Management of Marfan Syndrome and Related Disorders

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Management of Marfan syndrome and related disorders

Authors: Michael J Wright, MBChB, MSc, Heidi M Connolly, MD, FACC, FASE
Section Editor: Harry C Dietz, MD
Deputy Editor: Susan B Yeon, MD, JD, FACC
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2020. | This topic last updated: Apr 03, 2018.
INTRODUCTION
The Marfan syndrome (MFS, MIM #154700) is an autosomal dominant condition with a reported incidence of 1
in 3000 to 5000 individuals. There is a wide range of clinical severity associated with MFS. Although many
clinicians view the disorder in terms of classic ocular, cardiovascular, and musculoskeletal abnormalities, these
patients also demonstrate significant involvement of the lung, skin, and central nervous system.
The management and prognosis of MFS and related disorders will be reviewed here. The genetics,
pathogenesis, clinical manifestations, and diagnosis of MFS and related disorders and issues related to MFS
in pregnancy are discussed separately. (See "Genetics, clinical features, and diagnosis of Marfan syndrome
and related disorders" and "Pregnancy and Marfan syndrome".)
OVERVIEW
The prognosis of patients with MFS has improved with the use of beta blockers, restriction of vigorous physical
exercise, routine and noninvasive monitoring of aortic size, and elective surgical repair of the aorta. The
physiologic changes that occur during pregnancy are associated with an increased risk of aortic dilation and
dissection and therefore require more intensive monitoring. (See "Pregnancy and Marfan syndrome".)
The 2010 American College of Cardiology/American Heart Association/American Association for Thoracic
Surgery (ACC/AHA/AATS) guidelines for thoracic aortic disease include recommendations for MFS, Loeys-
Dietz Syndrome and other genetic syndromes affecting the aorta [1]. These guidelines are similar to the
recommendations for MFS published in 2001 by a Task Force of the European Society of Cardiology [2].
AORTIC MONITORING
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Monitoring of the aortic diameter is recommended to identify patients at high risk for aortic dissection.
How to measure — The aortic diameter measurement technique applied to follow a given patient should be
noted and used consistently during serial follow-up with nomograms specific for that technique applied when
determining deviation from the appropriate age- and size-matched normal population (generally expressed as
a Z-score, which designates number of standard deviations from the mean). Since normal aortic dimensions
vary with age and body surface area, these parameters should be taken into account (figure 1A-B).
Measurement conventions for aortic diameter differ for echocardiography as compared with computed
tomography (CT) or magnetic resonance imaging (MRI) and the measurement convention may also differ
within modalities from one institution to another.
Multiple conventions are applied when measuring the maximal dimension of the aortic root by
echocardiography. Examples include the leading-edge-to-leading-edge technique in diastole (a common
practice among adult cardiologists) or the inner-edge-to-inner-edge technique in systole (a common practice
among pediatric cardiologists). Similar variability can be observed when applying CT or MRI measurements,
depending upon whether or not contrast agents are utilized. In general, the external diameter is expected to be
2 to 4 mm larger than the internal diameter. There is also variation in practice with regard to whether the
external or internal diameter is operative when considering thresholds for surgical repair. (See 'Indications in
MFS' below.)
For MFS, aortic diameter at the sinuses of Valsalva is the key measurement since this is the segment that
generally dilates initially and is at greatest risk for aortic dissection. This measurement can usually be
monitored by echocardiography. Greater length of aortic dilation (ie, to the aortic arch versus limited to the
sinuses of Valsalva) can be associated with a worse prognosis [3].
Monitoring MFS — As recommended in the 2010 ACC/AHA/AATS guidelines for thoracic aortic disease,
patients with MFS should have echocardiography performed at the time of diagnosis and six months later to
determine the aortic root and ascending aortic diameters and their rate of enlargement [1].
We suggest performing cross-sectional aortic imaging with CT or MRI at the time of initial evaluation to confirm
that the aorta measurements determined by echocardiography are not underestimated, and also to identify
aortic or vascular disease that is not appreciated by echocardiography. When ascending aorta dimensions
measured by echocardiography and cross-sectional imaging correlate with each other and no additional aortic
disease is identified, cross-sectional imaging is repeated every three to five years and prior to elective
operation. This frequency should be increased if there is a family history of disease in the descending thoracic
or abdominal aorta in association with MFS.
In adults, if the aortic diameter is documented as stable over time, then annual imaging is recommended if the
aortic dimension is less than 45 mm. If the aortic diameter is ≥45 mm or shows significant growth over time,
then more frequent imaging is suggested (eg, twice yearly) and surgery may be indicated (see 'Indications in
MFS' below). More frequent imaging is also recommended if the aortic diameter shows rapid change (≥0.5
cm/year) or if there are concerns regarding heart or valve function [4].
For children with MFS, annual imaging is recommended if the aortic dimension is documented as stable over
time and not markedly enlarged. There are no validated age-specific absolute aortic diameters that can be
used to determine when more frequent imaging should be performed or when prophylactic aortic surgery is
indicated. It is recommended that aortic measurements be compared to the body surface area. Sonographic
measurement of aortic diameter should be performed annually as long as the increase in aortic size remains
proportional to the increase in body surface area. Twice-yearly measurements are recommended if aortic size
(expressed as a percentage increase) diverges from the height when expressed in the same fashion.
Individuals under 20 years of age with systemic findings suggestive of MFS, but without cardiovascular
involvement, should also have annual echocardiograms due to the potential risk of development of aortic
disease [4]. Adults with repeatedly normal and stable aortic measurements without a definitive genetic
predisposition for aortic enlargement but with a sense of predisposition based upon family history or borderline
aortic measurements can be seen at two- to three-year intervals.
Monitoring Loeys-Dietz and related syndromes — As recommended in the 2010 ACC/AHA/AATS

