Long‐term outcome in liver transplantation candidates with portopulmonary hypertension

Savale, Laurent; Sattler, Caroline; Coilly, Audrey; Conti, Filoména; Renard, Sébastien; Francoz, Claire; Bouvaist, Hélène; Féray, Cyrille; Borentain, Patrick; Jaïs, Xavier; Montani, David; Parent, Florence; O’Connell, Caroline; Hervé, Philippe; Humbert, Marc; Simonneau, Gérald; Samuel, Didier; Calmus, Yvon; Duvoux, Christophe; Durand, François; Duclos-Vallée, J.-C.; Sitbon, Olivier

doi:10.1002/hep.28990

Cited by 73 publications

(65 citation statements)

References 32 publications

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“…Included studies that reported survival data from a minimum of 20 patients. (14,(27)(28)(29)(30)32) *Unpublished data. Abbreviations: PH, pulmonary hypertension; N/A, not applicable.…”

Section: Discussionmentioning

confidence: 99%

“…(26,27) Post-LT resolution of POPH has been described, but clinical and hemodynamic worsening after LT has also been described. (28) Consequently, decisions regarding whether or not to pursue LT in patients with POPH, particularly in those without decompensated liver disease, are challenging. Recently, several studies have been published describing longterm outcomes of POPH following LT. (14,(27)(28)(29)(30) Although these experiences span a period of decades over which PAH therapeutic options have dramatically evolved, the cumulative reported experience in these studies has improved our understanding of both short-and long-term outcomes following LT. Posttransplant survival rates from these studies are summarized in Table 1.…”

Section: Myth: Little Is Known Regarding Long-term Posttransplant Outmentioning

confidence: 99%

“…In patients that survive this high-risk period, ~50% achieve a clinical and hemodynamic "cure" and are able to discontinue PAH therapy posttransplant without adverse effects. (28,31) Unfortunately, the individual response to LT, which ranges from clinical worsening to resolution of POPH, is unpredictable. According to a recent systematic review of the literature, the combination of PAHtargeted therapy and LT conferred the best prognosis in patients with POPH when LT was considered safe and feasible.…”

Section: Myth: Little Is Known Regarding Long-term Posttransplant Outmentioning

confidence: 99%

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The Myths and Realities of Portopulmonary Hypertension

DuBrock

Krowka

2020

Hepatology

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“…Included studies that reported survival data from a minimum of 20 patients. (14,(27)(28)(29)(30)32) *Unpublished data. Abbreviations: PH, pulmonary hypertension; N/A, not applicable.…”

Section: Discussionmentioning

confidence: 99%

Section: Myth: Little Is Known Regarding Long-term Posttransplant Outmentioning

confidence: 99%

Section: Myth: Little Is Known Regarding Long-term Posttransplant Outmentioning

confidence: 99%

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The Myths and Realities of Portopulmonary Hypertension

DuBrock

Krowka

2020

Hepatology

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“…Swanson et al reported superior 5‐year survival in patients receiving medical therapy before LT (67%) compared to patients who did not (25%). ( 1 ) Analysis of the other reports indicated that post‐LT outcome was numerically, although not statistically, better in studies including only patients who were pretreated ( 17,32 ) compared to studies reporting on a substantial ( 36 ) or intermediate ( 1,34 ) proportion of patients who were not medically pretreated. Pooled estimate rates of posttransplant deaths were 24% (95% CI, 15‐36) in pretreated patients versus 41% (95% CI, 21‐65) in patients who did not receive VM therapy (Q = 2.00; P = 0.16; Fig.…”

Section: Resultsmentioning

confidence: 97%

“…Eleven studies were performed in Europe, ( 15,23,25,29‐36 ) 13 in the United States, ( 1,13,14,16‐18,20‐22,24,26‐28 ) one in Canada, ( 19 ) and one in Europe/United States/Brazil (the only RCT ( 37 ) ). Two studies reported on the outcome in untreated patients with PoPH.…”

Section: Resultsmentioning

confidence: 99%

Vasomodulators and Liver Transplantation for Portopulmonary Hypertension: Evidence From a Systematic Review and Meta‐Analysis

