PURPOSE: Pulmonary artery aneurysm (PAA) is a rare disease. Proximal PAA occasionally induces some serious complications, including compression of the bronchus, dissection and/or rupture of the pulmonary artery, as well as a thrombus of the pulmonary artery. Recently, with the development of new drugs, the prognosis of patients with PAH has improved. We present a case of a patient with proximal PAA with severe PAH and successfully treated with treprostinil
METHODS: A case study. A 22 years old woman with idiopathic PAH had RHC performed in 11/2001 with MAP of 46 mmHg. She started treatment with inhaled prostacycline. After 3 months, RHC was repeated for progression of dyspnoea (MAP increased to 53 mmHg). In 2003, the patient was included in the SUPER study (sildenafil) and a follow up RHC was performed with MAP 48 mmHg. Since the patient was stable at WHO II, echocardiography examination was performed every 6 months. In 2007, PAA with a diameter of 6.42 cm was diagnosed by CT and ECHO. As the patient was stable, with no severe symptoms, stentgraph was technically impossible and cardiac surgery unsafe due to her PAH, continuous treatment with s.c. treprostinil was initiated.
RESULTS: The patient has been stable on s.c. treprostinil for 4 years, without any complications and with decreased PAA diameter of 5.3 cm
CONCLUSIONS: PAA combined with PAH is a rare but very serious condition. Treprostinil (s.c.) represents a possible treatment in patients mild symptoms and PAA with PAH.
CLINICAL IMPLICATIONS: Prostacycline treatment in patient with PAA and PAH
DISCLOSURE: The following authors have nothing to disclose: Hikmet Al-Hiti, Jiri Kettner, Vojtech Melenovsky, Josef Kautzner
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