04 February 2019 by Gulfer Okumus

10 years bosentan therapy in a patient with eisenmenger syndrome

Introduction:

Eisenmenger syndrome is the most severe and final form of shunt-associated pulmonary arterial hypertension (PAH). PAH secondary to systemic-pulmonary shunt occurs depending on the congenital heart disease CHD). The presence of PAH increases morbidity and reduces survival in patients with CHD. Some patients with Eisenmenger syndrome may be asymptomatic until adulthood without symptoms of pulmonary hypertension. Here, we report a case of Eisenmenger syndrome diagnosed 10 years ago and followed with a single PAH specific treatment.

Case:

A 43-years-old female patient with no previous complaint presented with exertional dyspnea in 2008. Trans thoracic echocardiography (TTE) showed a muscular type of ventricular septal defect (VSD), mitral regurgitation, severe pulmonary hypertension (systolic PAP: 120mmHg) and mild tricuspid regurgitation. She was desaturated in six minute walk test ( SpO2: 96%→ 77% ) and walk distance was 380 m. pro-BNP was 190 pg / mL. Functional capacity was III. In right heart catheterization, VSD, persistent left superior vena cava, precapillary severe pulmonary hypertension (mean PAP: 78mmHg) detected and pulmonary vascular resistance was 17 mmHg / lt / min. Bosentan was started as a monotherapy. During the follow-up period, clinical and FC improved and no side effect was observed with bosentan monotherapy. In the 10th year of the treatment, the FC of the patient is I. The current walking distance is 570 m and pro-BNP value is 98 pg / mL. Systolic PAB is 60 mmHg in TTE.

Conclusion:

PAH is a serious disease with poor prognosis and high mortality. However, with PAH specific treatment, satisfactory results can be obtained especially in patients with PAH associated CHD.

 

About the author


profile picture of Gulfer Okumus

Gulfer Okumus

Profesor doctor ( medical doctor)

Istanbul University, Istanbul Medical Faculty, Departmant of Pulmonary Disease

Turkey

Key Contributors

Gülfer Okumuş 1, Ayşegül Akçeli Şahin 1, Züleyha Bingöl 1, Ahmet Kaya Bilge 2, Murat İnanç 3 : 1. Istanbul University, Istanbul Faculty of Medicine, Department of Chest Diseases 2. Istanbul University, Istanbul Faculty of Medicine, Department of Cardiology 3. Istanbul University, Istanbul Faculty of Medicine, Department of Rheumatology


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