AORTIC INSUFFICIENCY AND OSTEOGENESIS IMPERFECTA

I am a 27 year old female who has Osteogenesis Imperfecta.(Type IV, I believe). I have had about 15-20 major fractures and I am 3 feet 11 inches tall. 3 years ago it was discovered that I have mitral valve prolapse in addition to Aortic Insufficiency. The latest test demonstrated a 2 - 2+ leakage rate. I am currently on 2.5 milligrams of Prinivil and am followed by a cardiologist once a year. I work full time and am constantly on the go with no restrictions placed on my exercise routine.

I am eager to find out information regarding the progression and treatment of this condition. When and if at what point is surgery required? What special considerations are necessary due to the OI? Prevention steps that I could be taking? Does exercise or increased heart rate affect this condition? Will I know if the leakage worsens( what symptoms) and how sudden can that happen? How often should I follow up with the cardiologist?
Other information regarding prevention, treatment or any other information that would be helpful.

I appreciate your support
Thank You
Lorriane Zelenz

[CCF CARDIO MD - DLB]

Connective tissue diseases  such as yours predispose to valve leakage. Medications like Prinivil are important to keep the blood pressure down and decrease the stress on the aortic valve. This makes the leakage much lesss likely to progress. Continued follow-up with your cardiologist is important to monitor the valve for deterioration - annually sounds reasonable, as long as you remain symptom free. If the leakage gets significantly worse, symptoms such as shortness of breath, particularly with physical exertion occur. A moderate degreee of exercise should not cause the valve leakage to worsen. Surgery would only be required if the leakage is severe (on an echocardiogram or cardiac catheterization) and if you have symptoms due to it. The OI does complicate surgery, especially in terms of wound healing, so it would be best to try to use medications to delay the need for surgery.

[janelle]

Dear Lorraine,
I am a 32 year old woman with mild OI. I had an aortic replacement in Feb of 99.  Thought you might want to know that there is some limited research in the medical data about OI and valve disease and surgery.  It sounds like your valve is doing well and hopefully you will not have to face replacement, but if it gets to that point, there are cardiac surgeons out there who have experience with the special complications OI patients can present.

I wish you the best.  If you'd like to chat, feel free to e-mail me.  ***@****

Janelle