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Vasoconstrictive signaling via endothelin receptors is not limited to primary pulmonary arterial hypertension, but has also been documented in secondary pulmonary hypertension due to congestive heart failure, including cardiac valve disease. The investigators aim to examine the clinical and physiologic effects of bosentan therapy in patients with secondary pulmonary hypertension due to severe, inoperable cardiac valve disease, using a single-center, prospective, open-label, non-randomized study of oral bosentan in outpatients with severe mitral stenosis due to childhood rheumatoid fever. Primary end-point will be exercise capacity at six months determined by six-minute walking distance and cardiopulmonary exercise testing. Secondary end-points will be symptomatic relief, echocardiographic left ventricular function and pulmonary pressure, serum pro-brain natriuretic peptide, and adverse events at six months.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| BOSENTAN | Drug | TWICE DAILY PERORAL BOSENTAN, 125 MG DAILY DIVIDED IN TWO DOSES DURING THE FIRST MONTH AND 250 MG DAILY DIVIDED IN TWO DOSES SUBSEQUENTLY. |
| Measure | Description | Time Frame |
|---|---|---|
| SIX MINUTE WALKING DISTANCE | CHANGE IN SIX MINUTE WALKING DISTANCE AFTER 6 MONTHS OF THERAPY COMPARED TO PRE-TREATMENT VALUE. | SIX MONTHS |
| MAXIMAL OXYGEN UPTAKE | CHANGE IN MAXIMAL OXYGEN UPTAKE AFTER 6 MONTHS OF THERAPY COMPARED TO PRE-TREATMENT VALUE. | 6 MONTHS |
| Measure | Description | Time Frame |
|---|---|---|
| ECHOCARDIOGRAPHIC PULMONARY PRESSURE | ECHOCARDIOGRAPHIC ASSESSMENT OF PEAK AND MEAN PULMONARY PRESSURE AT 6 MONTHS AFTER TREATMENT COMPARED WITH PRE-TREATMENT VALUES. | 6 MONTHS |
| ECHOCARDIOGRAPHIC LEFT VENTRICULAR FUNCTION |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| GEORGE VLACHOGIORGOS, MD PHD | Contact | 00306936192419 | georgevlacho@gmail.com |
| Name | Affiliation | Role |
|---|---|---|
| GEORGE VLACHOGIORGOS, MD PHD | GENERAL HOSPITAL OF CHALKIDA, GREECE | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| General Hospital of Chalkida | Recruiting | Chalcis | Evoia | 34100 | Greece |
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| ID | Term |
|---|---|
| D008946 | Mitral Valve Stenosis |
| D006333 | Heart Failure |
| ID | Term |
|---|---|
| D006349 | Heart Valve Diseases |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
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| ID | Term |
|---|---|
| D000077300 | Bosentan |
| ID | Term |
|---|---|
| D000096926 | Benzenesulfonamides |
| D013449 | Sulfonamides |
| D000577 | Amides |
| D009930 | Organic Chemicals |
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ECHOCARDIOGRAPHIC ASSESSMENT OF LEFT VENTRICULAR FUNCTION AT 6 MONTHS AFTER TREATMENT COMPARED WITH PRE-TREATMENT VALUES.
| 6 MONTHS |
| SERUM PRO-BNP | ASSESSMENT OF SERUM PRO-BRAIN NATRIURETIC PEPTIDE LEVELS AT 6 MONTHS AFTER TREATMENT COMPARED WITH PRE-TREATMENT VALUES. | 6 MONTHS |
| DYSPNEA | CHANGE IN BORG DYSPNEA INDEX COMPARED WITH PRE-TREATMENT VALUES. | 6 MONTHS |
| D001555 |
| Benzene Derivatives |
| D006841 | Hydrocarbons, Aromatic |
| D006844 | Hydrocarbons, Cyclic |
| D006838 | Hydrocarbons |
| D013450 | Sulfones |
| D013457 | Sulfur Compounds |
| D011743 | Pyrimidines |
| D006573 | Heterocyclic Compounds, 1-Ring |
| D006571 | Heterocyclic Compounds |