ArticleCase reports in pulmonology2013
Transition from hepatopulmonary syndrome to portopulmonary hypertension: a case series of 3 patients.
Article in Case reports in pulmonology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 28 citations in OpenAlex.
- Cardiac Syndromes in Liver Disease: A Clinical Conundrum.Journal of clinical and translational hepatology · 2023Review
- Quercetin ameliorates acute lung injury in a rat model of hepatopulmonary syndrome.BMC complementary medicine and therapies · 2022Article
- Article
- Pulmonary Hypertension that Developed During Treatment for Hepatopulmonary Syndrome and Pulmonary Arteriovenous Malformation.Internal medicine (Tokyo, Japan) · 2019Article
- Onset ages of hepatopulmonary syndrome and pulmonary hypertension in patients with biliary atresia.Pediatric surgery international · 2017Article
- Hepatopulmonary Syndrome and Liver Transplantation: A Recent Review of the Literature.Journal of clinical and translational hepatology · 2016Review
- Aggravation of hepatopulmonary syndrome after sildenafil treatment in a patient with coexisting portopulmonary hypertension.Korean circulation journal · 2015Article
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are the two major pulmonary vascular complications of liver disease. While HPS is characterized by low pulmonary vascular resistance, PPHTN is defined by the presence of elevated pulmonary vascular resistance. Given these seemingly opposing pathophysiologic mechanisms, these conditions were traditionally felt to be mutually exclusive. In this series, we present three patients with severe hepatopulmonary syndrome who had spontaneous resolution of their HPS with the subsequent development of PPHTN. To our knowledge, this is the largest case series presented of this phenomenon in nontransplanted patients. One proposed mechanism for the occurrence of this phenomenon involves dysregulation of the same vascular signaling pathway, which may lead to both pulmonary vascular dilatations and pulmonary arterial remodeling in the same patient. Another theory involves the possible differential binding of endothelin-1, a vasoactive signaling peptide that induces vasoconstriction when bound to receptor A and vasodilation when bound to receptor B. Although the mechanisms for this phenomenon remain unclear, it is important to be vigilant of this phenomenon as it may change the patient's overall treatment plan, especially in regard to appropriateness and timing of liver transplant.
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Registered trials
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