Hoarseness as a Rare Symptom of Idiopathic Pulmonary Arterial Hypertension Due to Ortner Syndrome: A Case Report

Objective: Rare disease Background: Idiopathic pulmonary arterial hypertension (IPAH) is the most common type of pulmonary arterial hypertension, affecting about 48 to 55 per million adults. Common symptoms include easy fatigability, dyspnea, chest pain, and syncope. Although hoarseness is a rare symptom, several, but limited, cases have been reported to be associated with IPAH due to Ortner syndrome, which is caused by paralysis of the left laryngeal nerve due to enlarged cardiovascular structures. Case Report: A 32-year-old man was admitted to the Cardiology Department with suspicion of pulmonary arterial hypertension. Paralysis of the left vocal fold was diagnosed, probably due to compression of the left laryngeal nerve, caused by cardiopulmonary structure (Ortner syndrome). Echocardiography revealed a high probability of pulmonary hypertension with dilatation of the pulmonary artery. Right heart catheterization indicated pre-capillary pulmonary hypertension. Then, several additional tests were performed to clear the causes of pulmonary hypertension, and a diagnosis of IPAH was made. Combined therapy with sildenafil, bosentan, and treprostinil was performed. After several months of therapy, the patient reported improvement in exercise tolerance and hoarseness remission, which seems to be a permanent effect, with no recurrence to date. Conclusions: This case report highlights the role of broad differential diagnosis of hoarseness, emphasizing rare cardiovascular diseases, such as IPAH. Hoarseness caused by the left recurrent laryngeal nerve palsy, resulting from pulmonary artery dilation, one of the most common Ortner syndrome causes, can be treated effectively with appropriate IPAH treatment, by reducing the diameter of the pulmonary trunk.