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Utility of hepatic vein waveform and transient elastography in patients with Budd–Chiari syndrome who require angioplasty

Utility of hepatic vein waveform and transient elastography in patients with Budd–Chiari syndrome... Rationale: Budd–Chiari syndrome (BCS), which causes congestive hepatopathy and aggravates cirrhosis, is typically treated by interventional angioplasty to ameliorate blood flow. X-ray venography is useful for the evaluation of inferior vena cava (IVC) stenosis and determination of treatment timing, but it is invasive and thus unsuitable for repeated examinations. The development of a simple method for the prediction of IVC stenosis would reduce the burden on patients with BCS. Patient concerns: We report here our experience of 2 patients with BCS who underwent percutaneous transluminal angioplasty (PTA). The first patient was a 39-year-old male who underwent PTA to expand his stenotic IVC. The second patient was a 19-year-old male who underwent PTA 3 times due to restenosis of his IVC. Diagnoses: Both patients were diagnosed with BCS with severe obstruction of the IVC. Interventions: We evaluated the hepatic vein (HV) waveform by Doppler ultrasonography and measured liver stiffness (LS) using transient elastography (TE) before and after PTA. Outcomes: In case 1, the phasic oscillation of the HV waveform recovered and the LS value decreased after PTA. Both improvements were maintained for ∼3 years, reflecting the long-term patency of the IVC. In case 2, the HV waveform and the LS value improved temporarily after PTA, but then deteriorated gradually. Monitoring of the HV waveform and LS value allowed retreatment prior to total occlusion of the IVC and abrogated the risk of intravascular needle puncture. Lessons: Monitoring of the HV waveform and the LS value enables safe management of patients with BCS who may require PTA. Abbreviations: BCS = Budd–Chiari syndrome, CT = computed tomography, HV = hepatic vein, IVC = inferior vena cava, LS = liver stiffness, MRI = magnetic resonance imaging, PTA = percutaneous transluminal angioplasty, RHV = right hepatic vein, TE = transient elastography, US = ultrasonography. Keywords: Budd–Chiari syndrome, congestive hepatopathy, elastography, hepatic vein waveform 1. Introduction Editor: N/A. TN and YS contributed equally to this work. Budd–Chiari syndrome (BCS) is a rare clinical disorder caused by The patients have provided informed consent for publication of the case. obstruction of the hepatic venous outflow tract, and can result in congestive hepatopathy. The resulting long-term liver congestion The authors report no conflicts of interest. triggers hepatic fibrosis, leading to cirrhosis and hepatocellular Department of Gastroenterology, Graduate School of Medicine, The University b [1–3] of Tokyo, Bunkyo-ku, Tokyo, Japan, Department of Clinical Laboratory carcinoma. In Asia, membranous obstruction of the inferior [4] Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, vena cava (IVC) is the major cause of BCS. Percutaneous Tokyo, Japan, Department of Radiology, International University of Health and transluminal angioplasty (PTA) is frequently performed to Welfare, School of Medicine, Minato-Ku, Department of Radiology, Graduate alleviate hepatic congestion in patients with BCS and membra- School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan. [5,6] nous obstruction. However, restenosis of the IVC often Correspondence: Hayato Nakagawa, Department of Gastroenterology, [7] occurs, even after successful treatment ; in cases of severe Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. (e-mail: hanakagawa-tky@umin.ac.jp). occlusion of the IVC, retreatment by intravascular needle puncture—an invasive procedure with the potential for severe Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc. This is an open access article distributed under the Creative Commons complications—is required. Therefore, the periodic monitoring Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and of IVC patency and determination of the appropriate timing of reproduction in any medium, provided the original work is properly cited. PTA prior to the development of severe IVC obstruction are How to cite this article: Nakatsuka T, Soroida Y, Nakagawa H, Okura N, Sato J, mandatory. Catheter venography is the most direct method for Akahane M, Sato M, Yatomi Y, Abe O, Tateishi R, Koike K. Utility of hepatic vein the assessment of IVC patency, but it is unsuitable for repeated waveform and transient elastography in patients with Budd–Chiari syndrome who examinations due to its invasiveness. Doppler waveform of the require angioplasty. Medicine 2019;98:45(e17877). hepatic vein (HV) revealed by abdominal ultrasonography (US) is Received: 24 April 