ArticleTransplantation direct2021
Causes and Circumstances of Death in Portopulmonary Hypertension.
Article in Transplantation direct, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it, 12 citations in OpenAlex.
- International Liver Transplantation Society practice guideline update on portopulmonary hypertension.Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society · 2026Guideline
- The Clinical Course of Portopulmonary Hypertension and Outcomes With Endothelin Receptor Antagonist Treatment: Observational Study of Data From the US Organ Procurement and Transplantation Network.Transplantation direct · 2024Article
- Portopulmonary Hypertension: An Updated Review.Transplantation direct · 2023Review
- Pulmonary Arterial Hypertension Associated with Portal Hypertension and HIV Infection: Comparative Characteristics and Prognostic Predictors.Journal of clinical medicine · 2023Article
- Liver Transplant Outcomes in Patients With Postcapillary Pulmonary Hypertension.Transplantation direct · 2022Article
- Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan.JGH open : an open access journal of gastroenterology and hepatology · 2022Article
- Portopulmonary hypertension: Current developments and future perspectives.Liver research (Beijing, China) · 2022Review
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
The causes and circumstances surrounding death are poorly studied in patients with portopulmonary hypertension (PoPH). We sought to determine the specific reasons for dying and characteristics surrounding this process in patients with PoPH.
methodsAll deaths of patients with PoPH followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the pulmonary hypertension team between 1996 and 2020.
resultsA total of 69 patients with PoPH (age 56.0 ± 8.9 y), with 49% females, were included. Causes of death were available in 52 (75%) patients, of these PoPH either directly or indirectly contributed to death in 13 of 52 (25%) of patients, meanwhile 39 of 52 (75%) of the patients died because of progressive liver disease and its related complications. Decompensated liver disease was the leading cause of death in this cohort 20 of 52 (38%), whereas 19 of 52 (37%) died because of conditions associated with liver disease. About half, 36 of 69 (52%) of patients died in a healthcare environment and 23 of 36 (64%) during a hospitalization at Cleveland Clinic. A total of 59 of 69 (74%) of patients received pulmonary arterial hypertension (PAH)-specific therapies. Six patients died after liver transplantation (in 3 death was related to PAH-related complications). Most of the patients in this cohort of PoPH patients were considered unsuitable for liver transplantation for a variety of reasons. Advanced healthcare directives were available in only 28% of patients.
conclusionsMost patients with PoPH died because of complications of their liver disease. PAH directly or indirectly contributed to death in a third of them. A quarter of them did not receive PAH-specific therapy before their death.
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Registered trials
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