with tetralogy of Fallot and their clinical complications. Materials and method: Exploratory, descriptive, quantitative, cross-sectional study, based on the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) initiative. This study involved the analysis of electronic medical records of children and adolescents under follow-up at a reference outpatient clinic in cardiology and pediatric cardiovascular surgery in northeastern Brazil. Data were collected from 2017 to 2019. Demographic and clinical variables and complications were evaluated, and the median, interquartile range, absolute and relative frequencies were calculated. Results: Out of 670 children and adolescents treated with congenital heart disease, 104 (15.5%) were diagnosed with tetralogy of Fallot. Most of these were male (59.6%) aged between 10 and 19 (49%). Among the complications identified we can mention ischemic stroke (6.7%), endocarditis (2.9%), and heart failure (1.9%). Conclusions: It is necessary that health services and professionals, especially nurses, are prepared to identify the signs and symptoms of tetralogy of Fallot and recognize the possible complications related to this congenital heart disease in order to promote quality health care.

Intracardiac Repair in Late Adolescent and Adult Tetralogy of Fallot — Early and Midterm Results from a Tertiary Care Centre

Abstract Introduction: In developing countries like India, it is common for late presentation of Tetralogy of Fallot (TOF) patients to a hospital as compared to that of developed countries. The objective of this study is to analyze the surgical outcome of TOF patients with age > 15 years. Methods: This is a retrospective descriptive study of the surgical outcomes of 45 adult patients undergoing correction for TOF. Epidemiology, symptomology, and preoperative evaluation were performed. Results: Most of the patients were male (33 [73%]). The median age was 21 years. A total of 42 (93.33%) patients had subaortic ventricular septal defect (VSD), while three (6.6%) patients presented with doubly committed VSD. The most common type of right ventricular outflow tract (RVOT) obstruction was combined infundibular and valvular types, accounting for 34 cases (75.5%). Six patients had infundibular RVOT obstruction, while three patients (6.6%) had predominantly valvular pulmonary stenosis. We performed trans-right atrial repair in 33 patients. Right atrium-pulmonary artery approach was used in five patients (11.1%). The most common postoperative complication was right bundle branch block, seen in 14 patients, with a mortality rate of 2% in the early postoperative period. We achieved excellent early and midterm survival results and significant improvement in functions and disease-free quality of life. Conclusion: Intracardiac repair in adult TOF can be performed with low mortality, less residual RVOT obstruction, and need for revision of RVOT far less frequent by using the Jhajhria Infundibular Resection Adequacy Assessment technique (JIRAAT) to assess for adequacy of infundibular resection.