Targeted therapy is required for management of pulmonary arterial hypertension after defect closure in adult patients with atrial septal defect and associated pulmonary arterial hypertension.
BACKGROUND
Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate.


RESULTS
We identified 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certified PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy significantly increased systemic blood flow (Qs) from the baseline (from 3.3 ± 0.6 L/minute to 4.2 ± 1.0 L/minute, P < 0.05) with a significant improvement in the World Health Organization Functional Class (WHO-FC; from 2.8 ± 0.4 to 1.6 ± 0.5, P < 0.05). The hemodynamic data before and after ASD closure without targeted therapy showed further elevation of pulmonary vascular resistance shortly after ASD closure (678 dyne · s/cm(5) to 926 dyne · s/cm(5)) in 1 case, as well as after a long time since ASD closure (491.0 ± 53.7 dyne · s/cm(5) to 1045.0 ± 217.8 dyne · s/cm(5)) in 2 cases. This worsening was reversed after the targeted therapy, accompanied by an increase in Qs and an improvement in WHO-FC in all cases.


CONCLUSIONS
Targeted therapy should be added to ASD closure in adult patients with ASD-PAH.
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo.
Takeo
T
Fujino
Atsushi
A
Yao
Masaru
M
Hatano
Toshiro
T
Inaba
Hironori
H
Muraoka
Shun
S
Minatsuki
Teruhiko
T
Imamura
Hisataka
H
Maki
Koichiro
K
Kinugawa
Minoru
M
Ono
Ryozo
R
Nagai
Issei
I
Komuro
D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D054087:Atrial Septum / Q000601:surgery; D000077300:Bosentan; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods; D019468:Disease Management; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives; D017079:Exercise Tolerance; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000503:physiopathology / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D007564:Japan; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D012189:Retrospective Studies; D013449:Sulfonamides / Q000008:administration & dosage; D016896:Treatment Outcome; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000008:administration & dosage
D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; C048081:beraprost; D011464:Epoprostenol; D000077300:Bosentan