guidelines, complete aortic imaging should be performed at the time of diagnosis and 6 months after in
patients with Loeys-Dietz syndrome or a confirmed genetic mutation associated with aortic aneurysms and
aortic dissections (eg, TGFBR1, TGFBR2, SMAD3, TGFB2, FBN1, ACTA2, or MYH11) to determine if aortic
enlargement is occurring.
If the aortic dimension is stable and no other specific problem in another vascular segment has been identified,
patients with Loeys-Dietz syndrome (potentially caused by mutations in TGFBR1, TGFBR2, SMAD3, TGFB2,
or TGFB3) should have serial MRI or CT angiography from the cerebrovascular circulation to the pelvis (with a
maximal interval between studies of two years) since they commonly develop aneurysms that are amenable to
prophylactic surgical management.
DRUG THERAPY
Beta blocker
Beta blocker recommendations — As noted in the 2010 American College of Cardiology/American Heart
Association/American Association for Thoracic Surgery (ACC/AHA/AATS) thoracic aorta guidelines, we
recommend beta blocker therapy for adults with MFS and aortic aneurysm to reduce the rate of aortic
dilatation unless contraindicated [1]. We also suggest beta blocker therapy for children with MFS and aortic
aneurysm, though data in children are inconclusive. Use of medication is commonly considered in individuals
documented to be at risk with demonstrated aortic root enlargement, a family history of aortic root
enlargement, or a mutation previously demonstrated to associate with aortic disease. The dose should be
adjusted to maintain the heart rate after submaximal exercise (eg, running up and down two flights of stairs)
<100 beats/minute in adults and <110 beats/minute in children [4].
Although propranolol was the first beta blocker demonstrated to slow aortic dilation, other beta blockers may
be used as well. Use of longer-acting agents, such as atenolol or metoprolol, with daily dosing may improve
compliance. Among pregnant women, labetalol or metoprolol are the preferred beta blockers since atenolol
may impair fetal growth. (See "Pregnancy and Marfan syndrome".)
A randomized trial is needed to determine the efficacy of beta blockers in children with MFS. In the absence of
such data, we suggest a beta blocker be given to all children with MFS. The dose should be adjusted to
maintain the heart rate at 110 beats/minute after submaximal exercise [5].
Patients who do not tolerate use of beta blocker may benefit from an angiotensin receptor blocker (preferably
losartan) [6] or an angiotensin converting enzyme inhibitor.
Beta blocker data — Beta blockers decrease myocardial contractility and pulse pressure and may also
improve the elastic properties of the aorta, particularly in patients with an aortic root diameter <40 mm [7,8].
Prophylactic treatment with beta blockers has been considered the standard of care in adults since the mid-
1990s, when the results of an open-label, randomized trial of 70 adults with classic MFS were published; the
patients were assigned to propranolol or no specific treatment [9]. The following observations were noted at
approximately ten years:
● Patients treated with propranolol (mean dose 212 mg/day) had a significantly slower rate of aortic
dilatation as reflected by a lower mean slope of the regression line for aortic root dimensions (0.023
versus 0.084 per year).
● Survival for the propranolol group was higher during the middle years of the trial and slightly but not
significantly better at the end of the trial. However, few patients reached a clinical end point (five in the
treatment group and nine in the control group).
Data supporting the use of beta blockers in children are less clear:
● In an observational study, 44 children and adolescents with MFS were followed for almost four years [10].
The 20 patients who were taking a beta blocker and the six patients taking a calcium channel blocker had
a slower absolute aortic growth rate (0.9 versus 1.8 mm/year in untreated patients) and aortic growth rate
adjusted for age and body size. Protection was not complete since 5 of the 26 treated patients had a
major cardiovascular complication requiring surgery over a four-year follow-up [10]. Of note, calcium
channel blocker therapy is generally avoided in patients with MFS. (See 'Avoid calcium channel blocker'
below.)
● A retrospective study of 63 children was conducted at two sites in which beta blocker therapy versus no
therapy was assigned according to local practice [11]. There was no difference in the rate of change in
aortic root measurements between the two groups at the end of the study. Only three patients underwent
aortic root replacement, one in the treatment group and two in the untreated group. There was a trend
toward more side effects in treated patients.
Therapy targeting the renin-angiotensin system — While data are limited, we suggest addition of an
angiotensin II receptor blocker (ARB; eg, losartan) as tolerated to beta blocker therapy to slow the rate of
aortic root dilation in patients with MFS. The 2010 ACC/AHA/AATS guidelines include a recommendation that
an ARB (losartan) is reasonable in this setting [1]. For a patient with thoracic aortic aneurysm (any cause), it is
reasonable to reduce blood pressure with beta blockers and angiotensin receptor blockers or angiotensin
converting enzyme (ACE) inhibitors to the lowest point the patient can tolerate without adverse effects.
Therapy targeting the renin-angiotensin system may attenuate the clinical manifestations of MFS by blocking
TGF-beta signaling [12].
The role of therapy targeting the renin-angiotensin system has been evaluated by several studies [6,12-16] but
an impact on clinical end points has not been established:
● ARB
• Added to beta blocker therapy – Studies evaluating the effect of adding an ARB (to standard beta
blocker therapy) have shown mixed results.
- Two randomized open-label trials in patients with MFS have reported reductions in aortic root
dilation rate with the ARB losartan added to beta blocker therapy at three-year follow-up [13,14].
- In contrast, a double-blind randomized trial found no significant difference between losartan and
placebo on aortic root dilation rate at median 3.5-year follow-up [16]. Most patients in both
treatment groups were also taking a beta blocker. One limitation of the study is inclusion of
patients who would not meet current diagnostic criteria for Marfan syndrome (eg, revised Ghent
nosology) since 22 percent of patients had no FBN1 mutation identified and many patients had
no baseline aortic root dilation.
• Comparison with beta blocker therapy − A randomized trial comparing losartan with atenolol in
608 children and adults (mean age 11.5 years) with MFS and aortic Z-scores >3.0 found no
significant difference in the rate of aortic root dilation between the two treatment groups over a three-
year period [15]. A comparable and significant decline in aortic root dilation relative to body surface
area was seen with both treatments. The three-year rates of aortic root surgery, aortic dissection,
death, and a composite of these events were similar in the losartan and atenolol treatment groups,
and the total numbers of events in both groups were small (19 versus 10 events). Importantly, this
study compared an atypically high dose of atenolol (mean 2.7 mg/kg/day and up to 4 mg/kg/day) with
a conventional dose of losartan (mean 1.3 mg/kg/day and up to 1.4 mg/kg/day).
● ACE inhibitor – Scant evidence is available on the effect of ACE inhibitor therapy in patients with MFS
[17,18]. A small randomized trial comparing perindopril with placebo (in addition to standard beta blocker
