Corresponding author:
Kento Shionoya
Gastroenterology Medicine Center, Shonan Kamakura General Hospital,
Okamoto 1370-1, Kamakura-shi, Kanagawa 247-8533, Japan, TEL:
+81-467-46-1717, FAX: +81-467-45-0190
E-mail: kentosh0812@gmail.com
Abstract
Hepatic arteriovenous malformation mostly occurs as a manifestation of
hereditary hemorrhagic telangiectasia. Clinical symptoms include
hyperpulsatile heart failure, hepatic encephalopathy, and ascites. We
present a case of hepatic arteriovenous malformation and atrial septal
defect causing heart failure.
Keywords
arteriovenous malformation; atrial septal defect; heart failure
1 CASE
A 77-year-old woman visited our hospital because of bilateral leg edema
and mild dyspnea. Blood test results showed normal liver function and
albumin levels, but high levels of ammonia. An echocardiogram revealed
atrial septal defect (ASD) and a right-left shunt associated with ASD
caused pulmonary artery dilation (Figure 1a and 1b). Cardiac
catheterization revealed pulmonary artery pressure was within the normal
range. Abdominal ultrasonography revealed that the intrahepatic portal
vein was prominent and increased blood flow, but no findings were
suggestive of liver cirrhosis (Figure 2). A contrast-enhanced CT showed
multiple hepatic arteriovenous malformations (AVM) (Figure 3). It was
thought that AVM and ASD caused capacitive loading of right heart system
and heart failure symptoms had occurred. The cause of high ammonia level
was thought to be hepatic AVM. Her leg edema and dyspnea improved, and
ammonia levels decreased with oral medications.
Hepatic AVM is a rare disease, mostly occurring as a manifestation of
hereditary hemorrhagic telangiectasia. Clinical symptoms include
hyperpulsatile heart failure, hepatic encephalopathy, and
ascites1,2. Hepatic arterial embolization has been
reported to be effective and to improve heart failure
symptoms3. Hepatic angioembolization or heart surgery
may be necessary in this case if heart failure symptoms worsen.
AUTHOR CONTRIBUTION
Kento Shionoya wrote and revised the manuscript, and involved in the
literature search. Makoto Kako supervised the case study. All authors
read and approved the final manuscript.
ACKNOWLEDGMENT
The authors wish to thank Ms. Fujii M for her excellent technical and
secretarial assistance.
FUNDING INFORMATION
None
CONFLICT OF INTEREST
The authors have no conflicts of interest to declare.
CONSENT
Written informed consent was obtained to publish this report in
accordance with the journal’s consent policy.
ETHICAL STATEMENT
Not applicable.