I am a 26 year old non-smoking, non-drinking female. I was diagnosed with Scimitar Syndrome at age 6 when they noticed I still had a heart murmor. A heart cath was done at that time. They came to the conclusion that since I was asymptomatic at the time, they'd just "sit and wait". Since then I've had routine EKGs, chest X-rays and echos. I recently had an echo done in April that showed mild to moderate tricuspid regurgitation, mild to moderate pulmonary hypertension, mild flattening of the interventricular septum, mild enlargement of the right atrium, mild to moderately enlarged right ventricle, and a trace of mitral regurgitation. Since then, I've had a spiral CT done which confirmed the Scimitar Syndrome. It showed right lung drainage to the inferior vena cava and the right atrium (via two separate passageways). My cardiologist would like to refer me to a pediatric cardiologist for a heart cath and surgery to correct.
The initial thought was to refer me to Denver Children's Hospital. However, I am very hesitant to go there due to their recent cardiology department issues (they've been in the paper a lot and their infant death rate following/during open-heart surgery was almost twice the national average). I've done a lot of reading on this and found a chat board online for people with Scimitar Syndrome. Everything I've read so far says that Philidelphia or Boston are the places to go to have this surgery. I guess I'm writing to you to ask for help. My current cardiologist is trying to work an insurance referral to cover wherever I want to go for the surgery. The problem is I don't know. This whole thing really scares me. I'm only 26, my husband and I just bought our first house, and we've got so much left to do still. Do you have any ideas as to how I could find the hospital that's done this procedure the most times successfully?
I have so many other questions... will my heart go back to normal after the surgery? How about my lungs? Will the hypertension go away? Will I get to keep both my lungs (a lot of the articles I've read said they often remove the right lung - though that is on infants)? Will I be able to have the children after the surgery? Can I pass on Scimitar Syndrome? How dangerous is the surgery? What are my chances for survival?
I don't expect you to answer all of my questions, but I would greatly appreciate any help you could give me - especially in finding a surgeon.
"Scimitar syndrome" is a complex consisting of partial anomalous pulmonary venous return, hypoplasia of the right ventricle, dextroposition of the the heart, pulmonary parenchymal abnormalities and anomalous system supply to the lower lobe of teh right lung from the aorta or its main branches. This complex has been called "scimitar syndrome" because of a characteristic finding on chest x-ray that looks like a scimitar. Surgical repair is the definative therapy for this syndrome and is relatively low risk.
As far a choosing a surgeon I would look at other things as well as mortality rates. Mortality rates may be higher in specialized hospitals because they are only treating the sickest patients. Therefore a high mortality rate by itself does not mean that a hospital or doctor is not good. I don't know any pediatric cardiac surgeons in Philadelphia or Boston but can give you the name of the one here. His name is Dr. Roger Mee and he is very well known and respected nationally and internationally. You can call make an appointment with him by calling 1-800-CCF-CARE and ask for extension 46915. Best wishes.
jen how are you doing? where did you decide to go? my daughter was born with scimitar and other cardiac anomalies. we are at boston now. she was born in january. they keep telling us that this syndrome is rare but people do live with it without even knowing. our issue is that she has other things wrong and we are living without knowing her chances of longevity with all her anomalies together,
i would be interested in haering about your situation.
keep in touch, ***@****
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