Clinical phenotypes and outcomes of precapillary pulmonary hypertension of sickle cell disease.
Eur Respir J
; 54(6)2019 12.
Article
in En
| MEDLINE
| ID: mdl-31537700
ABSTRACT
RATIONALE Precapillary pulmonary hypertension (PH) is a devastating complication of sickle cell disease (SCD). Little is known about the influence of the SCD genotype on PH characteristics. OBJECTIVES:
To describe clinical phenotypes and outcomes of precapillary PH due to SCD according to disease genotype.METHODS:
A nationwide multicentre retrospective study including all patients with SCD-related precapillary PH from the French PH Registry was conducted. Clinical characteristics and outcomes according to SCD genotype were analysed.RESULTS:
58 consecutive SCD patients with precapillary PH were identified, of whom 41 had homozygous for haemoglobin S (SS) SCD, three had S-ß0 thalassaemia (S-ß0 thal) and 14 had haemoglobin SC disease (SC). Compared to SC patients, SS/S-ß0 thal patients were characterised by lower 6-min walk distance (p=0.01) and lower pulmonary vascular resistance (p=0.04). Mismatched segmental perfusion defects on lung scintigraphy were detected in 85% of SC patients and 9% of SS/S-ß0 thal patients, respectively, and 50% of SS/S-ß0 thal patients had heterogeneous lung perfusion without segmental defects. After PH diagnosis, 31 patients (53%) received medical therapies approved for pulmonary arterial hypertension, and chronic red blood cell exchange was initiated in 23 patients (40%). Four patients were managed for chronic thromboembolic PH by pulmonary endarterectomy (n=1) or balloon pulmonary angioplasty (n=3). Overall survival was 91%, 80% and 60% at 1, 3 and 5â years, respectively, without influence of genotype on prognosis.CONCLUSIONS:
Patients with precapillary PH related to SCD have a poor prognosis. Thrombotic lesions appear as a major component of PH related to SCD, more frequently in SC patients.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pulmonary Embolism
/
Ventilation-Perfusion Scan
/
Hypertension, Pulmonary
/
Anemia, Sickle Cell
Type of study:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Adult
/
Aged
/
Female
/
Humans
/
Male
/
Middle aged
Country/Region as subject:
Europa
Language:
En
Journal:
Eur Respir J
Year:
2019
Document type:
Article
Affiliation country:
Francia