Initiation of Pulmonary Arterial Hypertension Specific Therapy Is Well Tolerated in Patients With Severe Lung Disease and Associated WHO Group III Pulmonary Hypertension

Aim. Using the prospective Registry data, to assess the effects of conventional and specific therapy on the clinical course and survival of the patients with pulmonary arterial hypertension (PAH). Material and methods. The study included 124 patients (mean age 38,2±13,7 years; 34 men and 78 women): 31 with idiopathic PAH (IPAH), 52 with Eisenmenger syndrome, 17 with inoperable chronic thromboembolic pulmonary hypertension, 9 with PAH and corrected congenital heart disease, 6 with PAH and systemic scleroderma, and 6 with PAH and HIV infection. Results. The cumulative one-year and three-year survival rates were 94% and 75%, respectively. Irrespective of the absence of right heart catheterisation and vasoreactive testing, 42,7% of the patients were treated with calcium antagonists. PAH-specific therapy was administered to 40,3% of the participants (64,5% and 21% of those with IPAH and Eisenmenger syndrome, respectively). PAH-specific therapy was associated with an increase in survival time. Conclusion. In PAH patients, the prognosis is linked to early administration of specific monotherapy and possible combination therapy. Developing a national registry of pulmonary hypertension will facilitate the assessment of the real-world demand for specific therapy and the related costs.

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Pulmonary hypertension

Oxford Textbook of Medicine ◽

10.1093/med/9780198746690.003.0374 ◽

2020 ◽

pp. 3695-3710

Author(s):

Nicholas W. Morrell

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Lung Disease ◽

Congenital Heart ◽

Heart Function ◽

Gene Encoding ◽

Pulmonary Arterial

Symptoms of unexplained exertional breathlessness or symptoms out of proportion to coexistent heart or lung disease should alert the clinician to the possibility of pulmonary hypertension, and the condition should be actively sought in patients with known associated conditions, such as scleroderma, hypoxic lung disease, liver disease, or congenital heart disease. Heterozygous germ-line mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR2) are found in over 70% of families with pulmonary arterial hypertension. Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest, and may be due to increased pulmonary vascular resistance (e.g. pulmonary arterial hypertension), increased transpulmonary blood flow (e.g. congenital heart disease), or increased pulmonary venous pressures (e.g. mitral stenosis). Exercise tolerance and survival in pulmonary hypertension is ultimately related to indices of right heart function, such as cardiac output.

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New potential diagnostic biomarkers for pulmonary hypertension

European Respiratory Journal ◽

10.1183/13993003.00187-2015 ◽

2015 ◽

Vol 46 (5) ◽

pp. 1390-1396 ◽

Cited By ~ 15

Author(s):

Svenja L. Tiede ◽

Henning Gall ◽

Oliver Dörr ◽

Marina dos Santos Guilherme ◽

Christian Troidl ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Growth Factor ◽

Pulmonary Arterial Hypertension ◽

Arterial Pressure ◽

Arterial Hypertension ◽

Lung Disease ◽

Pulmonary Arterial Pressure ◽

Diagnostic Biomarkers ◽

Mean Pulmonary Arterial Pressure ◽

Pulmonary Arterial

This study aimed to determine whether the vascular endothelial growth factor (VEGF) family members soluble VEGF receptor 1 (also called soluble fms-like tyrosine kinase 1 (sFlt-1)) and placental growth factor (PlGF) could be used as biomarkers for pulmonary hypertension (PH).Consecutive patients undergoing right heart catheterisation were enrolled (those with mean pulmonary arterial pressure ≥25 mmHg were classed as having PH; those with mean pulmonary arterial pressure <25 mmHg acted as non-PH controls). Plasma from the time of PH diagnosis was analysed for PlGF and sFlt-1 using enzyme immunoassays.In total, 247 patients with PH were enrolled: 62 with idiopathic pulmonary arterial hypertension (IPAH), 14 with associated pulmonary arterial hypertension (APAH), 21 with collagen vascular disease (CVD), 26 with pulmonary venous hypertension, 67 with lung disease-associated PH and 57 with chronic thromboembolic PH. The non-PH control group consisted of 40 patients. sFlt-1 plasma levels were significantly higher in patients with IPAH, APAH, CVD and lung disease-associated PH versus controls; PlGF levels were significantly higher in all PH groups versus controls. The combination of sFlt-1 and PlGF resulted in a sensitivity of 83.7% with specificity of 100% for pulmonary arterial hypertension. There was no association between sFlt-1 or PlGF and haemodynamic parameters, 6-min walking distance or survival.In summary, PlGF and sFlt-1 are promising diagnostic biomarkers for PH.

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