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Pre-capillary pulmonary hypertension

MedGen UID:
784220
•Concept ID:
C3696954
•
Disease or Syndrome
Synonym: Precapillary pulmonary hypertension
SNOMED CT: Precapillary pulmonary hypertension (697896007)
 
HPO: HP:0033578

Definition

Pre-capillary pulmonary hypertension is a haemodynamic condition characterized by elevated mean pulmonary artery pressure (mPAP over 20 mmHg) and pulmonary vascular resistance (PVR 3 Wood units or more) accompanied by normal pulmonary artery wedge pressure (PAWP not more than 15 mmHg). [from HPO]

Professional guidelines

PubMed

Treatment of pulmonary arterial hypertension: recent progress and a look to the future.
Humbert M, Sitbon O, Guignabert C, Savale L, Boucly A, Gallant-Dewavrin M, McLaughlin V, Hoeper MM, Weatherald J
Lancet Respir Med 2023 Sep;11(9):804-819. Epub 2023 Aug 14 doi: 10.1016/S2213-2600(23)00264-3. PMID: 37591298
Recent advances in the management of pulmonary hypertension with interstitial lung disease.
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
Treatment of pulmonary arterial hypertension: the role of prostacyclin and prostaglandin analogs.
Safdar Z
Respir Med 2011 Jun;105(6):818-27. Epub 2011 Jan 26 doi: 10.1016/j.rmed.2010.12.018. PMID: 21273054

Recent clinical studies

Etiology

Treatment of pulmonary arterial hypertension: recent progress and a look to the future.
Humbert M, Sitbon O, Guignabert C, Savale L, Boucly A, Gallant-Dewavrin M, McLaughlin V, Hoeper MM, Weatherald J
Lancet Respir Med 2023 Sep;11(9):804-819. Epub 2023 Aug 14 doi: 10.1016/S2213-2600(23)00264-3. PMID: 37591298
Pulmonary vascular disease in pulmonary hypertension due to left heart disease: pathophysiologic implications.
Omote K, Sorimachi H, Obokata M, Reddy YNV, Verbrugge FH, Omar M, DuBrock HM, Redfield MM, Borlaug BA
Eur Heart J 2022 Sep 21;43(36):3417-3431. doi: 10.1093/eurheartj/ehac184. PMID: 35796488Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Long-term survival outcome for pre-capillary pulmonary hypertension at a Japanese single center.
Sugiyama Y, Tahara N, Bekki M, Tahara A, Honda A, Maeda-Ogata S, Sun J, Igata S, Fukumoto Y
Can J Physiol Pharmacol 2020 Sep;98(9):644-652. Epub 2020 Mar 3 doi: 10.1139/cjpp-2019-0663. PMID: 32125894
Definition and classification of pulmonary hypertension.
Humbert M, Montani D, Evgenov OV, Simonneau G
Handb Exp Pharmacol 2013;218:3-29. doi: 10.1007/978-3-642-38664-0_1. PMID: 24092334

Diagnosis

Circulating apelin, IL22RA2 and VEGF in pre-capillary pulmonary hypertension.
Csósza G, Szűcs G, Rozgonyi Z, Csoma B, Losonczy G, Müller V, Karlócai K, Lázár Z
Physiol Int 2023 Dec 18;110(4):356-370. Epub 2023 Nov 17 doi: 10.1556/2060.2023.00264. PMID: 37975916
Recent advances in the management of pulmonary hypertension with interstitial lung disease.
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Long-term survival outcome for pre-capillary pulmonary hypertension at a Japanese single center.
Sugiyama Y, Tahara N, Bekki M, Tahara A, Honda A, Maeda-Ogata S, Sun J, Igata S, Fukumoto Y
Can J Physiol Pharmacol 2020 Sep;98(9):644-652. Epub 2020 Mar 3 doi: 10.1139/cjpp-2019-0663. PMID: 32125894
Combined post- and pre-capillary pulmonary hypertension in heart failure with preserved ejection fraction.
Dixon DD, Trivedi A, Shah SJ
Heart Fail Rev 2016 May;21(3):285-97. doi: 10.1007/s10741-015-9523-6. PMID: 26714826

