1. Introduction. Central European Journal of Medicine. Sadık Kadri Açikgöz 1, Gülten Taçoy 1*, Baran Önal 2, Beytullah Yıldırım 3, Atiye Çengel 1

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1 Cent. Eur. J. Med. 4(3) DOI: /s Central European Journal of Medicine Arterial embolisation and coiling for high-output heart failure and pulmonary hypertension ınduced by hepatic arteriovenous fistula in a patient with hereditary hemorrhagic telengiectasia Sadık Kadri Açikgöz 1, Gülten Taçoy 1*, Baran Önal 2, Beytullah Yıldırım 3, Atiye Çengel 1 1 Gazi University Faculty of Medicine, Cardiology Department, 2 Gazi University Faculty of Medicine, Radiology Department, 3 Gazi University Faculty of Medicine, Gastroenterology Department, Case Report Received 9 February 2009; Accepted 13 February 2009 Abstract: Hereditary hemorrhagic telangiectasia (HHT) is a genetic vascular disorder characterised by epistaxis, telangiectases, and visceral arteriovenous malformations. Hyperdynamic blood flow associated with arteriovenous malformations may lead to pulmonary hypertension, global heart failure, and valvular insufficiencies. We report a patient who had HHT with severe heart failure (New York Heart Association [NYHA] class III-IV) and pulmonary hypertension caused by an hepatic arteriovenous fistula. After successful transarterial embolisation of the right branch of the hepatic artery with polyvinyl alcohol (PVA) particles and coils, 4 to 7 mm in size, the patient was discharged with functional class II (NYHA) heart failure. Keywords: Hereditary hemorrhagic telengiectasia Hepatic arterivenous fistula Arterial embolisation Coiling Versita Warsaw and Springer-Verlag Berlin Heidelberg. 1. Introduction Hereditary hemorrhagic telangiectasia (HHT), or Osler- Weber-Rendu disease, is an autosomal-dominant genetic disorder characterized by epistaxis, widespread mucocutaneous and visceral telangiectasia, and arteriovenous malformations in multiple organ systems. The prevalence of HHT is 1 to 2 cases per Mutations that cause HHT can occur in the endoglin gene on chromosome 9, giving rise to HHT1, or in the activin receptor-like kinase 1 gene on chromosome 12, causing HHT2 [1]. The clinical presentation of the disease varies greatly depending on the features and location of vascular malformations and telangiectasia. Arteriovenous malformations frequently occur in the lungs, brain, kidney, and gastrointestinal tract. Hepatic involvement is relatively rare and encountered in about 8% to 31% of the patients with HHT [2], and specific criteria are required for the diagnosis of HHT [3]. In this case, the patient had high-output heart failure and pulmonary arterial hypertension caused by an hepatic arteriovenous malformation. The patient s cardiac status improved greatly after hepatic arterial coil embolisation, and beneficial effects persisted 18 months into the follow-up. * gtacoy@gmail.com 369

2 Arterial embolisation and coiling for high-output heart failure and pulmonary hypertension ınduced by hepatic arteriovenous fistula in a patient with hereditary hemorrhagic telengiectasia Figure 1. Electrocardiogram. Figure 2. Electrocardiogram. 2. Case Report A 67-year-old man was admitted with exertional dyspnea, palpitation, and pretibial edema that he had had for 2 years. His medical history was remarkable for HHT, which had been diagnosed 30 years previously on the basis of frequent epistaxis and gastrointestinal bleeding. He had undergone sclerotherapy for recurrent gastrointestinal bleeding 3 years before admission. Because of exertional dyspnea (NYHA Class III-IV) and pretibial edema that had worsened over a period of 2 months, he received a diagnosis of heart failure, and furosemide, digoxin, and perindopril were prescribed. One month before admission, a gastroscopy and colonoscopy revealed no sign of active gastrointestinal bleeding. His sister had had HHT. On physical examination, his blood pressure was 110/60 mm Hg, pulse 110 beats per minute, respiratory rate 16 breaths per minute, and temperature 36.5 C. Multiple telangiectatic lesions were noticed in the mouth and oropharynx. On cardiac auscultation, pansystolic murmurs, grade 2 out of 6, were audible at the apex and left lower sternal border. A systolic bruit was heard in the right upper abdominal quadrant, and crepitant rales were audible in the bilateral basal lung fields. Abdominal palpation revealed hepatomegaly, extending 5 cm below the right costal margin. Mild pretibial edema was also found. The blood chemical values showed a microcytic anemia (hemoglobin, 10,4 g/dl; mean corpuscular volume, 72,8 fl), mildly elevated level of creatinine (1,5 mg/dl), increased levels of alkaline phosphatase (ALP) (465 U/L) and gamma-glutamyl-transferase (GGT) (414 U/L), and hyperbilirubinemia (total bilirubin, 3,38 mg/dl; direct bilirubin, 1,53 mg/dl) with normal transaminases. The electrocardiogram on admission revealed atrial tachycardia with 2:1 conduction at a ventricular rate of 110 per minute, which spontaneously transformed into 370

