scholarly journals Evaluation, Diagnosis, and Classification of Pulmonary Hypertension

2021 ◽  
Vol 17 (2) ◽  
pp. 86-91
Author(s):  
Sarah Beshay ◽  
Ashrith Guha ◽  
Sandeep Sahay
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Options ◽  
High Morbidity ◽  
Clinical Classification ◽  
Careful Evaluation ◽  
The Past ◽  
Diagnosis And Classification ◽  
Expert Center ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) is a rare heterogenous disease characterized by elevated blood pressure in the lungs. Patients with PH require careful evaluation and management at an expert center. Understanding of the mechanisms underlying the development of PH has increased over the past two decades, and several treatment options for pulmonary arterial hypertension have emerged. Despite this progress, PH continues to carry high morbidity and mortality. The 6th World Symposium on Pulmonary Hypertension that occurred in late 2018 modified the clinical classification of PH into five groups. In this review, we focus on the evaluation and diagnosis of PH and discuss the updated clinical classification.

scholarly journals Classification of Pulmonary Hypertension

2014 ◽  
Vol 13 (1) ◽  
pp. 17-20
Author(s):  
Rogerio Souza ◽  
Gerald Simonneau
Keyword(s):  
Pulmonary Hypertension ◽  
Task Force ◽  
Management Strategies ◽  
Pathological Findings ◽  
Clinical Classification ◽  
Current Classification ◽  
Pulmonary Arterial ◽  
Current Classification System ◽  
Group 1

Classification of pulmonary hypertension groups patients with similar pathological findings, hemodynamic profiles, and management strategies. Minor modifications have been made to the current classification system, particularly within Group 1 pulmonary arterial hypertension. This article summarizes the published conclusions of the Fifth World Symposium of Pulmonary Hypertension task force that addressed the updated clinical classification of pulmonary hypertension.

The Evolving Classification of Pulmonary Hypertension

2016 ◽  
Vol 141 (5) ◽  
pp. 696-703 ◽  
Author(s):  
Michelle Foshat ◽  
Nahal Boroumand
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Presentation ◽  
Medical Diagnostics ◽  
Disease Classification ◽  
The Past ◽  
Current Classification ◽  
Pulmonary Arterial ◽  
Categorization Scheme ◽  
Underlying Pathology

Context.— An explosion of information on pulmonary hypertension has occurred during the past few decades. The perception of this disease has shifted from purely clinical to incorporate new knowledge of the underlying pathology. This transfer has occurred in light of advancements in pathophysiology, histology, and molecular medical diagnostics. Objectives.— To update readers about the evolving understanding of the etiology and pathogenesis of pulmonary hypertension and to demonstrate how pathology has shaped the current classification. Data Sources.— Information presented at the 5 World Symposia on pulmonary hypertension held since 1973, with the last meeting occurring in 2013, was used in this review. Conclusions.— Pulmonary hypertension represents a heterogeneous group of disorders that are differentiated based on differences in clinical, hemodynamic, and histopathologic features. Early concepts of pulmonary hypertension were largely influenced by pharmacotherapy, hemodynamic function, and clinical presentation of the disease. The initial nomenclature for pulmonary hypertension segregated the clinical classifications from pathologic subtypes. Major restructuring of this disease classification occurred between the first and second symposia, which was the first to unite clinical and pathologic information in the categorization scheme. Additional changes were introduced in subsequent meetings, particularly between the third and fourth World Symposia meetings, when additional pathophysiologic information was gained. Discoveries in molecular diagnostics significantly progressed the understanding of idiopathic pulmonary arterial hypertension. Continued advancements in imaging modalities, mechanistic pathogenicity, and molecular biomarkers will enable physicians to define pulmonary hypertension phenotypes based on the pathobiology and allow for treatment customization.

Pulmonary arterial hypertension

2020 ◽  
pp. 355-362
Author(s):  
Perry Elliott ◽  
Pier D. Lambiase ◽  
Dhavendra Kumar
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Clinical Features ◽  
Genetic Mutations ◽  
Clinical Classification ◽  
Pulmonary Arterial

This chapter covers pulmonary hypertension (PH), including its definitions and classifications. Pulmonary arterial hypertension (PAH) is a pre-capillary form of PH, and its clinical features and signs are described. The 2019 updated clinical classification of PH is included, along with hereditable PAH, associated genetic mutations, and clinical screenings.

scholarly journals FRI0152 PULMONARY HYPERTENSION IN NEWLY DIAGNOSED SPANISH PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: DATA FROM THE RELES COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 660.2-660
Author(s):  
J. Álvarez Troncoso ◽  
Á. Robles Marhuenda ◽  
F. Mitjavila Villero ◽  
F. J. García Hernández ◽  
A. Marín Ballvé ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Multivariable Analysis ◽  
Systolic Pressure ◽  
High Morbidity ◽  
Inception Cohort ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Pulmonary Arterial

