Double root translocation: Early experience on performance of translocated aortic and pulmonary root.

AIM: Transposition of great arteries with ventricular septal defect and pulmonary stenosis is a complex congenital cardiac malformation with varied treatment options. Double root translocation is one of the described techniques with several advantages over other techniques. Our goal of this study is to report our early experiences on the performance of translocated aortic and pulmonary root. METHODS: From 2016 to 2019, a total of 12 patients (seven boys and five girls) aged from one year to three years underwent double root translocation. All our patients were diagnosed with dextro-transposition of the great arteries (d-TGA)/double-outlet right ventricle (DORV), ventricular septal defect (VSD), and pulmonary stenosis (PS). One patient (8.33%) presented with (IL 2R CX), remaining 11 patients (91.66%) had (IL CX 2R) as their coronary pattern. Mean follow-up period was two years (1-3 years). RESULTS: At six months follow up, one patient died due to severe MR, one patient had severe TR and was taken back for surgery after two weeks post-surgery. Three patients had trivial aortic regurgitation (AR) and nine had no AR, two patients had trivial mitral regurgitation (MR) and 10 had no MR 11 patients had post-operative sinus rhythm and were in NYHA class 1. One patient with a complete heart block was supported with extracorporeal membrane oxygenator followed by tracheostomy. Redo TV repair was done in one patient. Peritoneal dialysis was accomplished in two patients. CONCLUSIONS: Double root translocation is a good alternative surgical treatment for TGA/DORV, VSD, and PS, where the pulmonary valve is small, but the leaflets are usable. Although the operation is technically demanding, our early follow-up on translocated root performance shows promising outcomes.

"Repair of common atrioventricular junction in isolation and when associated with other congenital heart defects".

Management of atrioventricular septal defects has grown leaps and bounds since the first correction of this malformation. Main reasons considered for the improvement are good understanding of lesion anatomy, precise preoperative diagnosis including imaging, progress in surgical technique, myocardial protection, and post-operative care. In this article we review the anatomy, pathophysiology, clinical features, associations, diagnosis, surgical therapy, complications, and results of atrioventricular septal defects.

Aortic root translocation: the Bex-Nikaidoh procedure.

Patients with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction are managed by various techniques. Traditional techniques include intracardiac baffling with a right ventricle to pulmonary artery conduit (Rastelli procedure) and the "reparation a l'etage ventriculaire" (REV) procedure. However, other techniques such as aortic root translocation (Bex-Nikaidoh procedure), pulmonary root translocation, and double root translocation are equivalent or in fact provide better results than the traditional techniques. In this review we will have a detailed discussion about aortic root translocation which allows a more normal and anatomically aligned left ventricular outflow tract. However, the operation is technically demanding, and coronary translocation remains one of the major challenges for successful root translocation.

Double-root transfer and the half-turned truncal switch.

Transposition of great arteries (TGA) or double-outlet right ventricle (DORV) with ventricular septal defect (VSD) and pulmonary stenosis (PS) is a complex congenital cardiac malformation, which can be treated by both univentricular and biventricular pathways. Biventricular repair includes Rastelli operation, reparation a l'etage ventriculaire (REV), pulmonary root translocation, and Nikaidoh procedures. Double-root translocation (DRT) technique and half-turned truncal switch technique (HTTS) are being considered as useful alternatives. In this review, we will see in detail about DRT and HTTS.

Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction.

A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures, and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful; oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one of the options in such patients.

Options for coronary translocation and other considerations in aortic root translocation (Bex-Nikaidoh procedure).

INTRODUCTION: The surgical options for patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction include intracardiac baffling with the right ventricle to pulmonary artery (PA) conduit (Rastelli procedure), "reparation a l'etage ventriculaire" or aortic root translocation (Bex-Nikaidoh procedure). The Bex-Nikaidoh procedure allows a more normal, anatomically aligned left ventricular outflow tract. However, the operation is technically demanding, and coronary translocation remains one of the major challenges for successful root translocation. METHODS: All patients who underwent aortic root translocation in a single institute over a period of 2 years from January 2015 to December 2017 were included in the study. Surgical technique and early outcomes are described with specific focus on the different observed coronary artery patterns and surgical strategies for translocation. RESULTS: Fourteen patients underwent aortic root translocation. The coronary artery patterns observed could be categorized into four different patterns based on the size of the pulmonary annulus and the relative position of the PA relative to the aorta. Successful translocation of the coronary arteries was achieved in every patient. Mean follow-up was 18.42 ± 9.22 months. There was no mortality and no reoperation during the follow-up period. CONCLUSION: The Bex-Nikaidoh procedure is a promising surgical option for TGA, VSD, and pulmonary stenosis. Good outcomes are achievable despite wide variations in anatomy using a tailored approach for coronary translocation.

Interesting case of left subclavian pseudoaneurysm in a child managed successfully.

Subclavian artery aneurysm is extremely rare, and further aneurysm in a 3-year-old child is more exceptional. Most common causes of subclavian artery aneurysms are atherosclerosis, poststenotic thoracic outlet syndrome, infectious, trauma, and inflammatory arteritis, and connective tissue disorders. Pseudoaneurysms may be due to trauma or any iatrogenic injury to subclavian artery. We present a case report of a 3-year-old male child presented to us with recurrent cough without expectoration for 4-months duration due to compression by a large subclavian artery aneurysm. Patient was successfully managed surgically through left thoracotomy approach. After operation, the patient improved symptomatically. Child was asymptomatic at 3-month follow-up.

Adherent Intrapericardial Teratoma: A Case Report.

Intrapericardial teratomas are rare tumours arising from all three germinal layers. They are mostly benign, but can lead to life-threatening complications such as cardiac tamponade in newborns, so surgical excision of the tumour should be done as early as possible once the diagnosis is made. We report a neonate diagnosed with a large intrapericardial teratoma, managed surgically with the help of cardiopulmonary bypass.

Cervicothoracic Thymic Cyst: An Unusual Presentation.

Cervicothoracic thymic cysts are rare and difficult to diagnose preoperatively. We report a case of a cervicothoracic thymic cyst presenting as a lateral neck mass and mimicking a laryngocele in a 3-year-old boy and its definitive management.

Unusual presentation of total anomalous systemic venous connection.

A 9-year-old girl who presented with dyspnea on exertion was diagnosed with total anomalous systemic venous connection to the left atrium (both venae cavae), no left superior vena cava, and a moderate-sized atrial septal defect with severe pulmonary arterial hypertension and ectopic atrial rhythm. She underwent septation of the common atrium using autologous pericardium, thereby rerouting the superior vena cava, inferior vena cava, and coronary sinus to the right atrium. Her postoperative course was uneventful. This case is reported for its rarity of presentation with severe pulmonary arterial hypertension and ectopic atrial rhythm.