What is Conn's Syndrome of the Adrenal Gland?Primary hyperaldosteronism (PA or “ALDO”) was first described by Dr. Jerome Conn (This is why the disease is sometimes referred to as Conn’s syndrome) and is the most frequent cause of secondary hypertension (Secondary hypertension means that it is potentially curable). It is most commonly caused by a benign aldosterone-producing adenoma (APA). Yes, the whole problem is caused by a small (but toxic) tumor of the adrenal gland that is almost always benign.
Conn's Syndrome is not Rare. It used to be thought that primary hyperaldosteronism was very rare. It is not, the reason was that doctors did not know about it and many patients were not feeling terribly sick (however, over time the disease damages your heart, vessels and brain), and doctors were not actively screening patients to find the disease. Also, many were taught in medical school and believed you cannot have primary hyperaldosteronism with a normal blood potassium. This is incorrect. Recent studies have reported that only a minority—less than a third—of patients are hypokalemic (low potassium) and thus, the presence of hypokalemia should not be considered necessary to test for primary hyperaldosteronism. Although the exact number is difficult to know, it is now in the modern era estimated that 4-10 % of all patients with high blood pressure have underlying primary hyperaldosteronism.
The importance of diagnosis of primary hyperaldosteronism is not just to identify patients who can potentially be cured of their blood pressure (yes, no more blood pressure medications, no more potassium pills) by a straightforward procedure, but to have them feel better and live longer. Almost all patients can be cured with a 30-40 minute mini-scope operation by an expert adrenal surgeon--we do this operation several times per week. This toxic (and very silent) disease can often be cured by a 20-30-minute operation. It may save you from developing future strokes, heart attacks, and arrhythmias (for instance atrial fibrillation), and premature death. Read more below about what is called “target organ damage” and reduced quality of life in primary hyperaldosteronism here.
The diagnosis of primary hyperaldosteronism follows a logic three step approach that is: 1. Screening for the disease with blood tests for aldosterone and potassium, 2. Confirming the disease with repeat blood tests and x-ray tests (CT Scan with contrast), 3. Perform subtype diagnosis
Symptoms of Conn's Syndrome; Primary HyperaldosteronismPatients with Conn's syndrome due to overproduction of the adrenal hormone aldosterone from an adrenal tumor are often without symptoms (asymptomatic). Often, the only sign that anything is wrong may by hypertension (mild to severe high blood pressure). Classicly, the blood pressure is mostly diastolic (the lower number is high while the systolic (the upper number) is normal. If hypokalemia is present (hypokalemia means low blood potassium; only about 9-37 % have low blood potassium levels) is present then the patient may have nocturia (urination at night time), polyuria (frequent urination), muscle cramps, and palpitations (raising heart beat).
Need for Screening for Conn's Syndrome and Aldosterone Producing Adrenal Tumors. As mentioned, 4-10% of all patients with high blood pressure have underlying primary hyperaldosteronism that nobody has figure out. It is approximated that 75 million Americans suffer from hypertension. This means that there are about 3-7.5 million Americans with primary hyperaldosteronism. Only a fraction of all these -- less than one tenth of one percent -- are being diagnosed, and an even smaller fraction are being offered surgery. With minimally invasive adrenalectomy, the vast majority of these patients can be cured in less than 30-40 minutes. Surgical removal of the adrenal tumor may save you from developing future strokes, heart attacks, and arrhythmias (for instance, atrial fibrillation), and premature death. This is why people with high blood pressure need to be screened for this disease.
Who Should be Tested for an Aldosterone-Secreting Adrenal Tumor and Conn's Syndrome?If you belong to any of the below groups, you need to be screened for primary hyperaldosteronism. Screening is easy. Ask our doctor, via a simple blood test, to check your plasma aldosterone concentration (PAC) and plasma renin activity (PRA). If dividing the PAC by the PRA: PAC/PRA (this is called ARR; aldosterone-renin ratio) the result is greater than 20, you may have primary hyperaldosteronism. This screening test is the ARR test. If you belong to any of the below groups, you need to be screened for primary hyperaldosteronism.
- Drug-resistant hypertension (i.e. blood pressure that is not controlled well with medications)
- Hypertension requiring many medications
- Hypertension requiring being on Spironolactone (Aldactone), Eplerenone (Inspra) or Amiloride
- Hypertension of any grade, especially without known risk factors such as advanced age, kidney problems, diabetes, obesity, etc.
- Hypertension at a young age (< 50 years of age)
- Hypertension with low blood potassium (hypokalemia)
- Hypertension with an adrenal incidentaloma (any adrenal tumor on CT scan)
- Hypertension and sleep apnea
- Hypertension and a family history of early-onset (< 50 years of age) hypertension or cerebrovascular accident (stroke) at a young age
- Hypertension in a family with any family member with an adrenal tumor (whether a Conn tumor, Cushing’s syndrome or pheochromocytoma/paraganglioma)
- Hypertension that is episodic (paroxysmal)