Marfan Syndrome and Pectus: Heart Checks and Treatment

You have Marfan syndrome, or a doctor has raised it, and your chest dips in or sticks out. A chest wall deformity is a recognized part of Marfan, sunken or pigeon, and at a Rome clinic that counted both, pigeon was the more common. The chest can even be the first clue to a diagnosis. But in Marfan, the aorta decides your health, not your chest. So heart checks come first, and any chest treatment gets planned with your Marfan team around them.

Worried right now? Skip to when it’s an emergency.

I was diagnosed with pectus excavatum at 15 and corrected it without surgery over about three years. The change was visible and measurable, but not complete. I’m not a doctor, and Marfan is one of the cases where I send people to their doctor first. Every medical point below names its source.

Why the aorta comes first

Marfan syndrome is a genetic condition of the connective tissue, the material that holds the body together. It comes from changes in the FBN1 gene, which carries the instructions for fibrillin, a protein in that tissue. The Marfan Foundation puts it at about 1 in 5,000 people, though estimates run from 1 in 3,000 to 1 in 15,000. About 3 in 4 inherit it from a parent. The rest are the first in their family.

It can bring long limbs, a curved spine (scoliosis), a slipped lens in the eye, a floppy heart valve and a widening aorta, and severity varies a lot, says NORD. There’s no cure. It’s managed and monitored, and the heart is why.

Heart and blood vessel problems affect about 9 in 10 people diagnosed, says the Marfan Foundation, and Cleveland Clinic names cardiovascular problems as the most common cause of death. The trouble usually starts at the aortic root, where the aorta, the body’s main artery, leaves the heart. It can slowly widen and then tear between the layers of its wall, an emergency called an aortic dissection.

In a Toronto study of 212 adults with Marfan who had heart MRI, those with a sunken chest had aortas of similar size to those without. They did have more mitral valve prolapse, where a valve between the heart’s left chambers bulges backward (57% against 27%), and a slightly larger, slightly weaker right ventricle. A sunken chest there meant a Haller index of 3.25 or more. That scan measure of chest depth averages about 2.5, and 3.25 is a common referral threshold, not an emergency.

The 2022 US aortic disease guideline recommends medicine to slow the aorta’s widening. Your cardiologist chooses it and decides on surgery.

The checks to ask for

A 2018 UK review by Fraser and colleagues says a chest visit may be the first chance to diagnose Marfan and start heart care early. Your team sets the schedule.

• An echocardiogram (echo). An ultrasound of the heart that measures the aortic root. The 2022 guideline, written for adults, advises one at diagnosis, another at 6 months, then yearly if the aorta is stable.
• CT or MRI of the aorta. Reasonable after the first echo, says the guideline.
• An eye exam. A slipped lens is one of the strongest clues. NORD advises yearly exams from early childhood.
• Genetics. A genetics referral and a test for FBN1 changes. The 2024 Pectus Care Guidelines say anyone at risk of an inherited connective tissue condition should be referred, and chest surgery should usually wait until a geneticist has done the work-up.
• Lung tests, if you’re breathless or your chest is severe.

How many people with Marfan have a sunken or pigeon chest?

About a third had a sunken chest in the two studies below that counted it on its own, and at least one other cohort reported a lower rate. The “up to 70%” in the Fraser review counts sunken and pigeon chests together, from a 2016 study of 146 patients at a Marfan referral center in Rome. Its patients were sent there by other doctors, so they may not look like everyone with Marfan.

Who was studied	What was counted, and how	Result
146 patients at one Marfan referral center, Rome, 2016	Chest checked on exam	Pigeon chest 39.7%, sunken or uneven chest 30.1%, either 69.9%
212 adults with Marfan having heart MRI, Toronto, 2023	Sunken chest, Haller index 3.25 or more	36%
407 people with Marfan in Denmark’s national registry, 1977 to 2014	Any pectus in hospital diagnosis codes	1 in 11
259 children with FBN1 Marfan, France, 2014	Any pectus, by age	43% at ages 0 to 6, 62% at 15 to 17
2024 Pectus Care Guidelines	Any documented pectus	About half

My plain reading: the “any pectus” figures swing from 1 in 11 in hospital codes, which likely miss mild chests, to about 70% on a specialist’s exam. I couldn’t find a figure for a whole population with Marfan. The French numbers also show the chest changing with growth.

Pigeon chest (pectus carinatum) also counts for more in the revised Ghent criteria, the scoring system doctors use for Marfan: 2 points against 1 for a sunken or uneven chest. An arm span over 1.05 times your height is another sign.

Have a sunken chest? How likely Marfan is

Not very. The 2024 guidelines estimate that about 5% of people with an apparently isolated pectus are later diagnosed with Marfan.

