Hypoxic Pulmonary Vasoconstriction and Chronic Lung Disease

2013 ◽  
Vol 12 (3) ◽  
pp. 135-144 ◽  
Author(s):  
Erik R. Swenson
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Disease ◽  
Hypoxic Pulmonary Vasoconstriction ◽  
World Health ◽  
Disease States ◽  
Pulmonary Vasoconstriction ◽  
Local Ventilation ◽  
Pulmonary Vascular Endothelium ◽  
Health And Disease ◽  
Health Organization

Hypoxic vasoconstriction in the lung is a unique and fundamental characteristic of the pulmonary circulation. It functions in health and disease states to better preserve ventilation-perfusion matching by diverting blood flow to better ventilated regions when local ventilation is compromised. As more areas of lung become hypoxic either with high altitude or global lung disease, then hypoxic pulmonary vasoconstriction (HPV) becomes less effective in ventilation-perfusion matching and can lead to pulmonary hypertension. HPV is intrinsic to the vascular smooth muscle and its mechanisms remain poorly understood. In addition, the pulmonary vascular endothelium, red cells, lung innervation, and numerous circulating vasoactive agents also affect the strength of HPV. This review will discuss the pathophysiology of HPV and address its role in pulmonary hypertension associated with World Health Organization Group 3 diseases. When sustained beyond many hours, HPV may initiate pulmonary vascular remodeling and lead to more fixed and less oxygen-responsive pulmonary hypertension if the hypoxic stimulus is maintained.

Pulmonary Hypertension - A Distinct Perspective

2021 ◽  
Vol 8 (26) ◽  
pp. 2349-2362
Author(s):  
Ankit Kumar Sahu ◽  
Sudeep Kumar
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Primary Treatment ◽  
World Health ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Vasoconstriction ◽  
Pulmonary Arterial ◽  
Health Organization ◽  
Pulmonary Vascular Remodelling

In this review, we have discussed pulmonary hypertension including definition, classification, pathophysiology, epidemiology, diagnosis and treatment of each class of PH as per latest available data and guidelines. Pulmonary hypertension is defined as an increase in mean pulmonary arterial pressure of ≥ 20 mmHg at rest. The clinical classification of pulmonary hypertension (as per world health organization) includes five different classes constructed on the basis of clinical causes of pulmonary hypertension. The main pathophysiology involved in the development of pulmonary hypertension of all types is prolonged pulmonary vasoconstriction caused by multiple factors which ultimately leads to pulmonary vascular remodelling (structural as well as functional). The primary treatment of pulmonary hypertension comprises of correction/control of underlying cause of pulmonary hypertension. The prevalence of pulmonary hypertension is increasing in developed as well as developing countries and thus its awareness is important for timely diagnosis and management of this chronic condition. The main aim of our review is to provide all the necessary information regarding pulmonary hypertension in one document. KEYWORDS Pulmonary Hypertension, Chronic Thromboembolism, Left Heart Disease, Lung Disease, Pulmonary Arterial Hypertension

‘Group 5’ pulmonary hypertension due to multiple and/or unclear mechanisms

ESC CardioMed ◽  
2018 ◽  
pp. 2554-2557
Author(s):  
Andrew J. Peacock
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Cardiac Dysfunction ◽  
World Health ◽  
Left Heart ◽  
Left Heart Failure ◽  
Pulmonary Vasoconstriction ◽  
Extrinsic Compression ◽  
Group 5 ◽  
Health Organization

World Health Organization group 5 pulmonary hypertension, that is, disorders that lead to pulmonary hypertension due to multiple or unclear mechanisms, is an interesting group because it combines several diseases with different aetiologies. A common feature of these diseases is that the mechanisms of pulmonary hypertension are poorly understood and may include different causes such as pulmonary vasoconstriction, proliferative vasculopathy, extrinsic compression, intrinsic occlusion, high-output cardiac dysfunction, vascular destruction. and left heart failure. In terms of treatment, the mantra is ‘treat the lung, not the pressure’.

scholarly journals Barriers and Solutions to Developing Future Therapies for Pulmonary Hypertension

2018 ◽  
Vol 17 (4) ◽  
pp. 159-165
Author(s):  
Brian Graham ◽  
Peter Fernandes ◽  
Sue Gu
Keyword(s):  
Clinical Trials ◽  
Pulmonary Hypertension ◽  
Phase 1 ◽  
Unmet Need ◽  
Orphan Drugs ◽  
World Health ◽  
Vasodilator Therapy ◽  
Scarce Resources ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary hypertension (PH) and its subset, pulmonary arterial hypertension (PAH), are rare diseases with a significant unmet need. Between the 1980s and 2010s, the 5-year survival rate for PAH after diagnosis improved from 34% to 65%,12 but remains unacceptably low. Since the introduction of vasodilator therapy, 34 important advances have been made in the understanding of the disease pathophysiology and development of targeted therapies. There are now 14 US Food and Drug Administration (FDA)-approved therapies that target 3 distinct pathways that contribute to PAH, and additional therapeutic targets are currently under investigation in phase 1, 2, and 3 clinical trials.5 However, there have been major challenges in PH medication development to date, including: 1) only one medication approved for pediatric PAH; 2) focusing on vasodilator therapy rather than targeting the underlying pathogenesis of the disease; 3) no medications approved for PH World Health Organization (WHO) Groups 2, 3, and 5; and 4) several recent high-profile clinical failures after promising preclinical studies.The focus and goal of the PH research community should be directed at identifying new options and solutions for patients. The field must ensure that the approaches used for clinical trials to develop orphan drugs maximize the scarce resources available for recruiting subjects, and are directed toward making safe and effective therapies available in a timely manner. Therefore, there is a critical need to coordinate and harmonize innovative approaches within the field, including strengthening translational research to deliver promising candidates and optimize the designs, endpoints, and biomarkers to conduct safe and efficient clinical trials.

