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Pulmonary arterial hypertension with positive acute response to NO challenge

MedGen UID:
1782938
•Concept ID:
C5539583
•
Disease or Syndrome
HPO: HP:0033423

Definition

A form of pulmonary arterial hypertension in which there is a reduction of mean pulmonary artery pressure (mPAP) at least 10 mmHg to reach an absolute value of mPAP not more than 40 mmHg with an increased or unchanged cardiac output (CO) in response to inhaled nitric oxide at 10-20 ppm. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary arterial hypertension with positive acute response to NO challenge

Professional guidelines

PubMed

Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension.
Piloto B, Fernandes CJCDS, Jardim C, Castro M, Alves-Jr JL, Souza R
J Bras Pneumol 2023;49(3):e20220337. Epub 2023 May 1 doi: 10.36416/1806-3756/e20220337. PMID: 37132695Free PMC Article

Recent clinical studies

Etiology

Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension.
Piloto B, Fernandes CJCDS, Jardim C, Castro M, Alves-Jr JL, Souza R
J Bras Pneumol 2023;49(3):e20220337. Epub 2023 May 1 doi: 10.36416/1806-3756/e20220337. PMID: 37132695Free PMC Article
Soluble CD163 in young sickle cell disease patients and their trait siblings: a biomarker for pulmonary hypertension and vaso-occlusive complications.
Tantawy AA, Adly AA, Ismail EA
Blood Coagul Fibrinolysis 2012 Oct;23(7):640-8. doi: 10.1097/MBC.0b013e3283573a42. PMID: 22885767

Diagnosis

Soluble CD163 in young sickle cell disease patients and their trait siblings: a biomarker for pulmonary hypertension and vaso-occlusive complications.
Tantawy AA, Adly AA, Ismail EA
Blood Coagul Fibrinolysis 2012 Oct;23(7):640-8. doi: 10.1097/MBC.0b013e3283573a42. PMID: 22885767

Therapy

Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension.
Piloto B, Fernandes CJCDS, Jardim C, Castro M, Alves-Jr JL, Souza R
J Bras Pneumol 2023;49(3):e20220337. Epub 2023 May 1 doi: 10.36416/1806-3756/e20220337. PMID: 37132695Free PMC Article

Prognosis

Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension.
Piloto B, Fernandes CJCDS, Jardim C, Castro M, Alves-Jr JL, Souza R
J Bras Pneumol 2023;49(3):e20220337. Epub 2023 May 1 doi: 10.36416/1806-3756/e20220337. PMID: 37132695Free PMC Article
Is There Value in Repeating Inhaled Nitric Oxide Vasoreactivity Tests in Patients with Pulmonary Arterial Hypertension?
Tooba R, Almoushref A, Tonelli AR
Lung 2020 Feb;198(1):87-94. Epub 2020 Jan 1 doi: 10.1007/s00408-019-00318-0. PMID: 31894408
Soluble CD163 in young sickle cell disease patients and their trait siblings: a biomarker for pulmonary hypertension and vaso-occlusive complications.
Tantawy AA, Adly AA, Ismail EA
Blood Coagul Fibrinolysis 2012 Oct;23(7):640-8. doi: 10.1097/MBC.0b013e3283573a42. PMID: 22885767

Clinical prediction guides

Soluble CD163 in young sickle cell disease patients and their trait siblings: a biomarker for pulmonary hypertension and vaso-occlusive complications.
Tantawy AA, Adly AA, Ismail EA
Blood Coagul Fibrinolysis 2012 Oct;23(7):640-8. doi: 10.1097/MBC.0b013e3283573a42. PMID: 22885767

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