A 37-year-old woman with paroxysmal atrial fibrillation, presented to the emergency
department with palpitations. Previously, a pulmonary venous isolation (PVI) was attempted
to control similar symptomatic episodes owing to atrial fibrillation. However, PVI
was unsuccessful owing to abnormal inferior vena cava (IVC) anatomy. Magnetic resonance
venography demonstrated an interrupted IVC with azygous continuation into the abdomen
and pelvis. The hepatic veins drained directly into the right atrium (Figure A). The azygos veins drained the right renal vein and iliac veins, whereas the hemiazygos
drained the left renal veins and then drained into the azygos vein. Other than the
venous anomalies, the patient also had polysplenia (Figure B), a midline liver and intestinal malrotation leading to the diagnosis of heterotaxy
syndrome (HTx) with left atrial isomerism (LAI). A transthoracic echocardiogram showed
no associated structural heart disease and another PVI was planned. With the help
of interventional radiology, transhepatic access to the right atrium was obtained.
Transesophageal echo was used to guide the transseptal access to the left atrium and
PVI was performed successfully with good result and complete isolation of all pulmonary
veins.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to The American Journal of the Medical SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Disorders of left-right asymmetry: heterotaxy and situs inversus.Am J Med Genet C Semin Med Genet. 2009; 151: 307-317https://doi.org/10.1002/ajmg.c.30228
- Outcomes of left atrial isomerism over a 28-year period at a single institution.J Am Coll Cardiol. 2000; 36: 908-916https://doi.org/10.1016/S0735-1097(00)00812-3
- Non-cardiac issues in patients with heterotaxy syndrome.Ann Pediatr Cardiol. 2014; 7: 187https://doi.org/10.4103/0974-2069.140834
Article info
Publication history
Published online: September 20, 2017
Accepted:
August 30,
2017
Received in revised form:
August 29,
2017
Received:
July 11,
2017
Footnotes
The authors have no financial or other conflicts of interest to disclose.
Identification
Copyright
© 2017 Southern Society for Clinical Investigation. Published by All rights reserved.