therapy) was retracted [19].
Other drug therapy — The potential role of other drug therapy in MFS has not been established. Statins have
been demonstrated to attenuate aortic root dilation in a mouse model of Marfan syndrome [20]. They appear to
act through reducing the excessive protein manufactured by vascular smooth muscle cells, which occurs in the
Marfan aorta. Human studies have not been reported.
Avoid calcium channel blocker — Although evidence is limited, preliminary animal and human data suggest
that calcium channel blocker therapy may increase the risk of aortic complications. Marfan mice treated with
calcium channel blockers demonstrated aneurysm expansion, rupture, and premature death. A study also
reported that patients with Marfan syndrome and other forms of inherited thoracic aortic aneurysm taking
calcium channel blockers display increased risk of aortic dissection and need for aortic surgery, compared with
patients on other medical agents [21].
RESTRICTION OF STRENUOUS ACTIVITY
An expert panel of the American Heart Association has made recommendations regarding permissible levels
of physical activity for patients with genetic cardiovascular diseases, including MFS [22]. As a general
principal, participation in recreational exercise, categorized as low to moderate intensity (approximately four to
six metabolic equivalents), can be recommended for many patients with MFS, though these suggestions do
not necessarily apply to patients who have had aortic root and/or valve replacement.
Recreational (noncompetitive) exercises that are of low and moderate intensity that are probably permissible
include the following: bowling, golf, skating (but not ice hockey), snorkeling, brisk walking on ground or
treadmill, stationary biking, modest hiking, and doubles tennis.
In general, patients with MFS are advised to avoid contact sports, exercise to exhaustion, and especially
isometric activities which entail the Valsalva maneuver [4]. Activities that are not advised or strongly
discouraged include [22]: isometric exercise (eg, weight lifting, sit-ups, pull-ups, push-ups), ice hockey, rock
climbing, windsurfing, and surfing. Scuba diving should be avoided due to an increased risk of pneumothorax
resulting from barotrauma [23].
Other activities that are thought to be of intermediate risk, and for which individual assessment is suggested,
include the following [22]: basketball (both full and half-court), racquetball, squash, running, skiing (downhill
and cross-country), singles tennis, touch (flag) football, soccer, baseball, softball, biking, lap swimming,
motorcycling, and horseback riding.
Parents of children with MFS face difficult decisions regarding participation in physical education classes.
Social pressure may necessitate inclusion of an affected child in a school-structured physical education
program. Parents and school officials should agree upon permitted and excluded activities for the affected
child. Physical education instructors must agree to abide by the activity restrictions and be able to monitor the
child's participation to avoid excessive exertion. It is not considered acceptable for children with MFS to
routinely simply observe exercise by their peers, as this practice is not benefiting the child and will promote
stigmatization. If the school is unwilling or unable to offer a suitable and sustainable program, the student
should be allowed to pursue an enriching activity in an alternative venue.
Recommendations in competitive athletes with MFS are presented separately. (See "Athletes: Overview of
sudden cardiac death risk and sport participation", section on 'Marfan syndrome'.)
AORTIC ROOT REPLACEMENT
Elective replacement
Rationale — Elective replacement of aortic root disease before critical enlargement is preferable to
emergency repair for marked dilatation or dissection. The importance of this approach was illustrated in a
series of 675 patients with MFS from Johns Hopkins in which the 30-day mortality for elective repair, urgent
repair (within seven days after a surgical consultation), or emergency repair (within 24 hours of consultation)
was 1.5, 2.6, and 11.7 percent, respectively [24]. In this series, 46 percent of the adults with aortic dissection
had an aortic root diameter of ≤65 mm at the time of operation; the authors suggested that prophylactic repair
be performed when the aortic diameter is well below this size.
Thus, surgical intervention should be considered, regardless of the patient's symptoms, in patients with
significant aortic root dilatation [25-27]. The goal is to improve survival. In a review of 231 patients who
underwent elective aortic root replacement at Johns Hopkins, the actuarial survival at five, ten, and twenty
years was 88, 81, and 75 percent, respectively [27].
Indications in MFS — The aortic root diameter at which elective surgery should be performed is uncertain.
The 2010 ACC/AHA/AATS guidelines recommend elective operation for patients with MFS at an external
diameter of ≥50 mm to avoid acute dissection or rupture [1]. Indications for repair at an external diameter less
than 50 mm include rapid growth (>5 mm/year), family history of aortic dissection at a diameter less than 50
mm, or presence of progressive aortic valve regurgitation.
One limitation of recommendations based upon absolute aortic root diameters is that predicted aortic root
diameter varies with body size [28] and age and may be smaller in women [29]. Smaller patients have
dissection at a smaller aortic root size and 15 percent of patients with MFS have dissection at a diameter less
than 50 mm [30]. Various strategies have been proposed to identify at risk patients with aortic size less than 50
mm [2,29,30].
The 2010 ACC/AHA/AATS thoracic aorta guidelines include the following recommendation for patients with
MFS and other genetic aortic diseases [1]. If the maximal cross-sectional area (πr2) in square centimeters of
the ascending aorta or root divided by the patient's height in meters is >10, surgical repair is reasonable. This
strategy was supported by a series of 103 patients with MFS who were managed using these criteria without
development of aortic dissection [30].
The optimal timing of elective aortic repair in children is also uncertain and adjustment for body size is
particularly relevant in this population. In children younger than 12 years of age, dissection is rare, irrespective
of aortic dilation. In these young patients, surgical indications are aneurysms that satisfy the adult criterion for
intervention, rapid enlargement (>10 mm/year), and progressive aortic insufficiency [31].
Indications in Loeys-Dietz syndrome — Patients with Loeys-Dietz syndrome tend to have severe
vascular disease and nearly all have aortic root aneurysms that lead to aortic dissection. Since aortic
dissection has been observed with aortic diameters <50 mm, repair is recommended at smaller diameters than
recommended for MFS [32].
The following recommendations are included in the 2010 ACC/AHA/AATS guidelines [1]:
● For adult patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation and an aortic
diameter of ≥4.2 cm by echocardiogram (internal diameter) or ≥4.4 to 4.6 cm by computed tomographic
imaging and/or magnetic resonance imaging (external diameter) it is reasonable to consider surgical
repair of the aorta.
● For young children with prominent craniofacial features of Loeys-Dietz syndrome, prophylactic surgery is
indicated if the aortic valve annulus is at least 18 to 20 mm and the aortic diameter exceeds the 99th
percentile for age.
Symptomatic aortic disease — Symptomatic expansion of a thoracic aortic aneurysm is generally an

