Abstract Introduction Ebstein anomaly is a rare congenital malformation of the tricuspid valve and right ventricle, characterized by apical displacement of the septal and posterior tricuspid leaflets. This results in atrialization of part of the right ventricle and may cause significant tricuspid regurgitation. Associated findings frequently include an atrial septal defect or patent foramen ovale, which may cause right-to-left shunting and cyanosis. Clinical presentation is highly variable: ranging from severe cyanosis and heart failure in neonates to asymptomatic adults. Report of case(s) A 67-year-old-man with history of Factor V Leiden and deep venous thromboembolisms was referred to Sleep clinic for complaints of insomnia. His Epworth Sleepiness Scale was 1. His Insomnia Severity Index was 22. Polysomnogram demonstrated central sleep apnea (CSA) with an apnea-hypopnea index of 21/hr. As part of the evaluation for his CSA, he underwent a cardiac evaluation. Echocardiogram showed a possible right ventricular apical mass. Subsequent cardiac MRI imaging revealed apical displacement of the septal leaflet of the tricuspid valve, morphologically consistent with an Ebstein anomaly, from which he had remained asymptomatic throughout his life. He underwent a CPAP titration and was prescribed CPAP 7cm H2O; however, he was unable to tolerate CPAP therapy and ultimately lost to follow-up. Conclusion Ebstein anomaly is the most common congenital tricuspid valve abnormality, but remains rare, accounting for less than 1% of congenital heart defects. To our knowledge this is the first case report describing central sleep apnea in a patient with Ebstein anomaly. CSA is most frequently associated with cardiovascular conditions like heart failure and atrial fibrillation, and neurological conditions including stroke, brainstem lesions, and chronic opioid use. CSA is prevalent in adults with congenital heart disease. In addition to ventilatory instability, impaired cardiac output, and altered chemoreflex sensitivity that are seen in the setting of ventricular dysfunction, patients with congenital heart disease may have additional susceptibility to developing CSA from residual shunts, cyanosis, and altered pulmonary vascular hemodynamics. This patient was asymptomatic from his congenital heart condition and his Ebstein anomaly was diagnosed incidentally during cardiac work up of his central sleep apnea. Support (if any)
Loui et al. (Fri,) studied this question.