2019 / Received in final form: 26 September 2019 / Accepted: 9 October 2019 classified into triphasic, biphasic, and monophasic patterns and is known to reflect several conditions and hemodynamics of the http://dx.doi.org/10.1097/MD.0000000000017877 1 Nakatsuka et al. Medicine (2019) 98:45 Medicine [8] liver. Liver stiffness (LS) measured by elastography, which is a restenosis of the IVC (Fig. 2A), we examined the HV waveform useful device for evaluation of liver fibrosis, also reflects the by Doppler US and assessed LS by TE. As expected, the HV [9,10] degree of liver congestion. In this report, we present the cases waveform exhibited a flattened monophasic pattern (Fig. 2B) and of 2 patients with BCS treated by PTA, in whom the combination the LS value was very high (35.3kPa). As X-ray venography of HV waveform assessment by Doppler US and LS measurement revealed complete obstruction of the IVC, intravascular needle by transient elastography (TE) facilitated evaluation of the puncture was performed, as in the first PTA (Fig. 2C). The therapeutic effect and monitoring for postoperative restenosis. pressure gradient across the obstruction was reduced from 11 to 8 mm Hg. On the day after PTA, the HV waveform remained monophasic (Fig. 2B), but the LS value had decreased to 21.3 2. Case presentations kPa. One month after PTA, the HV waveform had changed to a 2.1. Case 1 biphasic pattern (Fig. 2B) and the LS value had decreased further to 14.3 kPa. The site of IVC stenosis remained patent (Fig. 2B). A 39-year-old male with a history of a sustained low platelet After the second PTA, we monitored the HV waveform and LS count detected during health checkups presented to our hospital. value at 3–month intervals to detect restenosis of the IVC before it US performed at an outpatient clinic had suggested the presence progressed to complete obstruction. At 10 months after the of chronic liver disease with splenomegaly and collateral second PTA, the waveform of the RHV had a monophasic formation of the portal vein. Contrast–enhanced computed pattern (Fig. 2B) and the LS value had increased to 20.5 kPa. tomography (CT) revealed severe stenosis of the IVC; based on Furthermore, the IVC had narrowed to 4.7mm and contained a this finding, the patient was diagnosed with BCS. We obtained highly echoic structure (Fig. 2B). Based on these data, we HV waveforms using pulsed-wave Doppler devices as previously [11] suspected restenosis of the IVC and performed X-ray venogra- reported. The Doppler gate was placed on the right HV 1 to 3 phy. Although IVC stenosis had recurred, complete obstruction cm distal to the inferior vena cava by an intercostal approach and had not yet developed. The stenosis was treated successfully by a flattened monophasic pattern was detected that likely reflected balloon dilation without needle puncture (Fig. 2D). One month blocked transmission of cardiac pulsation from the right atrium after the third PTA, the HV waveform exhibited a triphasic to the HV due to IVC stenosis (Fig. 1A). In addition, TE pattern and the LS value was 8.4 kPa (Fig. 2B, E). (FibroScan) yielded an LS value of 17.8kPa, suggesting congestion and/or severe fibrosis of the liver. X-ray venography showed complete obstruction of the IVC, 3. Discussion and the pressures below and above the obstruction were 17 and 8 In patients with BCS, PTA can improve hepatic congestion and mm Hg, respectively (pressure gradient, 9mm Hg; Fig. 1B). The prevent liver fibrosis and portal hypertension; however, resteno- IVC obstruction was treated by intravascular needle puncture [7] sis often occurs after the procedure. Because complete followed by balloon dilation. This treatment decreased the obstruction of the IVC increases the risk of complications of pressure gradient between below and above the obstructed site to catheter treatment, timely intervention is critical. 2 mm Hg, indicating successful removal of the IVC obstruction. The HV waveform is correlated with the severity of several On the day after PTA, the waveform of the patient’s RHV, hepatic diseases. Several groups, including ours, have reported assessed by Doppler US, had changed to a biphasic pattern [11–14] that the HV waveform is associated with liver fibrosis. (Fig. 1A). His LS value decreased rapidly to 8.7 kPa, suggesting Liver fibrosis increases parenchymal stiffness and reduces HV that the high LS value before PTA was due to congestion of the wall compliance, which results in flattening of the HV waveform liver, rather than to liver fibrosis. One year after PTA, the as liver fibrosis progresses. The HV waveform is also a useful patient’s HV waveform had further improved to a triphasic [6,15,16] indicator of BCS. The loss of phasic oscillation in the HV pattern (Fig. 1A), and his LS value was 9.4 kPa. Three years