indication for prompt surgical intervention. An aortic dissection is an indication for emergency surgery when
the ascending aorta is involved (type A) or for complications associated with type B dissections. These issues
are discussed separately. (See "Management of thoracic aortic aneurysm in adults" and "Management of
acute aortic dissection".)
Surgical technique — Two approaches are used for aortic root replacement: a composite valve graft and
valve-sparing aortic root replacement.
Composite valve graft — Total aortic root replacement with a composite valve graft consisting of the aortic
root and a prosthetic aortic valve and reimplantation of the coronary arteries (Bentall procedure) (figure 2) was
the surgical procedure of choice for older children and adults in the past [25,27]. It has been associated with
5-, 10-, and 20-year survivals of 88, 81, and 75 percent, respectively [27]. Mitral valve repair or replacement
may be performed at the same procedure [25].
The ascending aorta is generally replaced from the valve to the mid-ascending aorta. Some centers advocate
aortic hemiarch replacement at the time of ascending aorta replacement for MFS patients. Data to support
routine use of this procedure are lacking.
The importance of adequate hospital experience as well as early referral for optimizing composite valve graft
outcomes was indicated by a study of 1962 composite valve grafts (percent MFS not known) in the United
Kingdom Heart Valve Registry [33]. Hospital volume of ≤8 composite valve graft procedures per year and
urgent or emergency surgery were among the risk factors for 30-day mortality.
Valve-sparing aortic root replacement — Surgical procedures that retain the patient's native aortic valve
are attractive alternatives to composite grafts. Two such operations are referred to as remodeling and (David)
reimplantation techniques (figure 3) [34-36]. The former attaches the aortic conduit to a cuff of native aorta just
above the aortic valve, while the latter reimplants the native valve into the aortic graft and attaches the
vascular graft to the left ventricular outflow tract.
While anticoagulation is generally recommended for some time after surgery, life-long anticoagulation is not
required for patients who undergo a valve-sparing procedure (see 'Postoperative issues' below). Thus, valve-
sparing surgery is a good option for women who desire to become pregnant and for other patients with relative
contraindications to long-term anticoagulation. Structurally abnormal valves constitute a potential
contraindication to valve-sparing surgery that requires individualized consideration.
Although these valve-sparing techniques are more complex than composite valve replacement, early
outcomes are similar when performed by experienced surgeons. In a report from an international registry of
151 patients undergoing aortic root surgery, those undergoing valve-sparing procedures had longer
cardiopulmonary bypass times than those undergoing valve replacement, but there were no in-hospital or 30-
day deaths in either group and there were similar complication rates [37].
Limited data on long-term results of valve-sparing surgery are available. Patients who are considering such
surgery should be advised that reoperation may be necessary. Reimplantation of the aortic valve appears to
provide more stable valve function than the remodeling technique as illustrated by the following series [36]:
● In the Johns Hopkins series of 372 patients with MFS undergoing aortic root replacement, five of 40
patients who underwent a remodeling valve-sparing procedure required late aortic valve replacement for
aortic insufficiency [38]. In contrast, all 44 patients who underwent a reimplantation valve-sparing
procedure had none or mild (1+) aortic regurgitation on follow-up.
● In the Toronto series of 103 patients with MFS undergoing aortic valve sparing procedures, three of 26
patients who underwent the remodeling procedure required subsequent aortic valve replacement (two for
aortic regurgitation and one for endocarditis) [39]. Of the 77 patients undergoing reimplantation, none
required subsequent aortic valve replacement and only two had greater than mild aortic regurgitation.
Surgical guidelines — As recommended in the 2010 ACC/AHA/AATS guidelines, patients with Marfan,
Loeys-Dietz or Ehlers Danlos syndromes (and others with dilated aortic root and sinuses of Valsalva) should
undergo excision of the sinuses together with a modified David reimplantation operation if feasible, or, if not,
root replacement with valved graft conduit [1].
Investigational approaches — We agree with the 2015 Professional Advisory Statement from the Marfan
Foundation that there are insufficient data on alternative approaches that reinforce rather than replace an
aortic aneurysm (eg, personalized external aortic root support [PEARS] procedure and "Florida sleeve" repair)
to support a recommendation for clinical use at the present time [40]. Potential advantages include
theoretically simpler technique and for PEARS, potential avoidance of use of cardiopulmonary bypass and
minimizing the size of the surgical incision. However, data on these procedures are limited to small numbers of
patients at a small number of centers with limited durations of follow-up [41-44]. An additional key
consideration in the risk-benefit assessment of the PEARS procedure is that PEARS has been applied at an
aortic dimension significantly lower than that at which aortic root replacement is generally considered for MFS.
Postoperative issues
Anticoagulation — Life-long anticoagulation is necessary for those receiving mechanical valves. In

