Hepatopulmonary Syndrome in Noncirrhotic Portal Hypertension

De, Binay K; Sen, Sambit; Sanyal, Ranjan

doi:10.7326/0003-4819-132-11-200006060-00023

Cited by 19 publications

(15 citation statements)

References 5 publications

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“…6 HPS associated with noncirrhotic portal hypertension is uncommon. [7][8][9][10] In such patients, some degree of accompanying noncirrhotic liver injury may or may not exist. Resolution of HPS after OLT has been documented in that setting.…”

Section: Commentsmentioning

confidence: 99%

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Krowka

2001

Liver Transplantation

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This report describes the unique development of pulmonary vascular dilatation and hypoxemia associated with portosystemic shunt in a pediatric liver transplant recipient. Ligation of the shunt resulted in resolution of hypoxemia. The outcome suggests that hepatic venous return to the pulmonary circulation is important in maintaining normal pulmonary vascular caliber.(2) Hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty. De BK, Sen S, Biswas PK, Sanyal R, Majumdar D, Biswas J. Gastroenterology 2000;118:192-196. (Reprinted with permission.) AbstractReports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis. However, hepatopulmonary syndrome has also been reported in cases of mild hepatic dysfunction or noncirrhotic portal hypertension, indicating that portal hypertension also plays a part in the pathogenesis. Liver transplantation remains the only therapeutic option of proven benefit. We describe 2 cases of hepatopulmonary syndrome in the setting of inferior vena cava (suprahepatic) obstruction, but with minimal hepatic dysfunction. After balloon cavoplasty, 1 patient showed, in addition to improvement of the features of hepatic outflow obstruction, significant reduction of dyspnea, cyanosis, and hypoxemia with arterial blood gas normalization within 2 weeks and intrapulmonary shunt reversal within 8 weeks. This implies that hemodynamic alterations (such as portal hypertension) independently contribute to the pathogenesis of hepatopulmonary syndrome in at least some of the cases. Therapies aimed at correcting these abnormal hemodynamics may be important in the treatment of this condition, especially when the hepatic functional status by itself does not warrant a liver transplant.

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Section: Commentsmentioning

confidence: 99%

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Krowka

2001

Liver Transplantation

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“…HPS has been reported to occur in other conditions of noncirrhotic liver injury. 2,3,8,9 HPS also has been shown after OLT, 5,10 although those patients had severe recurrent cirrhosis identified in association with HPS. In all those patients, HPS occurred in the setting of portal HTN, suggesting that portal HTN is the common denominator in the pathogenesis of HPS.…”

Section: Discussionmentioning

confidence: 96%

Hepatopulmonary syndrome occurring after orthotopic liver transplantation

Avendano

Flume

Baliga

et al. 2001

Liver Transplantation

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H epatopulmonary syndrome (HPS) is a wellknown consequence of chronic liver disease. 1 HPS is defined as a clinical triad of chronic liver disease, increased alveolar-arterial gradient while breathing room air, and evidence of intrapulmonary vascular dilations. 1 Most patients present with symptoms related to their liver disease (e.g., ascites or gastrointestinal bleeding), although some patients present with respiratory symptoms (e.g., dyspnea). 2 Typically, HPS occurs in patients with chronic liver disease resulting in cirrhosis, but it also has occurred in patients with noncirrhotic portal hypertension (HTN). 2,3 Orthotopic liver transplantation (OLT) has become a therapeutic option for patients with HPS, and patients often realize a regression of intrapulmonary shunts after OLT. 4 Recurrent HPS after OLT recently was reported in a patient who underwent OLT for severe HPS associated with nonalcoholic steatohepatitis. 5 HPS resolved after OLT, but the patient again experienced cirrhosis and subsequently developed HPS. We report a patient who developed severe HPS several years post-OLT, but unlike the typical presentation of HPS, our patient did not have cirrhosis or evidence of portal HTN. Case ReportA 9-year-old boy was referred for evaluation of progressive hypoxia. He was born with biliary atresia and underwent a Kasai procedure without success. He underwent OLT at the age of 7 months, which was complicated early by severe pneumonia caused by Pneumocystis carinii requiring mechanical ventilatory support, and later (4 years), by Epstein-Barr virusassociated lymphoproliferative disorder. This was treated with antiviral medications and a reduction in immunosuppression. One year later, a diagnosis of Hodgkin's lymphoma was made. His treatment consisted of splenectomy, radiotherapy to mediastinal lymph nodes (with lung shielding), and chemotherapy (courses of mechlorethamine, vincristine, procarbazine, and prednisone alternating with doxorubicin, bleomycin, vinblastine, and dacarbazine for 8 months).He was evaluated for respiratory symptoms at the age of 7 years. He had a nonproductive cough that worsened with exercise. Spirometry results were interpreted as normal, and room air arterial PO 2 was 63 mm Hg (alveolar-arterial gradient, 50 mm Hg). The favored diagnoses at that time included radiation and bleomycin pneumonitis, and the patient was treated with oral steroid therapy. Exertional dyspnea and arterial oxygen desaturation persisted, but remained stable.He was referred at 9 years for evaluation of malaise and progressive dyspnea with marked exercise intolerance. Room air arterial PO 2 was 55 mm Hg (alveolar-arterial gradient, 57.5 mm Hg), and he showed orthodeoxia. Arterial oxygen on a fraction of inspired oxygen of 1.0 was 428 mm Hg. Spirometry showed a forced vital capacity of 1.04 L (59% predicted), but with an expiratory time of only 0.5 seconds; previous test results had been normal, and this test also was interpreted as likely to be normal.Chest radiography showed a mild increase in perihilar interst...

