Ruling out pulmonary artery hypertension (PAH) should be considered in all patients with recurrent laryngeal nerve palsy, especially cases without a clear cause, according to a recent study published in Cureus.
Idiopathic PAH “is a rare but important cause of left recurrent laryngeal nerve palsy in young patients, and confirming the presence of a dilated [pulmonary artery] is essential for diagnosis,” the authors wrote.
This case report describes a 40-year-old woman previously diagnosed with PAH who was scheduled for lung transplantation due to severe physical limitations and disease progression despite multiple treatment approaches, including epoprostenol, macitentan, selexipag, and treprostinil.
She was evaluated to rule out infection foci in the ears, nose, and throat as part of the preoperative studies. Physical examination did not yield any important findings in these areas. Paralysis of the left vocal fold was found incidentally during flexible laryngoscopy. A more exhaustive physical examination identified mild hoarseness and a shortened maximum phonation time of 10 seconds.
Read more about PAH comorbidities
Computed tomography with contrast enhancement revealed dilations of the right ventricle, pulmonary trunk, and both pulmonary arteries, which resulted in a narrowed space between the aortic arch and the pulmonary arteries. These findings led the healthcare team to suspect left recurrent laryngeal nerve compression secondary to her underlying disease, yielding a diagnosis of cardiovocal syndrome associated with idiopathic PAH.
The researchers acknowledged another 23 cases of cardiovocal syndrome secondary to PAH in the literature. There was no apparent sex-dependent distribution, but a higher ratio may be present in younger patients since all reports described individuals aged less than 50 years.
Ruling out PAH in patients with recurrent laryngeal nerve palsy and assessing the indemnity of this nerve in patients with a previous diagnosis of PAH are of great importance, the researchers wrote. Although the patient in this report did not wish to undergo treatment for speech improvement, this finding could potentially dictate therapeutic approaches in other similar cases.
“Based on a literature review of cardiovocal syndrome caused by [idiopathic PAH], appropriate interventions for [idiopathic PAH] may effectively treat hoarseness caused by left recurrent laryngeal nerve palsy,” the authors concluded.
Ohi K, Suzuki J, Ikeda R, Kakuta R, Katori Y. Cardiovocal syndrome associated with idiopathic pulmonary arterial hypertension: a case report and literature review. Cureus. 2022;14(7):e27070. doi:10.7759/cureus.27070