Malignant Hypertension


Article Author:
Mario Naranjo


Article Editor:
Manju Paul


Editors In Chief:
Kranthi Sitammagari
Mayank Singhal


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
2/16/2019 11:41:51 AM

Introduction

Malignant hypertension is a term that has been used to describe patients with elevated blood pressure (BP) and multiple complications with a poor prognosis. Today, the term hypertensive crisis is used to describe patients who present with severe BP elevations as follow:

  • Systolic blood pressure (SBP) greater than 180 mm Hg 
  • Diastolic blood pressure (DBP) greater than 120 mm Hg) 

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.[1][2][3][4]

Etiology

There are multiple causes of malignant hypertension (hypertensive crisis) including the following:

  • Medication noncompliance
  • Renovascular diseases, such as renal artery stenosis, polyarteritis nodosa, and Takayasu arteritis
  • Renal parenchymal disease including glomerulonephritis, tubulointerstitial nephritis, systemic sclerosis, hemolytic-uremic syndrome, systemic lupus erythematosus
  • Endocrine dysfunction, such as pheochromocytoma, Cushing disease, primary hyperaldosteronism, renin-secreting tumor
  • Coarctation of aorta; drugs or other exposures, including cocaine, phencyclidine, sympathomimetics, erythropoietin, cyclosporine
  • Antihypertensive medication withdrawal
  • Amphetamines
  • Central nervous system disorders, such as head injury, cerebral infarction, and cerebral hemorrhage

Epidemiology

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%).

Pathophysiology

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.

History and Physical

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.[5][6][7]

Evaluation

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:

  • Electrocardiography
  • Chest x-Ray
  • Urinalysis
  • Electrolytes and creatinine
  • Cardiac biomarkers, when the acute coronary syndrome is suspected
  • Toxicology screen
  • CT/MRI of the brain, when head injury, neurologic symptoms, hypertensive retinopathy, nausea, or vomiting are present
  • Contrast CT/MRI of the chest or TEE, if aortic dissection is suspected

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.

Treatment / Management

Adequate therapy, including the choice of the medication and the BP target, changes depending on the specific hypertensive emergency and the affected organ.[8][9][10][11]

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:

  • Nicardipine, initial infusion rate 5 mg per hour, increasing by 2.5 mg per hour every 5 minutes to a maximum of 15 mg per hour
  • Sodium nitroprusside, 0.3 to 0.5 mcg/kg/minute, increase by 0.5 mcg/kg per minute every few minutes as needed to a maximum dose of 10 mcg/kg per minute
  • Labetalol 10 to 20 mg IV followed by bolus doses of 20 to 80 mg at 10-minute intervals until a target blood pressure is reached to a maximum 300-mg cumulative dose
  • Esmolol, initial loading dose 500 mcg/kg/minute over 1 minute, then 50 to 100 mcg/kg/minute to a maximum dose of 300 mcg/kg per minute.

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:

  • Acute Phase of an Ischemic CVA: The BP is usually not treated unless it is greater than 185/110 mmHg in patients who reperfusion therapy could be an option or greater than 220/120 mm Hg in patients who might not qualify for it. Consider labetalol or nicardipine infusion.
  • Acute Aortic Dissection: The SBP should be lowered to 120 mm Hg within 20 minutes, and a target heart rate around 60 beats per minute, to reduce aortic shearing forces. Treatment usually requires a beta blocker and a vasodilator. Options include esmolol, nicardipine, or nitroprusside.
  • An Intracerebral Hemorrhage: The goals of therapy are different and depend on the location and surgical approach.
  • Acute Myocardial Ischemia: Nitroglycerin is the drug of choice; do not use if the patient has taken phosphodiesterase inhibitors, including sildenafil or tadalafil, within the past 48 hours.

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.

Complications

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.

Pearls and Other Issues

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.

Enhancing Healthcare Team Outcomes

The management of malignant hypertension is multidisciplinary and includes an internist, nephrologist, cardiologist and a neurologist. for asymptomatic patients, regular overnight admission is adequate. Those with symptoms needs to be monitored and the specialists consulted to determine for presence of end organ injury.

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. 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. [12](Level V)


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Malignant Hypertension - Questions

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A 68-year-old man presents with a 4-day history of an intense headache, chest pain, blurry vision, and tea-colored urine. He says he ran out of his blood pressure medications and could not afford to buy more pills. His exam reveals blood pressure of 210/115, pulse of 111, and respiration of 20. The quick eye exam reveals flame hemorrhage and papilledema. What is the next step in his management?



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A 44-year-old African-American male complains of a headache and general malaise. He says he has not been feeling well for the past 48 hours. He has a history of hypertension and takes a diuretic. He appears pale with a blood pressure measuring 220/110 mmHg. Soon after, he develops chest pain and dies. Which of the following findings would associate with this disorder?



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Which of the following is false regarding malignant hypertension?



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Which of the following has the worst prognosis in a patient with hypertension?



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What is the best intervention for a 58-year-old African American female with a history of asthma presenting with headaches? She has a BP of 240/130 and papilledema.



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Which of the following is the best initial treatment for severe hypertension in an asthmatic?



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Malignant hypertension is always characterized by:



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Which symptoms may be present in a patient with malignant hypertension? Select all that apply.



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Malignant Hypertension - References

References

Yong B,Power DA, Malignant Hypertension Causing a Pulmonary-Renal Syndrome. Case reports in nephrology. 2018;     [PubMed]
Riemekasten G, [Progress in Systemic Sclerosis - Early, Targeted and Intensive Therapy is the Key to Success]. Deutsche medizinische Wochenschrift (1946). 2019 Feb;     [PubMed]
Mir D,Ardabilygazir A,Afshariyamchlou S,Sachmechi I, Malignant Hypertension in Association with Low Estrogen Dose Oral Contraceptives: Case Report and Review of Literature. Cureus. 2018 Jul 13;     [PubMed]
Dworakowska D,Grossman AB, Aggressive and malignant pituitary tumours: state-of-the-art Endocrine-related cancer. 2018 Nov 1;     [PubMed]
Kimura N,Takekoshi K,Naruse M, Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine. Journal of clinical medicine. 2018 Aug 27;     [PubMed]
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Smith M, Refractory Intracranial Hypertension: The Role of Decompressive Craniectomy. Anesthesia and analgesia. 2017 Dec;     [PubMed]
Brokmann JC,Rossaint R,Müller M,Fitzner C,Villa L,Beckers SK,Bergrath S, Blood pressure management and guideline adherence in hypertensive emergencies and urgencies: A comparison between telemedically supported and conventional out-of-hospital care. Journal of clinical hypertension (Greenwich, Conn.). 2017 Jul;     [PubMed]
Shah M,Patil S,Patel B,Arora S,Patel N,Garg L,Agrawal S,Jacobs L,Steigerwalt SP,Martinez MW, Trends in Hospitalization for Hypertensive Emergency, and Relationship of End-Organ Damage With In-Hospital Mortality. American journal of hypertension. 2017 Jul 1;     [PubMed]
van der Merwe W,van der Merwe V, Malignant hypertension: a preventable emergency. The New Zealand medical journal. 2013 Aug 16;     [PubMed]
Liu S,Song A,Zhou X,Kong X,Li WA,Wang Y,Liu Y, Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy: Literature review with one case report. Medicine. 2017 Nov;     [PubMed]

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