Abstract 15764: Evaluation of Pulmonary Hypertension World Health Organization Group II in the Perioperative Setting

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Martin Marak ◽  
Danielle Tatum ◽  
Denzil Moraes
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Risk Factor ◽  
World Health Organization ◽  
Independent Risk Factor ◽  
World Health ◽  
Intermediate Risk ◽  
Surgical Risk ◽  
Group Ii ◽  
Health Organization

Introduction: The World Health Organization (WHO) categorizes pulmonary hypertension (PHTN) into 5 groups. Group II classification denotes PHTN secondary to left-heart disease and accounts for nearly 75% of all cases. However, there is limited data regarding the effect of PHTN Group II status on outcomes in the perioperative setting. Hypothesis: PHTN WHO Group II is an independent risk factor for adverse cardiopulmonary events in the perioperative setting. Methods: Retrospective review of patients who underwent intra-abdominal surgery between January 2014 - August 2019 and had previously obtained an echocardiogram. PHTN Group II was defined as estimated pulmonary artery pressure (EPAP) > 30mmHg on echocardiogram . Other forms of PHTN were excluded. Major adverse cardiovascular events (MACE) were defined as heart failure exacerbation, arrhythmia, myocardial infarction, 30 day readmission, and death. Surgical risk was categorized as low (laparoscopic) or intermediate (open). Results: By echocardiogram findings, 65 of the 178 (36.3%) patients included were Group II PHTN. Between surgical risk classes, Group II PHTN was older (mean age 73.7 years v 60.5; P< 0.01), had more comorbidities including systolic (9.70% v 21.5%, P=0.03) and diastolic (22.1% v 34.5%, P < 0.01) heart failure, and were more likely to have a MACE ( 6.2% v 43.1%, P < 0.01). PHTN Group II patients with intermediate-risk surgeries demonstrated significantly more MACE than control (11.2% v 43.7%, P <0.01) without significant difference in comorbidities. Conclusions: Group II PHTN is an independent risk factor for MACE in patients undergoing intermediate risk surgery compared to non-PHTN counterparts. Additional studies involving severity of pulmonary hypertension may provide further insight into risk stratification.

scholarly journals Liver transplantation in a patient with hereditary haemorrhagic telangiectasia and pulmonary hypertension

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401989667
Author(s):  
Kentaro Ejiri ◽  
Satoshi Akagi ◽  
Kazufumi Nakamura ◽  
Naofumi Amioka ◽  
Keishi Ichikawa ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Pulmonary Hypertension ◽  
World Health Organization ◽  
Arteriovenous Malformations ◽  
Functional Class ◽  
Arteriovenous Shunt ◽  
World Health ◽  
Right Atrial ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Health Organization

Hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber syndrome is a systemic vascular disease with autosomal dominant inheritance, mucocutaneous telangiectasia, and repeated nasal bleeding due to vascular abnormalities. Hereditary haemorrhagic telangiectasia may occasionally lead to complications, including arteriovenous malformations and pulmonary hypertension. We present a case of a 52-year-old female patient with hereditary haemorrhagic telangiectasia who was referred to our hospital for treatment of pulmonary hypertension. She had been diagnosed with hereditary haemorrhagic telangiectasia during adolescence and was being followed up. Six months prior to presentation, she had undergone coil embolization for pulmonary haemorrhage due to pulmonary arteriovenous malformations. She was in World Health Organization functional class IV, with a mean of pulmonary arterial pressure of 38 mmHg, a pulmonary capillary wedge pressure of 10 mmHg, and a right atrial pressure of 22 mmHg. A contrast-enhanced computed tomography angiography showed large arteriovenous malformations in the liver. Right heart catheterization revealed an increase in oxygen saturation in the inferior vena cava between the supra- and infra-hepatic veins, low pulmonary vascular resistance, and high right atrial pressure. Hence, she was diagnosed with hereditary haemorrhagic telangiectasia with pulmonary hypertension due to major arteriovenous shunt resulting from arteriovenous malformations in the liver. Therefore, we considered liver transplantation as an essential treatment option. She underwent cadaveric liver transplantation after a year resulting in dramatic haemodynamic improvement to World Health Organization functional class I. Liver transplantation is a promising treatment in patients with hereditary haemorrhagic telangiectasia and pulmonary hypertension resulting from arteriovenous shunt caused by arteriovenous malformations in the liver.

scholarly journals Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401987715 ◽  
Author(s):  
Alexandra Arvanitaki ◽  
Maria Boutsikou ◽  
Anastasia Anthi ◽  
Sotiria Apostolopoulou ◽  
Aikaterini Avgeropoulou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Treatment Data ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

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