Cardiac Laterality: Surgical Results of Right Atrial Isomerism.

Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2-9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock-Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, p < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality.

Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries in a Criss-Cross Heart.

Congenitally corrected transposition of great arteries is an uncommon congenital heart disease characterized by discordance at both the atrioventricular and ventriculoarterial connections. A rare subgroup of patients with congenitally corrected transposition of the great arteries also has a criss-cross heart. The morphology of these patients represents a diagnostic challenge that requires critical analysis to perform a satisfactory surgical procedure. We present a case of a 12-year-old patient with the above mentioned anatomy who underwent physiologic repair.

Replacement of Obstructed Right Ventricle to Pulmonary Artery Conduits: The Modified Peel Operation.

Reconstruction of the right outflow tract with extracardiac conduits has made complete repair of complex cardiac malformations possible. However, reoperation is usually required for a right ventricle-to-pulmonary artery conduit obstruction. We describe a modified peel operation, where the sides and posterior half of the previously placed conduit are preserved, and a prosthetic roof is placed over the conduit remnant. This has been our current technique to manage conduit obstructions. It is a safe operation and to teach residents. This review aims to convey the technical details of each step of this technique.

Coronary Revascularization in Patients With Cardiac Sequelae of Kawasaki Disease at a Single Center.

Background: Kawasaki disease (KD) is a self-limited vasculitis that mainly affects infants and preschool-age children, characterized by coronary aneurysms and/or stenoses that may lead to the occurrence of ischemia, heart attack, and sudden death. This study aimed to evaluate the clinical and surgical results of pediatric patients with KD and cardiac sequelae who underwent coronary artery bypass grafting. Methods: A retrospective study was carried out. Patients with a diagnosis of KD and cardiac sequelae who underwent coronary artery bypass grafting from January 1, 2004, through March 31, 2021, were included: preoperative characteristics and conditions. Results: Ten patients were included, with a mean age of 6.4 ± 3.7 years; 80% males. Seven (70%) had the compromise of 2 coronary arteries, the most affected being the left anterior descendent artery (36.9%) with aneurysm and the right coronary artery (36.8%) with stenosis. 40% had preoperative moderate-to-severe ventricular dysfunction. The graft most frequently used was the right internal thoracic artery (47.6%), and the most frequent coronary bypass target vessel was the left anterior descending artery (38.1%). There was no early mortality or deaths during follow-up; 90% remained in functional class I. Among the cohort as a group, there was an overall improvement in postoperative ventricular function (P = .03). This persisted, being unchanged at subsequent follow-up (P = .95). Conclusions: Coronary artery bypass grafting is an appropriate treatment option for children with cardiac sequelae of KD, with excellent surgical results. Furthermore, this surgical procedure avoids the deterioration of left ventricular function in pediatric age.

Surgical outcomes among children with bicuspid aortic valve: 17 years of experience in a single center. First report in Mexico.

PURPOSE: To evaluate the clinical and surgical outcomes among children with bicuspid aortic valve who underwent cardiac surgery. METHODS: This observational and retrospective study included patients with a diagnosis of bicuspid aortic valve who underwent cardiac surgery between January 1, 2003, and March 31, 2020. Demographic characteristics and perioperative conditions were described. RESULTS: One hundred and sixteen patients were included, with a mean age of 12.4 ± 4.2 years; 63.2% were male. The most frequent diagnosis was congenital aortic stenosis (23.5%), followed by connective tissue disorders (16%). Mechanical aortic prostheses were used in 87.7% of cases, with a mean size of 21 ± 2.6 mm. The main factors associated with mortality were valve prosthesis dysfunction (odds ratio [OR]: 12.44; 95% confidence interval [CI]: 1.05-147.48; p = .04) and reoperation (OR: 24.29; 95% CI: 1.03-570.08; p = .04). The overall survival was 87.9%, with better outcomes among those who did not undergo reoperation (Log Rank, p = .01). CONCLUSIONS: Outcomes after aortic valve replacement in children with bicuspid aortic valve are excellent in the short and long term, regardless of using mechanical or biological prostheses.

Total anomalous pulmonary venous connection: 16 years of surgical results in a single center.

