Portopulmonary Hypertension

2017 ◽  
Vol 38 (05) ◽  
pp. 651-661 ◽  
Author(s):  
Jason Watherald ◽  
Olivier Sitbon ◽  
Laurent Savale
Keyword(s):  
Heart Failure ◽  
Portal Hypertension ◽  
Right Heart Failure ◽  
Pulmonary Vasculature ◽  
Heart Catheterization ◽  
Portopulmonary Hypertension ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Ventricular Afterload ◽  
The Impact

AbstractPortal hypertension may have major consequences on the pulmonary vasculature due to complex pathophysiological interactions between liver and lungs. Portopulmonary hypertension (PoPH) is characterized by the association of portal hypertension and pulmonary arterial hypertension (PAH). As progressive elevation of right ventricular afterload can lead to right heart failure, PoPH is a serious complication of portal hypertension, affecting functional status and prognosis of patients. Early detection by transthoracic echocardiography must be performed in symptomatic patients and in candidates for liver transplantation (LT). Right heart catheterization remains mandatory to confirm the diagnosis and exclude all other causes of elevated pulmonary pressures. The management of PoPH includes PAH-targeted therapies although few studies have evaluated these medications in this specific indication. The impact of LT on PoPH outcome remains complex and must be specified by future collaborative investigation. Although uncontrolled PoPH is associated with higher risk of postoperative right heart failure and death, stabilization, improvement, or normalization of pulmonary hemodynamics after LT seem to be achievable goals in selected patients with PoPH.

Portopulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2534-2537
Author(s):  
Olivier Sitbon ◽  
Laurent Savale
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Hepatopulmonary Syndrome ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Channel Blockers ◽  
Portopulmonary Hypertension ◽  
Right Heart ◽  
Pulmonary Haemodynamics

Portal hypertension, with or without liver disease, may have major consequences on pulmonary circulation due to complex pathophysiological interactions between the liver and the lungs. There are two distinct pulmonary vascular disorders associated with portal hypertension: hepatopulmonary syndrome, characterized by gas exchange impairment due to intrapulmonary shunts, and portopulmonary hypertension (PoPH), which is a particular form of pulmonary arterial hypertension (PAH). PoPH is a severe complication of portal hypertension, affecting functional status, exercise capacity, and survival of affected patients. Detection of PoPH by transthoracic echocardiography must be performed in symptomatic patients and in all candidates for liver transplantation. Right heart catheterization is mandatory to confirm the diagnosis of PoPH and exclude other causes of pulmonary artery pressure elevation, including fluid overload and high cardiac output, that are common features in portal hypertension. The management of PoPH is similar to that of other forms of PAH although very few trials have investigated the effects of PAH-targeted therapies in this particular indication. Anticoagulants and calcium channel blockers are usually not indicated in this setting. PAH-targeted medications can be used to improve pulmonary haemodynamics and bridge patients with severe PoPH to liver transplantation. However, the impact of liver transplantation on outcomes of patients with PoPH remains unpredictable. While uncontrolled PoPH is associated with a higher risk of perioperative right heart failure and death, stabilization, improvement, or normalization of pulmonary haemodynamics after liver transplantation seem to be achievable goals in selected patients with PoPH.

Pulmonary Hypertension Associated With Myocardial Amyloid Degeneration: a Case Report

2021 ◽  
Author(s):  
Lu Yan ◽  
Qixian Zeng ◽  
Changming Xiong ◽  
Zhihui Zhao ◽  
Qing Zhao ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Blood Vessels ◽  
Amyloid Fibrils ◽  
Right Heart Failure ◽  
Pulmonary Artery Hypertension ◽  
Pulmonary Vasculature ◽  
Vascular Involvement ◽  
Right Heart ◽  
Protein Deposits

Abstract Background: There is very little literature on Pulmonary hypertension associated with myocardial amyloid degeneration. At present, only 10 cases pulmonary hypertension cased by amyloid protein deposits in the pulmonary blood vessels have been reported by Eder et al. We reported a case that the patient was pulmonary artery hypertension combined with myocardial amyloid change. It’s aim to claims that pulmonary hypertension is most likely caused by amyloid fibrin deposition in pulmonary blood vessels.Case presentation: We report a case of a 65-year-old male patient with with AL and ATTR combined type amyloidosis who developed right heart failure because of severe pulmonary hypertension. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon finding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis.Conclusion: we present a men with amyloidosis who developed dyspnea and right heart failure and was diagnosed with pulmonary hypertension, most probably secondary to pulmonary vascular involvement by amyloid fibrils.

scholarly journals Risk stratification in PAH

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Paul A Corris
Keyword(s):  
Heart Failure ◽  
Pulmonary Arteries ◽  
Right Heart Failure ◽  
Response To Therapy ◽  
Pulmonary Vasculature ◽  
Short Term ◽  
Right Heart ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
Short Term Survival

[No abstract. Showing first paragraph of article]Pulmonary arterial hypertension (PAH) is a chronic disease of the pulmonary vasculature characterized by progressive narrowing of the pulmonary arteries leading to increased pulmonary vascular resistance, right heart failure, and ultimately premature death.There has been a significant improvement in the available medical therapeutic options in this field that have impacted the short-term survival and morbidity in these patients. However, the median survival post-diagnosis remains unacceptable at 7 years.Physicians’ ability to predict PAH disease progression and risk allows them to determine the patient’s prognosis, make informed adjustments to therapy, and monitor his or her response to therapy . If widely adopted, risk prediction can enhance the consistency of treatment approaches and improve the timeliness of referral for lung transplantation. This approach should lead optimal, directed care that ultimately reduces morbidity and improves mortality in patients with PAH.