contrast, short-term anticoagulation is generally recommended in the postoperative period, for those receiving
bioprosthetic valves, or valve-sparing surgery, regardless of type of repair. Low-dose aspirin is typically given
in addition to warfarin [45]. Most thromboembolic events occur within the first month after surgery. (See
"Antithrombotic therapy for surgical prosthetic heart valves and surgical valve repair: Indications".)
Antibiotic prophylaxis — Antibiotic prophylaxis against infective endocarditis is suggested in association

with dental procedures for patients who have had aortic valve replacement or prior endocarditis, consistent
with the 2014 American Heart Association/American College Cardiology valve guidelines and the 2012
European Society of Cardiology valve guidelines [46,47]. The potential benefit and risk of antibiotic prophylaxis
for patients with Marfan syndrome with aortic or mitral valve regurgitation without prior valve surgery remains
controversial, warranting individualized consideration and counseling. (See "Antimicrobial prophylaxis for the
prevention of bacterial endocarditis".)
Medical therapy — Medical therapy (beta blocker with suggested addition of an angiotensin II receptor
blocker) should be resumed postoperatively and continued indefinitely due to the recognized vulnerability of
the aorta remote from the site of aortic replacement. (See 'Drug therapy' above.)
Coronary ostial aneurysms — One complication of both composite and valve-sparing surgery is the
development of coronary ostial aneurysms. These aneurysms develop at the site of native coronary artery
reimplantation into the aortic graft as a result of stretch of the weakened wall of the coronary ostium and are
not likely to progress. In a report of 40 patients, ostial aneurysms were documented in 43 percent at a mean of
five years after surgery; aneurysms were more common in those ≤35 years of age [48]. Time after operation
did not influence the prevalence or size of coronary ostial aneurysms, suggesting that these are not
progressive.
Rarely, pseudoaneurysms occur at the site of coronary anastomosis; these usually require surgical repair.
Follow-up imaging — After initial surgical replacement of the ascending aorta, the arch and descending
aorta are vulnerable to development of aneurysms and dissections, indicating that MFS is a disease involving
the entire aorta. A significant number of patients require surgeries at other sites throughout the aorta [25].
Patients who have a dissection at the time of the first aortic surgery are more likely to require subsequent
aortic surgery than those who undergo prophylactic composite graft repair or valve sparing procedure.
Periodic imaging of the entire aorta is suggested indefinitely following initial aortic repair for aortic dissection or
after prophylactic repair. The 2010 ACC/AHA/AATS guidelines suggest imaging the aorta at one, three, six and
12 months post-dissection and then annually thereafter if stable [2]. Monitoring can be accomplished with MRI
or CT angiography. Use of the same modality at the same institution facilitates comparison of images over
time. If a thoracic aortic aneurysm is relatively stable and moderate in size, MRI is suggested over CT to
minimize the patient's radiation exposure.
Suggested indications for elective prophylactic replacement of the aortic arch or descending aorta segment
include [45]:
● Rapid increase in size (eg, >5 mm/year or 5 percent for adults, or >10 mm/year for children)
● Descending thoracic aortic diameter >55 to 60 mm
● Affected segment diameter twofold greater than adjacent segment or
● Symptoms related to aortic dilation
TREATMENT FOR OTHER COMPLICATIONS OF MFS
Other complications that require attention include:
● Ophthalmologic evaluation is suggested annually due to propensity to lens subluxation (ectopia lentis),
cataract, glaucoma, and retinal detachment [4].
• Eye care includes vision correction for myopia and photocoagulation for retinal tears and detachment.
Early monitoring and aggressive refraction is recommended for children with MFS to prevent
amblyopia [4]. (See "Refractive errors in children", section on 'Myopia' and "Amblyopia in children:
Classification, screening, and evaluation".)
• A dislocated lens should not be removed surgically unless more conservative means of correcting
vision are ineffective. Indications for surgical lens extraction include lens opacity with poor visual
function, refractive error not amenable to optical correction, impending complete luxation, and lens-
induced glaucoma or uveitis [4].
● Isolated mitral valve repair or replacement is recommended for severe mitral regurgitation with associated
symptoms or progressive left ventricular dilatation or systolic dysfunction (see "Indications for intervention
for severe chronic primary mitral regurgitation"). Recommendations for concomitant mitral valve repair at
the time of aorta replacement are being refined [49,50].
● Scoliosis may be initially treated with bracing but surgical correction is usually considered when the curve
exceeds 40 degrees. (See "Adolescent idiopathic scoliosis: Management and prognosis", section on
'Approach to surveillance and management'.)
● Surgery may be necessary for severe pectus deformities. (See "Pectus carinatum" and "Pectus
excavatum: Treatment".)
● Surgery may be necessary for recurrent pneumothoraces. (See "Pneumothorax in adults: Epidemiology
and etiology".)
● The arthropathy associated with joint laxity may require orthopedic correction.
● Pregnancy in women with MFS is discussed separately. (See "Pregnancy and Marfan syndrome".)
