A rare presentation of pediatric cardiac disease - Cardiovocal syndrome

Cardiovocal syndrome, also known as Ortner's syndrome, is a rare cause of left recurrent laryngeal nerve (LRLN) paralysis due to cardiac disease. Hoarseness and/or stridor secondary to the LRLN paralysis are rare signs of pediatric heart failure.1-3 Here we report two pediatric cases of this syndrome with underlying cardiac failure. After medical and surgical management of congestive heart failure, stridor and hoarseness improved and both cases recovered normal vocal cord function. Case 1: A 6-month-old girl with no relevant past medical history was referred for suspicion of croup syndrome. She sometimes had hoarseness and mild stridor when crying, and she started sweating excessively on her head at the age of 3 months. Three days before visiting the hospital, she had cough, wheezing, stridor when crying, and progressive feeding difficulty. The infant presented with compensated shock and impending respiratory failure with resting stridor, poor peripheral perfusion, gallop rhythms, and hepatomegaly. Chest radiography revealed significant cardiomegaly with increased pulmonary vascular markings and left atrial (LA) enlargement (Figure 1a). An echocardiogram showed significant left ventricle (LV) dysfunction, LA/LV dilatation, and pulmonary hypertension (PH) (Figure 1b). She was successfully intubated without complications by a pediatric intensivist and transferred to the pediatric intensive care unit. She was diagnosed with idiopathic dilated cardiomyopathy (DCM) and started treatment for heart failure, including mechanical ventilation and inotropic support. She was extubated on day 7 of admission but immediately had respiratory distress and inspiratory stridor, so high flow therapy was applied. Flexible laryngoscopy revealed a paralyzed left vocal cord at a fixed abduction position (Figure 1c). She continued to be treated for DCM with aggressive medical management, and her cardiac function improved very slowly. The hoarseness of voice and stridor resolved within 1 month, and follow-up laryngoscopy showed recovery of normal vocal cords function. Case 2: A 1-month-old girl was admitted to our hospital for evaluation of congenital heart disease. She was diagnosed with a ventricular septal defect (VSD) in utero. After birth, she gradually developed progressive tachypnea, poor feeding, and poor growth. A few days before admission, hoarseness and stridor during feeding became prominent. On examination, the infant was mildly distressed with tachycardia. A pansystolic grade 3/6 murmur was heard in her left 5th intercostal space, and her extremities were cold and sweaty. Chest radiography showed moderate cardiomegaly with increased pulmonary vascular markings and left atrial enlargement, and an ECG revealed biventricular hypertrophy. Echocardiography revealed a large perimembranous VSD, mildly reduced LV function, and PH. Flexible laryngoscopy showed left-sided vocal cord paralysis. The operation was performed 2 weeks after hospitalization. In perioperative management, an appropriately-sized cuffed tracheal tube was selected, and respiratory support with high-flow nasal cannula was prophylactically provided after extubating with mild sedation. Her hoarseness persisted for 2 weeks postoperatively, but her respiratory condition and growth rate improved. Hoarseness and stridor grades were resolved and returned to normal over the next 4 weeks. Follow-up laryngoscopy at 2 months of age showed an improvement in paralysis. In 1897, Norbert Ortner described three adult patients with mitral stenosis and LRLN paralysis, and the mechanism of nerve compression between the aorta and the enlarged pulmonary artery associated with PH was considered. Cardiovocal syndrome causes common respiratory symptoms in children such as hoarseness and stridor, and many of them may be overlooked as colds or aspirations. The initial diagnosis in case 1 was croup syndrome. It has not been reported previously in pediatric dilated cardiomyopathy.4 No particular heart diseases correlate with cardiovocal syndrome, but significant congestive left heart failure with PH is one of its principal pathophysiologies.2, 5 We consider that this syndrome is underestimated in infants who have congestive heart failure, because minor traction on the LRLN may occur, but it seems to be well compensated and asymptomatic. Persistent hoarseness and stridor can be some of the key signs for diagnosing heart failure. It is important to research the patient's clinical history actively, and flexible laryngoscopy may be advocated. If left vocal cord paralysis is visualized, physicians need to suspect Ortner's syndrome and predict the potential of pulmonary artery dilatation and increased pulmonary artery pressure. If the patient undergoes cardiac surgery in the future, postoperative management should be performed with attention to the transient exacerbation of upper airway obstruction. Naoki Fujiwara drafted the article. Koji Kanno, Yuki Ami, Masahiro Nishibata and Akio Kato gave conceptual advice. All authors have read and approved the final article. Written consent was obtained from the parents for the publication of this article. The authors declare no conflict of interest.