Marfan was found in 0.4% of 1,046 Danish surgical patients, 2.8% of 1,215 US surgical patients, and 5.3% of 241 New York children, some with pigeon chests, who were routinely sent for genetic and heart checks. Those groups and other linked syndromes are in my guide to pectus excavatum causes.

The dip on its own is a weak clue. In 355 children referred to a Dutch Marfan clinic, a sunken chest and arm span didn’t predict Marfan at that age. Younger age at referral, a slipped lens, a widened aorta and certain facial features did. The other signs and your family history matter more.

Loeys-Dietz and Ehlers-Danlos

In a Johns Hopkins series of 65 people with Loeys-Dietz syndrome, a related condition that also affects the aorta, 35 had a sunken chest and 16 had first been diagnosed with Marfan. The Ehlers-Danlos link to pectus rests mostly on small studies. My rule for all three: doctor first.

What the chest does to the heart and lungs

A sunken chest goes with some heart findings in Marfan, like the Toronto ones above, but those are associations, and it hasn’t been shown to change survival. More in pectus excavatum and heart problems.

In a Seoul study of 253 people with Marfan who had aortic surgery, 62 had a severe sunken chest (Haller index above 3.5) that had never been corrected. Their 10-year survival was 98.3%, against 96.8% for the others, not a significant difference, and readmissions for heart or lung problems didn’t differ. The one difference: more of them needed a breathing machine for longer after surgery (8.0% against 1.0%), a gap that shrank after adjustment.

In the Rome study, no patient with a sunken chest had any breathing dysfunction. If you’re breathless, the Fraser review lists a collapsed lung (pneumothorax) and sleep apnea among the other Marfan causes to check.

Treating the chest when you have Marfan

Marfan changes almost every chest treatment.

Option	What changes with Marfan	Source
Nuss procedure	Deeper chests, more bars left in longer, higher risk of the dip returning, no epidural	Redlinger 2010; Fraser 2018; 2024 guidelines
Chest surgery with heart surgery	One operation or staged; low complication rates in small series; debated	Raffa 2017; Davari 2026
Chest surgery after earlier heart surgery	Heart injury in 7% of one 75-patient series	Jaroszewski 2017
Vacuum bell	Not advised with any aortic root concern; Marfan is a listed reason not to use it	Fraser 2018; Haecker and Mayr 2006
Brace for a pigeon chest	Less success; longer wear may be needed	van Braak 2024; Fraser 2018
Timing for children	Usually after growth is nearly complete and a genetics work-up	Arn 1989; 2024 guidelines

Do you need to correct it?

Not necessarily. NORD says a sunken chest may be corrected for appearance or, in very rare severe cases, to avoid medical complications, and in the Seoul study, a severe uncorrected one didn’t change survival. My view: decide around your aorta and your symptoms, not out of fear, with your Marfan team.

Nuss procedure with Marfan

The Nuss procedure places a curved metal bar under the breastbone to push it out. The biggest Marfan numbers come from one large US series in Norfolk, Virginia: 1,192 repairs, 33 in people with Marfan.

• Their chests were deeper: a CT chest index of 8.75 on average, against 4.94 in patients without Marfan features.
• 58% needed two or more bars, against 29%.
• Wound infection ran 6% against 1.3%, a gap that wasn’t statistically significant.
• Surgeon-rated success (98%) and patient satisfaction (96%) in Marfan and Marfan-like patients matched the others.

The Fraser review adds that a Marfan chest is more supple, so the dip is more likely to return after any surgery. A 1989 Johns Hopkins study of older open repairs saw a more than moderate dip return in 11 of 28 people with Marfan, against 2 of 30 without. Bars generally stay in at least two years, but with Marfan, the 2024 guidelines say often about 4. More in pectus bar removal.

Pain relief changes too. Fraser says an epidural, pain medicine given through a thin tube in the back, isn’t recommended in Marfan because the membrane around the spinal cord is more fragile, which can lead to headaches.

When heart surgery is also needed

A 2017 review gathered 39 people with Marfan and a sunken chest from 27 publications, all having aortic surgery. Fixing both at once was most common, complications were uncommon, and nobody died. Other small series, some using the Ravitch (an open chest operation), look similar, including a 2026 one of 13 patients in Iran, 7 with Marfan, whose authors say one operation against two is still debated.

The Fraser review’s senior author prefers a modified “open” Nuss straight after the aortic surgery, which is expert opinion from one experienced surgeon.

Had heart surgery through a sternotomy, where the breastbone is cut open? In 75 patients who had the Nuss after a sternotomy for heart surgery at 14 centers, not only people with Marfan, the heart was accidentally injured in 5 (7%), and nobody died. Twelve (16%) needed the breastbone reopened first. Prof. Mustafa Yüksel, who reviews medical content on this site, co-authored that study.