after waveform indicates that cardiac movement is not transmitted to PTA, IVC restenosis had not occurred, the patient’sHV the HV, suggesting obstruction of hepatic outflow. Recovery of waveform remained triphasic, and his LS value had decreased phasic oscillation in the HV waveform can be used to assess the slightly to 7.2 kPa. [17,18] therapeutic effect of balloon angioplasty. TE is used routinely for the noninvasive assessment of liver fibrosis. Liver 2.2. Case 2 [9,10] congestion leads to an increased LS value as measured by TE ; An 11-year-old male was diagnosed with BCS at a pediatric therefore, TE can be used to assess the effect of PTA on hepatic [19] hospital due to abnormal results of liver function tests. He had congestion in patients with BCS. been followed at that hospital without interventional therapy, but We investigated the utility of the HV waveform and the LS 2 years after the diagnosis of BCS he presented to our hospital value measured by TE for the evaluation of the therapeutic effect with severe edema in the legs and eyelids. Magnetic resonance of PTA and monitoring of the clinical course after angioplasty. In imaging (MRI) revealed severe stenosis of the IVC due to a case 1, the patency of the IVC was maintained for several years membranous structure. X-ray venography showed complete after PTA, as indicated by a triphasic HV waveform and a low LS obstruction of the IVC; thus, intravascular needle puncture value. In case 2, the LS value increased gradually after the second followed by balloon dilation was performed. After PTA, the PTA while the HV waveform remained biphasic, indicating the diameter of the IVC had increased to 15mm and the RHV flow progression of hepatic congestion due to the development of IVC had changed from retrograde to normal antegrade. The patient stenosis. The HV waveform ultimately flattened, suggesting subsequently underwent routine follow-up at the pediatric severe restenosis of the IVC. Therefore, LS may be highly sensitive hospital where he had been diagnosed with BCS. for the detection of IVC restenosis, and the HV waveform may be At the age of 19 years, the patient was admitted to our hospital useful for determination of the timing of interventional therapy. due to the exacerbation of lower–leg edema accompanied by The finding of HV waveform must be carefully interpreted, as it splenomegaly and thrombocytopenia. Because MRI suggested reflects both cardiac and hepatic physiology as well as simple 2 Nakatsuka et al. Medicine (2019) 98:45 www.md-journal.com Figure 1. HV waveform before and after PTA in case 1. (A) The waveform of the RHV before and after PTA. The waveform was monophasic before treatment. One day after PTA, the HV waveform changed to biphasic and remained so for 1 month. One year after PTA, the HV waveform had adopted a normal triphasic pattern. (B) X-ray venography revealed complete obstruction of the IVC; thus, it was expanded by 14-gauge needle puncture followed by balloon dilation. After PTA, the contrast agent flowed from the IVC to the right atrium. (R)HV=(right) hepatic vein, IVC=inferior vena cava, PTA=percutaneous transluminal angiography. heartbeat. We have previously shown that HV waveforms can be HV waveform may not improve after PTA, because the phasic [11] detected even in patients with right heart dysfunction under waveform is lost as liver fibrosis progresses. Fontan circulation, thus circulatory dynamics might not greatly In conclusion, we report 2 cases of BCS in which the HV [14] influence the shape of the HV waveform. In BCS patients who waveform and the LS value determined by TE enabled the have already had cirrhosis due to prolonged liver congestion, the identification of IVC stenosis. Monitoring of the HV waveform 3 Nakatsuka et al. Medicine (2019) 98:45 Medicine Figure 2. Changes in the HV waveform and LS value in case 2. (A) Enhanced MRI showed severe stenosis of the IVC (arrow). (B) Ultrasound images of the IVC and waveform of the RHV before and after PTA. Before the second PTA, a membranous structure was detected in the IVC (arrow) and the HV waveform was monophasic. One day after PTA, the IVC had opened slightly while the HV waveform remained monophasic. One month later, the IVC had expanded to 11mm and a biphasic waveform was detected, which was maintained for 7 months. Ten months after the second PTA, the IVC had narrowed to 4.7mm, the HV waveform exhibited a monophasic pattern, and a highly echoic structure was present in the IVC (arrow). After the third PTA, the IVC had expanded to 12.6 mm and the HV waveform was triphasic. (C) X-ray venography during the second PTA. The completely occluded IVC was expanded by 14-gauge needle puncture followed by balloon dilation. (D) X-ray venography during the third PTA; the narrowed IVC was expanded by balloon dilation. (E) Changes in the LS value and HV waveform pattern between before and after PTA. (R)HV=(right) hepatic vein, IVC=inferior vena cava, LS=liver stiffness, MRI=magnetic resonance imaging, PTA= percutaneous transluminal angiography. 