But under most circumstances, most patients with Ebstein's anomaly will benefit from surgery at some point in time.ĭr Rihal: In the range of operative repairs that you perform, is this on the relatively straightforward end of the spectrum or the more complex end of the spectrum?ĭr Dearani: It is on the complex end of the spectrum. Fortunately, frank right-sided heart failure is very rare, and that would probably be the patient in whom conventional surgery is high risk, or, in some situations, it may be too late. The patient might take a few more naps or go to bed a little earlier. But many patients are not symptomatic and if you take the element of cyanosis out of it, probably the most common symptom is fatigue, and it can be very subtle. Clearly, when the patient is symptomatic, surgery is indicated. The timing of surgery with Ebstein's can be very difficult. This has been the topic of conversation and still is frequently the "buzz topic" at many of our meetings. Once Ebstein's anomaly has been identified, at some point in time most paients will need an operation.ĭr Rihal: Is it ever too late to operate on them? What is the ideal timing of surgery?ĭr Dearani: You have asked a sensitive question. Repair of Ebstein's Anomalyĭr Rihal: What proportion of patients with this anomaly eventually undergo surgery?ĭr Dearani: Eventually most of them will need surgery for symptomatic tricuspid regurgitation, right-sided heart failure, or either cyanosis or paradoxical embolism, which we may see in the adult patient. Fortunately, that is the exception, not the rule. So it may remain undetected until arrhythmias become apparent, or when the rare patient has frank right-sided heart failure. But when the atrial septal defect is small (eg, a patent foramen ovale) or absent, the patients have a delayed presentation because, as we know from many other disease entities, tricuspid regurgitation can be tolerated for extended periods of time. Newborns have right-to-left shunting and they are desaturated, and this usually prompts medical attention. In children, the most common clinical presentation is cyanosis, because an atrial septal defect is commonly present. Then, of course, there is everything in between. The presentation ranges from a highly symptomatic newborn who is ventilator dependent and needs urgent surgery to the other end of the spectrum, which is the 70-year-old who has made it through all of his or her adult years, who then has a chest X ray showing some cardiac enlargement, which prompts a cardiac workup, and it's a mild to moderate form of Ebstein's anomaly. What are the common presentations?ĭr Dearani: This is the most fascinating congenital anomaly. This has made it very challenging for cardiologists and surgeons to get into a comfort zone, because every patient is a little bit different.ĭr Rihal: We typically we think of Ebstein's anomaly as presenting in infancy or childhood, but over the course of my career, I have stumbled across a couple of patients in their adult years with previously undiagnosed Ebstein's anomaly. In contrast to other congenital defects such as a standard atrial septal defect or ventricular septal defect, which are very reproducible, the anatomic findings from patient to patient with Ebstein's anomaly differ significantly. From patient to patient, the anatomy differs. There is often a hole in the heart, usually an atrial septal defect or a patent foramen ovale. It is frequently associated with such arrhythmias as Wolff-Parkinson-White (WPW), and, with time, atrial arrhythmias. ![]() As a result, we end up with a myopathy of the right ventricle, a dysplastic, nonfunctional tricuspid valve, and other associated defects that can be related. But the tricuspid valve develops from the right ventricle, and the right ventricle is the underlying abnormality. Can you refresh our memory–what is Ebstein's anomaly? Why is it important?ĭr Dearani: Ebstein's anomaly is a malformation of the right ventricle, and most of the attention focuses on the tricuspid valve. This is an anomaly that we in the cardiology world have been learning about since the early days. It's nice to be here.ĭr Rihal: We are here to talk about Ebstein's anomaly. Joe, welcome.ĭr Joseph Dearani: Thank you, Chet. Dr Joseph Dearani is chair of the division of cardiovascular surgery at Mayo Clinic, Rochester, and he is a specialist in pediatric and adult congenital heart surgery. I have a very special guest with me today.
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