Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms : A Population-based Study
Rationale: Precapillary pulmonary hypertension (PH) is a rare and largely unrecognized complication of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF). Objectives: To describe characteristics and outcomes of MPN-associated PH. Methods: We report clinical, functional, and hemodynamic characteristics, classification, and outcomes of patients with PV, ET, or primary MF in the French PH registry. Measurements and Main Results: Ninety patients with MPN (42 PV, 35 ET, 13 primary MF) presented with precapillary PH with severe hemodynamic impairment, with a median mean pulmonary arterial pressure and pulmonary vascular resistance of 42 mm Hg and 6.7 Wood units, respectively, and impaired clinical conditions, with 71% in New York Heart Association functional classes III/IV and having a median 6-minute-walk distance of 310 m. Half of the patients were diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 PH. MF was preferentially associated with group 5 PH, whereas PV and ET were generally related to CTEPH. Proximal lesions were diagnosed in half of the patients with CTEPH. Thromboendarterectomy was performed in 18 selected patients with high risk of complications (5 early deaths). Overall survival at 1, 3, and 5 years was 67%, 50%, and 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively. Conclusions: PH is a life-threatening condition potentially occurring in MPN. There are multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH. Physicians should be aware that PH strongly affects the burden of patients with MPN, especially in group 5 PH, with unknown pathophysiological mechanisms.
| Errataetall: |
CommentIn: Am J Respir Crit Care Med. 2023 Sep 1;208(5):518-521. - PMID 37429036 |
|---|---|
| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2023 |
|---|---|
| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:208 |
|---|---|
| Enthalten in: |
American journal of respiratory and critical care medicine - 208(2023), 5 vom: 01. Sept., Seite 600-612 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Montani, David [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Chronic thromboembolic pulmonary hypertension |
|---|
| Anmerkungen: |
Date Completed 23.10.2023 Date Revised 24.10.2023 published: Print CommentIn: Am J Respir Crit Care Med. 2023 Sep 1;208(5):518-521. - PMID 37429036 Citation Status MEDLINE |
|---|
| doi: |
10.1164/rccm.202210-1941OC |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM358128862 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM358128862 | ||
| 003 | DE-627 | ||
| 005 | 20231226074104.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2023 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1164/rccm.202210-1941OC |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1193.xml |
| 035 | |a (DE-627)NLM358128862 | ||
| 035 | |a (NLM)37311222 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Montani, David |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms |b A Population-based Study |
| 264 | 1 | |c 2023 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 23.10.2023 | ||
| 500 | |a Date Revised 24.10.2023 | ||
| 500 | |a published: Print | ||
| 500 | |a CommentIn: Am J Respir Crit Care Med. 2023 Sep 1;208(5):518-521. - PMID 37429036 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Rationale: Precapillary pulmonary hypertension (PH) is a rare and largely unrecognized complication of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF). Objectives: To describe characteristics and outcomes of MPN-associated PH. Methods: We report clinical, functional, and hemodynamic characteristics, classification, and outcomes of patients with PV, ET, or primary MF in the French PH registry. Measurements and Main Results: Ninety patients with MPN (42 PV, 35 ET, 13 primary MF) presented with precapillary PH with severe hemodynamic impairment, with a median mean pulmonary arterial pressure and pulmonary vascular resistance of 42 mm Hg and 6.7 Wood units, respectively, and impaired clinical conditions, with 71% in New York Heart Association functional classes III/IV and having a median 6-minute-walk distance of 310 m. Half of the patients were diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 PH. MF was preferentially associated with group 5 PH, whereas PV and ET were generally related to CTEPH. Proximal lesions were diagnosed in half of the patients with CTEPH. Thromboendarterectomy was performed in 18 selected patients with high risk of complications (5 early deaths). Overall survival at 1, 3, and 5 years was 67%, 50%, and 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively. Conclusions: PH is a life-threatening condition potentially occurring in MPN. There are multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH. Physicians should be aware that PH strongly affects the burden of patients with MPN, especially in group 5 PH, with unknown pathophysiological mechanisms | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a chronic thromboembolic pulmonary hypertension | |
| 650 | 4 | |a essential thrombocythemia | |
| 650 | 4 | |a myeloproliferative neoplasms | |
| 650 | 4 | |a polycythemia vera | |
| 650 | 4 | |a pulmonary hypertension | |
| 700 | 1 | |a Thoré, Pierre |e verfasserin |4 aut | |
| 700 | 1 | |a Mignard, Xavier |e verfasserin |4 aut | |
| 700 | 1 | |a Jaïs, Xavier |e verfasserin |4 aut | |
| 700 | 1 | |a Boucly, Athénaïs |e verfasserin |4 aut | |
| 700 | 1 | |a Jevnikar, Mitja |e verfasserin |4 aut | |
| 700 | 1 | |a Seferian, Andrei |e verfasserin |4 aut | |
| 700 | 1 | |a Jutant, Etienne-Marie |e verfasserin |4 aut | |
| 700 | 1 | |a Cottin, Vincent |e verfasserin |4 aut | |
| 700 | 1 | |a Fadel, Elie |e verfasserin |4 aut | |
| 700 | 1 | |a Simonneau, Gérald |e verfasserin |4 aut | |
| 700 | 1 | |a Savale, Laurent |e verfasserin |4 aut | |
| 700 | 1 | |a Sitbon, Olivier |e verfasserin |4 aut | |
| 700 | 1 | |a Humbert, Marc |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t American journal of respiratory and critical care medicine |d 1994 |g 208(2023), 5 vom: 01. Sept., Seite 600-612 |w (DE-627)NLM074657305 |x 1535-4970 |7 nnns |
| 773 | 1 | 8 | |g volume:208 |g year:2023 |g number:5 |g day:01 |g month:09 |g pages:600-612 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1164/rccm.202210-1941OC |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 208 |j 2023 |e 5 |b 01 |c 09 |h 600-612 | ||