Doctor Mask

Several questions about Cor Triatriatum.?

My son is 15 mos. old right now, and was diagnosed as having Cor Triatriatum when he was 6 weeks old. They did see the membrane blocking his left atrium the day after he was born, because he also had VSD, the VSD has since healed, but I have several questions regarding his heart condition. I keep hearing that its a rare condition (my son’s pediatrician had to look up the term!) How rare is it? Secondly, as of our last appt. (Nov. 2006) the membrane that is in his left atrium was not disturbing or interupting his blood flow at all, and so his Dr. said they weren’t going to do anything until they needed to do something, but how long can this membrane be in his heart w/o causing any damage? I know that if its not impeding blood flow they’re not going to fix it, but how long can he stay like that? I’m afraid of the answer, but in order to fix cor triatriatum its Open Heart Surgery isn’t it?

Heather – It is true that cor triatriatum is a rare condition. Various hospitals have reported on how often they see it, and the prevalance is probably about 6 cases per million live births. The defect is not genetically transmitted and there have been no reports of families with multiple cases.

The problem with cor triatriatum arises when it obstructs the flow of oxygenated blood returning from the lungs and reentering the heart. However, the range of obstruction is very wide. On the one hand, people may present within the first days of life if there is NO way for the blood to get through the membrane. On the other hand, there are reports of people surviving into late adulthood (in one case the patient’s age was in the 70s) before the diagnosis is even made.

If there is no evidence that the membrane is causing significant obstruction, it is probably reasonable to just monitor with regular echocardiograms. Some cardiologists will “challenge” the patient by giving IV fluids and/or medicines to speed up the heart during an echocardiogram to see if this “unmasks” obstruction that could be caused by the membrane during physical activity.

If you have concerns, I would ask your son’s pediatrician for a referral to a pediatric cardiologist (or just seek a second opinion from a cardiologist on your own). Physicians will rarely take offense to such a request. The important thing is to make sure that YOU are comfortable with the plan! Right now, most cases of cor triatriatum are fixed with surgery, although there have been occasional reports of cases in which the repair was done with catheters. Whether this would be an option really depends on the specific configuration of your son’s heart and should be determined by a specialist!

Hope that helps some! Good luck to you both!

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