4 Nakatsuka et al. Medicine (2019) 98:45 www.md-journal.com on patency and survival in 177 Chinese patients from a single center. and the LS value may be valuable for follow-up of patients with Radiology 2013;2662:657–67. BCS, although further confirmatory studies are needed. [8] Scheinfeld MH, Bilali A, Koenigsberg M. Understanding the spectral Doppler waveform of the hepatic veins in health and disease. Radio- graphics 2009;297:2081–98. Author contributions [9] Millonig G, Friedrich S, Adolf S, Fonouni H, Golriz M, Mehrabi A, et al. Liver stiffness is directly influenced by central venous pressure. J Hepatol Conceptualization: Takuma Nakatsuka, Yoko Soroida, Hayato 2010;522:206–10. Nakagawa. [10] Colli A, Pozzoni P, Berzuini A, Gerosa A, Canovi C, Molteni EE, et al. Data curation: Takuma Nakatsuka, Yoko Soroida. Decompensated chronic heart failure: increased liver stiffness measured Investigation: Takuma Nakatsuka, Yoko Soroida. by means of transient elastography. Radiology 2010;2573:872–8. [11] Soroida Y, Nakatsuka T, Sato M, Nakagawa H, Tanaka M, Yamauchi Methodology: Takuma Nakatsuka, Yoko Soroida. N, et al. A novel non-invasive method for predicting liver fibrosis by Resources: Naoki Okura, Jiro Sato, Masaaki Akahane. quantifying the hepatic vein waveform. Ultrasound Med Biol 2019; Supervision: Masaya Sato, Yutaka Yatomi, Osamu Abe, 459:2363–71. Kazuhiko Koike. [12] Bolondi L, Li Bassi S, Gaiani S, Zironi G, Benzi G, Santi V, et al. Liver Writing – original draft: Takuma Nakatsuka, Yoko Soroida. cirrhosis: changes of Doppler waveform of hepatic veins. Radiology 1991;1782:513–6. Writing – review & editing: Hayato Nakagawa, Ryosuke [13] Kawanaka H, Kinjo N, Anegawa G, Yoshida D, Migoh S, Konishi K, Tateishi, Kazuhiko Koike. et al. Abnormality of the hepatic vein waveforms in cirrhotic patients Takuma Nakatsuka orcid: 0000-0002-5727-5385. with portal hypertension and its prognostic implications. J Gastroenterol Hepatol 2008;23(7 Pt 2):e129–36. [14] Nakatsuka T, Soroida Y, Nakagawa H, Shindo T, Sato M, Soma K, et al. References Identification of liver fibrosis using the hepatic vein waveform in patients with Fontan circulation. Hepatol Res 2019;493:304–13. [1] Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J [15] Hosoki T, Kuroda C, Tokunaga K, Marukawa T, Masuike M, Kozuka Med 2004;3506:578–85. T. Hepatic venous outflow obstruction: evaluation with pulsed duplex [2] Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, sonography. Radiology 1989;170(3 Pt 1):733–7. Bahr MJ, et al. Etiology, management, and outcome of the Budd-Chiari [16] Bolondi L, Gaiani S, Li Bassi S, Zironi G, Bonino F, Brunetto M, et al. syndrome. Ann Intern Med 2009;1513:167–75. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. [3] Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla Gastroenterology 1991;100(5 Pt 1):1324–31. DC, et al. Budd-Chiari syndrome: a review by an expert panel. J Hepatol [17] Ohta M, Hashizume M, Tomikawa M, Ueno K, Tanoue K, Sugimachi K. 2003;383:364–71. Analysis of hepatic vein waveform by Doppler ultrasonography in 100 [4] Kage M, Arakawa M, Kojiro M, Okuda K. Histopathology of patients with portal hypertension. Am J Gastroenterol 1994;892: membranous obstruction of the inferior vena cava in the Budd-Chiari 170–5. syndrome. Gastroenterology 1992;1026:2081–90. [18] Hirooka K, Hirooka M, Kisaka Y, Uehara T, Hiasa Y, Michitaka K, et al. [5] Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, et al. Doppler waveform pattern changes in a patient with primary Budd- Managing Budd-Chiari syndrome: a retrospective review of percutane- Chiari syndrome before and after angioplasty. Intern Med 2008;472: ous hepatic vein angioplasty and surgical shunting. Gut 1999;444: 91–5. 568–74. [19] Mukund A, Pargewar SS, Desai SN, Rajesh S, Sarin SK. Changes in liver [6] Valla DC. The diagnosis and management of the Budd-Chiari syndrome: congestion in patients with Budd-Chiari syndrome following endovas- consensus and controversies. Hepatology 2003;384:793–803. cular interventions: assessment with transient elastography. J Vasc Interv [7] Han G, Qi X, Zhang W, He C, Yin Z, Wang J, et al. Percutaneous Radiol 2017;285:683–7. recanalization for Budd-Chiari syndrome: an 11-year retrospective study http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Medicine Pubmed Central

Utility of hepatic vein waveform and transient elastography in patients with Budd–Chiari syndrome who require angioplasty

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Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc.
