PMID- 7453227
OWN - NLM
STAT- MEDLINE
DCOM- 19810327
LR  - 20221207
IS  - 0022-5223 (Print)
IS  - 0022-5223 (Linking)
VI  - 81
IP  - 2
DP  - 1981 Feb
TI  - Surgical management of patients with the Marfan syndrome and dilatation of the 
      ascending aorta.
PG  - 180-6
AB  - Until recently, surgical correction of Marfan defects of the aortic root has been 
      undertaken with some hesitancy because of the high perioperative risk. The 
      Division of Medical Genetics at Johns Hopkins follows about 300 patients with the 
      Marfan syndrome, and during the past 8 years 13 of these were referred to the 
      senior author (V.L.G.) for aortic valve replacement and repair of the ascending 
      aorta. Preoperatively, four of the 13 patients were in New York Heart Association 
      (NYHA) Class IV (3/4 required emergency operation), three patients were in Class 
      III, and six were in Class II. The aortic diameter at the mid-valve level ranged 
      from 5.3 cm to 8.2 cm on M-mode echocardiography. The first two patients received 
      a separate Bjork-Shiley prosthesis and a woven Teflon graft and the last 11 
      patients had a composite valve-graft with direct coronary implantation. There 
      were no hospital deaths. Follow-up ranges from 6 months to 8.1 years and is 
      complete for all 13 patients (mean of 23 months). Two late deaths occurred 2 and 
      20 months postoperatively from presumed arrhythmia and two late deaths occurred 
      at 4 and 6 months from endocarditis. The actuarial survival rate at 3 years is 
      61%. Some pathologists claim that the incidence of dissection of the aorta does 
      not increase with increasing aortic dilatation in the Marfan patient. Six of our 
      13 patients, however, had aortic dissection at the time of surgery (two DeBakey 
      Type I, three Type II, and one Type III). With the low hospital mortality that 
      can now be achieved in the Marfan patient, and with the relatively high incidence 
      of clinically unrecognized dissection, we fell that strong consideration should 
      be given to earlier prophylactic repair in these patients. Operation may be 
      indicated even in the asymptomatic Marfan patient with an aortic root diameter 
      greater than 5.5 cm.
FAU - McDonald, G R
AU  - McDonald GR
FAU - Schaff, H V
AU  - Schaff HV
FAU - Pyeritz, R E
AU  - Pyeritz RE
FAU - McKusick, V A
AU  - McKusick VA
FAU - Gott, V L
AU  - Gott VL
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Thorac Cardiovasc Surg
JT  - The Journal of thoracic and cardiovascular surgery
JID - 0376343
SB  - IM
MH  - Adult
MH  - Aortic Dissection/surgery
MH  - Aorta/pathology/*surgery
MH  - Aortic Aneurysm/surgery
MH  - Aortic Valve/*surgery
MH  - Aortic Valve Insufficiency/surgery
MH  - Aortic Valve Stenosis/surgery
MH  - Blood Vessel Prosthesis
MH  - Dilatation, Pathologic
MH  - Female
MH  - Heart Valve Prosthesis
MH  - Humans
MH  - Male
MH  - Marfan Syndrome/*surgery
MH  - Middle Aged
MH  - Postoperative Complications
EDAT- 1981/02/01 00:00
MHDA- 1981/02/01 00:01
CRDT- 1981/02/01 00:00
PHST- 1981/02/01 00:00 [pubmed]
PHST- 1981/02/01 00:01 [medline]
PHST- 1981/02/01 00:00 [entrez]
PST - ppublish
SO  - J Thorac Cardiovasc Surg. 1981 Feb;81(2):180-6.