Conn’s syndrome - A Silent Killer
April is adrenal disease awareness month, and today we would like to highlight a very silent but potentially lethal disease – Conn’ syndrome or primary hyperaldosteronism.
Hypertension, or high blood pressure, has been called a silent killer because often there are no associated symptoms. Individuals with high blood pressure can go undiagnosed for years if they do not have something tipping them off that their blood pressure is too high, such as persistent headaches, or even worse, a major cardiac event such as heart attack or stroke.
Imagine a busy parent of two children, working hard and long hours at his job to provide for his family, exercises when he can on the weekends (“weekend warrior”), and considers himself “active” because he can still keep up with the kids. Sounds familiar, right? What if this same dad does not have a primary care physician because he has not yet gotten around to it and does not know that he has been suffering with high blood pressure for years. Then, suddenly, he has a heart attack, or potentially worse, a stroke. Only until then, is he diagnosed with long-standing high blood pressure causing his cardiovascular collapse. We can see how high blood pressure has been termed the silent killer. This dad had no idea until an unexpected and catastrophic event occurred.
In roughly 5-10% (some studies mention a prevalence up to 20%) of individuals with high blood pressure, there may be an underlying tumor in the adrenal gland producing too much aldosterone. Hypertension, caused by an aldosterone-producing tumor, or Conn’s syndrome (primary hyperaldosteronism), has been discussed here. Even worse than the scenario above is the individual with known hypertension for years, being followed by his primary care physician, heart doctor, or kidney doctor, and being treated with multiple antihypertensive (“blood pressure”) medications, without a known cause for the high blood pressure. A silent, but deadly, adrenal tumor is over-producing aldosterone, which could have been removed with a 20-30 minute minimally invasive procedure. These scenarios drive home the points of having your blood pressure checked regularly, and if known to be high, obtain simple blood tests to ensure you are not missing a silent killer, Conn’s syndrome (other names include primary aldosteronism (PA), aldosterone-producing tumor, and “Aldo”). Dr. Carling edit: A recent study proved that only 2.7% of patients who should be screened for Conn’s syndrome as a cause of their hypertension are ever evaluated. This means that patients with high blood pressure needs to ask to be screened!
Conn’s syndrome - A Silent Killer
Primary aldosteronism can be the silent killer causing the silent killer, hypertension! Conn’s syndrome causing hypertension can increase the risk of stroke, heart attack and heart arrhythmias 10-fold (1,000%) compared to age-, sex- and blood pressure-matched patients with essential hypertension (high blood pressure not related to an adrenal tumor).
Too much aldosterone is toxic to our bodies, which can ultimately lead to targeted organ damage. One of the organs primarily targeted by too much aldosterone is the kidneys, which in turn leads to high blood pressure. Unfortunately, the kidneys are organs that do not bounce back after being repeatedly knocked down (long-standing exposure to too much aldosterone), silently killing those afflicted. With less than 5% of Conn’s syndrome patients being diagnosed in a timely manner, if at all (and an even smaller percentage being offered curative surgery), we can see how Conn’s syndrome has become a silent killer. No physician can know if something is there if they do not look for it!
Figure 1. Consequences of Target Organ Damage from Conn’s Syndrome- A Silent Killer
An aldosterone-producing adrenal tumor creates toxicity causing target organ damage. Traditionally, aldosterone has been considered the main regulator of water and electrolyte equilibrium due to its effects on the distal colon and collecting ducts of the kidneys. Aldosterone’s effects within other tissues have been historically less understood. However, more recent studies within human and animal models have provided new insights on how Conn’s syndrome silently kills, targeting the kidneys, heart, blood vessels, and central nervous system.
