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J Thorac Cardiovasc Surg 2009;137:760-761
© 2009 The American Association for Thoracic Surgery
Brief Communication |
a Department of Cardiothoracic Surgery, Medical University of Vienna, Vienna, Austria
b Department of Cardiology, Medical University of Vienna, Vienna, Austria
c Department of Dermatology, Medical University Tübingen, Germany
Received for publication November 11, 2007; revisions received March 3, 2008; accepted for publication March 23, 2008. * Address for reprints: H. J. Ankersmit, MD, Department of Cardiothoracic Surgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. (Email: hendrik.ankersmit@meduniwien.ac.at).
| The first 20% of the full text of this article appears below. |
Congenital type II atrial septal defect (ASD) is associated with precapillary pulmonary hypertension (PAH) in roughly 10% of cases.1
Principally, closure of the shunt lesion is recommended, and large ASDs must be repaired in early childhood to prevent Eisenmenger's syndrome. Once severe pulmonary hypertension or Eisenmenger's syndrome has developed, ASD closure is problematic due the increased risk of right ventricular failure and pulmonary hypertensive crisis.2
At this stage, heart–lung transplantation is the only surgical option. Nonsurgical strategies include supplemental oxygen, digitalis, anticoagulation, and vasodilator treatments.
In contrast to the traditional rule of inoperability of an ASD with severe pulmonary hypertension, single case reports have demonstrated that surgical correction of an ASD is feasible but requires longtime pre- and postoperative treatment with vasodilators.3-5
Endothelin receptor antagonists are a new class of vasoactive substances, some of which (eg, bosentan) are orally active. Bosentan has been shown to lower pulmonary artery pressure (PAP) and to induce reverse remodeling of the pulmonary arteries.
We report the case of a patient with type II ASD and severe
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