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Abstract

Rationale: Budd–Chiari syndrome (BCS), which causes congestive hepatopathy and aggravates cirrhosis, is typically treated by interventional angioplasty to ameliorate blood flow. X-ray venography is useful for the evaluation of inferior vena cava (IVC) stenosis and determination of treatment timing, but it is invasive and thus unsuitable for repeated examinations. The development of a simple method for the prediction of IVC stenosis would reduce the burden on patients with BCS. Patient concerns: We report here our experience of 2 patients with BCS who underwent percutaneous transluminal angioplasty (PTA). The first patient was a 39-year-old male who underwent PTA to expand his stenotic IVC. The second patient was a 19-year-old male who underwent PTA 3 times due to restenosis of his IVC. Diagnoses: Both patients were diagnosed with BCS with severe obstruction of the IVC. Interventions: We evaluated the hepatic vein (HV) waveform by Doppler ultrasonography and measured liver stiffness (LS) using transient elastography (TE) before and after PTA. Outcomes: In case 1, the phasic oscillation of the HV waveform recovered and the LS value decreased after PTA. Both improvements were maintained for ∼3 years, reflecting the long-term patency of the IVC. In case 2, the HV waveform and the LS value improved temporarily after PTA, but then deteriorated gradually. Monitoring of the HV waveform and LS value allowed retreatment prior to total occlusion of the IVC and abrogated the risk of intravascular needle puncture. Lessons: Monitoring of the HV waveform and the LS value enables safe management of patients with BCS who may require PTA. Abbreviations: BCS = Budd–Chiari syndrome, CT = computed tomography, HV = hepatic vein, IVC = inferior vena cava, LS = liver stiffness, MRI = magnetic resonance imaging, PTA = percutaneous transluminal angioplasty, RHV = right hepatic vein, TE = transient elastography, US = ultrasonography. Keywords: Budd–Chiari syndrome, congestive hepatopathy, elastography, hepatic vein waveform 1. Introduction Editor: N/A. TN and YS contributed equally to this work. Budd–Chiari syndrome (BCS) is a rare clinical disorder caused by The patients have provided informed consent for publication of the case. obstruction of the hepatic venous outflow tract, and can result in congestive hepatopathy. The resulting long-term liver congestion The authors report no conflicts of interest. triggers hepatic fibrosis, leading to cirrhosis and hepatocellular Department of Gastroenterology, Graduate School of Medicine, The University b [1–3] of Tokyo, Bunkyo-ku, Tokyo, Japan, Department of Clinical Laboratory carcinoma. In Asia, membranous obstruction of the inferior [4] Medicine, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, vena cava (IVC) is the major cause of BCS. Percutaneous Tokyo, Japan, Department of Radiology, International University of Health and transluminal angioplasty (PTA) is frequently performed to Welfare, School of Medicine, Minato-Ku, Department of Radiology, Graduate alleviate hepatic congestion in patients with BCS and membra- School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan. [5,6] nous obstruction. However, restenosis of the IVC often Correspondence: Hayato Nakagawa, Department of Gastroenterology, [7] occurs, even after successful treatment ; in cases of severe Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. (e-mail: hanakagawa-tky@umin.ac.jp). occlusion of the IVC, retreatment by intravascular needle puncture—an invasive procedure with the potential for severe Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc. This is an open access article distributed under the Creative Commons complications—is required. Therefore, the periodic monitoring Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and of IVC patency and determination of the appropriate timing of reproduction in any medium, provided the original work is properly cited. PTA prior to the development of severe IVC obstruction are How to cite this article: Nakatsuka T, Soroida Y, Nakagawa H, Okura N, Sato J, mandatory. Catheter venography is the most direct method for Akahane M, Sato M, Yatomi Y, Abe O, Tateishi R, Koike K. Utility of hepatic vein the assessment of IVC patency, but it is unsuitable for repeated waveform and transient elastography in patients with Budd–Chiari syndrome who examinations due to its invasiveness. Doppler waveform of the require angioplasty. Medicine 2019;98:45(e17877). hepatic vein (HV) revealed by abdominal ultrasonography (US) is Received: 24 April 2019 / Received in final form: 26 September 2019 / Accepted: 9 October 2019 classified into triphasic, biphasic, and monophasic patterns and is known to reflect several conditions and hemodynamics of the http://dx.doi.org/10.1097/MD.0000000000017877 1 Nakatsuka et al. Medicine (2019) 98:45 Medicine [8] liver. Liver stiffness (LS) measured by elastography, which is a restenosis of the IVC (Fig. 2A), we examined the HV waveform useful device for evaluation of liver fibrosis, also reflects the by Doppler US and