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We report a case of a man in his 50s, who presented with dry cough and breathlessness since 2–3 years which was gradually progressive, increased since past 1 month to grade IV, that is breathlessness even on sitting, and abdominal distension since 15 days. On examination, he had clubbing, bilateral pedal oedema with a bulging precordium. Abdominal examination revealed congestive hepatomegaly. A pansystolic murmur was heard on auscultation. Oxygen saturation in room air was 94%. There was history of one episode of haemoptysis. There was no history of diabetes or hypertension. The patient was a chronic alcoholic and tobacco chewer for 30 years.
An electrocardiograph showed atrial flutter with 2:1 conduction block, right bundle branch block and right axis deviation. Transthoracic echocardiography showed dilated right atrium and right ventricle, severe mitral and tricuspid regurgitation and severe pulmonary hypertension. Blood investigations were unremarkable except for low T3 levels. There was an inversion of the liver and abdominal structures to the contralateral side seen on abdominal ultrasound examination.
CT scan was further done to look for the cause of dilated right heart (video 1). Complete situs inversus with levocardia was noted, that is liver was on the left, spleen and stomach were on the right, bilobed right lung and trilobed left lung. Moderate cardiomegaly with mild pericardial effusion was noted. The morphological right atrium was on the left side, that is the atrium receiving the superior and inferior vena cava (SVC and IVC) was seen on the left side; and the morphological left atrium on the right side, that is the atrium receiving the four pulmonary veins was seen on the right side. The morphological right ventricle with coarse trabeculations, moderator band and muscular infundibulum was noted on the right side. The morphological left ventricle with a smooth septal wall and relatively fine trabeculae was on the left side (figure 1). The systemic venous blood entered the morphologically right atrium and a mitral valve in the morphologically left ventricle from which arose the pulmonary trunk. The pulmonary venous blood passed the left atrium and the tricuspid valve into a morphologically right but systemic ventricle from which arose the aorta. Venoatrial concordance was maintained, that is all four pulmonary veins were draining into the morphological left atrium and SVC and IVC are draining into morphological right atrium (figure 2). Thus, the morphological right atrium draining into the morphological left ventricle and the morphological left atrium draining into the morphological right ventricle was seen, suggestive of atrioventricular discordance. Aorta was seen arising from the morphological right ventricle and the main pulmonary artery was seen arising from the morphological left ventricle suggestive of ventriculoarterial discordance (figure 3). The aorta was seen anterior and to the right of the pulmonary artery. Right-sided aortic arch was noted, with mirroring of its branches, that is left brachiocephalic trunk, right common carotid artery and right subclavian artery (figure 4). The main pulmonary artery, its right and left branches were severely dilated, suggesting pulmonary hypertension. Major aortopulmonary collateral arteries (MAPCAs) were noted arising from the arch of aorta and descending aorta and supplying both the lungs. Early opacification of the inferior vena cava and hepatic veins in the arterial phase was noted suggesting tricuspid regurgitation. On the basis of the CT scan, a diagnosis of complete situs inversus with levocardia with congenitally corrected transposition of the great arteries (CCTGA) was made.1–3
Axial CT (A and B) illustrating a morphological right and systemic ventricle with coarse trabeculations, moderator band and muscular infundibulum are noted on the right side. Morphological left ventricle with smooth septal wall and relatively fine trabeculae is on the left side (black arrows). The morphological right atrium is draining into the morphological left ventricle and morphological left atrium draining into the morphological right ventricle suggestive of atrioventricular discordance. Oblique sagittal and coronal CT images (C and D) Illustrating the outflow of the aortic arch is shown in these images, coming from the morphological systemic right ventricle (white arrows).
Coronal CT image (A) shows the levocardia with situs inversus. The liver can be seen beneath the diaphragm on the patient’s left (black star). Axial CT image (B) shows pulmonary veins draining into morphological left atrium (black arrow). Coronal CT image (C) shows superior and inferior vena cava (arrows) draining into the morphological right atrium.
Oblique coronal (A), and oblique sagittal images (B and C) show the main pulmonary artery (MPA) arising from the morphological left ventricle (mLV) and aorta (AO) arising from morphological right ventricle (mRV).
Axial CT (A) illustrating a the right sided aortic arch and the left sided superior vena cava. Also seen are dilated pulmonary arteries. Axial CT image (B) shows the aorta is seen anterior and slightly to the right of the pulmonary artery.
The patient is being managed medically for the same. Catheter ablation has also been planned.
Patient’s perspective
I am happy to have undergone a CT scan which enabled my doctors to reach at a diagnosis and helped them to manage me better. I also now have a better understanding of the nature of my illness and the cause of repeated bouts of breathlessness.
Learning points
Situs inversus with levocardia is an extremely rare condition that is characterised by mirror-image position of the viscera with respect to situs solitus along with a cardiac apex on the left. It is seen in association with congenital heart disease in a large majority of patients.
Congenitally corrected transposition of the great arteries (CCTGA) may be seen with situs solitus or situs inversus. Situs solitus is seen in the majority of these patients, and only around 34% of cases have situs inversus.
Additional cardiac anomalies are commonly associated with CCTGA and seen in nearly 90% of all cases. If there is no coexistent intracardiac abnormalities coexist with CCTGA, these patients may go undiagnosed until adulthood.
Ethics statements
Patient consent for publication
Footnotes
Contributors AS and RDA have prepared the manuscript. ARJ and HJ have helped in editing it.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.