Exercise-Aggravated Hypoxemia and Orthodeoxia in Cirrhosis

Kennedy, Timothy C.; Knudson, Ronald J.

doi:10.1378/chest.72.3.305

Cited by 189 publications

(79 citation statements)

References 23 publications

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“…Hepatopulmonary syndrome was first described in 1884 by Fluckiger, who noted a relationship between the liver and the lung (Kennedy and Knudson 1977). In 1997, Kennedy and Knudson described "hepatopulmonary syndrome" (Yap et al 1999).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Manifestations in a Patient with Transaldolase Deficiency

et al. 2013

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Section: Discussionmentioning

confidence: 99%

Pulmonary Manifestations in a Patient with Transaldolase Deficiency

et al. 2013

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“…Although the first case was described in 1884, the term HPS was introduced only in 1977 by Kennedy. It is also combined with the occurrence of portal hypertension; however, this syndrome can be caused by every chronic disease of the liver, as well as some causes of acute liver damage [2][3][4]. In the subject literature HPS is estimated to be 4% to 32% of adult patients with the end-stage liver disease.…”

Section: Discussionmentioning

confidence: 99%

Dyspnoea, Cyanosis and Digital Clubbing in a 28-Year-Old Patient as a Result of Hepatopulmonary Syndrome

Zieliński

Hartleb

Sitek³

2017

Advances in Respiratory Medicine

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This paper presents a case of a young patient with cyanosis and digital clubbing, until then an active, sporty person. He sought medical assistance due to the growing dyspnoea and the drop of effort tolerance. Initially the diagnostic process focused on the confirmation of the suspicion of pulmonary fibrosis or another interstitial lung disease as causes of the respiratory failure. Due to the atypical presentation of the symptoms, reaching the final diagnosis of digestive system disease with lung involvement required a more thorough multifaceted diagnostics of a number of systems and organs.

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“…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. [11][12][13][14][15][16] Animal models of HPS have challenged the concept of a portal hypertensive basis to HPS; these were developed by causing injury to the liver (e.g., common bile duct ligation) or creating portal HTN (e.g., ligation of the portal vein). 17 The model of portal HTN without liver injury did not result in the development of HPS, leading the investigators to suggest that hepatic injury is necessary for HPS to develop.…”

Section: Discussionmentioning

confidence: 99%

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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Exercise-Aggravated Hypoxemia and Orthodeoxia in Cirrhosis

Cited by 189 publications

References 23 publications

Pulmonary Manifestations in a Patient with Transaldolase Deficiency

Pulmonary Manifestations in a Patient with Transaldolase Deficiency

Dyspnoea, Cyanosis and Digital Clubbing in a 28-Year-Old Patient as a Result of Hepatopulmonary Syndrome

Hepatopulmonary syndrome occurring after orthotopic liver transplantation

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