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Successful Two-Stage Repair Of Severe Pectus Excavatum And Aortic Insufficiency With Giant Aneurysm Of The Aortic Bulb In A Patient With Marfan Syndrome
Denise Marin1, Stefan Schulz-Drost2, Stephen Hohe1, Albert Schuetz1.
1Schoen Clinic Vogtareuth, Vogtareuth, Germany, 2Universitaetsklinikum Erlangen,Traumatology, Erlangen, Germany.

Background: Pectus excavatum (PE) is present in approximately two-thirds of patients with Marfan syndrome and is associated with congenital heart defects in 1.5% of these patients. Two possibilities exist to manage both lesions. First, there is the one-stage repair which carries the risk of excessive bleeding or wound infection. Second, there is the possibility for a two-stage repair, with the risk for aortic dissection between each procedure. We report on a patient with Marfan Syndrome who underwent successful two-stage repair of severe PE and then a Bentall procedure. Methods and Results: A 57 year old male with Marfan Syndrome and severe pectus excavatum (a) was admitted to our hospital with aortic insufficiency with an aortic bulb aneurysm. Transthoracic echocardiography showed an aortic valve insufficiency grade III-IV°. Computer tomography (CT) revealed a 65mm aortic bulb aneurysm displaced to the left hemithorax (b). The angle of the sternum and rib cage posed a problem to access the aorta and provide a safe operating field. Also, given the concerns regarding excessive bleeding and possible wound infections, we decided to perform a two-stage repair. First, the patient underwent a reconstruction of the PE with modified Nuss procedure in which the middle portion of the chest was straightened by using a titanium bar. Four months later we performed a successful Bentall procedure. To prepare the patient for cardiopulmonary bypass, the thorax was opened using an oscillating saw and the titanium bar was cut through with a side cutter. The aortic valve and aortic bulb were replaced with a 27mm valved-conduit (Medtronic ATS). The thorax was later closed conjoining the separated sternal halves were with titanium wires. The bar and osteosynthetic material were left in situ (d). The patient was extubated on the same day. Conclusions: Echocardiography and CT-scan examination aid the final decision as to the severity of cardiac involvement. We strongly believe a two-stage repair is more beneficial in cases of severe PE to avoid further complications during surgery.

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