3 S.K. Açikgöz et al. Figure 3. Radiograph of the chest. Figure 4. Hepatic angiogram. Figure 6. Hepatic angiogram. Figure 5. Hepatic angiogram. sinus tachycardia (Figure 1,2). Cardiomegaly and mild pulmonary congestion was found on a radiograph of the chest (Figure 3). Transthoracic echocardiography revealed a normal ejection fraction, mild left ventricular hypertrophy, biatrial and right ventricular dilatation (left atrium, 50 mm; right atrium, 67 mm; right ventricle, 48 mm), grade 1 mitral regurgitation and grade 3 tricuspid regurgitation, with a maximum systolic pulmonary artery pressure of 60 mm Hg. Hepatic arteriography was performed and revealed an enlarged hepatic artery and extensive hepatic arteriovenous malformations (Figure 4,5). No additional lesions were seen on the pulmonary angiogram. The arteriovenous malformations were assumed to be the cause of the high-output cardiac failure, and hepatic arterial embolisation was chosen as the therapeutic approach. Percutaneous transarterial embolisation of the right branch of the hepatic artery was successfully performed with polyvinyl alcohol (PVA) particles, 550 to 710 microns in size, and coils, 4 to 7 mm in size (Figure 6). The day after the procedure right abdominal pain, fluctuations in consciousness, and fever up to 38 C developed. A blood leukocyte count of 15,400 per milliliter was noted. The GGT, ALP, and bilirubin levels increased gradually, but the blood ammonia levels were normal. Samples for cultures were obtained, and empirical antibiotic treatment was started. An abdominal ultrasound study, electroencephalography, and magnetic resonance imaging of the brain revealed no signs of 371

4 Arterial embolisation and coiling for high-output heart failure and pulmonary hypertension ınduced by hepatic arteriovenous fistula in a patient with hereditary hemorrhagic telengiectasia disease. Two days later the patient s status improved, and on the fourth day his fever resolved; no bacterial growth was seen in the cultures. GGT and ALP levels decreased to the baseline values in 5 days, and the bilirubin level in 10 days. On the 15 th day, the results of blood tests were similar to the values on admission; there were no signs of heart failure on physical examination, and the patient s functional capacity was improved to NYHA class II. On echocardiographic evaluation, the maximum pulmonary artery pressure decreased to 40 mm Hg. The patient was discharged on the 16th day. After 6 months, his condition remained NYHA class II without additional symptoms. 3. Discussion Arteriovenous (AV) malformations in patients with HHT may lead to heart failure and pulmonary hypertension. Although hepatic involvement in HHT is as high as 73%, symptoms occur very rarely. The approaches to treatment of these patients are percutaneous embolisation or coiling, banding of the hepatic artery, or liver transplantation. Embolisation with metallic coil, gelatin sponge, and polyvinyl alcohol has been performed in cases of HHT [5-9]. In our case percutaneous transarterial embolisation of the right branch of the hepatic artery was successfully performed with PVA particles, 550 to 710 microns in size, and coils, 4 to 7 mm in size. Embolisation or ligation are effective therapeutic methods in patients who have communications between the hepatic artery and vein and may cause acute liver failure. Before embolisation procedures are instituted, the portal venous system should be evaluated to decrease postprocedural complications. Our patient had a hepatic arteriovenous malformation, and therefore, embolisation had been performed. After that procedure, our patient had right abdominal pain, fluctuations in consciousness, fever, leukocytosis, and increased levels of GGT, ALP, and bilirubin. An abdominal ultrasonographic evaluation was normal, and indicated that the liver damage was limited. Heart failure and pulmonary hypertension (PHT) are important factors in predicting the survival of patients with HHT. Therefore, the treatment approach must be chosen according to the patient s status. Long-term follow-up is required in patients with transcatheter embolisation and coiling due to frequent recanalization of arteriovenous malformations. High-output heart failure due to left-toright intrahepatic shunting has increased the morbidity rate, and the shunt output determines the symptomatic status [10,11]. In some patients with HHT, pulmonary arterial hypertension (PAH) may occur as a result of increased cardiac output due to systemic arteriovenous malformations in the liver or as a typical form of idiopathic pulmonary arterial hypertension. Although vasodilator treatment in HHT has not been recommended because of a potential increase in arteriovenous shunt volume, Bonderman et al. showed useful effects of treatment with bosentan [12]. Rigelsky et al. suggested that patients with HHT and PAH should be evaluated by analyzing the BMPR2 gene [13]. Garcia-Tsao et al. reported that the incidence of hepatic lesions in HHT in 8-13% and should be evaluated carefully [5,14]. HHT is an uncommon vascular disease with hepatic involvement and significantly increases morbidity. Transcatheter arterial embolization is a treatment approach that may effectively treat heart failure and pulmonary hypertension induced with AVM. References [1] Haitjema T, Westermann CJ, Overtoom TT, et al. Hereditary hemorrhagic telengiectasia (Osler-Weber- Rendu disease). New insights in pathogenesis, complications, and treatment. Arch Intern Med 156: , 1996 [2] Garcia-Tsao G, Korzenik JR, Young L, Henderson KJ, Jain D, Byrd B, PollakJS, White RI (2000) Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 343: [3] X. Shovlin CL, Guttmacher AE, Buscarini E, Faughan ME, Hyland RH, Westermann CJ, et al: Diagnostic cirteria for hereditary hemorrhagic telengectasia (Rendu-Osler-Weber syndrome). Am J Med Genet 2000;91:66-67 [4] Bourgeois N, Delcour C, Deviere J, Francois A, Lambert M, Crener M. Osler-Weber-Rendu disease associated with hepatic involvement and high-output heart failure. J Clin Gastroenterol 1990;12:236-8 [5] Chavan A, Galanski M, Wagner S, et al. Hereditary hemorrhagic telangiectasia:effective protocol for embolization of hepatic vascular malformationsexperience in five patients. Radiology 1998;209:735-9 [6] Roman CF, Cha SD, Incarvito J, Cope C, Maranhao V. Transcatheter embolization of hepatic arteriovenous fistula in Osler-Weber-Rendu disease : a case report. Angiology 1987;38:484-8 [7] Göthlin JH, Nordgard K, Johnson K, Nyman U. Hepatic telangiectasia in Osler s disease treated with arterial embolization. Report of 2 cases. Eur J 372

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