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Weekly Chest CasesArchive of Old Cases

Case No : 627 Date 2009-11-01

  • Courtesy of So Young Choi, Chang Hyun Lee / Seoul National University Hospital
  • Age/Sex 36 / M
  • Chief ComplaintDyspnea
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Hepatopulmonary syndrome
Radiologic Findings
Chest radiograph shows diffuse reticulonodular opacities in both lungs. CT images (lung window settings) show dilatation of peripheral pulmonary vessels. Liver shows cirrhotic change and there are splenic varices.
Brief Review
Hepatopulmonary syndrome is characterized by a triad of hypoxemia, hepatic dysfunction, and intrapulmonary vascular dilatation. Although the pathogenesis of vascular dilatation is unknown, some investigators have suggested that failure of the damaged liver to clear circulating vasodilators and inhibition of a circulating vasoconstrictive substance by the damaged liver.
Arterial hypoxemia in patients with hepatopulmonary syndrome is thought to occur because of ventilation-perfusion mismatch, limitations in oxygen diffusion due to vascular dilatation, and in some patients, intrapulmonary shunt through dilated vascular malformations.
On chest radiographs, intrapulmonary vascular dilation may manifest as small nodular or reticulonodular opacities, predominantly in the bases. The CT feature of hepatopulmonary syndrome is dilation of peripheral lung vessels with many extending to the pleura.
Lung perfusion imaging with 99mTc macroaggregated albumin can be used both in the detection of a right-to-left shunt and in the quantification of the degree of the shunt. In patients with normal pulmonary vasculature, nearly all the radioactive particles뾵hich typically range in size from 20 to 50 ?뾩odge in the pulmonary vascular bed, since pulmonary capillaries are only 8?5 ? in diameter. The presence of intrapulmonary vascular dilatations in patients with HPS allows passage of the radiolabeled macroaggregated albumin into the systemic circulation and results in the characteristic appearance of extrapulmonary uptake. Several studies that have evaluated prognostic factors in patients with HPS have shown that mortality is associated with higher shunt fractions, as measured with 99mTc macroaggregated albumin perfusion imaging.
References
1.Krowaka MJ, Cortese DA. Hepatopulmonary syndrome: current concepts in diagnostic and therapeutic considerations. Chest 194;47:897-902
2.Kim JH, Park CH, Pai MS, Hahn MH, Kim HJ. Hepatopulmonary syndrome in Gaucher disease with right-to-left shunt: evaluation and measurement using Tc-99m MAA. Clin Nucl Med 1999;24:164-166
3.Castro M, Krowaka MJ. Hepatopulmonary syndrome: a pulmonary vascular complication of liver disease. Clin Chest Med 1996; 17:35-48
4.Cremona G, Higenbottam TW, Mayoral V, et al. Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur Respir J 1995;8:1883-1885
5.Lee KN, Lee HA, Shin WW, Webb WR. Hypoxia and liver cirrhosis (hepatopulmonary syndrome) in eight patients: comparison of the central and peripheral pulmonary vasculature. Radiology 1999;211:549-53.
6.Oh YW, Kang EY, Lee NJ, Suh WH, Godwin JD. Thoracic manifestations associated with advanced liver disease. J Comput Assist Tomogr 2000;24:699-705
7.Ann N. Leung. Case 6: Hepatopulmonary symdome. Radiology 2003:229;64-67.
Please refer to
Case 113 Case 195
Keywords
Lung, Multiple organ, Vascular,

No. of Applicants : 90

▶ Correct Answer : 57/90,  63.3%
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - CH Sud Rnion , Reunion jean-baptiste Noel
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) YOOKYUNG KIM
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - Osaka University , Japan Osamu Honda
  • - Assam medical college , India Karunakaran M
  • - Samsung Medical Center , Korea (South) Eun Kim
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Goa Medical College , India Paresh Desai
  • - Assam Medical College , India Pramod K.G
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - NTUH , Taiwan Kuei-pin Chung
  • - CHRU Lille , France guillaume lefebvre
  • - CHRU lille , France manuel toledano
  • - SMG-SNUH boramae medical center , Korea (South) Kwang Nam Jin
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Kang buk samsung hospital, department of radiology , Korea (South) Hyojin Lim
  • - Dong-A university hospital , Korea (South) Sang-yun LEE
  • - Gangnam Severance Hospital , Korea (South) Min Jeong Kim
  • - NOUR EL-ISLAM RADIOLOGY CENTER , Egypt Elsayed Mousa
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
  • - hospital Sao Paulo , Brazil israel missrie
  • - Seoul St Mary , Korea (South) Jin Kyoung Oh
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Samsung Medical Center , Korea (South) Ju Won Lee
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - KING KHALID UNIVERSITY HOSPITAL , Saudi Arabia Elbagir Nasser
  • - ROHINI Scan Centre RSC. Ambattur, Chennai , India Umapathi Mahesh
  • - Hospital M de Deus , Brazil Thiago Bento da Silva
  • - SMC , Korea (South) Jihoon Cha
  • - CHU caen , Canada nicolas gautier
  • - Saga University , Japan Ryoko Egashira
  • - Seoul Nationial University Bundang Hospital , Korea (South) Kyoung Jin Oh
  • - Department of Radiology, University of Alabama at Birmingham , United States Yukihiro Nagatani
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - CHRU LILLE , France Vittorio Pansini
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - McGill University Health Center , Canada Alexandre Semionov
  • - uniradiology , Australia bimal parameswaran
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OKUBO
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - InRad - HCFMUSP S Paulo , Brazil Natanael Ahn
  • - hospital 12 de octubre , Spain sergio alonso
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - IRSA , France jean-luc BIGOT
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Uzunkopru Devlet Hastanesi , Turkey Armagan Sarac
  • - NASA SCANS , India RAKESH BHATIA
  • - SacrCoeur , Canada Andrea Ojanguren
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - UZ GASTHUISBERG , Belgium Johan COOLEN
  • - PITTWATER RADIOLOGY , Australia Saurabh Khandelwal
  • - PITTWATER RADIOLOGY , Australia Julie Arora
  • - Armed Forces Chun-Cheon Hospital , Korea (South) Chaehun Lim
▶ Correct Answer as Differential Diagnosis : 3/90,  3.3%
  • - partner, aditya imaging centre , India vivek patel
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - vinayaka missions medical college , India k bhaskar
▶ Semi-Correct Answer : 1/90,  1.1%
  • - MD SKNMC & GH,Pune,Maharashtra, INDIA , India Sushant Bhadane
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