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Pulmonary arterial hypertension associated with portal hypertension

MedGen UID:
742247
•Concept ID:
C1868851
•
Disease or Syndrome
Synonyms: PAH associated with portal hypertension; POPH; Portopulmonary hypertension
SNOMED CT: Portopulmonary hypertension (445237003); Pulmonary arterial hypertension associated with portal hypertension (445237003)
Modes of inheritance:
No inheritance data available
MedGen UID:
989178
•Concept ID:
CN307043
•
Finding
Source: Orphanet
No information is available in the scientific literature on heredity of the clinical entity.
See: This record
No inheritance data available (Orphanet)
 
Orphanet: ORPHA275813

Definition

Pulmonary arterial hypertension associated with portal hypertension (PAH-PH) is a form of pulmonary arterial hypertension (PAH), characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of portal hypertension. [from ORDO]

Professional guidelines

PubMed

Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
Hollatz TJ, Musat A, Westphal S, Decker C, D'Alessandro AM, Keevil J, Zhanhai L, Runo JR
Liver Transpl 2012 Jun;18(6):686-95. doi: 10.1002/lt.23407. PMID: 22315210

Recent clinical studies

Etiology

Does Portopulmonary Hypertension Impede Liver Transplantation in Cirrhotic Patients? A French Multicentric Retrospective Study.
Reymond M, Barbier L, Salame E, Besh C, Dumortier J, Pageaux GP, Bureau C, Dharancy S, Vanlemmens C, Abergel A, Woehl Jaegle ML, Magro P, Patat F, Laurent E, Perarnau JM
Transplantation 2018 Apr;102(4):616-622. doi: 10.1097/TP.0000000000001981. PMID: 29077657
Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
Hollatz TJ, Musat A, Westphal S, Decker C, D'Alessandro AM, Keevil J, Zhanhai L, Runo JR
Liver Transpl 2012 Jun;18(6):686-95. doi: 10.1002/lt.23407. PMID: 22315210
Sildenafil therapy is associated with improved hemodynamics in liver transplantation candidates with pulmonary arterial hypertension.
Gough MS, White RJ
Liver Transpl 2009 Jan;15(1):30-6. doi: 10.1002/lt.21533. PMID: 19109844
Hemodynamics and survival of patients with portopulmonary hypertension.
Kawut SM, Taichman DB, Ahya VN, Kaplan S, Archer-Chicko CL, Kimmel SE, Palevsky HI
Liver Transpl 2005 Sep;11(9):1107-11. doi: 10.1002/lt.20459. PMID: 16123953

Diagnosis

Does Portopulmonary Hypertension Impede Liver Transplantation in Cirrhotic Patients? A French Multicentric Retrospective Study.
Reymond M, Barbier L, Salame E, Besh C, Dumortier J, Pageaux GP, Bureau C, Dharancy S, Vanlemmens C, Abergel A, Woehl Jaegle ML, Magro P, Patat F, Laurent E, Perarnau JM
Transplantation 2018 Apr;102(4):616-622. doi: 10.1097/TP.0000000000001981. PMID: 29077657

Therapy

Does Portopulmonary Hypertension Impede Liver Transplantation in Cirrhotic Patients? A French Multicentric Retrospective Study.
Reymond M, Barbier L, Salame E, Besh C, Dumortier J, Pageaux GP, Bureau C, Dharancy S, Vanlemmens C, Abergel A, Woehl Jaegle ML, Magro P, Patat F, Laurent E, Perarnau JM
Transplantation 2018 Apr;102(4):616-622. doi: 10.1097/TP.0000000000001981. PMID: 29077657
Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
Hollatz TJ, Musat A, Westphal S, Decker C, D'Alessandro AM, Keevil J, Zhanhai L, Runo JR
Liver Transpl 2012 Jun;18(6):686-95. doi: 10.1002/lt.23407. PMID: 22315210
Sildenafil therapy is associated with improved hemodynamics in liver transplantation candidates with pulmonary arterial hypertension.
Gough MS, White RJ
Liver Transpl 2009 Jan;15(1):30-6. doi: 10.1002/lt.21533. PMID: 19109844

Prognosis

Does Portopulmonary Hypertension Impede Liver Transplantation in Cirrhotic Patients? A French Multicentric Retrospective Study.
Reymond M, Barbier L, Salame E, Besh C, Dumortier J, Pageaux GP, Bureau C, Dharancy S, Vanlemmens C, Abergel A, Woehl Jaegle ML, Magro P, Patat F, Laurent E, Perarnau JM
Transplantation 2018 Apr;102(4):616-622. doi: 10.1097/TP.0000000000001981. PMID: 29077657
Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension.
Hollatz TJ, Musat A, Westphal S, Decker C, D'Alessandro AM, Keevil J, Zhanhai L, Runo JR
Liver Transpl 2012 Jun;18(6):686-95. doi: 10.1002/lt.23407. PMID: 22315210

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