assessed LS by TE. As expected, the HV [9,10] degree of liver congestion. In this report, we present the cases waveform exhibited a flattened monophasic pattern (Fig. 2B) and of 2 patients with BCS treated by PTA, in whom the combination the LS value was very high (35.3kPa). As X-ray venography of HV waveform assessment by Doppler US and LS measurement revealed complete obstruction of the IVC, intravascular needle by transient elastography (TE) facilitated evaluation of the puncture was performed, as in the first PTA (Fig. 2C). The therapeutic effect and monitoring for postoperative restenosis. pressure gradient across the obstruction was reduced from 11 to 8 mm Hg. On the day after PTA, the HV waveform remained monophasic (Fig. 2B), but the LS value had decreased to 21.3 2. Case presentations kPa. One month after PTA, the HV waveform had changed to a 2.1. Case 1 biphasic pattern (Fig. 2B) and the LS value had decreased further to 14.3 kPa. The site of IVC stenosis remained patent (Fig. 2B). A 39-year-old male with a history of a sustained low platelet After the second PTA, we monitored the HV waveform and LS count detected during health checkups presented to our hospital. value at 3–month intervals to detect restenosis of the IVC before it US performed at an outpatient clinic had suggested the presence progressed to complete obstruction. At 10 months after the of chronic liver disease with splenomegaly and collateral second PTA, the waveform of the RHV had a monophasic formation of the portal vein. Contrast–enhanced computed pattern (Fig. 2B) and the LS value had increased to 20.5 kPa. tomography (CT) revealed severe stenosis of the IVC; based on Furthermore, the IVC had narrowed to 4.7mm and contained a this finding, the patient was diagnosed with BCS. We obtained highly echoic structure (Fig. 2B). Based on these data, we HV waveforms using pulsed-wave Doppler devices as previously [11] suspected restenosis of the IVC and performed X-ray venogra- reported. The Doppler gate was placed on the right HV 1 to 3 phy. Although IVC stenosis had recurred, complete obstruction cm distal to the inferior vena cava by an intercostal approach and had not yet developed. The stenosis was treated successfully by a flattened monophasic pattern was detected that likely reflected balloon dilation without needle puncture (Fig. 2D). One month blocked transmission of cardiac pulsation from the right atrium after the third PTA, the HV waveform exhibited a triphasic to the HV due to IVC stenosis (Fig. 1A). In addition, TE pattern and the LS value was 8.4 kPa (Fig. 2B, E). (FibroScan) yielded an LS value of 17.8kPa, suggesting congestion and/or severe fibrosis of the liver. X-ray venography showed complete obstruction of the IVC, 3. Discussion and the pressures below and above the obstruction were 17 and 8 In patients with BCS, PTA can improve hepatic congestion and mm Hg, respectively (pressure gradient, 9mm Hg; Fig. 1B). The prevent liver fibrosis and portal hypertension; however, resteno- IVC obstruction was treated by intravascular needle puncture [7] sis often occurs after the procedure. Because complete followed by balloon dilation. This treatment decreased the obstruction of the IVC increases the risk of complications of pressure gradient between below and above the obstructed site to catheter treatment, timely intervention is critical. 2 mm Hg, indicating successful removal of the IVC obstruction. The HV waveform is correlated with the severity of several On the day after PTA, the waveform of the patient’s RHV, hepatic diseases. Several groups, including ours, have reported assessed by Doppler US, had changed to a biphasic pattern [11–14] that the HV waveform is associated with liver fibrosis. (Fig. 1A). His LS value decreased rapidly to 8.7 kPa, suggesting Liver fibrosis increases parenchymal stiffness and reduces HV that the high LS value before PTA was due to congestion of the wall compliance, which results in flattening of the HV waveform liver, rather than to liver fibrosis. One year after PTA, the as liver fibrosis progresses. The HV waveform is also a useful patient’s HV waveform had further improved to a triphasic [6,15,16] indicator of BCS. The loss of phasic oscillation in the HV pattern (Fig. 1A), and his LS value was 9.4 kPa. Three years after waveform indicates that cardiac movement is not transmitted to PTA, IVC restenosis had not occurred, the patient’sHV the HV, suggesting obstruction of hepatic outflow. Recovery of waveform remained triphasic, and his LS value had decreased phasic oscillation in the HV waveform can be used to assess the slightly to 7.2 kPa. [17,18] therapeutic effect of balloon angioplasty. TE is used routinely for the noninvasive assessment of liver fibrosis. Liver 2.2. Case 2 [9,10] congestion leads to an increased LS value as measured by TE ; An 11-year-old male was diagnosed with BCS at a pediatric therefore, TE can be used to assess the effect of PTA on hepatic [19] hospital due to abnormal results of liver function tests. He had congestion in patients with BCS. been followed at that hospital without interventional