PURPOSE: The aim of the study was to analyze the surgical outcome of patients with total anomalous pulmonary venous connection (TAPVC) who underwent cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of TAPVC undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2003 and June 30, 2019 were included. Descriptive statistics were calculated, as well as a bivariate analysis of the variables associated with mortality. A logistic regression model was included to determine risk factors associated with the main outcome and survival was analyzed using the Kaplan-Meier method. RESULTS: A total of 5314 patients diagnosed with congenital heart disease (CHD) underwent surgery, 414 (7.8%) were patients with TAPVC, with an average age of 17.1 ± 34.6 months, 58.2% were male. It was frequent in infants (61.6%) and preschool (19.6%). Predominant type was supracardiac TAPVC (47.4%). Pulmonary venous obstruction (PVO) occurred in 32.1%. Risk factors associated with mortality were infracardiac TAPVC (odds ratio [OR]: 3.26; 95% confidence interval [CI]: 1.17-9.03; p = .02), PVO (OR: 2.56; 95% CI: 1.05-6.22; p = .03) and postoperative mechanical ventilation (OR: 1.005; 95% CI: 1.002-1.008; p = .01). Overall survival was 87.2%, with better outcomes in adolescents (100%), children (94.1%), mixed TAPVC (96%), and cardiac TAPVC (91.9%; p < .001). CONCLUSIONS: The survival of our institution after surgical correction of TAPVC is similar to that of other referral centers, where patients with infracardiac TAPVC and newborns worse outcomes. All patients must undergo a rigorous evaluation to determine an adequate repair strategy.

Origen anómalo de una de las ramas de la arteria pulmonar (OAAP): tratamiento quirúrgico y resultados / Anomalous origin of one of the branches of the pulmonary artery (AOPA): Surgical treatment and outcome

Resumen Introducción: El origen anómalo de una de las ramas de la arteria pulmonar procedente de la aorta ascendente es poco frecuente. Objetivo: Identificar las características clínicas y quirúrgicas de los pacientes sometidos a reimplante de la rama afectada. Método: Se realizó un estudio observacional, transversal, descriptivo, retrospectivo y retrolectivo, en el que se incluyeron los pacientes diagnosticados de origen anómalo de alguna de las ramas de la arteria pulmonar y que fueron tratados mediante cirugía correctiva en el Instituto Nacional de Cardiología Ignacio Chávez, en el periodo del 1 de enero de 2003 al 31 de enero de 2019. De los expedientes se extrajeron las características demográficas, los antecedentes quirúrgicos, los diagnósticos, los reportes ecocardiográficos y tomográficos, la técnica quirúrgica y el estado posquirúrgico. Resultados: Se encontraron nueve pacientes sometidos a cirugía de corrección, con un promedio de edad de 2 ± 2 años, un peso de 11.4 ± 1.5 kg y una talla de 82 ± 15 cm. El 67% fueron de sexo masculino. La rama afectada con más frecuencia fue la derecha; el 68% se originaban directamente de la aorta y el 42% de un conducto arterioso persistente. La corrección con circulación extracorpórea tuvo un tiempo promedio de pinzamiento aórtico de 35 minutos y de soporte circulatorio de 45 minutos. Se utilizó el implante directo o con material sintético. Las complicaciones fueron falla ventricular, sangrado, arritmias y neumonía nosocomial. La mortalidad fue del 11%. Conclusiones: El tratamiento quirúrgico para el reimplante de la rama anómala de la arteria pulmonar es el procedimiento de elección, y con cuidados quirúrgicos y posteriores tiene un buen pronóstico a mediano y largo plazo.

Abstract Introduction: The anomalous origin of one of the branches of the pulmonary artery from the ascending aorta is rare. Objective: To identify the clinical and surgical characteristics of the patients undergoing reimplantation of the affected branch. Method: An observational, cross-sectional, descriptive, retrospective and retrolective study was performed; patients diagnosed with anomalous origin of one of the branches of the pulmonary artery and treated by corrective surgery at the Instituto Nacional de Cardiología Ignacio Chavez, in the period from January 1, 2003 to January 31, 2019, were included. It was extracted from the files: demographic characteristics, surgical antecedents, diagnoses, echocardiographic and tomographic reports, surgical technique and post-surgical status. Results: Nine patients underwent correction surgery, with an average age of 2 ± 2 years, 11.4 ± 1.5 kg and height 82 ± 15 cm; 67% were male. The most frequent affected branch was the right, 68% originated directly from the aorta and 42% from a patent ductus arteriosus. The correction with extracorporeal circulation had an average aortic cross-clamp of 35 minutes and circulatory support of 45 minutes, the direct or synthetic implant was used. The complications were ventricular failure, bleeding, arrhythmias and nosocomial pneumonia. Mortality was 11%. Conclusions: Surgical treatment for reimplantation of the anomalous branch of the pulmonary artery is the procedure of choice, which with surgical and subsequent care has a good prognosis in the medium and long term.

