PoTS and Pectus Excavatum in 2022, Causes, Treatment, Symptoms + Can Surgery Help?

Evidence Based This post has medical citations

Many people with pectus excavatum develop PoTS (Postural Orthostatic Tachycardia Syndrome) symptoms but are undiagnosed probably because of the chest deformity. The deformity can mask PoTS symptoms. Most people with PoTS hate the feeling of being lightheaded, tired, and having heart palpitations frequently. They want to undergo Nuss hoping it will fix their sunken chest and PoTS.

WHAT Is POTS

PoTS is Postural Orthostatic Tachycardia Syndrome. Patients with PoTS feel tired and don’t have much energy during the day. The T in PoTS stands for Tachycardia. It means a fast heartbeat, one of the signs of this condition is when somebody changes their posture from laying down to standing up. The heart beats excessively fast. That’s postural orthostatic Tachycardia.

The S of the PoTS is the syndrome. It collects symptoms including dizziness, fatigue, headache, and tummy upset. This is what gets people down. This condition makes people feel tired and dizzy, rundown, and uncomfortable with headaches and tummy aches. Anybody can be affected, but there seem to be two kinds.

POTS IS REAL

PoTS are symptoms predominantly caused by an exaggerated heart rate increase in response to standing. Even though not many people have heard of this, PoTS is a common problem. PoTS is real and relates to the involuntary nervous system and points out we don’t know everything in medical science. Doctors research a lot about PoTS. They can see many things, but not all patients are aware. People need to know that PoTS is real and explains a lot of chronic fatigue in teenagers.

IS POTS TREATABLE?

PoTS is treatable. Most patients with PoTS as teenagers do get better. They should be able to look forward to a complete and good recovery. Various treatments can treat it, facilitate healing, and help them function better while recovering. Some medicines seem to work better when they get medical treatment. It is also essential to know it’s not just about medicine.

TREATMENT

Treatment of PoTS is complicated because, despite the relatively common condition, there are no large randomized controlled trials of any specific treatment. It is generally believed that a collaborative multidisciplinary approach will be helpful. This will include physicians, psychologists, and physical and occupational therapists who may work fast rather than a physician helping the patient manage the disease independently. However, this is not always available.

3 SPECIFIC TREATMENTS

PoTS needs changes in diet, more salts, more fluids, and the need to exercise. Their bodies tell them they are too tired and can’t exercise. We need to overcome that physical feeling and get them to exercise to recondition and rehabilitate. When people use these non-medicine ways in conjunction with good medicine, they do better. Specific treatments can be divided into three categories.

1. ACTIVITY & EXERCISE TRAINING

This is the most commonly advised treatment. But any regiment should initially avoid the upright position and instead rely on equipment such as rowing machines and static bicycles. Weight training, particularly on the legs and core, will be beneficial too. Patients should avoid deconditioning, such as extended breaks in their exercise regimen during cold weather.

Managing PoTS through lifestyle and exercising can be tricky. Also, some people with PoTS and pectus excavatum use inhalers before exercising. Even though it doesn’t help much, it still helps reduce some of the PoTS symptoms.

2. WATER AND SALT INTAKE

The most straightforward advice here is to increase water and salt intake. Sometimes even using salt tablets. IV infusions of saline are occasionally used during periods of terrible symptoms. However, regularly scheduled saline infusions are not recommended. Vascular compressions stockings can prevent venous pulling of blood in the legs upon standing. The medication fluid cortisone is sold under the brand name Fluorine.

SIDE EFFECTS

  • Edema
  • Supine hypertension
  • Low potassium.

For some patients focusing on activity and volume expansion is sufficient to control the symptoms, but in others is not enough. For those, there is a third category.

3. Autonomic Medications

  • Midodrine
  • Propanolol
  • Ivabradine
  • Clonidine
  • Pyridostigmine

All these have various advantages and disadvantages. For example, propanol and clonidine may lower blood pressure, exacerbating dizziness and syncope symptoms.

DOES PECTUS EXCAVATUM CAUSE POTS?   

I wanted to find out whether PoTS can be caused by pectus excavatum. I also researched whether fixing pectus excavatum will also fix PoTS.

Unfortunately, there isn’t any scientific literature that proves the connection between pectus excavatum and PoTS. I had to look through various pectus excavatum forums and see people’s experiences with pectus excavatum surgery and PoTS.

Pectus deformities can be related to many other illnesses you may not know about.

DR. J THOUGHTS ON POTS WITH PECTUS EXCAVATUM

I stumbled upon a comment on Reddit about how a patient with pectus arcuatum and PoTS was told by Dr. J (one of the world’s best pectus excavatum surgeons) how surgery might improve PoTS symptoms.

She also said PoTS is significantly worsened by pectus excavatum, and repairing the deformity can improve the symptoms. Still, PoTS is a lifetime disorder, and surgery can’t enhance its symptoms immediately.