Screening of family members, genetic counseling, and prenatal genetic testing are discussed separately. (See
"Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Screening
relatives'.)
MANAGEMENT OF RELATED CONDITIONS
Periodic follow-up is indicated in patients with the MASS phenotype (mitral valve prolapse, mild aortic
dilatation, striae atrophica, one or more skeletal features (picture 1)), mitral valve prolapse syndrome, or
ectopia lentis syndrome (ELS), including annual cardiovascular imaging to monitor aortic size and mitral
regurgitation and annual ophthalmological evaluation. Patients with either MASS phenotype or ELS should be
counseled regarding their potential development of aortic disease and the risk of more severe disease
(including aortic aneurysm) in their offspring. (See "Genetics, clinical features, and diagnosis of Marfan
syndrome and related disorders".)
Careful cardiovascular and ophthalmologic follow-up is also indicated in individuals <20 years old diagnosed
with potential MFS or nonspecific connective tissue disorder, since additional clinical features may emerge in
such patients.
PROGNOSIS
The life span of untreated patients with the classic MFS was approximately 32 years in 1972. However,
improved therapy has resulted in marked increases in life expectancy to 72 years in 1993 [25,51]. Beta
blockers, noninvasive aortic imaging and elective aortic root repair have all contributed to this improvement in
survival. For reasons that are not well understood, life expectancy is significantly lower in men than in women.
A family history of premature death or aortic surgery may identify patients at increased risk. In one study of
108 patients from 33 multigenerational families, those in the highest quartile for aortic size were more likely to
have such a family history than those in the lowest quartile (>80 versus <10 percent) [52].
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around the world
are provided separately. (See "Society guideline links: Aortic dissection" and "Society guideline links: Marfan
syndrome".)
INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics
patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer
the four or five key questions a patient might have about a given condition. These articles are best for patients
who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient
education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to
12th grade reading level and are best for patients who want in-depth information and are comfortable with
some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these
topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on
"patient info" and the keyword(s) of interest.)
● Basics topic (see "Patient education: Marfan syndrome (The Basics)")
SUMMARY AND RECOMMENDATIONS
● In children with Marfan syndrome (MFS), sonographic measurement of aortic diameter should be
performed annually as long as the increase in aortic size remains proportional to the increase in body
surface area. Twice-yearly measurements are recommended if aortic size indexed to body surface area
exceeds the expected proportion. More frequent imaging is indicated once the absolute aortic dimension
approaches a surgical threshold or to monitor progression of significant valve or ventricular dysfunction.
(See 'Monitoring MFS' above.)
● In adults with MFS, yearly sonographic measurement of aortic root diameter (after confirming that
sonographic aortic measurement correlates with cross- sectional imaging measurement) is recommended
as long as the diameter is <45 mm; more frequent monitoring is recommended if the aortic root diameter
is ≥45 mm, if the aortic diameter shows rapid change (≥0.5 cm/year), or if there are concerns regarding
heart or valve function. (See 'Monitoring MFS' above.)
● Patients with Loeys-Dietz syndrome and related disorders should have serial magnetic resonance
imaging from the cerebrovascular circulation to the pelvis. If the aortic and other vascular dimensions are
stable after initial and first six-month follow-up, annual reassessment is generally recommended, since
they commonly develop aneurysms that are amenable to prophylactic surgical management. (See
'Monitoring Loeys-Dietz and related syndromes' above.)
● High-intensity exercise, exercise that involves bursts of activity (eg, sprinting), or activities likely to cause
marked increases in blood pressure (eg, weight lifting or weight training) should be avoided for patients
with MFS and related disorders. (See 'Restriction of strenuous activity' above.)
● Low- and moderate-intensity noncompetitive recreational activities may be recommended for patients with
MFS and related disorders. The participation of children in school-related physical education programs
should be individualized.
● We recommend a beta blocker for adults with MFS and aortic aneurysm (Grade 1B). We suggest beta
blocker therapy for children with aortic aneurysm (Grade 2C). The dose should be individually titrated to
limit heart rate to less than 100 or 110 beats per minute for adults or children, respectively, following
submaximal exercise. (See 'Beta blocker' above.)
● We suggest addition of an angiotensin receptor blocker to beta blocker therapy in patients with MFS with
aortic aneurysm as tolerated (Grade 2C). (See 'Therapy targeting the renin-angiotensin system' above.)
● For patients with MFS, elective aortic repair is associated with reduced mortality when compared with
urgent or emergency repair. Elective surgical intervention should be considered when an adult has an
aortic root diameter of ≥50 mm and a child has a disproportionately rapid increase in aortic diameter when
