Hepatopulmonary syndrome associated with Budd-Chiari syndrome/ Budd-Chiari sendromu ile birliktelik gosteren hepatopulmoner sendrom.
Budd-Chiari Syndrome (BCS) is a rare cause of portal hypertension. Occlusion of inferior vena cava (IVC) or hepatic vein (HV) causes BCS and leads to centrilobular congestion and necrosis of liver. We present a cyanotic patient with BCS associating with hepatopulmonary syndrome (HPS).
A 15-year-old girl admitted with dyspnea and cyanosis. She was referred to our clinic with the prediagnosis of congenital heart disease. On examination, there was cyanosis of the skin and mucosa; clubbing of the fingers and toes (Fig.1) and a grade 2/6 systolic murmur. Electrocardiography showed right axis deviation. Chest X-ray and thoracal computed tomography findings are presented in Figure 2. The pulmonary veins in the lower parts of the lungs were prominent and enlarged.
Her laboratory results revealed; hemoglobin, 17.9 g/dl; platelets, 120000/[mm.sup.3], white blood cell count, 5000/[mm.sup.3]. Blood glucose level was 73 mg/dl, alanine aminotransferase, 29 U/L; aspartate aminotransferase, 38 U/L; gamma-glutamyl transferase, 52 U/L; alkaline phosphatase, 490 U/L; total protein, 7.6 mg/dl; albumin, 3.9 mg/dl; total bilirubin, 3.32 mg/dl; direct bilirubin, 1.02 mg/dl. Arterial blood gas analysis revealed; pH, 7.403; PC02, 30.9 mmHg; P02, 31.1 mmHg; S02, 54.9%; HC03, 18.9 mmol/L; MetHb, 0.9 %. Hepatitis A, B and C, cytomegalovirus and toxoplasma antibodies were negative. Paroxysmal nocturnal hemoglobinuria panel, ol-antitrypsin, blood copper, ceruloplasmin, sweat test, protein C and S, antithrombin III were in normal limits. Factor V Leiden mutation, prothrombin 20210A mutation were negative.
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After echocardiography revealed normal findings, contrast-enhanced echocardiography was performed. Contrast medium, seen as micro bubbles, after appeared in right chambers, appeared in left heart in a period of 4-6 heart beats.
Oxygen saturation by digital pulse oximetry was 72-74%. With [0.sub.2] (2lt/min, nasal cannula), it was 78%. It was 65-69 % in upright position and 50-55 % with exertion.
Pulmonary angiography showed dilated capillaries (Fig. 3). On abdominal ultrasound and CT (Fig. 4a), multiple large nodules in liver and splenomegaly were seen (vertical length: 150 mm). Esophagogastroduodenoscopy revealed esophageal varicose veins. Doppler ultrasound showed normal course of IVC. Liver biopsy revealed prominent dilatation of sinusoids around central veins (Fig. 5) which was compatible with venous outflow obstruction. Vena cavography and hepatic venography showed that there was not a web in IVC and the course of right HV and its branches were abnormal (Fig. 4b). Partial liver transplantation was planned for patient.
For patients with cyanosis, intracardiac and intrapulmonary shunts should be considered. Contrast-enhanced echocardiography is useful for discrimination (1).
Differential diagnosis of intrapulmonary shunts includes two conditions: pulmonary arteriovenous malformations (PAVM) and HPS (2). PAVM are characterized by abnormal communications between the pulmonary arteries and veins (3). Approximately two thirds of PAVM occur in hereditary hemorrhagic teleangiectasia (2).
The triad of 'HPS' are liver disease, hypoxemia with a Pa02<70 mmHg while breathing room air and evidence of intrapulmonary vascular dilatations (4). Patients with HPS can present with either hepatic (80%) or pulmonary symptoms (20%) (5). Most common pulmonary symptom is dyspnea, which may accompany platypnea, and/or orthodeoxia. Platypnea and orthodeoxia are not pathognomonic for HPS. But, association with liver dysfunction strongly suggests HPS.
Hepatopulmonary syndrome (HPS) is associated with many types of liver diseases. Association with BCS is rare (6). Budd-Chiari Syndrome (BCS) which is due to hepatic outflow obstruction, occur in a variety of conditions, particularly prothrombotic states. Occlusion of a single HV is usually silent (7). Overt BCS generally requires the occlusion of at least two HV. Enlargement of the caudate lobe is common because blood is shunted through it directly into IVC. Large nodules in liver have been described in literature as benign regenerative nodules associated with BCS (8). In our patient, symptoms of HPS were dominant. BCS showed a severe and chronic course because of insidious progress of HPS. Caudate lobe hypertrophy, large nodules in the liver, hepatic venography findings and liver biopsy were compatible with BCS.
In series of Gentil-Kocher et al. (9), all children with BCS had hepatomegaly and 3 children had acute refractory ascites. Liver function tests were normal in most of them. Dilawari et al. (10) suggested that children usually do not have acute BCS, and chronic BCS in children and adolescents is similarto BCS in adults.
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This is a rare case of BCS that showed clinical features of HPS before clinical findings of liver dysfunction. Because of her deep cyanosis due to HPS, differential diagnosis with cyanotic congenital heart diseases, Eisenmenger syndrome and pulmonary hypertension was required.
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(8.) Wanless IR. Regenerative nodules in Budd-Chiari syndrome. Hepatology 1994; 19: 1391.
(9.) Gentil-Kocher S, Bernard 0, Brunelle E Hadchouel M, Maillard JN, Valayer J, et al. Budd-Chiari syndrome in children: report of 22 cases. J Pediatr 1988; 113: 30-8.
(10.) Dilawari JB, Bambery R Chawla Y, Kaur U, Bhusnurmath SR, Malhotra HS, et al. Hepatic outflow obstruction (Budd- Chiari Syndrome). Experience with 177 patients and a review of the literature. Medicine 1994; 73: 21-36.
Address for Correspondence/Yazisma Adresi: Dr. Deniz N. Cagdas, Department of Pediatric Cardiology, Yiiksek ihtisas Education and Research Hospital Ankara, Turkey Phone: +90 312 306 17 24 Fax: +90 312 312 41 20 E-mail: firstname.lastname@example.org
F. Aysenur Pac, Deniz N. Cagdas, Meral Akdogan *, Neslihan Inci Zengin **, Nurgul Sasmaz *
From Departments of Pediatric Cardiology, and Internal Medicine * Section of Gastroenterology ** Section of Pathology; Yuksek Ihtisas Education and Research Hospital, Ankara, Turkey
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|Title Annotation:||Case Reports/Olgu Sunumlari|
|Author:||Pac, F. Aysenur; Cagdas, Deniz N.; Akdogan, Meral; Zengin, Neslihan Inci; Sasmaz, Nurgul|
|Publication:||The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)|
|Date:||Jun 1, 2010|
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