OrtnerâÂÂs Syndrome as First Clinical Presentation of Severe Pulmonar Arterial Hypertension

Abstract

A 40 year old female, non-smoker with a past medical history of urticaria and allergic rhinitis presented with hoarseness of voice of 3 months duration without any accompanying symptoms or limitation of daily activities. Direct laryngoscopy identified palsy of the left vocal cord. The magnetic resonance imaging of the chest indicated abnormal signal intensity in the left vocal cord. The thoracic computed tomography revealed only an enlargement of the pulmonary artery, while transthoracic echocardiography estimated systolic pulmonary artery pressure of 75 mmHg. Pulmonary function tests were compatible with pulmonary vascular disease and cardiopulmonary exercise test showed exercise limitation of cardiocirculatory origin (maximum O2 consumption 60% of pred.). Catheterization of the right heart showed precapillary pulmonary hypertension (mean pulmonary artery pressure 40 mmHg) with a positive response to vasoreactivity test. A diagnosis of idiopathic pulmonary arterial hypertension (IPAH) was made, with hoarseness of voice as the only manifestation of the disease. The hoarseness of voice is believed to be the result of the compression of the left recurrent laryngeal nerve between aorta and the dilated pulmonary artery. This is an unusual case of cardiovocal syndrome (or Ortner’s syndrome) in a patient with IPH who had no perception of dyspnea or limitation of daily activities.