Conn’s syndrome silently killing your kidneys
Due to elevated aldosterone levels, oftentimes long-standing from delayed diagnosis, and the fact that the kidneys do not bounce back well from repeated insult, many Conn’s syndrome referrals to us are already showing signs of kidney damage (increased creatinine and decreased glomerular filtration rate [GFR]). Many animal model studies of aldosterone excess have shown that the increased aldosterone is a direct promoter of kidney disease. The good news is that adrenalectomy (by the world’s foremost expert) has proven effective in preventing further deterioration of kidney function in patients with Conn’s syndrome regardless of how high the blood pressure has become. No one wants to be dependent on a dialysis machine. So, if you have high blood pressure, and worsening kidney function, you need to make sure you do not have an aldosterone-producing tumor slowly killing your kidneys.
Figure 2. Too much aldosterone is very toxic to the small vessels of the kidney leading to chronic kidney disease and kidney failure.
Conn’s syndrome silently killing your heart
Too much aldosterone can cause heart inflammation, fibrosis (scarring), and enlargement. This has been demonstrated in animal models, where increased aldosterone promoted inflammatory damage within the heart muscle. This damage led to the heart muscle remodeling, with subsequent heart enlargement, independent of elevated blood pressure. If the heart is bigger, it is working harder to effectively pump blood.
Studies in human patients also suggest that primary hyperaldosteronism is associated with increased incidence and severity of left ventricular hypertrophy (the chamber of the heart that pumps blood to the rest of the body grows too big and does not function properly). The aldosterone effect, regardless of the inevitable blood pressure elevation, demonstrated that those with Conn’s syndrome will have significantly greater heart damage (even after correction for age, gender, duration of disease, and severity of hypertension) compared to those with high blood pressure and no aldosterone-producing adrenal tumor. Conn’s syndrome, the silent killer, strikes again!
Figure 3. Take care of your heart. You only have one. Make sure you do not have an adrenal tumor producing hormones injuring your heart, and heart muscle.
Conn’s syndrome silently killing your blood vessels
Aldosterone excess is toxic to the inner lining of blood vessels leading to an increased risk of heart attack, stroke, and arrhythmia (atrial fibrillation being the most common). Ultimately, with any of these conditions, premature death may occur. Conn’s syndrome, leading to inflammation within the lining of your vessels (arteries and veins), and as a result, deficits in blood transport throughout the body via these vessels, is not good for your overall health. Aldosterone will cause problems with relaxation of your vessels leading to vascular stiffness. In patients with Conn’s syndrome, research has proven that blood vessels have more scarring, contributing to increased vessel wall thickness, decreased ability for the vessel to maintain its shape and function (elasticity), and increased vascular resistance (high blood pressure). If a 40-year-old has these undetected problems going on within her blood vessels, she is experiencing premature vascular aging. And, depending on how long this silent adrenal tumor has been wreaking havoc, she really has the blood vessels and heart of a 60-, 70-, or 80-year-old!
Figure 4. Aldosterone and other adrenal hormones affect the fine vessels of the brain. Damage to these vessels leads to stroke and brain injury.
Conn’s syndrome silently killing your brain and Central Nervous System (CNS)
The brain is considered one of the main targets of aldosterone. Increased aldosterone levels in the brain and CNS can increase blood pressure. Excess aldosterone-mediated CNS damage occurs in the vascular bed and can predispose to a catastrophic acute cerebrovascular event such as stroke. To prevent a devastating injury to the brain, those with Conn’s syndrome should be treated surgically to remove the adrenal gland harboring the aldosterone-producing tumor. Too much aldosterone also affects how you think and feel. Conn’s syndrome causes anxiety, depression, fatigue, psychological distress, reduced activity, emotional mood swings, and body aches and pains.
The bottom line is to have your blood pressure checked regularly, and if it is high and not able to be easily controlled, consider checking your aldosterone and renin levels. Have an endocrine surgeon interpret your data to see if you are a candidate for life-saving surgery. Don’t let Conn’s syndrome continue to be a silent killer.
Additional Resources:
- Learn more about the Carling Adrenal Center
- Learn more about Dr. Tobias Carling
- Learn more about our sister surgeons at the Norman Parathyroid Center, Clayman Thyroid Center and Scarless Thyroid Surgery Center
- Learn more about the Hospital for Endocrine Surgery