Therapy

Treatment of pulmonary arterial hypertension: recent progress and a look to the future.
Humbert M, Sitbon O, Guignabert C, Savale L, Boucly A, Gallant-Dewavrin M, McLaughlin V, Hoeper MM, Weatherald J
Lancet Respir Med 2023 Sep;11(9):804-819. Epub 2023 Aug 14 doi: 10.1016/S2213-2600(23)00264-3. PMID: 37591298
Recent advances in the management of pulmonary hypertension with interstitial lung disease.
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
Determinants of altered left ventricular suction in pre-capillary pulmonary hypertension.
Chiba Y, Iwano H, Tsuneta S, Tsujinaga S, Meyers B, Vlachos P, Ishizaka S, Motoi K, Aoyagi H, Tamaki Y, Tanemura A, Murayama M, Yokoyama S, Nakabachi M, Nishino H, Kaga S, Kamiya K, Ohira H, Tsujino I, Anzai T
Eur Heart J Cardiovasc Imaging 2022 Sep 10;23(10):1399-1406. doi: 10.1093/ehjci/jeab285. PMID: 35019957
Combined post- and pre-capillary pulmonary hypertension in heart failure with preserved ejection fraction.
Dixon DD, Trivedi A, Shah SJ
Heart Fail Rev 2016 May;21(3):285-97. doi: 10.1007/s10741-015-9523-6. PMID: 26714826
Treatment of pulmonary arterial hypertension: the role of prostacyclin and prostaglandin analogs.
Safdar Z
Respir Med 2011 Jun;105(6):818-27. Epub 2011 Jan 26 doi: 10.1016/j.rmed.2010.12.018. PMID: 21273054

Prognosis

Recent advances in the management of pulmonary hypertension with interstitial lung disease.
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Long-term survival outcome for pre-capillary pulmonary hypertension at a Japanese single center.
Sugiyama Y, Tahara N, Bekki M, Tahara A, Honda A, Maeda-Ogata S, Sun J, Igata S, Fukumoto Y
Can J Physiol Pharmacol 2020 Sep;98(9):644-652. Epub 2020 Mar 3 doi: 10.1139/cjpp-2019-0663. PMID: 32125894
Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis.
Launay D, Montani D, Hassoun PM, Cottin V, Le Pavec J, Clerson P, Sitbon O, Jaïs X, Savale L, Weatherald J, Sobanski V, Mathai SC, Shafiq M, Cordier JF, Hachulla E, Simonneau G, Humbert M
PLoS One 2018;13(5):e0197112. Epub 2018 May 15 doi: 10.1371/journal.pone.0197112. PMID: 29763468Free PMC Article
Combined post- and pre-capillary pulmonary hypertension in heart failure with preserved ejection fraction.
Dixon DD, Trivedi A, Shah SJ
Heart Fail Rev 2016 May;21(3):285-97. doi: 10.1007/s10741-015-9523-6. PMID: 26714826

Clinical prediction guides

The potential of cystatin C as a predictive biomarker in pulmonary hypertension.
Duan A, Huang Z, Zhao Z, Zhao Q, Jin Q, Yan L, Zhang Y, Li X, Zhang S, Hu M, Gao L, An C, Luo Q, Liu Z
BMC Pulm Med 2023 Aug 26;23(1):311. doi: 10.1186/s12890-023-02595-1. PMID: 37633906Free PMC Article
Dynamic aspects of ventricular interaction during exercise in HFpEF and in pre-capillary pulmonary hypertension.
Claeys M, Petit T, Bogaert J, La Gerche A, Los J, Delcroix M, Willems R, Claessen G, Claus P
ESC Heart Fail 2023 Feb;10(1):650-660. Epub 2022 Nov 24 doi: 10.1002/ehf2.14216. PMID: 36424844Free PMC Article
The comorbidity burden and disease phenotype in pre-capillary pulmonary hypertension: The contributing role of obstructive sleep apnea.
Duan A, Huang Z, Hu M, Zhao Z, Zhao Q, Jin Q, Yan L, Zhang Y, Li X, An C, Luo Q, Liu Z
Sleep Med 2023 Jan;101:146-153. Epub 2022 Nov 2 doi: 10.1016/j.sleep.2022.10.029. PMID: 36395719
Recent advances in the management of pulmonary hypertension with interstitial lung disease.
Waxman AB, Elia D, Adir Y, Humbert M, Harari S
Eur Respir Rev 2022 Sep 30;31(165) Epub 2022 Jul 12 doi: 10.1183/16000617.0220-2021. PMID: 35831007Free PMC Article
Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079

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