Portopulmonary Hypertension in Nontransplanted Patients : Results of the Largest US Single-Institution Registry
Copyright © 2022 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved..
OBJECTIVE: To explore clinical characteristics, risk profiles, and outcomes of patients with portopulmonary hypertension (PoPH) who have contraindications to liver transplant (LT).
METHODS: From the largest US single-institution registry of patients with PoPH, we analyzed 160 patients who did not receive LT between 1988 to 2019. Pulmonary arterial hypertension (PAH)-pertinent characteristics, hemodynamic features, treatments, and risk stratification were compared at baseline, first follow-up visit, and censor/death time.
RESULTS: Median survival for the entire cohort was 27.5 months from the diagnosis of PoPH. Overall survival was 89%, 77%, 51%, and 38% at 6 months, 1 year, 3 years, and 5 years, respectively. Survival was significantly affected by the severity of liver disease (P<.001). Most patients received PAH-specific therapies (136 [85%]), predominantly monotherapy (123 [77%)]. With treatment, significant improvements were noted in World Health Organization functional class (P=.04), 6-minute walk distance (P<.001), right ventricular function (P<.001), pulmonary vascular resistance (P<.001), and Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) Lite 2 score (P=.02) univariately. Per European Society of Cardiology risk stratification, no patient met full criteria for low risk at baseline or at follow-up. In a multivariate Cox risk model, 6-minute walk distance, right atrial pressure, pulmonary capillary wedge pressure, bilirubin level, and Model for End-Stage Liver Disease-sodium score of 15 or higher were associated with increased risk of death.
CONCLUSION: Patients with PoPH who did not undergo LT had a poor prognosis. This persisted despite use of PAH-specific therapies and significant improvements in hemodynamics, echocardiography parameters of right ventricle function, 6-minute walk distance, and World Health Organization functional class.
| Errataetall: |
CommentIn: Mayo Clin Proc. 2022 Dec;97(12):2189-2191. - PMID 36464461 |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:97 |
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| Enthalten in: |
Mayo Clinic proceedings - 97(2022), 12 vom: 15. Dez., Seite 2236-2247 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Cajigas, Hector R [VerfasserIn] |
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Date Completed 06.12.2022 Date Revised 09.12.2022 published: Print-Electronic CommentIn: Mayo Clin Proc. 2022 Dec;97(12):2189-2191. - PMID 36464461 Citation Status MEDLINE |
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| doi: |
10.1016/j.mayocp.2022.08.020 |
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| 500 | |a CommentIn: Mayo Clin Proc. 2022 Dec;97(12):2189-2191. - PMID 36464461 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2022 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved. | ||
| 520 | |a OBJECTIVE: To explore clinical characteristics, risk profiles, and outcomes of patients with portopulmonary hypertension (PoPH) who have contraindications to liver transplant (LT) | ||
| 520 | |a METHODS: From the largest US single-institution registry of patients with PoPH, we analyzed 160 patients who did not receive LT between 1988 to 2019. Pulmonary arterial hypertension (PAH)-pertinent characteristics, hemodynamic features, treatments, and risk stratification were compared at baseline, first follow-up visit, and censor/death time | ||
| 520 | |a RESULTS: Median survival for the entire cohort was 27.5 months from the diagnosis of PoPH. Overall survival was 89%, 77%, 51%, and 38% at 6 months, 1 year, 3 years, and 5 years, respectively. Survival was significantly affected by the severity of liver disease (P<.001). Most patients received PAH-specific therapies (136 [85%]), predominantly monotherapy (123 [77%)]. With treatment, significant improvements were noted in World Health Organization functional class (P=.04), 6-minute walk distance (P<.001), right ventricular function (P<.001), pulmonary vascular resistance (P<.001), and Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) Lite 2 score (P=.02) univariately. Per European Society of Cardiology risk stratification, no patient met full criteria for low risk at baseline or at follow-up. In a multivariate Cox risk model, 6-minute walk distance, right atrial pressure, pulmonary capillary wedge pressure, bilirubin level, and Model for End-Stage Liver Disease-sodium score of 15 or higher were associated with increased risk of death | ||
| 520 | |a CONCLUSION: Patients with PoPH who did not undergo LT had a poor prognosis. This persisted despite use of PAH-specific therapies and significant improvements in hemodynamics, echocardiography parameters of right ventricle function, 6-minute walk distance, and World Health Organization functional class | ||
| 650 | 4 | |a Journal Article | |
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| 700 | 1 | |a Cartin-Ceba, Rodrigo |e verfasserin |4 aut | |
| 700 | 1 | |a DuBrock, Hilary |e verfasserin |4 aut | |
| 700 | 1 | |a Swanson, Karen |e verfasserin |4 aut | |
| 700 | 1 | |a Vargas, Hugo E |e verfasserin |4 aut | |
| 700 | 1 | |a Keaveny, Andrew P |e verfasserin |4 aut | |
| 700 | 1 | |a Watt, Kymberly D |e verfasserin |4 aut | |
| 700 | 1 | |a Krowka, Michael |e verfasserin |4 aut | |
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