compared with the rate of increase in body surface area even if the diameter is ≤50 mm. (See 'Indications
in MFS' above.)
● For adult patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation and an aortic
diameter of ≥4.2 cm by echocardiogram (internal diameter) or ≥4.4 to 4.6 cm by computed tomographic
imaging and/or magnetic resonance imaging (external diameter) it is reasonable to consider elective
surgical repair of the aorta. (See 'Indications in Loeys-Dietz syndrome' above.)
● For young children with prominent craniofacial features of Loeys-Dietz syndrome, prophylactic surgery is
indicated if the aortic valve annulus is at least 18 to 20 mm and the aortic diameter exceeds the 99th
percentile for age. (See 'Indications in Loeys-Dietz syndrome' above.)
● For patients undergoing aortic root replacement, composite aortic root and valve replacement and valve-
sparing surgical techniques are considerations, and the choice of operation is dependent upon patient
specific considerations. (See 'Elective replacement' above.)
ACKNOWLEDGMENT
The UpToDate editorial staff would like to thank Dr. Catherine M. Otto for her contributions as Section Editor to
previous versions of this topic review.
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27. Gott VL. Antoine Marfan and his syndrome: one hundred years later. Md Med J 1998; 47:247.
28. Roman MJ, Devereux RB, Kramer-Fox R, O'Loughlin J. Two-dimensional echocardiographic aortic root
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30. Svensson LG, Khitin L. Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic
patients with Marfan syndrome. J Thorac Cardiovasc Surg 2002; 123:360.
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35. Kallenbach K, Karck M, Pak D, et al. Decade of aortic valve sparing reimplantation: are we pushing the
limits too far? Circulation 2005; 112:I253.
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52:438.
37. Volguina IV, Miller DC, LeMaire SA, et al. Valve-sparing and valve-replacing techniques for aortic root
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2009; 137:1124.
38. Cameron DE, Alejo DE, Patel ND, et al. Aortic root replacement in 372 Marfan patients: evolution of
operative repair over 30 years. Ann Thorac Surg 2009; 87:1344.
39. David TE, Armstrong S, Maganti M, et al. Long-term results of aortic valve-sparing operations in patients
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and other congenital disorders associated with aortic root aneurysms. Heart 2014; 100:1571.
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aortic insufficiency due to root aneurysms. Ann Thorac Surg 2009; 87:1161.
43. Treasure T, Takkenberg JJ, Golesworthy T, et al. Personalised external aortic root support (PEARS) in
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47. Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology
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Topic 8151 Version 19.0
GRAPHICS
Ascending aorta dimensions for men
Fifth and 95th percentiles based upon echocardiographic leading-edge-to-leading-edge diameters of the sinus of Valsalva and
ascending aorta in 815 men with normal cardiac findings on transthoracic echocardiogram.
Reproduced from: Biaggi P, Matthews F, Braun J, et al. Gender, age, and body surface area are the major determinants of ascending aorta
dimensions in subjects with apparently normal echocardiograms. J Am Soc Echocardiogr 2009; 22:720. Illustration used with the permission
of Elsevier Inc. All rights reserved.
Graphic 97882 Version 2.0
Ascending aorta dimensions in women
Fifth and 95th percentiles based upon echocardiocardiographic leading-edge-to-leading-edge diameters of the sinus of Valsalva and
ascending aorta in 984 women with normal cardiac findings on transthoracic echocardiogram.
Reproduced from: Biaggi P, Matthews F, Braun J, et al. Gender, age, and body surface area are the major determinants of ascending aorta
dimensions in subjects with apparently normal echocardiograms. J Am Soc Echocardiogr 2009; 22:720. Illustration used with the permission
of Elsevier Inc. All rights reserved.
Aortic root replacement with a composite valved graft
A composite valved graft (Bentall) procedure involves replacement of the aortic root with a
graft and aortic valve replacement with a prosthetic valve (mechanical as shown here, or
bioprosthetic), with reimplantation of the coronary arteries.
Used with permission of Mayo Foundation for Medical Education and Research. All rights
reserved.
Valve-sparing aortic root
The figures show a close-up view of a valve-sparing aortic root replacement showing excision of the
affected tissue down to the annulus (A), and reconstruction with a tube graft and coronary artery
reimplantation (B).
Skeletal features of Marfan syndrome
Individuals with Marfan syndrome have an increased arm span to height ratio. Arm span is measured from finger tip to finger
tip with the arms abducted to 90 degree from the trunk as shown.
Photographers: Rick Guidotti and Tim Joyce. Reproduced with permission from The Marfan Foundation (Marfan.org).
Contributor Disclosures
Michael J Wright, MBChB, MSc Nothing to disclose Heidi M Connolly, MD, FACC, FASE Nothing to disclose Harry C
Dietz, MD Nothing to disclose Susan B Yeon, MD, JD, FACC Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by
vetting through a multi-level review process, and through requirements for references to be provided to support the
content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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Monitoring Loeys-Dietz and related syndromes — As recommended in the 2010 ACC/AHA/AATS

