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Year : 2008
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: 11 | Issue : 2 | Page
: 140 |
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Authors' reply |
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Shahzad G Raja
Department of Cardiothoracic Surgery, Harefield Hospital, Hill End Road, Harefield, Middlesex UB9 6JH, United Kingdom
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How to cite this article: Raja SG. Authors' reply. Ann Card Anaesth 2008;11:140 |
The Editor,
The readers of our article have highlighted another emerging indication of bosentan therapy. [1] Portopulmonary hypertension (PPHT) occurs in 2% to 8% of the patients with cirrhosis. Imbalance between vasodilating (decreased pulmonary expression of eNOS and prostacyclin I2) and vasoconstrictive agents (increased expression of endothelin[ET]-1 and angiotensin 1) may be responsible for misguided angiogenesis and pulmonary hypertension. Although prostacyclin analogues are efficacious, adverse effects in terms of safety, tolerability, and drug delivery occur. Bosentan could perhaps be the therapy of choice for patients with PPHT because it not only decreases pulmonary hypertension but also diminishes portal hypertension. [2] Adding further to what the readers have mentioned in their letter, I can confirm that recently, low-dose bosentan therapy (initially twice, 31.25 mg/day; and then, 62.5 mg/day) has also been reported to be effective for the treatment of portopulmonary hypertension and renal insufficiency in a patient with Child C cirrhosis as well. [3]
However, it is important to remember that presently there are few data on effects of long-term bosentan treatment for this new indication. The available evidence contributes to a building sense of excitement that bosentan may be an effective treatment in PPHT. However, the safety and dosage of bosentan for this new indication has still not been established. Moreover, bosentan therapy for this novel indication will require continuous exposure to possibly larger doses, suggesting that adverse effects may be more widespread, especially given that the ET receptors are distributed in a variety of tissues. Because so many novel therapies in the past have not lived up to their initial promise, we should protect our patients (and ourselves) and refrain from empirically administering bosentan for this emerging indication at present. Perhaps it will be prudent to wait for the results of a rigorous, blinded, placebo-controlled, multicentre randomised clinical trial confirming efficacy and safety of long-term bosentan use for treatment of PPHT before routinely prescribing it for all patients with cirrhosis.
References | |  |
1. | Tempe DK, Datt V, Datta D. Bosentan for the treatment of portopulmonary hypertension. Ann Cardiac Anaesth 2008;11:139. |
2. | Colle I, Van Steenkiste C, Geerts A, Van Vlierberghe H. Hepatopulmonary syndrome and portopulmonary hypertension: whats new? Acta Gastroenterol Belg 2007;70:203-9. [PUBMED] |
3. | Barth F, Gerber PJ, Reichen J, Dufour JF, Nicod LP. Efficiency and safety of bosentan in child C cirrhosis with portopulmonary hypertension and renal insufficiency. Eur J Gastroenterol Hepatol 2006;18:1117-9. [PUBMED] [FULLTEXT] |

Correspondence Address: Shahzad G Raja Department of Cardiothoracic Surgery, Harefield Hospital, Hill End Road, Harefield, Middlesex UB9 6JH United Kingdom
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-9784.41596

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