Peer Review #2 of "Role of echocardiography in screening for portopulmonary hypertension in liver transplant candidates: a meta-analysis (v0.1)"
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Objectives.To demonstrate the screening value of echocardiography for portopulmonary hypertension (POPH) in liver transplant candidates. Design. Systematic review and meta-analysisBackground.POPH is a complication of end-stage liver disease that adversely affects the outcome of orthotopic liver transplant.There are no specific symptoms in the early stage of POPH.POPH reduce the survival rate of patients with end-stage liver disease specially if they are not diagnosed.Therefore, early detection may improve prognosis.The objective of this study is to explore the screening value of echocardiography on liver transplant candidates for screening of POPH compared to right heart catheterization (RHC).Method.PubMed, EMBASE and the Cochrane Library were searched by two independent reviewers for potentially eligible studies published up to 30 June 2019 to retrieve data based on per-patient analysis.STATA, Meta-DiSc, and RevMan were applied to perform this meta-analysis. Results.Our search yielded 1576 studies, of which 11 satisfied the inclusion criteria.The pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR) and area under the summary receiver operating characteristic (SROC) curve (AUC) of echocardiography for POPH were 0.85 (95% CI, 0.65-0.94),0.83 (95% CI, 0.73-0.90),4.99 (95% CI, 3.03-8.21),0.19 (95% CI, 0.07-0.46),and 0.91 (95% CI, 0.88-0.93),respectively.Deeks' funnel plot did not indicate the existence of publication bias (P= 0.66).Conclusions.Echocardiography, a noninvasive modality, provides superior screening for POPH, but the diagnosis of POPH still requires RHC.Keywords:
Portopulmonary hypertension
Portopulmonary hypertension
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Key Points 1 How do physicians decide which patients with pulmonary vascular disease will benefit from liver transplantation? 2 Studies on patients with pulmonary vascular disease are limited and the findings and recommendations may not apply to all practice sites. 3 All patients with hypoxemia, liver disease, and pulmonary vasodilation do not have hepatopulmonary syndrome (HPS). 4 Not all patients with hepatopulmonary syndrome will benefit from liver transplantation. 5 The mean pulmonary artery pressure (mPAP) may not be an accurate predictor of mortality in patients with portopulmonary hypertension. 6 The effects of pulmonary vasodilators on the outcome of patients with portopulmonary hypertension (PPHTN) is still unconfirmed but promising. (Liver Transpl 2004;10:S54–S58.)
Portopulmonary hypertension
Hepatopulmonary syndrome
Liver disease
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We report a case of portopulmonary hypertension in which the pulmonary hypertension resolved after initial orthotopic liver transplantation. Portopulmonary hypertension recurred when the transplanted liver failed and again resolved after a second liver transplantation. Intravenous epoprostenol was administered perioperatively to control the pulmonary hypertension in both instances.
Portopulmonary hypertension
Orthotopic liver transplantation
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Portopulmonary hypertension
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Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are distinct clinical entities that may complicate liver disease. Although HPS and PPHTN are different, several reports describe 6 patients in whom both conditions have occurred, either concurrently or sequentially, sometimes with the onset of PPHTN after liver transplantation. The current report extends this sparse experience by reporting 2 patients who underwent liver transplantation for HPS and who developed pulmonary hypertension after liver transplantation. This experience calls for better understanding of the pathogenesis of HPS and PPHTN and ways to better predict their occurrence. Liver Transpl 12:1278-1282, 2006. © 2006 AASLD.
Hepatopulmonary syndrome
Portopulmonary hypertension
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A A A A AA A A A A A A A A AA A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A A A A A A A A A A A AA A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A A
Sudden Death
Safeguarding
Clustering coefficient
Socialization
Gratification
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Pulmonary arterial hypertension (PAH) that occurs in the setting of cirrhosis and portal hypertension is referred to as portopulmonary hypertension (PPHTN). Liver transplantation (LTx) is curative, but the presence of moderate-to-severe PPHTN may be a contraindication for transplantation because of the elevated risk of peri- and post-transplantation morbidity and mortality. We report a successful liver transplantation in a patient with liver cirrhosis after treatment of moderate-to-severe PPHTN with a combination of the dual endothelin receptor antagonist bosentan and the specific phosphodiesterase-5 inhibitor sildenafil.
Portopulmonary hypertension
Contraindication
Endothelin receptor antagonist
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Portopulmonary hypertension (PPHTN) is one of the most devastating consequences of end-stage liver cirrhosis. When a patient has moderate to severe PPHTN, his or her candidature for liver transplantation is denied. Here we report a successful adult-to-adult living donor liver transplantation (LDLT) in a patient with moderate to severe PPHTN. The patient was a 58-yr-old female who was diagnosed with end-stage liver cirrhosis due to chronic hepatitis C. Preoperative evaluation revealed that the patient had moderate to severe PPHTN. Her mean pulmonary artery pressure (mPAP) was 35-47 mmHg without treatment. Continuous epoprostenol therapy was introduced to lower the mPAP. She underwent LDLT using an extended right hepatic lobe graft which was donated by her daughter. Prolonged artificial ventilation was necessary until postoperative day (POD) 25, after which her general condition gradually improved. By POD 72, she was in good condition and was allowed to leave the hospital. Currently, 1 yr after the operation, she visits the outpatient clinic regularly and enjoys a normal life. It should be noted, however, that the PPHTN markedly improved but did not completely resolve, as assessed by right heart catheterization 1 yr after successful LDLT. Liver Transpl 12:481–484, 2006. © 2006 AASLD.
Portopulmonary hypertension
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Summary Background Liver transplantation is potentially a life‐saving therapeutic intervention for patients with portopulmonary hypertension and hepatopulmonary syndrome. However, due to limited data, listing criteria for patients with these conditions have not been clearly established. Indeed, this has led some to speculate that transplantation may not be appropriate in cases of moderate‐to‐severe portopulmonary hypertension and severe hepatopulmonary syndrome. Aim To critically discuss the utility of LT for the treatment of hepatopulmonary syndrome and portopulmonary hypertension. Methods A literature search was conducted in 2012 on PubMed, Ovid Embase, Ovid Medline and Scopus using the following search terms: hepatopulmonary syndrome, portopulmonary hypertension, pulmonary arterial hypertension, liver transplantation. Relevant manuscripts were included in the review. Results Liver transplantation has established itself as an effective treatment for selected patients with hepatopulmonary syndrome and portopulmonary hypertension. A multidisciplinary team approach incorporating focused strategies (both pre‐ and post‐operatively) aimed at improving oxygenation in patients with hepatopulmonary syndrome has led to a dramatic improvement in patient outcomes. Additionally, careful patient selection and the use of targeted pulmonary vascular therapies are successfully being used to treat portopulmonary hypertension and ‘bridge’ patients to successful liver transplantation. Conclusions Liver transplantation is an effective therapy for patients with hepatopulmonary syndrome and portopulmonary hypertension. However, rigorous screening and early identification of these conditions allied with aggressive pre‐operative optimisation of physiology and diligent post‐operative care are imperative to ensuring a good outcome.
Portopulmonary hypertension
Hepatopulmonary syndrome
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Hepatopulmonary syndrome and portopulmonary hypertension are the most common pulmonary vascular complications in patients with cirrhosis. Usually but not universally mutually exclusive, they each may present prior to liver transplantation and, if severe enough, may be a contraindication to transplant. However, there have been a number of case reports describing patients developing pulmonary hypertension de novo after liver transplantation. This report describes one such patient from our institution and reviews the medical literature describing this unusual clinical entity.
Portopulmonary hypertension
Contraindication
Hepatopulmonary syndrome
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