Porres-Aguilar M, Bartolome SD, Duarte-Rojo A

Language: English
References: 7
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Text Extraction

Portopulmonary hypertension (PoPH) is a serious lung vascular complication of portal hypertension. Current definition criteria for PoPH include the following:

• Presence of portal hypertension with or without cirrhosis.
• Presence of pulmonary arterial hypertension (PAH) diagnosed by right heart catheterization (RHC); and absence of an alternative cause for PAH. Hemodynamic criteria for PAH, as based on RHC, must include a mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest, and pulmonary vascular resistance (PVR) › 240 dynes/ sec/cm^-5 [PVR = mPAP - PAOP/cardiac output (CO) x 80] or › 3 Wood units (Wood units= mPAP – PAOP/CO).^1,2 Ideally, pulmonary arterial occlusion pressure (PAOP) should be ≤ 15 mmHg, and whenever this criterion is not met, the transpulmonary gradient (TPG) must be › 12 mmHg, reflecting the presence of true PAH in association with venous hypertension.

REFERENCES

1. Rodriguez-Roisin R, Krowka MJ, Herve P, Fallon MB. Pulmonary- hepatic vascular disorders. Eur Respir J 2004; 24: 860-80.

2. Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Charlton MR, Duarte-Rojo A. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev 2012; 21: 223-33.

3. Krowka MJ, Miller DP, Barst RJ, Taichman D, Dweik RA, Badesch DB, McGoon MD. Portopulmonary hypertension: a report from the US-based REVEAL registry. Chest 2012; 141: 906-15.

4. Krowka MJ, Mandell MS, Ramsay MA, Kawut SM, Fallon MB, Manzarbeitia C, Pardo M Jr, et al. Hepatopulmonary syndrome and portopulmonary hypertension: a report of the multicenter liver transplant. Liver Transpl 2004; 10: 174-82.

5. Krowka MJ, Fallon MB, Mulligan DC, Gish RG. Model of endstage liver disease (MELD) exception for portopulmonary hypertension. Liver Transpl 2006; 12: S114-S116.

6. Goldberg DS, Batra S, Sahay S, Kawut SM, Fallon MB. MELD exceptions for portopulmonary hypertension: Current policy and future implementation. Am J Transplant 2014. Doi: 10.1111/ajt.12783 [Epub ahead of print].

7. Porres-Aguilar M, Mukherjee D. Cardiopulmonary hemodynamics for accurate diagnosis of portopulmonary hypertension: A redefinition to consider. Hepatology 2014. Doi: 10.1002/hep.27234 [Epub ahead of print].