RESTRICTION OF STRENUOUS ACTIVITY

AORTIC ROOT REPLACEMENT

Symptomatic aortic disease — Symptomatic expansion of a thoracic aortic aneurysm is generally an

Anticoagulation — Life-long anticoagulation is necessary for those receiving mechanical valves. In

Antibiotic prophylaxis — Antibiotic prophylaxis against infective endocarditis is suggested in association

TREATMENT FOR OTHER COMPLICATIONS OF MFS

Other complications that require attention include:

MANAGEMENT OF RELATED CONDITIONS

SOCIETY GUIDELINE LINKS

INFORMATION FOR PATIENTS

● Basics topic (see "Patient education: Marfan syndrome (The Basics)")

SUMMARY AND RECOMMENDATIONS

1. Hiratzka LF, Bakris GL, Beckman JA, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM

40. file:///C:/Users/syeon/Downloads/PEARS%20STATEMENT-%20Final.pdf (Accessed on September 19, 2

Topic 8151 Version 19.0

Ascending aorta dimensions for men

Graphic 97882 Version 2.0

Ascending aorta dimensions in women

Graphic 97883 Version 2.0

Aortic root replacement with a composite valved graft

Graphic 117684 Version 1.0

Valve-sparing aortic root

Graphic 107677 Version 2.0

Skeletal features of Marfan syndrome

Graphic 108185 Version 1.0

Conflict of interest policy

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