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“…9,10,12,49,50 The frequency among non-cirrhotic patients with portal hypertension has been found to be lower, ranging from 8% to 9.7%. 29,30 Among cases of chronic hepatitis with no cirrhosis or portal hypertension, the occurrence rate is 1.1%. 31 In 2004, a paper was published suggesting standardization for diagnosing HPS, 5 and this has contributed towards future comparisons between different studies.…”

Section: Diagnosing Of Hpsmentioning

confidence: 99%

“…[25][26][27] Confirming the possibility that IPVD might develop in non-cirrhotic patients with portal hypertension, HPS has been diagnosed in this population, although with a lower occurrence rate than among patients with cirrhosis. [28][29][30] HPS has also been described in non-cirrhotic patients with chronic viral hepatitis and with normal portal pressure, 31 as well as during the evolution of severe acute liver failure. 32 …”

Section: Chronic Liver Disease and Liver Transplantationmentioning

confidence: 99%

Hepatopulmonary syndrome: an update

Macedo

Lopes

2009

Sao Paulo Med. J.

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Hepatopulmonary syndrome (HPS) is a clinical threesome composed of liver disease, intrapulmonary vascular dilatation (IPVD) and arterial gas abnormalities. Its occurrence has been described in up to 32% of cirrhotic candidates for liver transplantation. It also affects non-cirrhotic patients with portal hypertension. Its pathogenesis is not well defined, but an association of factors such as imbalance in the endothelin receptor response, pulmonary microvascular remodeling and genetic predisposition is thought to lead to IPVD. Diagnosis is based on imaging methods that identify these dilatations, such as contrast echocardiography or perfusion scintigraphy with RESUMOA síndrome hepatopulmonar (SHP) é considerada uma tríade clínica composta de doença hepática, dilatações vasculares intrapulmonares (IPVD) e alterações de gases arteriais. Há descrição de sua ocorrência em até 32% dos cirróticos candidatos ao transplante de fígado, acometendo também não cirróticos com hipertensão portal. Sua etiopatogenia não está bem definida, mas se especula que a associação de fatores como o desequilíbrio na resposta dos receptores vasculares de endotelina, o remodelamento microvascular pulmonar e a predisposição genética propiciem as IPVD. O diagnóstico baseia-se em métodos de imagem que identifiquem essas dilatações, como a ecocardiografia com contraste ou a cintilografia de perfusão com 99m Tc, além da análise de gases arteriais, para identificar elevação da diferença alvéolo-arterial de O 2 ou hipoxemia. Não existe tratamento medicamentoso eficaz e sua resolução completa ocorre apenas com o transplante de fígado. A importância do diagnóstico da SHP está em priorizar os candidatos ao transplante, uma vez que sua presença está associada ao pior prognóstico. O objetivo deste artigo é fazer revisão das teorias de etiopatogenia da

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Hepatopulmonary Syndrome in Noncirrhotic Portal Hypertension

Cited by 19 publications

References 5 publications

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Hepatopulmonary syndrome and extrahepatic vascular abnormalities

Hepatopulmonary syndrome occurring after orthotopic liver transplantation

Hepatopulmonary syndrome: an update

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