Navigation Links
A combination therapy of 3 vasodilators may treat portopulmonary hypertension

Combination therapy of Prostacyclin, Sildenafil, and Bosentan helped a young male patient with severe portopulmonary hypertension improve enough to receive a liver transplant. It was also used post-transplant to help him maintain his health. These findings are published in the March issue of Liver Transplantation, a journal by John Wiley & Sons. The article is also available online at Wiley Interscience (

Portopulmonary hypertension (PPHTN) is pulmonary arterial hypertension associated with liver disease or portal hypertension. Our knowledge about treatment options for this condition is limited by lack of randomized controlled trials, concerns about drug safety and the inability to reverse pulmonary disease. Liver transplant is an effective treatment for many patients with mild-to-moderate PPHTN characterized by a mean pulmonary artery pressure (mPAP) <35mmHg; but it is often not offered to patients who have severe cases (mPAP >50mmHg).

Researchers led by Mark Austin of Kings College Hospital describe a case study of a 26-year-old male patient who had severe refractory PPHTN and non-cirrhotic portal hypertension. He was treated with sequential, then combination, therapy of the vasodilators Iloprost, Sildenafil, and Bosentan. His condition improved dramatically, with his mPAP falling from 70 to 32mmHg. He underwent liver transplantation, and continued on the therapy for two years post-transplant, when his mPAP fell even further to 28mmHg. He currently continues on dual combination therapy of Sildenafil and Bosentan, and his doctors hope to eventually withdraw both agents.

This is the first reported patient with severe PPHTN using this combination of vasodilator therapy as a bridge to liver transplantation and then as maintenance in the post-transplant phase. This regimen may enable liver transplantation in similar patients in the future, without long-term consequences, the authors conclude.

An accompanying editorial by Karen Swanson of the Mayo Clinic writes, It is easy to see why combination therapy may make sense as we do not yet have a means of determining which predominant mechanism is influencing which patient and, which medications might be most beneficial specifically in POPH.

She laments the fact that POPH is excluded from every clinical trial involving pulmonary hypertension, especially given its prevalence, and worries about the cost of triple-drug therapy when we dont even have good prospective data on individual drug therapy.

Only by following multi-center prospective protocols in POPH will we ever have more meaningful answers to the ideal course of treatment in these patients, she concludes. Until then, we continue to extrapolate our thoughts from single center case reports and case series. Can we move on?


Contact: Amy Molnar

Related medicine news :

1. In Combination With FOLFOX4, the Effect of Erbitux and Avastin On Survival Rates Will Drive Prescription Decisions in the Treatment of Stage III Colon Cancer
2. Combination of Thyroid Medicines Not Necessary
3. Combination therapy improves survival for certain prostate cancer patients
4. Enlarged Prostate Treatment Study Shows Combination of AVODART(R) and Tamsulosin Provides Greater Benefit Than Either Medication Alone
5. Drug combination shrinks breast cancer metastases in brain
6. Xeloda(R), Taxotere(R) and Herceptin(R) Combination Study Suggests Clinical Benefit in Invasive Breast Cancer with Shortened Treatment Cycle
7. 3-drug combination extremely promising as first-line therapy for multiple myeloma
8. Pharmion and MethylGene Present Favorable Clinical Data From Combination MGCD0103/Vidaza(R) Study in Myelodysplastic Syndromes and Acute Myelogenous Leukemia at The 49th American Society of Hematology (ASH) Meeting
9. ZOLINZA (vorinostat) in combination with bortezomib demonstrated clinical activity
10. Drug combination effective against multiple myeloma, researchers show
11. HIV drug resistance risk in mothers reduced by combination of common drugs
Post Your Comments:
(Date:10/13/2017)... ... ... Yisrayl Hawkins, Pastor and Overseer at The House of Yahweh, has released a ... books in the Holy Scriptures, Revelation. The Book of Revelation paints a picture of ... have tossed it off as mere rubbish, but Yisrayl Hawkins says that is because ...
(Date:10/12/2017)... ... October 12, 2017 , ... First Healthcare ... program management, will showcase a range of technology and learning solutions at the ... Convention and Expo to be held October 14–18, 2017 at the Mandalay Bay ...
(Date:10/12/2017)... ... 12, 2017 , ... The American College of Medical Informatics (ACMI) will present ... the Opening Session of AMIA’s Annual Symposium in Washington, D.C. AMIA’s Annual Symposium ... Collen, a pioneer in the field of medical informatics, this prestigious award is presented ...
(Date:10/12/2017)... ... October 12, 2017 , ... Leading pediatric oncology experts at ... D.C., for the 49th Congress of the International Society of Paediatric Oncology ... the Center for Cancer and Blood Disorders at Children’s National, and Stephen ...
(Date:10/12/2017)... ... October 12, 2017 , ... Vohra ... care advancements to physician colleagues, skilled nursing facility medical directors and other clinicians ... Waters of Wound Care." , "At many of these conferences we get to ...
Breaking Medicine News(10 mins):
(Date:9/27/2017)... , Sept. 27, 2017  DarioHealth Corp. (NASDAQ: DRIO), a leading ... announced that its MyDario product is expected to appear on The Dr. ... The Dr. Oz Show airs in your area: ... The nine-time Emmy award-winning, The Dr. Oz Show ... The segment ...
(Date:9/23/2017)... Janssen Biotech, Inc. (Janssen) announced today that ... U.S. Food and Drug Administration (FDA) for the Biologics ... treatment of moderately to severely active rheumatoid arthritis (RA). ... needed to further evaluate the safety of sirukumab in ... "We are disappointed ...
(Date:9/19/2017)... , Sept. 19, 2017 HistoSonics, Inc., a venture-backed medical device company developing a ... targeted tissues, announced three leadership team developments today:   ... ... ... Veteran medical device executive Josh Stopek , ...
Breaking Medicine Technology: