Chapter 26. Systemic and Pulmonary Hypertension

Classification of acute systemic hypertension into categories facilitates management:

1. Hypertensive emergency: elevated blood pressure (BP) associated with target organ dysfunction such as aortic dissection, acute pulmonary edema, acute coronary syndrome, acute renal failure, severe preeclampsia, hypertensive encephalopathy, subarachnoid hemorrhage, intracranial hemorrhage, acute ischemic stroke, and sympathetic crisis. Immediate recognition and treatment are required but therapeutic goals vary considerably.

2. Hypertensive urgency: a clinical presentation associated with severe elevations in blood pressure without progressive target organ dysfunction. The arbitrary numerical criterion of ≥180/110 mm Hg is often cited as an indication for treatment, when in fact the clinical benefit of such treatment is not well defined (see Emergency Department Care and Disposition section).

The clinician must ensure that the BP cuff size is appropriate for the patient's size; a small cuff relative to the arm size produces a falsely elevated reading.

Essential historic features include a prior history of HTN; noncompliance with BP medications; cardiovascular, renal, or cerebrovascular disease; diabetes; hyperlipidemia; chronic obstructive pulmonary disease or asthma; and a family history of HTN.

Precipitating causes such as pregnancy, illicit drug use (cocaine and methamphetamines), or decongestants should be considered. Patients should be asked about central nervous system symptoms (headaches, visual changes, weakness, seizures, and confusion), cardiovascular symptoms (chest pain, palpitations, dyspnea, syncope, pedal edema, or tearing pain radiating to the back or abdomen), and renal symptoms (anuria, edema, or hematuria). The patient should be examined for evidence of papilledema, retinal exudates, neurologic deficits, seizures, or encephalopathy; the presence of these findings may constitute a hypertensive emergency in the setting of elevated blood pressure. The patient also should be assessed for carotid bruits, heart murmurs, gallops, asymmetrical pulses or unequal blood pressures (coarctation vs aortic dissection), pulsatile abdominal masses, and pulmonary rales. Hypertensive encephalopathy is characterized by altered mental status in the setting of acute hypertension, and may be accompanied by headache, vomiting, seizures, visual disturbances, papilledema, or hematuria. In the pregnant (or postpartum) patient, the clinician should look for hyperreflexia and peripheral edema, suggesting preeclampsia.

Testing should be guiding by presenting symptoms, the most cost effective test is urinalysis. Renal impairment may present as hematuria, proteinuria, red cell casts, or elevations in blood urea nitrogen, creatinine, and potassium levels. An electrocardiogram may show ST- and T-wave changes consistent with coronary ischemia (see Chapter 18), electrolyte abnormalities, or left ventricular hypertrophy. A chest x-ray may help identify congestive heart failure (see Chapter 22), or aortic dissection (see Chapter 27). In patients with neurologic compromise, computed tomography of the head may show ischemic changes, edema, or blood (see Chapter 141). A urine or serum drug screen may identify illicit drug use. A pregnancy test should be done on all hypertensive women of childbearing potential.

Patients with hypertensive emergencies require O2 supplementation, cardiac monitoring, and intravenous access. After attention to the ABCs of resus-citation, the treatment goal is to reduce arterial pressure gradually in the following clinical situations, with attention to the therapeutic goal.

1. Aortic dissection: reduce force of contraction and sheer forces first with β-blockers, reduce heart rate to approximately 60 beats/min, reduce BP below 140 mm Hg systolic, and ideally, below 120 systolic (range of 100 to 120 mm Hg) if tolerated by the patient. Recommended first agents include esmolol (300 micrograms/kilogram IV bolus followed by a 50 micrograms/kilogram/min infusion) or labetalol (20 milligram IV over 2 min followed by subsequent doses of 20 to 40 milligrams IV every 10 min as needed up to 300 milligrams maximum). If β-blockers are contraindicated, use verapamil 5 to 10 milligrams IV, or diltiazem 0.25 milligram/kilogram IV over 2 min, to reduce heart rate. Follow β-blockers with vasodilators as needed to achieve desired blood pressure reduction. Nicardipine can be started by IV infusion: start at a rate of 5 milligrams/h. If target BP is not achieved in 5 to 15 min, increase dose by 2.5 milligrams/h every 5 to15 min until target pressure or the maximum dose of 15 milligrams/h is reached. Alternatively, use nitroprusside IV infusion: 0.3 to 0.5 microgram/kilogram initial infusion, increase by increments of 0.5 microgram/kilogram/min; titrate to desired effect.

2. Acute hypertensive pulmonary edema: reduce BP by no more than 20% to 30%. First agent of choice is nitroglycerin, sublingual 0.4 milligram, up to 3 doses, paste 1 to 2 inches, or IV infusion, start 5 micrograms/min, increase by 5 micrograms/min every 3 to 5 min to 20 micrograms/min; if no response at 20 micrograms/min, increase by 10 micrograms/min every 3 to 5 min, up to 200 micrograms/min. Alternatives include enalaprilat IV, 0.625 to 1.25 milligrams over 5 min every 4 to 6 hours, titrate at 30 min intervals to a maximum of 5 milligrams every 6 hours, nicardipine (see dose above, #1) or nitroprusside (see dose above, #1).

3. Acute coronary syndrome: if BP is above 160 mm Hg systolic, reduce BP no more than 20% acutely. Start with nitroglycerin (see dose above, #2) or metoprolol 50 to 100 milligrams PO every 12 hours, or 5 milligrams IV every 5 to 15 min up to 15 milligrams. Avoid IV β-blockers if patient at risk for cardiogenic shock.

4. Acute sympathetic crisis: treat to relieve symptoms, start with benzodiazepines first. Follow with nitroglycerin (see dose above, #2), or phentolamine, bolus load: 5 to 15 milligrams IV.

5. Acute renal failure: for BP above 180/110 mm Hg, reduce BP by no more than 20% acutely. Recommend agents include labetalol (see dose above, #1) nicardipine (see dose above, #1) or fenoldopam, start 0.1 microgram/kilogram/min, titrate to desired effect every 15 min, range 0.1 to 1.6 micrograms/kilogram/min.

6. Preeclampsia: For BP above 160/110 mm Hg, use labetalol (see dose above, #1). Hydralazine, 5 to 10 milligrams IV, is less predictable, but commonly used.

7. Hypertensive encephalopathy: For BP above 180/110 (in the setting of immunosuppressive drugs, symptomatic BP may be lower), reduce BP by no more than 20% acutely. Recommended agents include nicardipine (see dose above, #1) labetalol (see dose above, #1), fenoldopam (see dose above, #5), or nitroprusside (see dose above, #1).

8. Subarachnoid hemorrhage: reduce systolic pressure below 160 mm Hg or MAP below 130 mm Hg to prevent rebleeding. Recommended agents include nicardipine (see dose above, #1) labetalol (see dose above, #1), or esmolol (see dose above, #1).

9. Intracranial hemorrhage: to reduce hemorrhage growth, for patients with evidence of increased intracranial pressure (decreased level of consciousness, evidence of midline shift or hematoma volume >30 mL on CT imaging.) reduce MAP to 130 mm Hg. In patients for whom there is no suspicion of increased intracranial pressure, treatment may be intensified to a MAP of 110 mm Hg, or a systolic pressure of 150 to 160 mm Hg. Recommended agents include nicardipine (see dose above, #1) labetalol (see dose above, #1), or esmolol (see dose above, #1).

10. Acute ischemic stroke: If fibrinolytic therapy is planned, reduce BP below 185/110 mm Hg; if no fibrinolytic therapy is planned and BP remains elevated on repeat measures, reduce BP below 220/120 mm Hg. More intensive lowering can be accomplished safely (however, pharmacologic reduction below 160/100 is not advised). Recommended agents include labetalol (see dose above, #1), nicardipine (see dose above, #1), or nitroglycerin paste, 1 to 2 inches.

11. For hypertensive urgency, useful agents include oral labetalol 200 to 400 milligrams, repeated every 2 to 3 hours; oral captopril 25 milligrams every 4 to 6 hours; sublingual nitroglycerin spray or tablets (0.3 to 0.6 milligram); or clonidine, 0.2 milligram oral loading dose, followed by 0.1 milligram/h until the DBP is below 115 mm Hg, or a maximum of 0.7 milligram.

12. For asymptomatic patients with severe hypertension, with BP above the 180 to 200 systolic range, or above 110 to 120 diastolic range, starting an oral agent at discharge should be considered. The choice of the oral agent should be based on coexisting conditions, if any. Diuretics, such as hydrochlorothiazide 25 milligrams/d, should be used in most patients with uncomplicated HTN. For patients with angina, postmyocardial infarction, migraines, or supraventricular arrhythmias, a β-blocker should be considered, such as metoprolol 50 milligrams orally 2 times daily. Angiotensin-converting enzyme inhibitors such as lisinopril, start at 10 milligrams daily, can be used in those with heart failure, renal disease, recurrent strokes, or diabetes mellitus. Restarting a noncompliant patient on a previously established regimen is a recommended strategy.

Children often will have nonspecific complaints such as throbbing frontal headache or blurred vision. Physical findings associated with HTN are similar to those found in adults.

The most common etiologies in this age group are renovascular lesions and pheochromocytoma. The decision to treat a hypertensive emergency in a child is based on the BP and associated symptoms. Urgent treatment is required if the BP exceeds prior measurements by 30%. Alternatively, if prior measurements are not known, childhood hypertension is defined as diastolic or systolic blood pressure ≥95th percentile on a standardized table. The goal is to reduce the BP by 25% within 1 hour in acutely symptomatic patients. Preferred medications for the control of hypertensive emergencies in children include labetalol, 0.2 to 1.0 milligram/kilogram per dose, up to 40 milligrams/dose, or an infusion of 0.25 to 3.0 milligrams/kilogram/h; nicardipine, 1 to 3 micrograms/kilogram/min; or, if prior drugs fail, nitroprusside, 0.5 to 10.0 micrograms/kilogram/min. The treatment of pheochromocytoma is surgical excision and managing the BP with α-adrenergic blockers such as phentolamine. Pediatric HTN that requires intervention in the emergency department will likely require admission.

Although the diagnosis of pulmonary hypertension cannot be made in the ED, suspicion of primary or secondary pulmonary hypertension as a cause of dyspnea, chest pain, or syncope can affect ED evaluation, consultation, or disposition. Pulmonary hypertension is a pathologic condition characterized by elevation of the pulmonary vascular pressure, which compromises right ventricular function. There can be an isolated increase in pulmonary arterial pressure or an elevation of both arterial and venous pressure. The hemodynamic parameter that defines pulmonary hypertension is a median pulmonary artery pressure of >25 mm Hg at rest or >30 mm Hg during effort. The most common symptoms are dyspnea, fatigue, syncope, and chest pain. Typically, this disorder will be seen in association with other cardiovascular or pulmonary disorders such as chronic obstructive pulmonary disease, left ventricular dysfunction or disorders associated with hypoxemia. Treatment of the underlying disorder is the only management indicated in the emergency setting, for example, oxygen for conditions producing hypoxia. Patients may be on calcium channel blockers chronically, or for primary pulmonary hypertension, patients may have arrangements for home infusions of epoprostenol (prostacyclin).