Current Management of Ebstein’s Anomaly in the Adult

Abstract

Ebstein's anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein's anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. Pre-pregnancy evaluation should be considered in all Ebstein's anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. Most acyanotic Ebstein's anomaly patients are able to tolerate pregnancy with manageable or no complications. Those patients with mild anomalies and no right heart dilation can participate in sports whereas those with severe anomalies are discouraged from competitive sports. Physical activity as tolerated is important in all patients with adult congenital heart disease. Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery. Longitudinal clinical follow-up in all Ebstein's anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. This should be ideally performed in collaboration with an adult congenital heart center of excellence.

Wide complex tachycardia in a patient with Ebstein's anomaly

“…Figure (B) demonstrates sinus rhythm with a normal PR interval and fractionated QRS complex, known as a “splinter pattern,” typical among patients with Ebstein's anomaly . This bizarre QRS morphology is thought to be due to either infra‐Hisian conduction disease or abnormal activation of the atrialized right ventricle.…”

“…While Wolff–Parkinson–White syndrome is commonly associated with Ebstein's anomaly, BBR‐VT has only rarely been reported in this disease . Presently, we report a case of BBR‐VT with RBBB pattern, which is even less common.…”

Peer Review #1 of "Recent advances and challenges on application of tissue engineering for treatment of congenital heart disease (v0.2)"

“…Here the right ventricle is usually hypoplastic. It would usually be comorbid with a patent ductus arteriosus [61].…”

“…Done for patients with a hypoplastic right ventricle. The right atrium is surgically connected to the pulmonary artery [61].…”

Ebstejn's anomaly in patients perioperative period during a non-cardiac surgery operation

“…Preporuke kardioloških udruženja su uvođenje obavezne profilakse infektivnog endokarditisa i tromboembolijske 11,12,13 profilakse. Izbor vrste anestezije kada god je moguće su regionalne tehnike anestezije -spinalna, epiduralna i kombinovana spinalno-epiduralna.…”