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:6/26/2016)... NC (PRWEB) , ... June 26, 2016 , ... Brent Kasmer, a legally blind and ... to be personalized through a fitness app. The fitness app plans to fix the two ... currently only offer a one size fits all type program , They don’t ...
(Date:6/25/2016)... ... ... Austin residents seeking Mohs surgery services, can now turn to Dr. Jessica Scruggs ... for medical and surgical dermatology. , Dr. Dorsey brings specialization to include Mohs surgery, ... Micrographic Surgery completed by Dr. Dorsey was under the direction of Glenn Goldstein, MD, ...
(Date:6/25/2016)... ... ... First Choice Emergency Room , the largest network of independent freestanding ... of its new Mesquite-Samuell Farm facility. , “We are pleased to announce Dr. ... James M. Muzzarelli, Executive Medical Director of First Choice Emergency Room. , Dr. ...
(Date:6/25/2016)... ... , ... On Friday, June 10, Van Mitchell, Secretary of the Maryland Department ... in recognition of their exemplary accomplishments in worksite health promotion. , The Wellness at ... Wellness Symposium at the BWI Marriott in Linthicum Heights. iHire was one of 42 ...
(Date:6/24/2016)... Marne, Michigan (PRWEB) , ... June 24, 2016 , ... ... To deal with these feelings, many turn to unhealthy avenues, such as drug or ... Center of Marne, Michigan, has released tools for healthy coping following a traumatic event. ...
Breaking Medicine News(10 mins):
(Date:6/23/2016)... Roche (SIX: RO, ROG; OTCQX: RHHBY) announced that it ... (procalcitonin) assay as a dedicated testing solution for people ... Roche is the first IVD company in the U.S ... assessment and management. PCT is a sepsis-specific ... blood can aid clinicians in assessing the risk of ...
(Date:6/23/2016)... MOUNTAIN VIEW, Calif. , June 23, 2016 ... 9:00 a.m. CST on Thursday, July 7, 2016 , , ... ) , , , , EXPERT PANELISTS:  ... Sciences, Nitin Naik; Senior Industry Analyst, Christi Bird; Senior Industry Analyst, ... The global pharmaceutical industry is witnessing ...
(Date:6/23/2016)... 2016 The vast majority of dialysis patients ... Treatments are usually 3 times a week, with treatment ... travel time, equipment preparation and wait time.  This regimen ... for patients who are elderly and frail.  Many elderly ... rehabilitation centers for some duration of time. ...
Breaking Medicine Technology: