An extremely rare clinical entity: congenitally corrected transposition with situs ınversus and single coronary artery presented with complete atrioventricular block in a young man

2018 ◽  
Vol 28 (5) ◽  
pp. 759-761
Author(s):  
Omer Faruk Cirakoglu ◽  
Ali Bayraktar ◽  
Muhammet Rasit Sayin
Keyword(s):  
Coronary Artery ◽  
Atrioventricular Block ◽  
Situs Inversus ◽  
Single Coronary Artery ◽  
Complete Atrioventricular Block ◽  
Rare Form ◽  
Complete Atrioventricular ◽  
Congenitally Corrected Transposition ◽  
Single Coronary Artery Anomaly ◽  
Corrected Transposition

AbstractCongenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

The prenatal diagnosis of, and short-term outcome for, patients with congenitally corrected transposition

2004 ◽  
Vol 14 (3) ◽  
pp. 265-276 ◽  
Author(s):  
Enrico Chiappa ◽  
Angelo Micheletti ◽  
Andrea Sciarrone ◽  
Gianni Botta ◽  
Piero Abbruzzese
Keyword(s):  
Atrioventricular Block ◽  
Tricuspid Regurgitation ◽  
Fetal Life ◽  
Complete Atrioventricular Block ◽  
Initial Examination ◽  
Term Outcome ◽  
Favorable State ◽  
Complete Atrioventricular ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Congenitally corrected transposition is a rare congenital anomaly, with only a few cases diagnosed and reported prenatally even in the largest fetal series. To determine the morphologic features and outcome for the lesion as recognized during fetal life, we reviewed the fetal and postnatal echocardiograms and medical records of 11 consecutive cases of congenitally corrected transposition. These were identified among 230 (4.7%) consecutive cases of structural cardiac disease referred to our fetal cardiology unit over a period of 4 years. The mean gestational age at diagnosis was 24.7 weeks. Reasons for referral were suspected complete transposition, abnormal position of the heart, and bradyarrhythmias. Associated cardiac lesions included an abnormal cardiac position in 6 cases, ventricular septal defect in 8, obstruction of the subpulmonary outflow tract in 6, tricuspid valvar displacement in 5, and complete atrioventricular block in 2. Only 3 of the cases had mild tricuspid regurgitation prior to birth. Termination was chosen in 4 cases with severe obstruction to pulmonary flow. Of the remaining cases, 2 patients died at 3 and 12 months after birth, respectively. Both developed significant tricuspid regurgitation associated with unexpected major arrhythmias. The remaining 5 patients are alive and relatively well at a mean follow-up of 25.4 months. An epicardial pacemaker was inserted in 1 because of complete atrioventricular block. We conclude that prenatal counseling must be guarded following the diagnosis of congenitally corrected transposition, even in fetuses with an apparently favorable state at initial examination. Some of these cases may undergo major and unexpected changes, particularly with regard to cardiac rhythm and tricuspid valvar function, with concomitant significant changes in prognosis.

scholarly journals What is the Diagnosis?

2020 ◽  
Vol 32 (4) ◽  
pp. 275-277
Author(s):  
Jose Mario Baggio Junior ◽  
Joubert Ariel Pereira Mosquera ◽  
Luis Gustavo Ferreira Gomes ◽  
Wagner Luis Gali ◽  
Alvaro Valentim Lima Sarabanda
Keyword(s):  
Atrioventricular Block ◽  
Functional Capacity ◽  
Pacemaker Implantation ◽  
Complete Atrioventricular Block ◽  
Narrow Qrs ◽  
The Face ◽  
Complete Atrioventricular ◽  
Generator Replacement ◽  
Corrected Transposition

Patient T.D., 23 years old, female, with corrected transposition of the great arteries, complete atrioventricular block with narrow QRS, submitted to bicameral pacemaker implantation at 13 years of age, with generator replacement three years ago. She has a good functional capacity, but complains of fatigue in the face of intense effort.

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