The call came one afternoon in June 2009. I remember exactly where I was and what went through my mind when my brother told me, “The fluid around Mom’s heart has increased and is causing her heart to fail.” I was at the top of the stairs looking down at my front door, realizing these were the very words I’d been dreading for 9 years.
I was afraid my mother was dying. Diagnosed in 2000 with a small pericardial effusion, she’d been worked up for every conceivable cause, from rheumatoid arthritis to cancer. All the test results were negative. The doctors were baffled but not too concerned, so her effusion was deemed idiopathic.
Every 6 months, when my mother went for routine echocardiograms, I’d wait for the results with fingers and toes crossed. The conclusion was always “No change.” This caused conflicting emotions for our family. It left us wondering what was causing the effusion, yet relieved it hadn’t grown larger—until that day 4 years ago when we learned the effusion not only had enlarged but had brought on cardiac tamponade. (See How pericardial effusion progresses to cardiac tamponade.)
How pericardial effusion progresses to cardiac tamponade
The pericardium is a thin sac that surrounds the heart and its blood vessels. A small amount of lubricating fluid separates its inner and outer layers. Pericardial effusion results from an increase in this lubricating fluid.
If fluid accumulation comes on rapidly or the effusion is large, cardiac tamponade may occur. Tamponade reduces the force of myocardial contractions because fluid trapped in the pericardial space compresses the heart, in turn reducing ventricular filling and causing hemodynamic compromise. About 7% to 10% of patients with pericardial effusions are at risk for cardiac tamponade, which is a medical emergency. The risk of death from cardiac tamponade depends on the speed of diagnosis and treatment and the underlying cause. Untreated, the condition is fatal.
Cardiac tamponade is the most severe manifestation of hemodynamic compromise caused by a tense pericardial effusion. Pericardial effusion with tamponade should be considered in any patient with shortness of breath, chest pain or trauma, or an autoimmune, cardiac, or infectious disorder. Typically, the patient has dyspnea, tachycardia, jugular vein distention, chest pain, pulsus paradoxus, rapid breathing, swelling of the abdomen or other areas, dizziness, and anxiety. In more severe cases, arterial hypotension and shock may occur.
Causes of pericardial effusion
Pericardial effusion is a common finding in clinical practice. In some cases, its origin is apparent. Causes include:
- infection (bacterial, viral, fungal, or parasitic)
- human immunodeficiency virus
- hemorrhage into the pericardial space (caused by chest trauma, myocardial rupture or infarction, aortic rupture, or cardiopulmonary bypass surgery)
- radiation therapy
- renal failure
- autoimmune diseases (such as hypothyroidism, inflammatory bowel disease, systemic lupus erythematosus, and rheumatoid arthritis).
Occasionally, the cause remains unknown. Inflammatory signs and symptoms (such as chest pain, fever, and pericardial friction rub) predict idiopathic pericarditis. Chronic idiopathic pericardial effusion is diagnosed when the fluid lasts more than 3 months with no apparent cause.
The amount of pericardial fluid needed to impair heart function depends on the fluid accumulation rate and pericardial compliance. Just 150 mL of rapidly accumulating fluid can severely compromise cardiac output, whereas 1,000 mL of gradually accumulating fluid may not significantly affect heart function because of adaptive pericardial stretching.
Pericardial effusion and cardiac tamponade can be diagnosed from the patient history, physical exam, chest X-ray, electrocardiography, and echocardiography.
In most cases, pericardial effusion can be managed medically with anti-inflammatory drugs, corticosteroids, colchicine, and diuretics. In an emergency, pericardiocentesis or pericardial window surgery may be required.
- In pericardiocentesis, fluid is aspirated from the pericardium via a needle inserted just below the breast bone and into the pericardial sac. Echocardiography guides needle positioning and helps monitor fluid drainage. However, fluid may return depending on the underlying cause of the effusion, and long-term drainage may be indicated. Emergency pericardiocentesis is done for life-threatening hemodynamic changes of cardiac tamponade. Nonemergency pericardiocentesis in stable patients is done for diagnostic, palliative, or prophylactic reasons.
- Pericardial window surgery may be indicated when longer-term drainage is needed. A fistula or window is made from the pericardium into the pleural cavity. This allows the effusion to drain from around the heart into the chest cavity, where it is less dangerous and usually reabsorbed by the body. Typically, a cardiothoracic surgeon cuts a small hole in the pericardium below the sternum, through a subxiphoid incision or between the ribs on the left side of the chest.
My mother had an emergency pericardiocentesis. The cardiologist removed a cup of fluid from her pericardial sac. She was admitted overnight to the telemetry unit and discharged the next day feeling much better. Fluid analysis showed inflammation and nothing diagnostic. But 3 days later, she noticed ankle edema, mild to moderate chest discomfort, and shortness of breath. An echocardiogram confirmed that the pericardial effusion and cardiac tamponade were back—this time with a vengeance. She was sent immediately to a cardiothoracic surgeon, who performed pericardial window surgery. She spent the next 3 days on the telemetry unit with a subxiphoid drain with closed drainage.
After her discharge and the expected 6-week recovery period, her condition continued to deteriorate. For months she was weak and anorexic, and she lost weight. She had tremendous headaches and so much pain in her chest, neck, shoulders, and upper back that she couldn’t care for herself. I feared she was dying. Follow-up echocardiography showed a small pericardial effusion and lab tests revealed an elevated sedimentation rate (ESR), indicating inflammation; her hematocrit and hemoglobin values wre below normal.
She was referred to a rheumatologist. The rheumatologist reviewed my mother’s test results, performed a physical exam, obtained a history—and quickly diagnosed polymyalgia rheumatica (PMR).
The link between pericardial effusion and polymyalgia rheumatica
As my mother’s case shows, PMR should be considered in patients who continue to decline after they’ve been treated for pericardial effusion. The cause of PMR is unknown. Some experts believe an autoimmune process may play a role. Sudden onset of intense inflammation sometimes suggests an infectious cause, but no causative agent has been found.
Signs and symptoms of PMR include severe, symmetrical pain and stiffness in the neck, shoulder, and pelvic girdles, along with painful restriction of motion, malaise, anorexia, weight loss, moderate fever, asthenia, difficulty rising from the bed and toilet, and difficulty performing activities of daily living. Chest pain, malaise, early-morning stiffness, mild anemia (characterized by decreased hematocrit and hemoglobin), and an ESR above 40 mm/hour are classic PRM manifestations.
In the United States, average incidence of PMR is 1 in every 133 persons ages 50 and older. The condition affects more women than men, and incidence increases with age. It affects more whites than persons of other races. Because PMR is more common in those of northern European descent, researchers suspect a genetic component. Other risk factors are age older than 50 and presence of giant-cell arteritis (GCA). (See A link between polymyalgia and giant-cell arteritis.)
A link between polymyalgia rheumatica and giant-cell arteritis
Polymyalgia rheumatica (PMR) may occur independently of giant-cell arteritis (GCA), or may accompany, precede, or follow it. GCA is a chronic systemic inflammatory vasculitis most often affecting the external carotid branches. It can be hard to diagnose due to lack of specific tests.
Signs and symptoms of GCA include headache, shoulder and hip pain, jaw pain after chewing, fever, blurred vision, scalp tenderness, cough, throat pain, tongue pain, weight loss, and stroke. Aortic dissection from mural weakness caused by GCA has been reported. Blindness can occur if GCA isn’t treated promptly.
In rare cases like my mother’s, PMR causes cardiac manifestations. This diagnosis should be considered in patients older than age 50 who have pericarditis or idiopathic pericardial or pleural effusions. Recognizing this uncommon manifestation is crucial, because patients who have it typically respond well to corticosteroids.
Usually, PMR is self-limiting. Without treatment, patients may feel markedly ill and suffer an impaired quality of life. Treatment goals are to control painful myalgia and muscle stiffness and resolve constitutional features of the disease.
Corticosteroids are the treatment of choice and can bring complete or near-complete symptom resolution. For mild PMR, steroids can dramatically improve symptoms within a few months. In any patient with PMR, if improvement doesn’t occur within 1 week after starting steroids, the clinician should suspect GCA.
Studies show prednisone given as a starting dose of 15 to 20 mg once in the morning with food brings remission in most patients. Slow tapering (less than 1 mg per month) is associated with fewer relapses. Patients should have frequent follow-up care and regular ESR monitoring. Because relapse is more common during the first 18 months of therapy and within 1 year of completing corticosteroid therapy, all patients should be followed for 1 year after steroid weaning.
Risks associated with long-term corticosteroid therapy include osteoporosis, weight gain, skin bruising, blood pressure and blood glucose elevations, depression, cardiac failure, increased infection risk, cataracts, glaucoma, and Cushing syndrome. Assess the patient for changes in blood pressure, cognition, mood, and sleep patterns.
Supplemental vitamin D (800 to 1,000 IU) and calcium (1,500 mg in divided doses) are recommended when patients begin long-term medium to high daily doses of corticosteroids. Patients should have baseline bone density tests and biannual bone densitometry. Bisphosphonate therapy should be considered in patients taking corticosteroid doses above 7.5 mg/day for more than 6 months and in those at high risk for fracture. Advise patients on high-dose or long-term corticosteroid therapy to perform weight-bearing exercises.
With prompt diagnosis and proper therapy, patients have an excellent prognosis. Although PMR generally isn’t associated with serious complications, patients who receive corticosteroids are at risk for adverse effects of long-term steroid therapy; all patients are at risk for GCA.
Average duration of PMR is 3 years, although exacerbations may occur, especially if treatment is tapered too rapidly. About 25% of treated patients relapse. With adequate treatment, survival is similar to that of unaffected patients of the same age. However, some research shows an increased mortality from vascular disease among men with PMR beyond the initial 2 years after diagnosis.
Within 3 days of starting corticosteroids, my mother’s symptoms improved dramatically, proving her PMR diagnosis was correct. Follow-up echocardiography showed her pericardial effusion had disappeared. Now, 3 years later, she has been completely weaned from prednisone. Despite some days when she has pain and fatigue, she has recovered. Interestingly, she recently took a week-long course of prednisone for contact dermatitis, during which she had fewer “bad days.” This tells us that although her PMR is gone, it will never be forgotten.
Editor’s note: The author’s mother has given the author permission to share her story.
Kennedy S. Polymyalgia rheumatica and giant cell arteritis: an in-depth look at diagnosis and treatment. J Am Acad Nurse Pract. 2012;24(5):277-85.
van Hecke O. Polymyalgia rheumatica—diagnosis and management. Aust Fam Physician. 2011;40(5):303-6.
Jamie Anderson is a case manager at CVPH Medical Center in Plattsburgh, New York.