Cardiac surgery in patients with atrial isomerism: Long-term results and outcomes.

PURPOSE: The aim of the study was to identify, determine, and analyze the clinical and surgical outcomes of patients with atrial isomerism (AI) undergoing cardiac surgery. METHODS: A retrospective study was carried out. Patients with diagnosis of AI undergoing cardiac surgery at the National Institute of Cardiology Ignacio Chávez, from January 1, 2010 and March 31, 2020 were included; demographic characteristics and perioperative conditions of the patients were considered. RESULTS: Sixty-five patients were included, with an average age of 6.4 ± 4.9 years, 50.8% males. Thirty-eight (58.5%) had right atrial isomerism (RAI) and 27 (41.5%) had left atrial isomerism (LAI); univentricular physiology (78.5%) predominated. Atrioventricular septal defect (AVSD) in RAI and septal defects in LAI were identified as the main associated defects. The most common surgical procedures performed were modified Blalock-Taussig shunt (MBTS) (27.6%), MBTS with total anomalous pulmonary venous connection (TAPVC) repair (15.3%) and total cavopulmonary connection (TCPC) with an extracardiac conduit fenestrated (10.8%); 100% RAI required a univentricular approach, while in LAI it was 48.1%. Overall survival was 92.3%, with 100% survival in LAI with biventricular physiology and 86.8% in RAI with univentricular physiology. CONCLUSIONS: The survival of our institution is similar to that of other referral centers, where patients with LAI had a better evolution than RAI; in addition, the univentricular approach was required in all with RAI. Patients with AI must undergo a rigorous evaluation to determine an adequate repair strategy, considering univentricular RAI with a high possibility of morbidity and mortality.

[Isomorfismo cardiaco: Una perspectiva multidisciplinaria]. / Atrial isomerism: A multidisciplinary perspective

Atrial isomerism describes complex anatomical findings with defects in the determination of lateralization; being a rare situation, with a prevalence of 1 in every 10.000 to 20.000 live births, with an incidence of up to 4% of all cardiac malformations. The diagnosis can be made in the neonatal age; however, clinical presentation is nonspecific. Depending on the spectrum of malformations, complex and invasive diagnostic tools may be required. Treatment is varied and can range from palliative surgery in view of univentricular physiology to total correction surgery for biventricular repair.

El isomorfismo cardiaco describe hallazgos anatómicos complejos con defectos en la determinación de la lateralización. Es una situación poco frecuente, con prevalencia de 1 en cada 10,000 a 20,000 nacidos vivos, con incidencia hasta del 4% de todas las malformaciones cardiacas. El diagnóstico puede realizarse en la etapa neonatal; sin embargo, el cuadro clínico es inespecífico. De acuerdo con el espectro de malformaciones se pueden necesitar medios diagnósticos complejos e invasivos. El tratamiento es variado y puede ir desde la cirugía paliativa en vista de una fisiología univentricular hasta una cirugía de corrección total para una reparación biventricular.

Impact of the COVID-19 Pandemic on Congenital Cardiac Surgeries at a National Referral Center in Mexico.

Severe acute respiratory syndrome coronavirus 2, the etiologic agent of coronavirus disease 2019, has caused more than 160 million infections globally. The experience of our department showed that the execution of a strict surgery protocol, universal severe acute respiratory syndrome coronavirus 2 screening, surgical prioritization, and an orderly reactivation of the cardiac surgery program is a feasible way to keep congenital heart diseases surgery program.

Rehabilitation of pulmonary arteries: Hybrid treatment in the unilateral absence of the pulmonary artery.

BACKGROUND: Unilateral abscence of pulmonary artery is a rare congenital heart disease. If these anomaly is unrecognized and treated the risk of pulmonary hypertension in the contralateral lung can be lethal for the patients. AIMS: To report the successful sequential hybrid-surgical approach of two cases of rescue of pulmonary branches only fed by ductus arteriosus. MATERIALS & METHODS: Description of two rare cases of absence of a unilateral pulmonary branch successfully treated in our institution. RESULTS, DISCUSSSIION AND CONCLUSION: The timely identification of these cases and the strategic planning of interventional and surgical hybrid sequential treatment of these rare cases is effective.