40. Hoarseness of voice as a primary presentation of pulmonary hypertension: A rare case of Ortner’s (cardio-vocal) syndrome

Abstract

Ortner’s (cardio-vocal) syndrome is a rare disease entity that involves compression of the left recurrent laryngeal nerve due to enlargement of cardiovascular structures. The syndrome was first described by Norbert Ortner (an Austrian physician) in 1897 and is usually reported to be associated with mitral stenosis. However, we report a rare case when a 20-year-old girl presented with hoarseness of voice and was found to have an enlarged main pulmonary trunk due to pulmonary hypertension that was compressing the left recurrent laryngeal nerve. The case highlights a rare presentation of a relatively common disease and stresses the importance of detailed cardiac workup in a patient with hoarseness of voice. We present the case and review the literature. A 20-year-old lady was referred from primary health center due to complaints of hoarseness of voice for the last four months. She reported only mild exertional palpitations and shortness of breath. She did not report any previous cardiac problem in childhood. Examination revealed a loud pulmonary component of 2nd heart sound, a pan-systolic murmur at the tricuspid area, and a palpable thrill. There was no evidence of right heart failure or any stigmata of connective tissue disease. Electrocardiography showed sinus rhythm with dominant R-wave in lead V1 indicating right ventricular hypertrophy. Laboratory tests did not reveal any abnormality. 2-D transthoracic echocardiograhy revealed normal left ventricle in size and function, right ventricular hypertrophy (6 mm thickness of free wall), mild to moderate tricuspid regurgitation, pulmonary hypertension (pulmonary pressure 60–65 mmHg) and an enlarged main pulmonary trunk. Computed tomography (CT) of the chest showed dilated main pulmonary trunk (max diameter 42 mm). An angiogram excluded pulmonary emboli. The patient was also seen by the Otolaryngology team in view of the main complaint of hoarseness of voice. Indirect laryngoscopy showed left vocal cord palsy. CT scan of the neck excluded a mass lesion or enlarged lymph nodes. We concluded that the enlarged pulmonary trunk secondary to pulmonary hypertension has compressed the left recurrent laryngeal nerve as it passes between the aorta and the pulmonary artery, thus causing left vocal cord paralysis. The case has been referred to the tertiary center where she will be reviewed by the pulmonary hypertension team and the thoracic surgeons, and will be followed in our clinic. Ortner’s syndrome is rare. Although aortic aneurysms are the most common risk factor, pulmonary hypertension with dilated pulmonary artery can compress the left recurrent laryngeal nerve and cause hoarseness of voice. The case clearly identifies the importance of detailed cardiac workup in patients presenting with hoarseness. Early diagnosis helps in guiding immediate treatment and may alleviate the distressing symptoms.