Background:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiorgan involvement. Pulmonary hypertension (PH) is an uncommon manifestation with high morbidity and mortality whose characteristics, prevalence and evolution in SLE are not completely defined.Objectives:Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to to identify the factors associated with pulmonary hypertension (PH) in systemic lupus erythematosus (SLE).Methods:Prospective observational study on a multicenter Spanish inception cohort. Patients with SLE, diagnosed by the American College of Rheumatology (ACR) criteria, since January 2009, who had at least one transthoracic echocardiogram (TTE) performed were selected. Demographic data, diagnostic criteria, follow-ups, treatments and SLEDAI were analyzed.Results:Of 289 patients diagnosed with SLE with TTE performed, 15 (5.2%) patients were identified to have PH. Mean age was 56,9±7,7 years, of which 93,3% (14) were women and 80% (12) Caucasian. The ACR score at diagnosis was 4.66. Mean SLEDAI was 15. Only 5 patients had dyspnea at the time of diagnosis. Mean pulmonary arterial systolic pressure was 49.2±5.6 mmHg. Among the PH, 4 patients had pericarditis (26.6%), 3 (20%) valvulopathies (1 antiphospholipid syndrome), 1 patient pulmonary embolism and 1 shrinking lung. Multivariable analysis indicated that pericarditis (odds ratio (OR)=2.53), and valvulopathies (OR 8.96) were independently associated with the development of PH in SLE. Having PH was associated with older age at diagnosis (p<0.001), more dyspnea (p<0.001), higher ESR (p=0.007), more serositis (p<0.001), higher SLEDAI (p=0.011), higher SLICC (p <0.001), higher number of admissions (p=0.006) and higher mortality (p=0.003).Conclusion:PH in SLE is a serious comorbidity with high mortality. In the RELES cohort it was associated with increased disease activity, pericarditis and valvulopathies. Performing TTE in patients with SLE may favor early diagnosis and treatment.References:[1]Kim JS, Kim D, Joo YB, et al. Factors associated with development and mortality of pulmonary hypertension in systemic lupus erythematosus patients.Lupus. 2018;27(11):1769–1777.[2]Bazan IS, Mensah KA, Rudkovskaia AA, et al. Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.Respir Med. 2018;134:42–46.Disclosure of Interests:Jorge Álvarez Troncoso: None declared, Ángel Robles Marhuenda: None declared, Francesca Mitjavila Villero: None declared, Francisco José García Hernández: None declared, Adela Marín Ballvé: None declared, Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Gonzalo Salvador Cervelló: None declared, Eva Fonseca: None declared, Isabel Perales Fraile: None declared, Guillermo Ruiz-Irastorza: None declared

scholarly journals Pulmonary hypertension in pregnancy

2021 ◽  
Vol 10 (3) ◽  
pp. 1259
Author(s):  
K. Durga ◽  
S. Yuvarajan ◽  
R. Praveen ◽  
Antonious Maria Selvam ◽  
Yashoda . ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Phosphodiesterase Inhibitors ◽  
High Morbidity ◽  
Therapeutic Abortion ◽  
Hypertension In Pregnancy ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Receptor Blockers ◽  
Pulmonary Arterial ◽  
In Pregnancy

Pulmonary hypertension is defined as an increase in mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest as assessed by right heart catheterisation. Pulmonary hypertension in pregnancy is known to be associated with significantly high morbidity and mortality rate which ranges between 30% and 56%. So during pregnancy, efforts to be made to diagnose common medical ailments that can be complicated by pulmonary hypertension. Bedside 2D Echo and thoracic ultrasound are the strongly recommended in these patients to diagnose early and prevent the devastating complications. Relevant blood investigations need to be sent to diagnose the underlying etiology and to assess the prognosis. Cardiac catheterization is the gold standard investigation of choice for pulmonary hypertension. But it is 1 performed in very few cardiac centres in developing countries. In India diagnosis largely depends on echocardiography. It should be made clear to women at the time of their PAH diagnosis that pregnancy is not recommended due to the high maternal and fetal risks. If a woman with known PHT become pregnant, counselling should be given for therapeutic abortion. If they are willing for therapeutic abortion, it should be done before 22 weeks of gestation. All women with PHT should be initiated on PAH specific therapies (prostanoids, ccbs, phosphodiesterase inhibitors) except endothelin receptor blockers as it is teratogenic. Pregnancy in PAH is difficult to manage and needs mutidisciplanary team. Pregnancy is not recommended in women with PAH and appropriate counselling to be done to the mother and their relatives. 

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

New Treatment Options for Lymphangioma in Infants and Children

2002 ◽  
Vol 111 (12) ◽  
pp. 1066-1075 ◽  
Author(s):  
Chantal M. Giguère ◽  
Nancy M. Bauman ◽  
Richard J. H. Smith
Keyword(s):  
Lymphatic System ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Therapeutic Interventions ◽  
Lymphatic Malformations ◽  
Diagnostic Measures ◽  
The Past ◽  
New Treatment ◽  
In Infants And Children

Lymphangiomas are congenital malformations of the lymphatic system. These lesions occur most often in the head and neck area, and their treatment continues to be a challenge. Fortunately, a number of advances have occurred in the diagnosis and management of lymphatic malformations in the past decade. The purpose of this article is to clarify the embryology, pathogenesis, histopathology, and classification of these lesions, as well as to describe their various forms of clinical presentation. We provide a complete review of the diagnostic measures available and thoroughly discuss new therapeutic interventions proposed to treat lymphangiomas.

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