scholarly journals A case report of late physiologic repair of congenitally corrected transposition of the great arteries and pulmonary stenosis in a severely cyanotic patient: better late than never

2021 ◽  
Author(s):  
Paolo Ferrero ◽  
Massimo Chessa ◽  
Alessandro Varrica ◽  
Alessandro Giamberti
Keyword(s):  
Nyha Class ◽  
Pulmonary Stenosis ◽  
Pacemaker Implantation ◽  
Medical Attention ◽  
Transposition Of Great Arteries ◽  
Surgical Risk ◽  
Situs Solitus ◽  
First Time ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract Background Patients with congenitally corrected transposition of great arteries (ccTGA) not infrequently seek medical attention for the first time late in life. Optimal management of natural history ccTGA is debated and must be tailored. Case summary A 38 years old male patient was referred to our centre because of severe cyanosis and worsening dyspnoea. Investigations disclosed: situs solitus, mesocardia, double discordance, large ventricular septal defect (VSD), severe pulmonary stenosis, no significant atrioventricular valves regurgitation. The patient underwent physiologic repair: VSD closure, placement of a left ventricle to pulmonary artery conduit and epicardial atrio-biventricular pacemaker implantation. The conduit was intentionally undersized to promote tricuspid valve continence. Postoperative course was uneventful, trans-thoracic echocardiography showed good biventricular function without significant tricuspid regurgitation. At one month after discharge the patients is in NYHA class II. Discussion Management of late presenter patients with ccTGA depends on the associated lesion and estimation of surgical risk. In selected patients markedly symptomatic physiologic repair is a rationale option, providing a normal saturation and biventricular circulation with a significantly lower surgical risk as compared with anatomic repair.

scholarly journals Congenitally corrected transposition of great arteries: a case series of five unoperated African children

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Bernard Obongonyinge ◽  
Judith Namuyonga ◽  
Hilda Tumwebaze ◽  
Twalib Aliku ◽  
Peter Lwabi ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Pulmonary Stenosis ◽  
Valve Regurgitation ◽  
Ventricular Failure ◽  
Tricuspid Valve Regurgitation ◽  
Transposition Of Great Arteries ◽  
Anatomic Repair ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract Background Congenitally corrected transposition of great arteries (ccTGA) is rare. It is commonly associated with ventricular septal defect (VSD), pulmonary stenosis and heart block. Early anatomic repair is recommended between 3 and 6 months of age to prevent development of tricuspid valve regurgitation and systemic right ventricular failure. Case presentation We retrospectively identified five cases of ccTGA. Cases were between one and 13 years of age. All the cases were unoperated. Four of the five cases had associated intracardiac defects/complications. These included: VSD, pulmonary stenosis, tricuspid valve regurgitation, right ventricular systolic dysfunction and heart block. Conclusion These cases demonstrate the challenges of access to early diagnosis and surgery in a low resource setting. This delay in anatomic repair leads to complications of tricuspid valve regurgitation and systemic right ventricular failure.

Serial changes of tricuspid regurgitation after anatomic repair for congenitally corrected transposition

2020 ◽  
Vol 58 (1) ◽  
pp. 163-170 ◽  
Author(s):  
Tsubasa Furuya ◽  
Takaya Hoashi ◽  
Masatoshi Shimada ◽  
Kenta Imai ◽  
Motoki Komori ◽  
...  
Keyword(s):  
Tricuspid Regurgitation ◽  
Pacemaker Implantation ◽  
Hospital Death ◽  
Transposition Of Great Arteries ◽  
Anatomic Repair ◽  
Atrial Switch ◽  
The Mean ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract OBJECTIVES The aim of this study was to reveal the serial changes in tricuspid regurgitation (TR) after anatomic repair for congenitally corrected transposition of great arteries. METHODS Between 1995 and 2018, 48 patients underwent anatomic repair (atrial/arterial switch in 14 patients, atrial switch and Rastelli in 34 patients). The mean age and weight of the patients during anatomic repair was 33 (interquartile range 21.8–62.1) months and 12 (10.3–16.3) kg. The preoperative TR was less than mild in 15 patients (31.3%), mild-to-moderate in 29 patients (60.4%) and more-than-moderate in 4 patients (8.3%). Ebsteinoid dysplasia of the tricuspid valve (TV) was observed in 7 patients (14.6%). During the study period, no patient underwent TV surgery or bidirectional Glenn anastomosis at the time of anatomic repair. RESULTS There was 1 in-hospital death and 1 late death. The follow-up was completed by other surviving patients, with a median follow-up period of 12.1 years (5.9–18.1). The overall survival, reoperation-free survival and freedom from permanent pacemaker implantation rate at 15 years were 94.3%, 74.3% and 81.5%, respectively. The mean TR grade was 2.0 (1.0–2.6) preoperatively, 2.0 (1.0–2.0) at 1 year, 2.0 (2.0–2.0) at 5 years and 2.0 (2.0–2.0) at 10 years after anatomic repair. A Cox proportional hazards model showed that association of Ebsteinoid dysplasia of the TV, type of anatomic repair and previous pulmonary artery banding did not affect freedom from death or the more-than-moderate TR rate. There were 2 patients who underwent TV surgery after the anatomic repair for severe TR; TV repair was successfully done for 1 patient, the other required semi-closure of TV and one and one-half ventricle conversion. CONCLUSIONS TR remained subclinical or improved in the majority of patients after anatomic repair without TV repair. However, there were a few patients whose TR progressed to severe or massive, then required TV surgery after anatomic repair. Although exposure was difficult, TR was sometimes repairable following atrial switch, otherwise, one and one-half ventricle repair conversion would be the choice of treatment.

scholarly journals Augmented-reality computed tomography-guided transcatheter pacemaker implantation in dextrocardia and congenitally corrected transposition of great arteries

2018 ◽  
Vol 25 (3) ◽  
pp. 412-413 ◽  
Author(s):  
Maksymilian P. Opolski ◽  
Ilona M. Michałowska ◽  
Bartosz A. Borucki ◽  
Barbara Nicińska ◽  
Łukasz Szumowski ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Augmented Reality ◽  
Pacemaker Implantation ◽  
Transposition Of Great Arteries ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

scholarly journals Low Dose Combined Spinal-Epidural Anesthesia: An Anesthesia Technique for Cesarean Section in a Patient Suffering from Congenitally Corrected Transposition of Great Arteries and Severe Pulmonary Stenosis

2021 ◽  
Author(s):  
Dhruv Jain ◽  
Kaushal Kumar ◽  
Abishek Singh ◽  
Jyotsna Punj
Keyword(s):  
Cesarean Section ◽  
Epidural Anesthesia ◽  
Low Dose ◽  
Pulmonary Stenosis ◽  
Transposition Of Great Arteries ◽  
Symptomatic Management ◽  
Combined Spinal Epidural Anesthesia ◽  
Spinal Epidural ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

A primigravida, aged 22 years, at 39th gestational week was hospitalized with dyspnea since onset of labor. On systemic evaluation and echocardiography, she was diagnosed to have congenitally corrected transposition of the great arteries (CCTGA) along with severe pulmonic stenosis and bidirectional ventricular and atrial septal defects. A multidisciplinary team provided initial symptomatic management, stratified the risk and planned for a cesarean section. A low-dose combined sequential spinal-epidural anesthesia with invasive monitoring was used which provided excellent surgical condition with stable intraoperative and postoperative hemodynamics.

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