Portopulmonary hypertension in the current era of pulmonary hypertension management

Savale, Laurent; Guimas, M.; Ebstein, Nathan; Fertin, Marie; Jevnikar, Mitja; Renard, Sébastien; Horeau-Langlard, Delphine; Tromeur, Cécile; Chabanne, Céline; Prévôt, Grégoire; Chaouat, Ari; Moceri, Pamela; Artaud-Macari, Élise; Degano, Bruno; Trésorier, Romain; Boissin, Clément; Bouvaist, Hélène; Simon, Anne-Claire; Riou, Marianne; Favrolt, Nicolas; Palat, S.; Bourlier, Delphine; Magro, Pascal; Cottin, Vincent; Bergot, Emmanuel; Lamblin, Nicolas; Jaïs, Xavier; Coilly, Audrey; Durand, François; Francoz, Claire; Conti, Filoména; Hervé, Philippe; Simonneau, Gérald; Montani, David; Duclos‐Vallée, Jean‐Charles; Samuel, Didier; Humbert, Marc; Groote, P. De; Sitbon, Olivier

doi:10.1016/j.jhep.2020.02.021

Cited by 96 publications

(146 citation statements)

References 27 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 (14,(27)(28)(29)(30)32) *Unpublished data.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%

“…More recently, studies have found that PVR is more important than mPAP in predicting survival in POPH. (14,22) Additionally, studies suggest that LT can be safely accomplished in patients with an elevated mPAP if PVR is normal and right ventricular FIg. 1.…”

Section: Myth: Mpap Must Be <35 MM Hg To Enable a Safe Ltmentioning

confidence: 99%

“…(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%

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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: Mpap Must Be <35 MM Hg To Enable a Safe Ltmentioning

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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“…Historically, PoPH was thought to be 5-10% of all patients with PAH (10,11). More recently, French authors have reported that the proportion of newly diagnosed (incident) patients with PoPH is as high as 15% of all patients with PAH, and is continuing to rise as wider screening practices are adopted (12). PoPH is thought to be present in anywhere between 2 and 10% of patients with portal hypertension (6,13).…”

Section: Demographics and Clinical Characteristics Of Patients With Pophmentioning

confidence: 99%

“…Although the hemodynamic profile of patients with PoPH is better than in patients with idiopathic/familial PAH (IPAH/HPAH), their overall mortality is similar or worse (12,(20)(21)(22). The French Pulmonary Hypertension Registry (FHPR) shows a 1, 3, and 5-years survival of 84, 69, and 51%, respectively, for patients with PoPH (12), which is similar to the survival data for patients with IPAH, HPAH and anorexigen associated PAH (23). In contrast, analysis of the US based REVEAL registry showed that patients with PoPH had significantly worse survival when compared to patients with IPAH/FPAH: 67 vs. 85% at 2 years, and 40 vs. 64% at 5 years (Figure 1) (20).…”

Section: Demographics and Clinical Characteristics Of Patients With Pophmentioning

confidence: 99%

Portopulmonary Hypertension: From Bench to Bedside

et al. 2020

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Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies.

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Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease

Valle

DuBrock

2021

Comprehensive Physiology

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Portopulmonary hypertension in the current era of pulmonary hypertension management

Cited by 96 publications

References 27 publications

The Myths and Realities of Portopulmonary Hypertension

The Myths and Realities of Portopulmonary Hypertension

Portopulmonary Hypertension: From Bench to Bedside

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease

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