Korean J Med.  2009 Aug;77(Suppl 1):S68-S72.

Congestive heart failure due to an iatrogenic arteriovenous fistula in a patient with neurofibromatosis-1

• Choi ER
• Lee SJ
• Kim SM
• Choi HJ
• Ki CS
• Choi YH
• Kim DK

Abstract

A 39-year-old woman, who 3 months prior had undergone sono-guided biopsy for pelvic masses, was admitted to our hospital with dyspnea. After the procedure, a large arteriovenous (AV) fistula was detected. The patient developed dyspnea with pain and swelling of the right leg and subsequently visited our hospital. Multiple cafe-au-lait spots and subcutaneous nodules were noted and a continuous bruit was heard in the right pelvic area. Transthoracic echocardiography revealed marked dilatation of the right atrium, right ventricle, and inferior vena cava. An angiogram showed multiple AV fistulas between the right iliac arteries and veins. The patient underwent aortoiliofemoral bypass surgery. Follow-up echocardiography after surgery showed dramatic improvement in heart function. Molecular analysis revealed a novel variation (c.4270-1G>a) at the 24th intron-exon splicing consensus sequence of the neurofibromin 1 (NF1) gene. This is a rare case of AV fistula that developed after biopsy and caused right-sided heart failure in a patient with neurofibromatosis.

Keyword

Arteriovenous fistula; Congestive heart failure; Neurofibromatosis type 1

MeSH Terms

Adult
Arteriovenous Fistula
Biopsy
Cafe-au-Lait Spots
Consensus Sequence
Dilatation
Dyspnea
Echocardiography
Estrogens, Conjugated (USP)
Female
Fistula
Follow-Up Studies
Heart
Heart Atria
Heart Failure
Heart Ventricles
Humans
Iliac Artery
Leg
Neurofibromatoses
Neurofibromatosis 1
Neurofibromin 1
Veins
Vena Cava, Inferior
Cafe-au-Lait Spots
Estrogens, Conjugated (USP)
Neurofibromin 1