et al. 2020

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Background and Aims Untreated portopulmonary hypertension (PoPH) carries a poor prognosis. Previous reports have described vasomodulator (VM) therapy and liver transplantation (LT) as treatment options. We aimed to provide summary estimates on the endpoints of pulmonary hemodynamics and survival in patients with PoPH, treated with different modalities. Approach and Results We performed a systematic review with meta‐analysis of mainly observational and case‐control studies describing no treatment, VM, LT, or VM + LT in patients with PoPH. Twenty‐six studies (1,019 patients) were included. Both VM and VM + LT improve pulmonary hemodynamics. A substantial proportion of patients treated with VM become eligible for LT (44%; 95% confidence interval [CI], 31‐58). Pooled estimates for 1‐, and 3‐year postdiagnosis survival in patients treated with VM were 86% (95% CI, 81‐90) and 69% (95% CI, 50‐84) versus 82% (95% CI, 52‐95) and 67% (95% CI, 53‐78) in patients treated with VM + LT. Of note, studies reporting on the effect of VM mainly included Child‐Pugh A/B patients, whereas studies reporting on VM + LT mainly included Child‐Pugh B/C. Seven studies (238 patients) included both patients who received VM only and patients who received VM + LT. Risk of death in VM‐only–treated patients was significantly higher than in patients who could be transplanted as well (odds ratio, 3.5; 95% CI, 1.4‐8.8); however, importantly, patients who proceeded to transplant had been selected very strictly. In 50% of patients, VM can be discontinued post‐LT (95% CI, 38‐62). Conclusions VM and VM + LT both improve pulmonary hemodynamics and prognosis in PoPH. In a strictly selected subpopulation of cases where LT is indicated based on severe liver disease and where LT is considered safe and feasible, treatment with VM + LT confers a better prognosis. Considering successful VM, 44% can proceed to LT, with half being able to postoperatively stop medication.

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Diagnosis and Treatment of Pulmonary Arterial Hypertension

Ruopp

Cockrill

2022

JAMA

241

144

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ulmonary arterial hypertension (PAH) is a life-threatening disorder characterized by elevated pressure in the pulmonary arteries due to increased pulmonary vascular resistance. 1 Symptoms of PAH are nonspecific but commonly include dyspnea on exertion and fatigue. 2 The estimated prevalence of PAH is 10.6 per 1 million adults in the US. Untreated, PAH typically progresses to right ventricular failure and death. 3 Treatment has significantly improved outcomes in the last decade. 4 The diagnosis should be considered in any patient presenting with unexplained exertional dyspnea. This Review summarizes current evidence regarding diagnosis and treatment of PAH. MethodsPubMed was searched for English-language articles from 1985 through December 30, 2021, using search terms for pulmonary arterial hypertension. Articles were selected for inclusion based on relevance to current clinical practice. Randomized clinical trials, large longitudinal observational studies, and more recent articles were prioritized. Bibliographies of retrieved articles were searched for other relevant articles. Of the articles identified, 99 were included, consisting of 23 randomized clinical trials, 3 meta-analyses and systematic reviews, 36 observational studies, 16 registry-based studies, and 21 clinical practice guidelines or other reports. Definition and Classification of Pulmonary HypertensionThe clinical classification of pulmonary hypertension (PH), of which PAH is a subtype, is important for understanding the approach to both diagnosis and treatment of PAH. Pulmonary hypertension is currently defined by a mean pulmonary artery pressure greater than IMPORTANCE Pulmonary arterial hypertension (PAH) is a subtype of pulmonary hypertension (PH), characterized by pulmonary arterial remodeling. The prevalence of PAH is approximately 10.6 cases per 1 million adults in the US. Untreated, PAH progresses to right heart failure and death.OBSERVATIONS Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 20 mm Hg and is classified into 5 clinical groups based on etiology, pathophysiology, and treatment. Pulmonary arterial hypertension is 1 of the 5 groups of PH and is hemodynamically defined by right heart catheterization demonstrating a mean pulmonary artery pressure greater than 20 mm Hg, a pulmonary artery wedge pressure of 15 mm Hg or lower, and a pulmonary vascular resistance of 3 Wood units or greater. Pulmonary arterial hypertension is further divided into subgroups based on underlying etiology, consisting of idiopathic PAH, heritable PAH, drug-and toxin-associated PAH, pulmonary veno-occlusive disease, PAH in long-term responders to calcium channel blockers, and persistent PH of the newborn, as well as PAH associated with other medical conditions including connective tissue disease, HIV, and congenital heart disease. Early presenting symptoms are nonspecific and typically consist of dyspnea on exertion and fatigue. Currently approved therapy for PAH consists of drugs that enhance the nitric oxide-cyclic guanosine m...

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Long‐term outcome in liver transplantation candidates with portopulmonary hypertension

Abstract: Stabilization or reversibility of PoPH seems to be an attainable goal using the combination of pulmonary arterial hypertension-targeted therapies and LT in patients who are transplantation candidates. (Hepatology 2017;65:1683-1692).

Cited by 73 publications

References 32 publications

The Myths and Realities of Portopulmonary Hypertension

The Myths and Realities of Portopulmonary Hypertension

Vasomodulators and Liver Transplantation for Portopulmonary Hypertension: Evidence From a Systematic Review and Meta‐Analysis

Diagnosis and Treatment of Pulmonary Arterial Hypertension

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