Evaluation of treatment with bosentan in patients with carcinoid heart disease: single center study.
BACKGROUND
The primary aim of this study was to evaluate a combined therapeutic intervention, including the dual endothelin receptor antagonist bosentan, in patients with carcinoid heart disease (CaHD). The efficacy of the treatment protocol was investigated using serological, echocardiographic, and clinical markers.


METHODS
Since 2003, 40 patients with neuroendocrine tumours were identified; 14 had echocardiographic findings consistent with CaHD. Six of the 14 patients with CaHD and a New York Heart Association (NYHA) functional class >or= III received bosentan and were eligible for inclusion in this study.


RESULTS
N-terminal pro-brain natriuretic peptide (NT-pro-BNP) had decreased 6 months after treatment with bosentan (median: 646 pg/ml vs. 400.5 pg/ml; p = 0.02); the right ventricular systolic pressure had decreased after 3 and 6 months (median: 69 mmHg vs. 61 mmHg, p = 0.02; median: 69 mmHg vs. 48.5 mmHg, p = 0.02); the 6-minute walk distance (6MWD) had significantly improved after 3 and 6 months of treatment (median: 293.5 vs. 406.5 m; p = 0.02; median: 293.5 vs. 578.5 m; p = 0.02). The NYHA functional class improved in 5/6 patients receiving bosentan.


CONCLUSIONS
Combined treatment with bosentan is effective in patients with CaHD, based on functional class, 6MWD, and NT-pro-BNP. Further clarification of the CaHD fibrosis pathogenesis is needed to facilitate development of targeted antifibrotic therapeutic agents.
Lafaras
Christos T
CT
Theagenion Cancer Hospital, Thessaloniki, Greece. iatros@the.forthnet.gr
Mandala
Eudokia M
EM
Platogiannis
Dimitrios N
DN
Saratzis
Athanasios N
AN
Barbetakis
Nikolaos G
NG
Paraskevopoulos
Panagiotis P
PP
Ilonidis
George C
GC
Bischiniotis
Theodoros S
TS
D000368:Aged; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D000077300:Bosentan; D002275:Carcinoid Heart Disease / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy*; D003131:Combined Modality Therapy; D015150:Echocardiography, Doppler; D065128:Endothelin Receptor Antagonists; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D005770:Gastrointestinal Neoplasms / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010051:Ovarian Neoplasms / Q000188:drug therapy* / Q000473:pathology; D010446:Peptide Fragments / Q000097:blood; D013449:Sulfonamides / Q000009:adverse effects / Q000627:therapeutic use*
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D010446:Peptide Fragments; D013449:Sulfonamides; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000077300:Bosentan