HOW SURGERY CAN HELP

Depending on the cavity severity, a person’s heartbeat with pectus excavatum can be noticed by looking at the chest, right above the left flaring rib.

When the chest is corrected surgically, the PoTS shouldn’t feel as bad, mainly because you won’t be able to feel every heartbeat throughout the day. Surgery will hopefully get rid of the sensations of palpitations.

POTS AND CONNECTIVE TISSUE DISORDERS

Also, muscular-skeletal issues, such as joint hypermobility, and connective tissue disorder, are pretty standard for PoTS. There is evidence that pectus excavatum is common in people with Ehlers-Danlos syndrome (EDS) and Marfan Syndrom, with a high co-morbidity with PoTS.

Surgeons say that patients with pectus excavatum with Marfans or EDS are less likely to have PoTS disappear entirely after surgery.

However, expect a huge relief once the lungs and heart are relieved from the deformity’s compression. This will make it easier to manage symptoms by exercising.

YOU’RE NOT ALONE

You are not alone in this. There are many fellow people with pectus excavatum that experience this daily. I am surprised there isn’t any scientific research about the connection between pectus excavatum and PoTS.

WOMEN WITH PECTUS EXCAVATUM AND POTS

On the r/PectusExcavatum forum, many of us with pectus excavatum have PoTS. Also, many women with PoTS were unaware they had pectus excavatum until early adulthood or late teenage years.

This makes a lot of sense as the symptoms of pectus excavatum generally worsen during this period. Adult PoTS mainly affect women between the ages of 30 and age 50. In addition, a lot of females are underdiagnosed with pectus excavatum.

TYPICAL TEENAGER WITH POTS AND PECTUS EXCAVATUM

PoTS also affects teenagers with pectus excavatum. It usually happens about a year during their growth spurt at the beginning of their puberty changes. Usually, they are high-achieving teenagers.

The majority are girls, but it can be boys too. They’re what parents think they’re ideal children achieving hard work and thriving in life, and then they get sick like a typical sickness.

But instead of bouncing back from it, it puts their involuntary nervous system out of balance. They feel tired and run down, their blood flow is disturbed, unable to get blood at the right place at the right time.

So, they stand up and feel dizzy. They can’t move their food through their intestines well and feel rundown and uncomfortable.

TREATMENT FOR TEENAGERS

There are ways to treat PoTS. There have been some studies on adults to see what works. However, few studies on teenagers say what works to get better from PoTS.

Doctors asked patients who successfully treated PoTS what worked effectively for them. They knew how they were treating them based on their science, so they surveyed a year after seeing them and asked how they were doing.

NOT WELL KNOWN

PoTS isn’t very well known. It was discovered and recognized about 20 years ago and first recognized just about ten years ago in teenagers. It is newly identified and not known by everybody. Many patients with PoTS look normal.

They’re high-achieving, happy, friendly people who have trouble getting motivated, getting out of bed, and doing much. Some people looking at the surface would blame it on depression or anxiety.

That’s all in their head. Often the patients have gotten the impression from other healthcare providers that there is nothing wrong and it’s all in their head.

If they’re significantly impacted, it can be explained by what is going on with their involuntary nervous system and can be pointed toward recovery.

MEDICINE

People treated with one particular medicine were doing better than those not treated with that medicine. That pointed toward a better understanding of what medicine could help. This medicine is a beta-blocker and works well for PoTS in adults and, often, in teenagers.

Most of us think beta-blockers are for older people with heart diseases and high blood pressure. One of the reasons this works is because they tighten up blood vessels, so they aren’t floppy.

Even when people stand up and have to pump their blood against gravity, the tightened-up blood vessels will be able to do so to keep blood circulating and do better. Doctors found using beta-blockers to be very effective. Two medicines are used to stimulate circulations.

These are either beta-blockers or alpha medicine. Both are pretty effective, but almost everybody gets a lot better on beta-blockers, and more than half but not everybody gets better on alpha medicine.

Learning from patients and surveying how they were a year after doctors saw them, they knew to put better emphasis on beta-blockers in treatment.

When doctors saw these results a while back, they were analyzing data. They realized they needed to push beta-blockers more, so they gave beta-blockers to some and nitrogen to others.

Beta-blockers are used as the first line of medical treatment for PoTS in Mayoclinic. The clinical features of PoTS are divided into how directly related to cardiovascular response and those whose relationship to hemodynamics is less direct.

