TY - JOUR
T1 - Evolving dilemmas and management of portopulmonary hypertension
AU - Krowka, Michael J.
PY - 2006/8
Y1 - 2006/8
N2 - Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.
AB - Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.
KW - Echocardiography
KW - Liver transplantation
KW - Pulmonary hypertension
UR - http://www.scopus.com/inward/record.url?scp=33746569076&partnerID=8YFLogxK
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U2 - 10.1055/s-2006-947294
DO - 10.1055/s-2006-947294
M3 - Review article
C2 - 16850376
AN - SCOPUS:33746569076
SN - 0272-8087
VL - 26
SP - 265
EP - 272
JO - Seminars in liver disease
JF - Seminars in liver disease
IS - 3
ER -