Malignant hypertension is a term that has been used to describe patients with elevated blood pressure (BP) and multiple complications (End organ damage) with a poor prognosis. Today, the term hypertensive crisis is used to describe patients who present with severe BP elevations as follow:
The diagnosis can be further classified as a hypertensive emergency when severe elevation in BP is associated with end-organ damage, or hypertensive urgency when severe hypertension occurs without it. Prompt treatment of BP can prevent a hypertensive emergency and consequently, serious life-threating complications.
In order to make a diagnosis of malignant hypertension, papilledema has to be present. In malignant hypertension, the key is to lower the blood pressure within a few hours.
There are multiple causes of malignant hypertension (hypertensive crisis) including the following:
Hypertensive emergencies are unusual, with a projected incidence of 1 to 2 cases per million per year. A recent investigation showed that the estimated number of visits due to this condition and the rate per million per adult, emergency department (ED) visits have more than doubled from 2006 to 2013. Few examples include eclampsia (2%), cerebral infarction (39%), and acute pulmonary edema (25%).
Hypertensive emergencies occur when a relatively rapid elevation of BP develops in a short period. Increase in systemic vascular resistance by an increase in vasoconstriction mechanisms through renin-angiotensin activation, pressure natriuresis, hypoperfusion, and ischemia are the most common culprits of end-organ damage. The classic vascular feature is fibrinoid necrosis of the small vessels. In addition, red cell destruction is common as they pass through these obstructed vessels leading to microangiopathic hemolytic anemia. Another feature of a hypertensive emergency is the loss of autoregulation in the brain, which can present as hypertensive encephalopathy.
Most patients have persistently-elevated BP for years before presenting with hypertensive emergency. Initial questioning should focus on finding indicators related to end-organ damage including a headache, nausea or vomiting, visual disturbances, chest or back pain, dyspnea, orthopnea, or visual disturbances. Review all prescription and nonprescription medications, adherence, and time from the last dose. Ask about recreational drug use, such as amphetamines, cocaine, phencyclidine.
On physical exam, confirm BP on both arms using appropriately-sized blood pressure cuff. Funduscopic exam findings may include hemorrhages, exudates or papilledema. Assess for murmurs and gallops or other signs of heart failure. Look for evidence of pulmonary edema, abdominal bruits. Neurological findings can include stupor, seizures, delirium, agitation.
The history and physical examination are really important in patients presenting with a very elevated BP, or an acute rise over a previously normal baseline, even if the presenting BP is less than 180/120 mm Hg. Furthermore, the following evaluation should be performed to find the presence of end-organ damage in association with targeted clinical symptoms or signs:
It is often easiest to categorize hypertensive emergencies by the organ that is being damaged. The evaluation above can usually identify the at-risk target organ and can direct both, the target BP and the promptness with which the target is achieved.
The cardiac exam may reveal the presence of an MI, CHF or pulmonary edema. Concentric left ventricular hypertrophy is often present, including the 4th heart sound. Always check the blood pressure in both arms to rule out aortic dissection. Patients may also have bruit in the neck and groin
CNS exam may reveal a headache, visual changes, vomiting, confusion, and seizures. The eye exam may reveal soft exudates, flame-shaped hemorrhages, and papilledema
The renal exam may present with oliguria and the GI symptoms may include nausea, vomiting and vague abdominal pain.
It is not recommended to decrease the BP too fast or too much, as ischemic damage can occur in vascular territories that have become habituated with the elevated level of BP. For the majority of hypertensive emergencies, mean arterial pressure (MAP) should be reduced by approximately 10 to 20% within the first hour and by another 5% to 15% over the next 24 hours. This often results in a target BP of less than 180/120 mm Hg for the first hour and less than 160/110 mm Hg for the next 24 hours, but rarely less than 130/80 mm Hg during that time frame.
Common intravenous (IV) medications and doses used to treat hypertensive emergencies include:
If there is any possibility of over or underestimation of BP using frequent noninvasive cuff measurements or if the end-organ damage is life-threatening, consider arterial catheterization for precise, second-to-second measurements allowing for more careful medication titration.
The major exceptions to gradual BP lowering over the first day are:
After a suitable period, often 8 to 24 hours, of BP control at a target, oral medications are usually given, and the initial intravenous therapy is tapered and discontinued.
ACEP Guidelines for managing hypertension in the ED
The prognosis of patients with malignant hypertension is guarded. Five-year survivals of 75-84% have been reported with treatment; without treatment, the life expectancy is less than 24 months. Most deaths are a result of heart failure, stroke or renal failure.
Multiple complications can arise when target organs are affected, including encephalopathy, intracerebral hemorrhage, acute myocardial infarction, acute heart failure, pulmonary edema, unstable angina, dissecting aortic aneurysm, acute kidney injury, and vision loss.
Most patients with severely elevated BP have no acute end-organ damage (hypertensive urgency). Nevertheless, some patients have signs and symptoms of acute, ongoing injury, which is recognized as hypertensive emergency or formerly as malignant hypertension.
It is generally not recommended to decrease the BP too quickly or too much as ischemic damage can occur in vascular territories that have become habituated with the elevated level of BP. For most hypertensive emergencies, the MAP should be reduced gradually by approximately 10 to 20% within the first hour, and by a further 5% to 15% over the next 24 hours, with a final goal of approximately 25% reduction compared with baseline.
Many patients have a poorly controlled essential or secondary hypertension. Long-term management turns out to be the primacy once the hypertensive crisis has been addressed.
The management of malignant hypertension requires an interprofessional team that includes an internist, nephrologist, cardiologist and a neurologist. Untreated elevated blood pressure has high morbidity and mortality, including enormous healthcare costs. The primary care providers including the nurse practitioner should regularly monitor blood pressure and emphasize medication compliance.
For asymptomatic patients, admission is not recommended. Those with symptoms need to be monitored and the specialists consulted to determine for the presence of end-organ injury.
It is not recommended to decrease the BP too fast or too low, as ischemic damage can occur in vascular territories that have become habituated with the elevated level of BP. For the majority of hypertensive emergencies, mean arterial pressure (MAP) should be reduced by approximately 10 to 20% within the first hour and by another 5% to 15% over the next 24 hours.
All patients with hypertension should be encouraged to follow up in the outpatient clinic with a cardiology nurse. In addition, adherence to a low salt diet is beneficial. The members of the interprofessional team should continually encourage patients to remain compliant with their blood pressure medications and also monitor the blood pressure at home.
The outcome for most patients with malignant hypertension in the short term is good but in the long term exacerbations are common. Strokes, vision loss, kidney damage, and adverse cardiac events are known to occur in a number of patients who are not compliant with therapy. (Level V)
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