therapy, but We investigated the utility of the HV waveform and the LS 2 years after the diagnosis of BCS he presented to our hospital value measured by TE for the evaluation of the therapeutic effect with severe edema in the legs and eyelids. Magnetic resonance of PTA and monitoring of the clinical course after angioplasty. In imaging (MRI) revealed severe stenosis of the IVC due to a case 1, the patency of the IVC was maintained for several years membranous structure. X-ray venography showed complete after PTA, as indicated by a triphasic HV waveform and a low LS obstruction of the IVC; thus, intravascular needle puncture value. In case 2, the LS value increased gradually after the second followed by balloon dilation was performed. After PTA, the PTA while the HV waveform remained biphasic, indicating the diameter of the IVC had increased to 15mm and the RHV flow progression of hepatic congestion due to the development of IVC had changed from retrograde to normal antegrade. The patient stenosis. The HV waveform ultimately flattened, suggesting subsequently underwent routine follow-up at the pediatric severe restenosis of the IVC. Therefore, LS may be highly sensitive hospital where he had been diagnosed with BCS. for the detection of IVC restenosis, and the HV waveform may be At the age of 19 years, the patient was admitted to our hospital useful for determination of the timing of interventional therapy. due to the exacerbation of lower–leg edema accompanied by The finding of HV waveform must be carefully interpreted, as it splenomegaly and thrombocytopenia. Because MRI suggested reflects both cardiac and hepatic physiology as well as simple 2 Nakatsuka et al. Medicine (2019) 98:45 www.md-journal.com Figure 1. HV waveform before and after PTA in case 1. (A) The waveform of the RHV before and after PTA. The waveform was monophasic before treatment. One day after PTA, the HV waveform changed to biphasic and remained so for 1 month. One year after PTA, the HV waveform had adopted a normal triphasic pattern. (B) X-ray venography revealed complete obstruction of the IVC; thus, it was expanded by 14-gauge needle puncture followed by balloon dilation. After PTA, the contrast agent flowed from the IVC to the right atrium. (R)HV=(right) hepatic vein, IVC=inferior vena cava, PTA=percutaneous transluminal angiography. heartbeat. We have previously shown that HV waveforms can be HV waveform may not improve after PTA, because the phasic [11] detected even in patients with right heart dysfunction under waveform is lost as liver fibrosis progresses. Fontan circulation, thus circulatory dynamics might not greatly In conclusion, we report 2 cases of BCS in which the HV [14] influence the shape of the HV waveform. In BCS patients who waveform and the LS value determined by TE enabled the have already had cirrhosis due to prolonged liver congestion, the identification of IVC stenosis. Monitoring of the HV waveform 3 Nakatsuka et al. Medicine (2019) 98:45 Medicine Figure 2. Changes in the HV waveform and LS value in case 2. (A) Enhanced MRI showed severe stenosis of the IVC (arrow). (B) Ultrasound images of the IVC and waveform of the RHV before and after PTA. Before the second PTA, a membranous structure was detected in the IVC (arrow) and the HV waveform was monophasic. One day after PTA, the IVC had opened slightly while the HV waveform remained monophasic. One month later, the IVC had expanded to 11mm and a biphasic waveform was detected, which was maintained for 7 months. Ten months after the second PTA, the IVC had narrowed to 4.7mm, the HV waveform exhibited a monophasic pattern, and a highly echoic structure was present in the IVC (arrow). After the third PTA, the IVC had expanded to 12.6 mm and the HV waveform was triphasic. (C) X-ray venography during the second PTA. The completely occluded IVC was expanded by 14-gauge needle puncture followed by balloon dilation. (D) X-ray venography during the third PTA; the narrowed IVC was expanded by balloon dilation. (E) Changes in the LS value and HV waveform pattern between before and after PTA. (R)HV=(right) hepatic vein, IVC=inferior vena cava, LS=liver stiffness, MRI=magnetic resonance imaging, PTA= percutaneous transluminal angiography. 4 Nakatsuka et al. Medicine (2019) 98:45 www.md-journal.com on patency and survival in 177 Chinese patients from a single center. and the LS value may be valuable for follow-up of patients with Radiology 2013;2662:657–67. BCS, although further confirmatory studies are needed. [8] Scheinfeld MH, Bilali A, Koenigsberg M. Understanding the spectral Doppler waveform of the hepatic veins in health and disease. Radio- graphics 2009;297:2081–98. Author contributions [9] Millonig G, Friedrich S, Adolf S, Fonouni H, Golriz M, Mehrabi A, et al. Liver stiffness is directly influenced by central venous pressure. J Hepatol Conceptualization: Takuma Nakatsuka, Yoko Soroida, Hayato 2010;522:206–10. Nakagawa. [10] Colli A, Pozzoni P, Berzuini A, Gerosa A, Canovi C, Molteni EE, et al. Data curation: Takuma Nakatsuka, Yoko Soroida. Decompensated chronic heart failure: increased