Marfan's syndrome: patients died young, mainly from heart problems.

Clinical bottom line (level 4)

Patients with Marfan's syndrome had a greatly shortened life expectancy. Most died from cardiac problems (mainly aortic dilatation).

Murdoch et al: New England Journal of Medicine 1972; 286 (15): 804-808

Expires December 2004

The study

Case series with ?objective ?blinded outcomes, not adjusted for confounding factors, not validated in an independent set of patients.

Setting: teaching hospital, USA

74 patients (aged ?, 65% male) with Marfan's syndrome (classical picture, or with a family member with Marfan syndrome, and a dislocated lens, aortic regurgitation or typical skeletal features) who had died

Outcomes studied:

cause of death: any cardiac problem
aortic dilatation or associated complication
aortic rupture or dissection
aortic dissection
unknown

Cause of death was determined by autopsy report or death certificate.

The evidence

outcome	time to outcome	number of patients/total number	% (95% CI)
cause of death: any cardiac problem	?	53/74	72% (61% to 82%)
aortic dilatation or associated complication	?	39/74	53% (41% to 64%)
aortic rupture or dissection	?	20/74	27% (17% to 37%)
aortic dissection	?	8/74	11% (3.7% to 18%)
unknown	?	18/74	24% (15% to 34%)

men: probability of surviving 16 years:
- age 10 years: 84%
- age 20 years: 60%
- age 30 years: 57%
- age 40 years: 50%
women: probability of surviving 16 years:
- age 10 years: 87%
- age 20 years: 84%
- age 30 years: 74%
- age 40 years: 34%

Comments

Are these results still true today? Patients in the study were form 1903 to 1972- life expectancy has increased over this time.

Citation

Murdoch JL, Walker BA, Halpern BL, et al: Life expectancy and causes of death in the Marfan syndrome. New England Journal of Medicine 1972; 286 (15): 804-808

_{Clinical Question.}

_Patient	_{Marfan's syndrome}
_{Intervention or Exposure}
_Outcome	_{life expectancy}