Effectiveness of Phosphodiesterase‐5 Inhibitor Therapy for Portopulmonary Hypertension

Fisher, J.H.; Chau, Cathy; Kron, Amie; Granton, John

doi:10.1155/2015/810376

Cited by 30 publications

(20 citation statements)

References 32 publications

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“…Small case series suggest that sildenafil improves functional capacity and increases cardiac output. [576][577][578] It should be noted that sildenafil can precipitate variceal bleeding and as such, caution should be exercised. 579 Prostacyclin analogues.…”

Section: Medical Treatmentmentioning

confidence: 99%

EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis

Angeli¹,

Bernardi

Villanueva³

et al. 2018

Journal of Hepatology

2,054

1,750

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The natural history of cirrhosis is characterised by an asymptomatic compensated phase followed by a decompensated phase, marked by the development of overt clinical signs, the most frequent of which are ascites, bleeding, encephalopathy, and jaundice. The following Clinical Practice Guidelines (CPGs) represent the first CPGs on the management of decompensated cirrhosis. In this context, the panel of experts, having emphasised the importance of initiating aetiologic treatment for any degree of hepatic disease at the earliest possible stage, extended its work to all the complications of cirrhosis, which had not been covered by the European Association for the Study of the Liver guidelines, namely: ascites, refractory ascites, hyponatremia, gastrointestinal bleeding, bacterial infections, acute kidney injury, hepatorenal syndrome, acute-on-chronic liver failure, relative adrenal failure, cirrhotic cardiomyopathy, hepatopulmonary syndrome, and porto-pulmonary hypertension. The panel of experts, produced these GPGs using evidence from PubMed and Cochrane database searches providing up to date guidance on the management of decompensated cirrhosis with the only purpose of improving clinical practice.

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Section: Medical Treatmentmentioning

confidence: 99%

EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis

Angeli¹,

Bernardi

Villanueva³

et al. 2018

Journal of Hepatology

2,054

1,750

View full text Add to dashboard Cite

show abstract

“…There are limited data evaluating the long-term survival of patients with PPH managed with medical therapy alone. Recent study from UK showed that phosphodiesterase 5 inhibitors were the most frequently used targeted therapy (63%) followed by prostacyclin analogs (12.7%), and endothelin receptor antagonists (10%) [32].…”

Section: Treatmentmentioning

confidence: 99%

“…Phosphodiesterase-5 inhibitor therapy is efficacious in other causes of WHO group I pulmonary arterial hypertension [32]. They inhibit the growth of pulmonary vascular smooth muscle cells and lower mean pulmonary artery pressure and pulmonary vascular resistance by mediating vasodilation through guanosine monophosphate [2,27].…”

Section: Phosphodiesterase 5 Inhibitorsmentioning

confidence: 99%

“…A recent single center retrospective study showed that sildenafil therapy resulted in improvement of WHO functional class with significant decrease in PVR, mPAP, and increase in cardiac output but no change in 6-min walk test over the period of 6 months treatment [32]. A recent retrospective study of all patients with PPH treated by oral pulmonary vasoactive drugs (PVD) (bosentan, ambrisentan, sildenafil, tadalafil) showed that oral PVD improved MPAP, PVR, and 6-min walk distance [36].…”

Section: Phosphodiesterase 5 Inhibitorsmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Complications of Liver Cirrhosis: A Concise Review

Than¹

2017

Liver Cirrhosis - Update and Current Challenges

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Pulmonary complications, in the form of hepatopulmonary syndrome (HPS), portopulmonary hypertension (PPH), and hepatic hydrothorax (HH), are rare occurrences in patients with portal hypertension and liver cirrhosis. These complications are associated with high morbidity and mortality. The only effective therapy is liver transplantation in patients who are suitable. In this chapter, each condition will be outlined in detail from clinical presentations to diagnosis and treatment as well as the challenges that clinicians may have encountered in managing patients with these complications.

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“…Both drugs appear to be well tolerated in POPH and do not adversely affect hepatic function. In the large Canadian sildenafil series (n = 20 POPH), treatment was successful (PVR decreased by a mean of -236 dynes.s.cm -5 at 6 months) and the drug was used in 2 patients with increased pulmonary artery wedge pressure (PAWP), but their outcome data was not reported 51 .…”

Section: Pde-5 Inhibitionmentioning

confidence: 99%

The Liver-Lung Vascular Connection

Krowka¹

2017

BRN

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Since the 1950's and 1960's it is well known that liver dysfunction can induce two distinct lung vascular disorders. We commonly refer to these disorders as hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). This concise review is intended to update the clinician on the current understanding and management of HPS and POPH that has evolved over the last 10 years. Each disorder is discussed in 5 parts: 1) diagnostic criteria/screening/clinical presentation; 2) pathophysiology; 3) epidemiology/natural history; 4) medical management; and 5) implications for liver transplant (LT). A summary table is provided to compare these clinically significant pulmonary vascular disorders.

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Effectiveness of Phosphodiesterase‐5 Inhibitor Therapy for Portopulmonary Hypertension

Abstract: Phosphodiesterase-5 inhibitor therapy improved hemodynamics and functional class at six months in a cohort of patients with portopulmonary hypertension.

Cited by 30 publications

References 32 publications

EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis

EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis

Pulmonary Complications of Liver Cirrhosis: A Concise Review

The Liver-Lung Vascular Connection

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