liver stiffness measured Investigation: Takuma Nakatsuka, Yoko Soroida. by means of transient elastography. Radiology 2010;2573:872–8. [11] Soroida Y, Nakatsuka T, Sato M, Nakagawa H, Tanaka M, Yamauchi Methodology: Takuma Nakatsuka, Yoko Soroida. N, et al. A novel non-invasive method for predicting liver fibrosis by Resources: Naoki Okura, Jiro Sato, Masaaki Akahane. quantifying the hepatic vein waveform. Ultrasound Med Biol 2019; Supervision: Masaya Sato, Yutaka Yatomi, Osamu Abe, 459:2363–71. Kazuhiko Koike. [12] Bolondi L, Li Bassi S, Gaiani S, Zironi G, Benzi G, Santi V, et al. Liver Writing – original draft: Takuma Nakatsuka, Yoko Soroida. cirrhosis: changes of Doppler waveform of hepatic veins. Radiology 1991;1782:513–6. Writing – review & editing: Hayato Nakagawa, Ryosuke [13] Kawanaka H, Kinjo N, Anegawa G, Yoshida D, Migoh S, Konishi K, Tateishi, Kazuhiko Koike. et al. Abnormality of the hepatic vein waveforms in cirrhotic patients Takuma Nakatsuka orcid: 0000-0002-5727-5385. with portal hypertension and its prognostic implications. J Gastroenterol Hepatol 2008;23(7 Pt 2):e129–36. [14] Nakatsuka T, Soroida Y, Nakagawa H, Shindo T, Sato M, Soma K, et al. References Identification of liver fibrosis using the hepatic vein waveform in patients with Fontan circulation. Hepatol Res 2019;493:304–13. [1] Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J [15] Hosoki T, Kuroda C, Tokunaga K, Marukawa T, Masuike M, Kozuka Med 2004;3506:578–85. T. Hepatic venous outflow obstruction: evaluation with pulsed duplex [2] Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, sonography. Radiology 1989;170(3 Pt 1):733–7. Bahr MJ, et al. Etiology, management, and outcome of the Budd-Chiari [16] Bolondi L, Gaiani S, Li Bassi S, Zironi G, Bonino F, Brunetto M, et al. syndrome. Ann Intern Med 2009;1513:167–75. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. [3] Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla Gastroenterology 1991;100(5 Pt 1):1324–31. DC, et al. Budd-Chiari syndrome: a review by an expert panel. J Hepatol [17] Ohta M, Hashizume M, Tomikawa M, Ueno K, Tanoue K, Sugimachi K. 2003;383:364–71. Analysis of hepatic vein waveform by Doppler ultrasonography in 100 [4] Kage M, Arakawa M, Kojiro M, Okuda K. Histopathology of patients with portal hypertension. Am J Gastroenterol 1994;892: membranous obstruction of the inferior vena cava in the Budd-Chiari 170–5. syndrome. Gastroenterology 1992;1026:2081–90. [18] Hirooka K, Hirooka M, Kisaka Y, Uehara T, Hiasa Y, Michitaka K, et al. [5] Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, et al. Doppler waveform pattern changes in a patient with primary Budd- Managing Budd-Chiari syndrome: a retrospective review of percutane- Chiari syndrome before and after angioplasty. Intern Med 2008;472: ous hepatic vein angioplasty and surgical shunting. Gut 1999;444: 91–5. 568–74. [19] Mukund A, Pargewar SS, Desai SN, Rajesh S, Sarin SK. Changes in liver [6] Valla DC. The diagnosis and management of the Budd-Chiari syndrome: congestion in patients with Budd-Chiari syndrome following endovas- consensus and controversies. Hepatology 2003;384:793–803. cular interventions: assessment with transient elastography. J Vasc Interv [7] Han G, Qi X, Zhang W, He C, Yin Z, Wang J, et al. Percutaneous Radiol 2017;285:683–7. recanalization for Budd-Chiari syndrome: an 11-year retrospective study

Journal

Medicine – Pubmed Central

Published: Nov 11, 2019

References

  • The Budd-Chiari syndrome
  • Etiology, management, and outcome of the Budd-Chiari syndrome
  • Budd-Chiari syndrome: a review by an expert panel
  • Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome
  • Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting
  • The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies
  • Percutaneous recanalization for Budd-Chiari syndrome: an 11-year retrospective study on patency and survival in 177 Chinese patients from a single center
  • Understanding the spectral Doppler waveform of the hepatic veins in health and disease
  • Liver stiffness is directly influenced by central venous pressure
  • Decompensated chronic heart failure: increased liver stiffness measured by means of transient elastography
  • A novel non-invasive method for predicting liver fibrosis by quantifying the hepatic vein waveform
  • Liver cirrhosis: changes of Doppler waveform of hepatic veins
  • Abnormality of the hepatic vein waveforms in cirrhotic patients with portal hypertension and its prognostic implications
  • Identification of liver fibrosis using the hepatic vein waveform in patients with Fontan circulation
  • Hepatic venous outflow obstruction: evaluation with pulsed duplex sonography
  • Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound
  • Analysis of hepatic vein waveform by Doppler ultrasonography in 100 patients with portal hypertension
  • Doppler waveform pattern changes in a patient with primary Budd-Chiari syndrome before and after angioplasty
  • Changes in liver congestion in patients with Budd-Chiari syndrome following endovascular interventions: assessment with transient elastography