Case Report
Complete transposition of the great arteries (d-TGA) is a congenital cyanotic cardiac malformation characterized by atrioventricular concordance and ventriculoarterial discordance, resulting in two parallel circuits with systemic oxygen-free blood on one side and oxygenated blood on the other (1). In order to be compatible with life, either the presence of ductus arteriosus or communication between atria or ventricles is required (1). In un-operated patients, even in the presence of atrial septal defect (ASD), the mean age of death was found to be 9 month (2). Here, we presented a case of un-operated d-TGA with a large secundum type ASD accompanied by dextrocardia, situs inversus with abnormal origin of the coronary artery, pulmonary artery (PA) aneurysm and pulmonary hypertension (PH) who survived to late adulthood, 46 year-old. To the best of our knowledge, it is the first un-operated case that survived to late adulthood in this complex anatomy.
A 46-year-old male was admitted to our outpatient clinic with complaints of shortness of breath and palpitations. He had a regular pulse of 125/min, arterial blood pressure of 128/86 mmHg and respiratory rate of 19 breaths/min. His resting oxygen saturations were around 83% on room air and dropping to 78% with exercise. Finger clubbing, bluish discoloration on the nail bed and lips were observed.
On cardiovascular examination, the apex beat was on the right and there was a wide, fixed split second heart sound with pulmonic ejection click at the right upper parasternal border.
His blood work showed elevated hemoglobin and hematocrit levels of 19% and 59% g/dL respectively and brain natriuretic peptide level of 379 ng/L.
Chest X-ray revealed presence of cardiomegaly, dextrocardia and pointed that base-apex, aortic arch and gastric air bubble was on the right (Figure-1).
Echocardiography showed mirror image dextrocardia, atrial and ventricular situs inversus, 29 mm ostium secundum ASD with bidirectional shunt. Tricuspid annular plane systolic excursion was normal. Moderate mitral and pulmonary valve insufficiency was observed. ASD with bidirectional shunt was confirmed by transesophageal echocardiography too (Figure-2).
CT scan demonstrated an aneurysm of the PA with a diameter of 60 mm (Figure-3).
In cardiac magnetic resonance imaging (CMR), aorta was arising anterior and leftward to the PA and originated from base of the right-sided hypertrophic ventricle with increased trabeculation. No contractile deficit was seen in systemic ventricle. There was aneurysmatic PA originated from the left-sided ventricle (Figure-4).
In right heart catheterization, aortic and mixed venous oxygen saturation was 85.9% and 68.2% respectively. RV end-diastolic pressure was 7 mmHg. Left heart catheterization was performed by reaching the PA through the ASD. PA oxygen saturation was 93.5%. Mean PA and LV end-diastolic pressure was 27 mmHg and 5 mmHg respectively. Pulmonary vascular resistance (PVR) was not calculated because evaluation of PVR with hemodynamic and saturation data obtained by cardiac catheterization may not be reliable in d-TGA-ASD physiology. On coronary angiography, coronary artery ostia positions were mirror image of the normal orientation and coronary arteries were free from obstructions but anomalously left circumflex artery originated from the right sinus Valsalva (Figure-5).