J Cardiovasc Ultrasound.  2011 Sep;19(3):159-162. 10.4250/jcu.2011.19.3.159.

Transient Use of Oral Bosentan Can Be an Additional Option to Reduce Pulmonary Arterial Hypertension in a Patient with Severe Pulmonary Arterial Hypertension Associated with Atrial Septal Defect

• Park YK
• Park JH
• Yu JH
• Kim JH
• Lee JH
• Choi SW
• Jeong JO
• Seong IW

• KMID: 1980373
• DOI: http://doi.org/10.4250/jcu.2011.19.3.159

Abstract

Atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) is thought to preclude shunt closure. However, there are several reports that vasodilator treatment is associated with good clinical outcome in these patients, recently. We report a case of good clinical outcome in a patient with ASD and severe PAH successfully treated with operative closure of ASD and subsequent use of oral bosentan medication. This case supports that the corrective repair of ASD and an oral bosentan treatment can be one of the treatment options in the selected patients with severe PAH associated with ASD.

Keyword

Atrial septal defect; Pulmonary arterial hypertension; Bosentan

MeSH Terms

Heart Septal Defects, Atrial
Humans
Hypertension
Hypertension, Pulmonary
Sulfonamides
Hypertension, Pulmonary
Sulfonamides

Figure

• 

  Fig. 1 The transthoracic echocardiogram shows markedly dilated right ventricle and dysfunction (A) end-diastole, and (B) end-systole. There is significant shunt between left and right atria through the septal defect and the measured defect size was 1.5 cm (arrows, C). The measured maximal tricuspid regurgitation velocity is 4.7 m/sec suggesting severe pulmonary arterial hypertension (estimated pulmonary arterial systolic pressure is 98 mmHg, D).

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  Fig. 2 The initial echocardiography reveals D-shaped left ventricle (A) end-diastole, and (B) end-systole. The follow-up transthoracic echocardiogram taken 31 months after the surgery demonstrates markedly decreased right ventricular size and disappeared right ventricular dysfunction (C) end-diastole, and (D) end-systole.

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