Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.
OBJECTIVE
Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France.


METHODS
This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of >3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines.


RESULTS
Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 +/- 17 mm Hg and TPR 1,007 +/- 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00).


CONCLUSIONS
This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.
Hachulla
Eric
E
Hôpital Claude-Huriez, Lille, France. ehachulla@chru-lille.fr
Gressin
Virginie
V
Guillevin
Loïc
L
Carpentier
Patrick
P
Diot
Elisabeth
E
Sibilia
Jean
J
Kahan
André
A
Cabane
Jean
J
Francès
Camille
C
Launay
David
D
Mouthon
Luc
L
Allanore
Yannick
Y
Tiev
Kiet Phong
KP
Clerson
Pierre
P
de Groote
Pascal
P
Humbert
Marc
M
D000328:Adult; D000368:Aged; D000465:Algorithms; D006328:Cardiac Catheterization; D042241:Early Diagnosis; D015150:Echocardiography, Doppler; D005260:Female; D005602:France; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology*; D008297:Male; D008403:Mass Screening / Q000379:methods; D008875:Middle Aged; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*