Assessment and management of patients with portopulmonary hypertension undergoing liver transplantation - 08/08/23
, Michael Toolan a, Jeremy S Nayagam b, Deepak Joshi c, Brian J Hogan d, Colm McCabe e, f, Philip Marino g, Sameer Patel h, i
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Highlights |
• | PoPH occurs in 5-8.5% of LT candidates and has important implications for the eligibility, feasibility and outcomes of LT. |
• | Early recognition of PoPH is key - screen all LT candidates with TTE and confirm diagnosis and assess severity with RHC. |
• | PAH-specific pharmacological therapy can improve haemodynamics, facilitate eligibility for LT and reduce mortality. |
• | Improvement in pulmonary haemodynamics and the discontinuation of PAH-specific pharmacotherapy is often achievable post-LT. |
• | Further research required to define optimal risk stratification and pharmacotherapy strategies in LT candidates with PoPH. |
Abstract |
Portopulmonary hypertension (PoPH) is defined as the presence of otherwise unexplained pre-capillary pulmonary hypertension in patients with portal hypertension of cirrhotic or non-cirrhotic aetiology. PoPH occurs in at least 5–8.5% of patients being worked up for a liver transplant (LT) and its prevalence is thought to be increasing. Uncontrolled PoPH prior to LT is associated with high perioperative morbidity and mortality, with severe PoPH being considered a contraindication to LT. Early recognition and appropriate management of PoPH in patients being considered for LT is therefore imperative to achieve optimal outcomes. This review provides a detailed overview of: the epidemiology, prognosis and pathophysiology of PoPH; clinical assessment, screening and diagnostic approach; and pre-, peri‑ and post-transplant management of PoPH in patients undergoing LT. The current evidence base in this area is limited. This review particularly focuses on the evidence both supporting and challenging current practices and highlights areas for future research.
Le texte complet de cet article est disponible en PDF.Keywords : Portopulmonary hypertension, liver transplantation, pulmonary arterial hypertension, preoperative evaluation, preoperative optimization, postoperative complications
Abbreviations : 6MWD, BMP9, BNP, cGMP, CI, CpcPH, CO, DLCO, DPG, ECG, ECMO, ERA, ERS, ESC, ET-1, FAC, FPAH, HPS, ILTS, IPAH, IpcPH, IPVD, IVC, LT, MAA, MAP, MELD, mPAP, mRAP, PA, PADP, PAH, PASP, PAWP, PDE5, PFT, PH, PoPH, PRS, PVR, RA, RAD, RBBB, RCT, RHC, RV, RVH, RVSP, sGC, TAPSE, TBS, TIPSS, TOE, TPG, TRV, TTE, UKELD, WHO, WU
Plan
Vol 12
Article 100169- novembre 2023 Retour au numéro
Article précédent
- Contents
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