INTRODUCTION
Intrahepatic interruption of
the inferior vena cava (IVC) with azygos vein continuation is a rare
congenital aberration, occurring in 0.6% of patients with other cardiac
defects as situs abnormalities and dextrocardia.1 In
most instances, patients are asymptomatic. They might be noticed as
incidental findings following imaging investigations. We present a case
of situs inversus totalis and dextrocardia associated with interrupted
inferior vena cava (IVC) and azygos vein continuation in a patient with
concomitant long-persistent atrial fibrillation. The treatment of atrial
fibrillation (AF) has been considered at high risk for percutaneous
interventions and thus referred to our institute for surgical bilateral
thoracoscopic epicardial ablation and left atrial appendage (LAA)
exclusion.
The patient is a 45-years
old male with symptomatic long-standing persistent atrial fibrillation
(LsP-AF) who received multiple electrical cardioversions for AF
recurrences since 2003. Class I antiarrhythmic drugs failed to restore
sinus rhythm thus, according to the current ESC 2020 Guidelines for AF
treatment 2, the patient was scheduled for
transcatheter pulmonary veins (PV) isolation.3Pre-operative chest X-RAY showed the presence of a complete situs
inversus dextrocardia (SID). (Figure 1A)
Then, a thoraco-abdominal CT scan was performed to rule out other
anatomical abnormalities. A concomitant intrahepatic interruption of the
IVC was described with the renal veins draining into the azygos vein
which was directly collecting blood from the lower body and draining
posteriorly into the superior vena cava (SVC). In adjunct, the hepatic
veins were draining directly into the right atrium (RA). Nonetheless,
regular anatomy of the left atrium (LA) with two right and two left PVs
was depicted (Figure 1B, 1C).
The patient was deemed not suitable for percutaneous ablation (PA)
because of the complex anatomy and was then referred to our Institution
for surgical thoracoscopic ablation. Transesophageal echocardiogram was
performed to rule out thrombus in the LAA prior to
surgery.