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).