Background: Ebstein's anomaly is a rare and complex heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly often is diagnosed prenatally, due to its severe cardiomegaly. Some individuals with this anomaly do not experience complications until adulthood and even then, they have mostly minor complaints like exercise intolerance. An atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases.
Case presentation: This report presents a case of surgical intervention for a 38-year-old female with Ebstein's anomaly symptomatic with moderate-severe pulmonary regurgitation and foramen ovale apertum.
Conclusions: Ebstein anomaly is a complicated form of congenital heart disease with variable clinical presentations. The anesthetic plan must also focus on maintenance of RV function and avoidance of increase in PVR. Reversible causes of increased PVR, such as acidemia, hypoxemia, and hypercarbia must be avoided. Agents that lower PVR, such as nitrates, and nitric oxide may be beneficial in patients with severe pulmonary hypertension.
With a sound knowledge of the cardiac anatomy, accurate scheming of surgical outcomes, routine follow-ups, multidisciplinary team approach, and better management, an experienced center can ultimately improve the prognosis of such patients.
2. Hamaoka K, Onaka M, Tanaka T, Onouchi Z. Congenital ventricular aneurysm and diverticulum in children. Pediatr Cardiol 1987; 8: 169-75.
3. Sealy WC. The cause of the hemodynamic disturbances in Ebstein's anomaly based on observations at operation. Ann Thorac Surg 1979; 27:536-46
4. Reddy S, Sharma R. Ebstein's anomaly of tricuspid valve with rheumatic mitral stenosis. Int J Cardiol 2009; 33: 52-4.
5. Gentles TL, Calder AL, Clarkson PM, Neutze JM. Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve. Am J Cardiol 1992; 69: 377-81
6. Jayaprasad N, Thomas V, Madhavan S, Rajesh G, Francis J, Venugopal K. A rare association of Ebstein's anomaly of tricuspid valve with rheumatic mitral stenosis. Echocardiography 2007; 24:176
7. Fuster V, Ryden LE, Cannom DS, et al. ACC/AHA/ESC 2006 guidelines for the management of patients with atrial fibrillation: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to revise the 2001 guidelines for the management of patients with atrial fibrillation): Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation 2006; 114: e257-354.
8. Lazorishinets VV, Glagola MD, Stychinsky AS, Rudenko MN, Knyshov GV. Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly. Eur J Cardiothorac Surg 2000; 18: 487-90.
9. Brancaccio G, Chauvaud S, Carpentier A. Pre and Postoperative evaluation of the incidence of arrhythmia in patients undergoing corrective intervention for Ebstein anomaly. Ital Heart J Suppl 2000;1: 1173-9.
10. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC Jr. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg 1992; 104: 1195-202.
11. Vester EG. Clinical-electrophysiologic effects of magnesium, especially in supraventricular tachycardia. Herz 1997; 22: 40-50.
12. Sharpe MD, Dobkowski WB, Murkin JM, Klein G, Guiraudon G, Yee R. The electrophysiologic effects of volatile anesthetics and sufentanil on the normal atrioventricular conduction system and accessory pathways in Wolff-Parkinson-White syndrome. Anesthesiology 1994; 80: 63-70.
13. Elsten JL, Kim YD, Hanowell ST, Macnamara TE. Prolonged induction with exaggerated chamber enlargement in Ebstein's anomaly. Anesth Analg 1981; 60: 909-10.
14. Butterworth JF 4th, Prielipp RC, Royster RL, et al. Dobutamine increases heart rate more than epinephrine in patients recovering from aortocoronary bypass surgery. J Cardiothoracic Vasc Anesth 1992; 6:535- 41.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.