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

2021 ◽  
pp. 1-22
Author(s):  
Kathryn del Valle ◽  
Hilary M. DuBrock
Keyword(s):  
Liver Disease ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Portopulmonary Hypertension

scholarly journals Transition from Hepatopulmonary Syndrome to Portopulmonary Hypertension: A Case Series of 3 Patients

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Radhika Zopey ◽  
Irawan Susanto ◽  
Igor Barjaktarevic ◽  
Tisha Wang
Keyword(s):  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Treatment Plan ◽  
Case Series ◽  
Vascular Complications ◽  
Subsequent Development ◽  
Hepatopulmonary Syndrome ◽  
Portopulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Differential Binding

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are the two major pulmonary vascular complications of liver disease. While HPS is characterized by low pulmonary vascular resistance, PPHTN is defined by the presence of elevated pulmonary vascular resistance. Given these seemingly opposing pathophysiologic mechanisms, these conditions were traditionally felt to be mutually exclusive. In this series, we present three patients with severe hepatopulmonary syndrome who had spontaneous resolution of their HPS with the subsequent development of PPHTN. To our knowledge, this is the largest case series presented of this phenomenon in nontransplanted patients. One proposed mechanism for the occurrence of this phenomenon involves dysregulation of the same vascular signaling pathway, which may lead to both pulmonary vascular dilatations and pulmonary arterial remodeling in the same patient. Another theory involves the possible differential binding of endothelin-1, a vasoactive signaling peptide that induces vasoconstriction when bound to receptor A and vasodilation when bound to receptor B. Although the mechanisms for this phenomenon remain unclear, it is important to be vigilant of this phenomenon as it may change the patient's overall treatment plan, especially in regard to appropriateness and timing of liver transplant.

Impact of Hepatopulmonary Syndrome in Liver Transplantation Candidates and the Role of Angiogenesis

2021 ◽  
pp. 2102304
Author(s):  
Steven M. Kawut ◽  
Michael J. Krowka ◽  
Kimberly A. Forde ◽  
Nadine Al-Naamani ◽  
Karen L. Krok ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Functional Class ◽  
Hazard Ratio ◽  
Arterial Oxygen ◽  
Risk Of Death ◽  
Increased Risk

Hepatopulmonary syndrome affects 10–30% of patients with cirrhosis and portal hypertension. We evaluated the serum angiogenic profile of hepatopulmonary syndrome and assessed the clinical impact of hepatopulmonary syndrome in patients evaluated for liver transplantation.The Pulmonary Vascular Complications of Liver Disease 2 study was a multicentre, prospective cohort study of adults undergoing their first liver transplantation evaluation. Hepatopulmonary syndrome was defined as an alveolar-arterial oxygen gradient ≥15 mmHg (≥20 mmHg if age >64 years), positive contrast-enhanced transthoracic echocardiography, and absence of lung disease.We included 85 patients with hepatopulmonary syndrome and 146 patients without hepatopulmonary syndrome. Patients with hepatopulmonary syndrome had more complications of portal hypertension and slightly higher Model for End-stage Liver Disease-Na score compared to those without hepatopulmonary syndrome (median [interquartile range] 15 [12, 19] versus 14 [10, 17], p=0.006). Hepatopulmonary syndrome patients had significantly lower six minute walk distance and worse functional class. Hepatopulmonary syndrome patients had higher circulating angiopoietin-2, Tie2, tenascin-C, c-kit, VCAM-1, and von Willebrand factor levels, and lower E-selectin levels. Patients with hepatopulmonary syndrome had an increased risk of death (hazard ratio 1.80 [1.03–3.16], p=0.04) which persisted despite adjustment for covariates (hazard ratio 1.79 [1.02–3.15], p=0.04). This association did not vary based on levels of oxygenation reflecting the severity of hepatopulmonary syndrome.Hepatopulmonary syndrome was associated with a profile of abnormal systemic angiogenesis, worse exercise and functional capacity, and an overall increased risk of death.

scholarly journals Recent advances in the approach to hepatopulmonary syndrome and portopulmonary hypertension

2021 ◽  
Vol 84 (1) ◽  
pp. 95-99
Author(s):  
S Raevens ◽  
A Geerts ◽  
L Devisscher ◽  
H Van Vlierberghe ◽  
C Van Steenkiste ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Negative Impact ◽  
Vascular Complications ◽  
Hepatopulmonary Syndrome ◽  
Medical Intervention ◽  
Pulmonary Vascular Disease ◽  
Portopulmonary Hypertension ◽  
Pulmonary Vasodilation ◽  
Recent Advances ◽  
Impact On Survival

Liver disease, cirrhosis and portal hypertension can be complicated by pulmonary vascular disease, which may affect prognosis and influence liver transplantation (LT) candidacy. Pulmonary vascular complications comprise hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). Although these two conditions develop on a same background and share a common trigger, pulmonary responses are distinct and occur at different anatomical sites of the pulmonary circulation. HPS affects 10-30% of patients referred for LT, and is characterized by gas exchange abnormalities due to pulmonary vasodilation and right-to-left shunting. POPH occurs in 5%, and is defined by pulmonary arterial hypertension due to increased pulmonary vascular resistance, which leads to hemodynamic failure. Even though HPS and POPH may have a substantial negative impact on survival, both entities are clinically underrecognized and frequently misdiagnosed. Without intervention, the 5-year survival rate is 23% in HPS and 14% in POPH. Their presence should be actively sought by organized screening in patients presenting with dyspnea and in all patients on the waitlist for LT, also because clinical symptoms are commonly non-specific or even absent. LT may lead to resolution, however, advanced stages of either HPS or POPH may jeopardize safe and successful LT. This implicates the need of proper identification of HPS and POPH cases, as well as the need to be able to successfully ‘bridge’ patients to LT by medical intervention. A review article on this topic has been published in this journal in 2007 (1). This updated review focuses on recent advances in the diagnosis and management of these 2 liver-induced pulmonary vascular disorders and incorporates results from our recent work.

scholarly journals Estrogen Signaling and Portopulmonary Hypertension: The Pulmonary Vascular Complications of Liver Disease Study (PVCLD2)

Hepatology ◽  
2020 ◽  
Author(s):  
Nadine Al‐Naamani ◽  
Michael J. Krowka ◽  
Kimberly A. Forde ◽  
Karen L. Krok ◽  
Rui Feng ◽  
...  
Keyword(s):  
Liver Disease ◽  
Vascular Complications ◽  
Estrogen Signaling ◽  
Portopulmonary Hypertension ◽  
Disease Study

The lung in liver disease: hepatopulmonary syndrome and portopulmonary hypertension

2019 ◽  
pp. 262-277
Author(s):  
Sebastiano Emanuele Torrisi ◽  
Valentin Fuhrmann ◽  
Dirk Skowasch ◽  
Michael Kreuter
Keyword(s):  
Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Portopulmonary Hypertension

Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism

2021 ◽  
Vol 14 (9) ◽  
pp. e244805
Author(s):  
Eman Alabsawy ◽  
Yassen Serry ◽  
Sreelakshmi Kotha ◽  
Philip Berry ◽  
Giovanni Tritto
Keyword(s):  
Liver Disease ◽  
Fatty Liver Disease ◽  
Hepatopulmonary Syndrome ◽  
Progressive Dyspnoea ◽  
Alcoholic Fatty Liver ◽  
End Stage Liver Disease ◽  
Pituitary Dysfunction ◽  
Vascular Dilatation ◽  
End Stage ◽  
Alcoholic Fatty Liver Disease

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%–47%. Chronic liver disease has been described in patients with hypothalamic–pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

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