POTS SYMPTOMS WITH PECTUS EXCAVATUM

CARDIOVASCULAR SYMPTOMS

These are universal among patients and can include:

  • Lightheadedness
  • Palpitations
  • Exercise intolerance
  • Syncope in a minority of patients

NON-CARDIOVASCULAR SYMPTOMS

These are not present in all patients. But when they are, they include:

  • Fatigue
  • Anxiety
  • Tremor
  • Headache
  • Mental clouding often referred to patients as brain fog
  • Variety of symptoms such as nausea and bloating

DEVELOPMENT OF POTS

The pathogenesis of PoTS is complex and poorly understood. Multiple mechanisms can lead to the syndrome resulting in various responses to different treatments between different patients.

More than one mechanism can be present in one person. Some clinicians and researchers describe three subtypes of PoTS based on the suspected mechanism in an individual patient.

COMMON POTS SUBTYPES

  • Neuropathic
  • Hypovolemic
  • Hyperadrenergic

Some PoTS experts use these subtypes to guide treatment decisions. In contrast, others don’t find this framework helpful because none of these mechanisms or subtypes are mutually exclusive, and many patients have a more mixed subtype.

Some clinicians think it may be more beneficial to characterize the syndrome rather than classify them into one of the three categories in such patients.

DIAGNOSIS

When diagnosing PoTS, the most challenging step is suspecting it in the first place. Some of its symptoms are the same as pectus excavatum. Many doctors are not taught about the PoTS in medical school.

But once the disease is suspected, the patients have often suggested the diagnosis. The diagnostic criteria are very straightforward.

HEART RATE INCREASE

The patients should have sustained heart rate increase upon standing by:

  • At least 40 beats per minute if between the ages of 12 and 19
  • At least 30 if 20 or older.

Plus, symptoms are withstanding and absent orthostatic low blood pressure, meaning the blood pressure can’t prominently drop upon standing. In this case, an increased heart rate is usually an appropriate response to low blood pressure.

These last criteria can be tricky because the mechanisms behind orthostatic low blood pressure and PoTS can coexist in the same patient. Yet, the two syndromes cannot be diagnosed in the same patient by the current definition.

TILT TABLE TEST

Doctors can order a tilt table test to document the heart rate increase in the places available. Tilt table testing protocols vary between institutions. For example, there is not one specific PoTS protocol that everyone agrees upon, but they are all roughly similar.

HOW IT’S DONE

The tilt table itself is a bed in the clinic or hospital. The patient will lie down and be strapped on mainly to prevent falling over if they faint during the test. The patient will be hooked to cardiac and blood pressure monitors.

A pulse oximeter may measure oxygen levels though PoTS do not impact oxygenation. The patient is left supine for 10 minutes to establish a baseline heart rate and blood pressure.

At this point, the table is tilted upwards, most commonly to an angle of 70 degrees, at which point they are observed for changes in heart rate and blood pressure. A patient’s PoTS will experience a heart rate increase while the blood pressure is minimally infected.

These changes reverse when the bed is tiled back down. In contrast, there is also an increase in heart rate in patients with orthostatic hypotension though not usually as pronounced or rapid in growth.

But more importantly, from a diagnostic perspective, patients with low blood pressure while standing have a prominent decrease in systolic and diastolic blood pressure.

OTHER TESTINGS

Other tests that doctors should order on patients with suspected Pots include

  • CBC to rule out anemia,
  • Thyroid function tests to rule out hyperthyroidism. Doctors investigate alternative explanations for lightheadedness and palpitations like Wolf-Parkinson-White syndrome and Hypertrophic cardiomyopathy. An ambulatory ECG monitor worn at home can better characterize the frequency of tachycardia episodes and help rule out our diagnosis called inappropriate sinus tachycardia. The heart always beats too fast regardless of position, which is believed to be a separate clinical entity.

An echocardiogram may be ordered if syncope or shortness of breath are prominent features of the presentation or if there are unusual findings on the physical exam. And where available, doctors might do autonomic testing, including:

Thermoregulatory sweat test detects autonomic neuropathy and supine in upright plasma epinephrine and norepinephrine levels. But these specialized tests are readily necessary to make a diagnosis of Pots.

DIFFERENTIAL DIAGNOSIS

The diagnosis, which can present similarly to PoTS:

  • Orthostatic hypotension that has different causes
  • Dehydration
  • Hyperthyroidism
  • Inappropriate sinus tachycardia
  • Recurrent vasovagal syncope
  • Deconditioning
  • Anxiety disorders
  • Chronic fatigue syndrome.

Importantly, just as the mechanisms of PoTS are not mutually exclusive, these diagnoses are not mutually exclusive either. For example, a patient with PoTS might also have chronic fatigue, anxiety, vasovagal syncope, etc.

PROGNOSIS

Finally, there is the prognosis. Although the symptoms can be very frustrating and activity limiting, no known deaths are directly related to PoTS. Symptoms get better over time but can also continue throughout the years.

Article by:

Mihail Veleski

My name is Mihail Veleski. I used to suffer from the pectus excavatum deformity during my teenage years.

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