Treatment of the Budd-Chiari Syndrome (BCS) Caused by Membranous Obstruction of Inferior Vena Cava with Percutaneous Transluminal Angioplasty: Report of an Amazing Relation between Undiagnosed BCS and Infertility
From the Cardiovascular Research Center, Avicenna (Bu-Ali) Research Institute, Mashhad University of Medical Science, Mashhad, Iran, and the Interventional Cardiology Ward, Imam Reza Hospital, Mashhad University of Medical Science, Mashhad, Iran. The author reports no conflicts of interest regarding the content herein. Manuscript submitted February 7, 2009, provisional acceptance given April 15, 2009, final version accepted April 17, 2009. Address for correspondence: Mahmoud Ebrahimi, MD, Interventional Cardiology Ward, Imam Reza Hospital, Mashhad University of Medical Science, Mashhad, Iran. E-mail: Ebrahimi_cardiology@yahoo.com
J INVASIVE CARDIOL 2009;21:E166-E167 Budd-Chiari syndrome (BCS) is an infrequent, usually life-threatening disorder.1,2 It is characterized by hepatic venous outflow obstruction at the level of hepatic venules, large hepatic veins, inferior vena cava (IVC), or right atrium leading to congestive liver syndromes (hepatomegaly, ascites, abdominal pain and eventual liver failure).3 Early decompression is very essential to prevent liver dysfunction and death.4 The primary therapeutic approach includes medical management and relief of hepatic venous outflow obstruction. This might be achieved by surgery, percutaneous interventional techniques and even liver transplantation in selected patients, especially those with fulminant hepatic failure.3–6 We hereby report the early and late results of percutaneous balloon angioplasty in an infertile patient with BCS caused by a membranous web in the IVC. Case Description. A 23-year-old female was referred to our center by a hepatologist with abdominal pain, ascites, hepatomegaly and dilated abdominal veins. She had been followed up by numerous diagnostic examinations and therapies for infertility during the past 4 years. The final diagnosis was unexplained infertility. Abdominal sonographic examinations reported an inferior vena caval web, so she was referred for percutaneous transluminal angioplasty. Interventional technique. After establishing venous access from the right femoral and internal jugular veins, simultaneous bidirectional venacavography was performed, which revealed a membranous web-like lesion in the superior portion of the IVC (Figure 1). Obtaining an adequate control view, we crossed the web with the guidewire, and successful dilatation was performed using balloons of progressively increasing size (Figures 2 A and B), with no stent deployment. The patient received anticoagulants after the procedure. Immediately subsequent to the successful balloon angioplasty, the pressure gradient between the IVC and the right atrium declined from 15 mmHg to zero, and the profuse superficial and deep collateral vein tortuosity disappeared (Figures 3 A and B). The patient’s symptoms were relieved and her ascites disappeared. After 3 months, the patient became pregnant and she subsequently delivered of a healthy baby without any problems. All clinical and laboratory findings are normal at present and were normal throughout the 4-year follow up. Discussion. Idiopathic membranous stenosis and strictures of the IVC are the most frequent causes of BCS according to several reports.9,10 Balloon dilatation is the treatment of choice for membranous BCS. For patients with thick and sizable lesions, surgical membrane resection should be considered.11 Patients with normal coagulation status and membranous IVC obstruction respond successfully to treatment without signs of portal vein thrombosis.9 Female infertility is a noteworthy medical problem that in many cases is of unidentified etiology.7,8 Since the liver is the principal organ for estrogen removal and hormone regulation, its health is essential for fertility.12 There are several reports of successful pregnancy13–15 and twin pregnancies16–19 after liver transplantation, which indicates that the return of normal hepatic function plays an important role in pregnancy; optimal recovery of the menstrual cycle due to reinstated hepatic estrogen metabolism may be the explanation.20 In the case of our patient, perhaps due to probable compensation of the liver tissue the diagnosis of BCS was neglected until the development of ascites and presentation of superficial abdominal collateral over the 4-year follow-up period before the procedure (liver profile tests were in the upper normal limit at that time). This case study shows that percutaneous intervention for membranous obstruction of the IVC is a feasible and effective method of treatment that provides excellent long-term restoration of physiological hepatic circulation and function. With consideration of the important role of hepatic function in pregnancy, BCS — even in its asymptomatic stages — should be considered and sought as a rare, but potentially reversible, cause of infertility.
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