Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses.
A subgroup of patients with idiopathic pulmonary arterial hypertension (IPAH) has severely reduced diffusing capacity of the lung for carbon monoxide (DLCO) and poor prognosis. Their characteristics are currently unknown. The aim of this study is to contrast clinical characteristics and treatment responses of IPAH-patients with a severely reduced and more preserved DLCO. Retrospectively, 166 IPAH patients were included and grouped based on a DLCO cut-off value of 45% pred (IPAH(<45%) and IPAH(≥45%)). Clinical characteristics, treatment responses and survival were compared. IPAH(<45%) were older, more often male, had a more frequent history of coronary disease and a higher tobacco exposure. Forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity, total lung capacity and alveolar volume values were slightly lower and computed tomography scan abnormalities more prevalent in patients with a low DLCO. Age and number of pack years were independently associated with DLCO < 45% pred. IPAH(<45%) showed no different haemodynamic profile, yet worse exercise performance and a worse survival rate, which were both related to age, sex and the presence of coronary disease. To conclude, a severely reduced DLCO in IPAH is associated with advanced age and a greater tobacco exposure. These patients have a worse exercise performance despite a similar hemodynamic profile. We confirm the decreased survival in this patient group and now show that this poor outcome is related to age, sex and the presence of coronary disease.
Institute for Cardiovascular Research, VU University Medical Center, Amsterdam.
Pia
P
Trip
Esther J
EJ
Nossent
Frances S
FS
de Man
Inge A H
IA
van den Berk
Anco
A
Boonstra
Herman
H
Groepenhoff
Edward M
EM
Leter
Nico
N
Westerhof
Katrien
K
Grünberg
Harm-Jan
HJ
Bogaard
Anton
A
Vonk-Noordegraaf
D000328:Adult; D000368:Aged; D002248:Carbon Monoxide / Q000737:chemistry; D003324:Coronary Artery Disease / Q000150:complications; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology* / Q000628:therapy*; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011650:Pulmonary Alveoli / Q000502:physiology; D011653:Pulmonary Diffusing Capacity; D011658:Pulmonary Fibrosis / Q000473:pathology; D012189:Retrospective Studies; D012907:Smoking / Q000009:adverse effects; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity; D016896:Treatment Outcome; D014797:Vital Capacity
D002248:Carbon Monoxide