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 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.

Linked mechanical and biological aspects of remodeling in mouse pulmonary arteries with hypoxia-induced hypertension

2005 ◽  
Vol 288 (3) ◽  
pp. H1209-H1217 ◽  
Author(s):  
Ryan W. Kobs ◽  
Nidal E. Muvarak ◽  
Jens C. Eickhoff ◽  
Naomi C. Chesler
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Large Diameter ◽  
Pulmonary Arteries ◽  
Right Heart Failure ◽  
Effective Elastic Modulus ◽  
Pulmonary Vasculature ◽  
Right Heart ◽  
Vascular Impedance ◽  
Biological Aspects

Right heart failure due to pulmonary hypertension causes significant morbidity and mortality. To study the linked vascular mechanical and biological changes that are induced by pulmonary hypertension, we mechanically tested isolated left main pulmonary arteries from mice exposed to chronic hypobaric hypoxia and performed histological assays on contralateral vessels. In isolated vessel tests, hypoxic vessels stretched less in response to pressure than controls at all pressure levels. Given the short length and large diameter of the pulmonary artery, the tangent Young's modulus could not be measured; instead, an effective elastic modulus was calculated that increased significantly with hypoxia [(280 kPa (SD 53) and 296 kPa (SD 50) for 10 and 15 days, respectively, vs. 222 kPa (SD 35) for control; P < 0.02)]. Hypoxic vessels also had higher damping coefficients [(0.063 (SD 0.017) and 0.054 (SD 0.014) for 10 and 15 days, respectively, vs. 0.033 (SD 0.016) for control; P < 0.002)], indicating increased energy dissipation. The increased stiffness with hypoxia correlated with an increase in collagen thickness (percent collagen multiplied by wall thickness) as well as the sum of elastin and collagen thicknesses measured histologically in the artery wall. These results highlight the mechanobiological changes in the pulmonary vasculature that occur in response to hypoxia-induced pulmonary hypertension. Furthermore, they demonstrate significant vascular mechanical and biological changes that would increase pulmonary vascular impedance, leading to right heart failure.

Inhibition of mTOR by rapamycin does not improve hypoxic pulmonary hypertension-induced right heart failure in old mice

2021 ◽  
pp. 111395
Author(s):  
Benjamin D. McNair ◽  
Jacob A. Schlatter ◽  
Ross F. Cook ◽  
Musharraf Yusifova ◽  
Danielle R. Bruns
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Right Heart ◽  
Hypoxic Pulmonary Hypertension ◽  
Old Mice

scholarly journals Genistein, a Soy Phytoestrogen, Reverses Severe Pulmonary Hypertension and Prevents Right Heart Failure in Rats

Hypertension ◽  
2012 ◽  
Vol 60 (2) ◽  
pp. 425-430 ◽  
Author(s):  
Humann Matori ◽  
Soban Umar ◽  
Rangarajan D. Nadadur ◽  
Salil Sharma ◽  
Rod Partow-Navid ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Severe Pulmonary Hypertension ◽  
Right Heart

Efficacy and safety of a calcium sensitizer, levosimendan, in patients with right heart failure due to pulmonary hypertension

2017 ◽  
Vol 12 (4) ◽  
pp. 1518-1525 ◽  
Author(s):  
Rong Jiang ◽  
Qin‐Hua Zhao ◽  
Wen‐Hui Wu ◽  
Rui Zhang ◽  
Ping Yuan ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Heart Failure ◽  
Efficacy And Safety ◽  
Right Heart ◽  
Calcium Sensitizer

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 Critical Care Management of Decompensated Right Heart Failure in Pulmonary Arterial Hypertension Patients – An Ongoing Approach

2021 ◽  
Vol 7 (3) ◽  
pp. 170-183
Author(s):  
Ioan Tilea ◽  
Andreea Varga ◽  
Anca-Meda Georgescu ◽  
Bianca-Liana Grigorescu
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Care Management ◽  
Right Heart Failure ◽  
Right Heart ◽  
Continuous Increase ◽  
Pulmonary Arterial

Abstract Despite substantial advancements in diagnosis and specific medical therapy in pulmonary arterial hypertension patients’ management, this condition continues to represent a major cause of mortality worldwide. In pulmonary arterial hypertension, the continuous increase of pulmonary vascular resistance and rapid development of right heart failure determine a poor prognosis. Against targeted therapy, patients inexorable deteriorate over time. Pulmonary arterial hypertension patients with acute right heart failure who need intensive care unit admission present a complexity of the disease pathophysiology. Intensive care management challenges are multifaceted. Awareness of algorithms of right-sided heart failure monitoring in intensive care units, targeted pulmonary hypertension therapies, and recognition of precipitating factors, hemodynamic instability and progressive multisystem organ failure requires a multidisciplinary pulmonary hypertension team. This paper summarizes the management strategies of acute right-sided heart failure in pulmonary arterial hypertension adult cases based on recently available data.

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