Vacuum bell with Marfan

The Fraser review is direct: with any concern about the aortic root’s size, the vacuum bell is “not advised as a safe treatment option”.

Marfan was listed as a reason not to use the bell in the original 2006 Basel series and by the bell’s maker. Not every surgeon treats it as an absolute no: in a 2024 survey of chest wall surgeons, one answer per hospital, 32.5% of those who answered listed Marfan as a reason not to use it. I couldn’t find a study testing the vacuum bell in people with Marfan.

I sell a custom vacuum bell, and that doesn’t change my rule: with Marfan, I send people to their doctor first. Don’t start a vacuum bell or a brace unless your Marfan team and a chest wall surgeon agree.

Bracing a pigeon chest

A custom brace presses a raised chest back in, usually over at least a year, says the Fraser review, which suggests longer wear with Marfan. In an Amsterdam study of 631 braced patients, Marfan was linked to less success. More in pectus carinatum brace therapy.

Timing for children

Surgery usually waits until growth is nearly complete and, the 2024 guidelines add, until a geneticist has investigated. In the Johns Hopkins study, a third of the Marfan patients had their chest repaired before their Marfan was diagnosed. More for parents in pectus in babies and kids.

Questions to ask your team

• What’s my aortic size, and is it growing?
• Is heart surgery likely later, and should the chest plan wait for it or join it?
• Have I had an eye exam, a genetics work-up and, if needed, lung tests?
• Is a vacuum bell or brace safe for me?
• Is the surgeon experienced with Marfan, with heart surgery backup on site?
• How long will the bars stay in?
• What pain relief will I get without an epidural?

Exercise, sport and weight

Be active. The Marfan Foundation’s April 2026 guidance says most children can and should be physically active and encourages activity for most adults, at a moderate, comfortable pace, chosen with your care team.

What carries more risk: powerlifting, high-impact contact sports like tackle football, and scuba diving, says the Marfan Foundation. Cleveland Clinic adds exercising to exhaustion and holding a strain, like planks, wall sits or heavy lifts you strain through.

On muscle, the 2026 sheet encourages steady, mid-range strengthening and warns against extreme joint stretching, which matters for stretching routines. A 2021 Marfan Foundation article said building muscle isn’t recommended, as it can contribute to the aorta widening. My reading: light-to-moderate strengthening planned with your team, yes. A program aimed at getting bigger, no.

Gaining weight is often hard with Marfan, especially when young, and the Marfan Foundation suggests a dietitian. I cover it in Marfan syndrome and weight gain.

Does a sunken chest shorten life with Marfan?

Not in the Seoul study above, the only pectus outcome study in Marfan I found, though it’s one group of Korean surgical patients.

Cleveland Clinic now says life expectancy with Marfan is close to that of people without it, though lower in men than in women. The Marfan Foundation says that with treatment, you have “a good chance of avoiding a life-threatening situation”.

In 1972 data, the average age at death was 32. A 1995 study of 417 patients at four US referral centers put it at 41, and median survival at 72 years, against 48 in 1972. Those numbers come from referral patients decades ago and aren’t a forecast for you.

Recent studies still show higher death rates, mainly from the aorta. In a Norwegian group of 84 adults followed for about 11 years, 16 died, a death rate 5.24 times that expected for the general Norwegian population. Eight of those 16 deaths were linked to the aorta, and 32% of survivors hadn’t been followed up as recommended. That last number is the one you control. Keep your scans.

For pectus on its own, Cleveland Clinic says there’s no evidence it limits life expectancy, as I cover in pectus excavatum problems later in life.

When it’s an emergency

Call 911 (999 in the UK) or your local emergency number now if you have any of these. Don’t drive yourself.

• palpitations that won’t stop
• palpitations with chest pain, shortness of breath, feeling faint or fainting
• sudden chest pain or pressure that doesn’t go away
• chest pain that spreads to your arm, neck, jaw or back
• chest pain with sweating, feeling sick, light-headedness or breathlessness

That list follows the NHS advice on chest pain and palpitations, as in my heart problems guide.

With Marfan, add one more. The NHLBI and the Marfan Foundation say aortic dissection pain is sudden and severe, in the center of the chest, the stomach or the back, often called sharp, tearing or ripping, and it may move. You may also feel light-headed, look pale or have a fast heartbeat. Call 911 (999 in the UK) and say you have Marfan.

What to do next

• Marfan suspected, not confirmed. See your doctor and ask about an echo, an eye exam and a genetics referral before anything is done to your chest.
• Diagnosed and weighing chest treatment. Take the questions above to your Marfan team and a chest wall surgeon with Marfan experience. Finding the best pectus surgeon helps with the second.
• Cleared by your team and curious where your chest stands. For a sense of how significant it is and which routes might fit, take my free 1-minute quiz.

The chest is the part you can see. The aorta is the part to watch.

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