scholarly journals Rapidly progressive respiratory failure after helminth larvae ingestion

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Anahit A. Zeynalyan ◽  
Balaji Kolasani ◽  
Chetan Naik ◽  
Christopher J. G. Sigakis ◽  
Leann Silhan ◽  
...  
Keyword(s):  
Heart Failure ◽  
Respiratory Failure ◽  
Right Heart Failure ◽  
Laboratory Evaluation ◽  
Heart Catheterization ◽  
Self Administration ◽  
High Resolution Computed Tomography ◽  
Right Heart ◽  
Progressive Respiratory Failure ◽  
Helminth Therapy

Abstract Background Self-administration of helminths has gained attention among patients as a potential but unproven therapy for autoimmune disease. We present a case of rapidly progressive respiratory failure in a patient with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH) as a result of self-administration of parasitic organisms. Case A 45-year-old woman with a history of interstitial lung disease and PAH due to limited cutaneous SSc presented to pulmonary clinic with worsening dyspnea, cough, and new onset hypoxemia. Three months prior to presentation she started oral helminth therapy with Necator americanus as an alternative treatment for SSc. Laboratory evaluation revelaed eosinophilia and elevated IgE levels. IgG antibodies to Strongyloides were detected. High resolution computed tomography of the chest revealed progressive ILD and new diffuse ground glass opacities. Transthoracic echocardiogram and right heart catheterization illustrated worsening PAH and right heart failure. The patient was admitted to the hospital and emergently evaluated for lung transplantation but was not a candidate for transplantation due to comorbidities. Despite aggressive treatment for PAH and right heart failure, her respiratory status deteriorated, and the patient transitioned to comfort-focused care. Conclusion Although ingestion of helminths poses a risk of infection, helminth therapy has been investigated as a potential treatment for autoimmune diseases. In this case, self-prescribed helminth ingestion precipitated fatal acute worsening of lung inflammation, hypoxemia, and right heart dysfunction, highlighting the risk of experimental helminth therapy in patients, especially those with underlying respiratory disease.

scholarly journals Acute decompensated pulmonary hypertension

2017 ◽  
Vol 26 (146) ◽  
pp. 170092 ◽  
Author(s):  
Laurent Savale ◽  
Jason Weatherald ◽  
Xavier Jaïs ◽  
Constance Vuillard ◽  
Athénaïs Boucly ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Right Heart Failure ◽  
Right Heart ◽  
Adaptation Capacity ◽  
Ventricular Afterload ◽  
Time Specific

Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from impaired right ventricular filling and/or reduced right ventricular flow output. This clinical picture results from an imbalance between the afterload imposed on the right ventricle and its adaptation capacity. Acute decompensated pulmonary hypertension is associated with a very poor prognosis in the short term. Despite its major impact on survival, its optimal management remains very challenging for specialised centres, without specific recommendations. Identification of trigger factors, optimisation of fluid volume and pharmacological support to improve right ventricular function and perfusion pressure are the main therapeutic areas to consider in order to improve clinical condition. At the same time, specific management of pulmonary hypertension according to the aetiology is mandatory to reduce right ventricular afterload. Over the past decade, the development of extracorporeal life support in refractory right heart failure combined with urgent transplantation has probably contributed to a significant improvement in survival for selected patients. However, there remains a considerable need for further research in this field.

scholarly journals Rationale and design of the Pan African Pulmonary hypertension Cohort (PAPUCO) study: implementing a contemporary registry on pulmonary hypertension in Africa

BMJ Open ◽  
2014 ◽  
Vol 4 (10) ◽  
pp. e005950 ◽  
Author(s):  
Friedrich Thienemann ◽  
Anastase Dzudie ◽  
Ana O Mocumbi ◽  
Lori Blauwet ◽  
Mahmoud U Sani ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Systolic Pressure ◽  
Right Heart Failure ◽  
Developed Countries ◽  
Pulmonary Vasculature ◽  
Karnofsky Performance Score ◽  
Care Providers ◽  
Right Heart ◽  
Right Heart Catheterisation

IntroductionPulmonary hypertension (PH) is a devastating, progressive disease with increasingly debilitating symptoms and usually shortened overall life expectancy due to a narrowing of the pulmonary vasculature and consecutive right heart failure. Little is known about PH in Africa, but limited reports suggest that PH is more prevalent in Africa compared with developed countries due to the high prevalence of risk factors in the region.Methods and analysisA multinational multicentre registry-type cohort study was established and tailored to resource-constraint settings to describe disease presentation, disease severity and aetiologies of PH, comorbidities, diagnostic and therapeutic management, and the natural course of PH in Africa. PH will be diagnosed by specialist cardiologists using echocardiography (right ventricular systolic pressure >35 mm Hg, absence of pulmonary stenosis and acute right heart failure), usually accompanied by shortness of breath, fatigue, peripheral oedema and other cardiovascular symptoms, ECG and chest X-ray changes in keeping with PH as per guidelines (European Society of Cardiology and European Respiratory Society (ESC/ERS) guidelines). Additional investigations such as a CT scan, a ventilation/perfusion scan or right heart catheterisation will be performed at the discretion of the treating physician. Functional tests include a 6 min walk test and the Karnofsky Performance Score. The WHO classification system for PH will be applied to describe the different aetiologies of PH. Several substudies have been implemented within the registry to investigate specific types of PH and their outcome at up to 24 months. Data will be analysed by an independent institution following a data analyse plan.Ethics and disseminationAll local ethics committees of the participating centres approved the protocol. The data will be disseminated through peer-reviewed journals at national and international conferences and public events at local care providers.

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