Does a 72-year-old patient with smoldering myeloma, presenting with severe pitting edema and shortness of breath, and a 2-D echo showing significant pericardial thickening, increased echo density, and moderate pericardial effusion suggestive of constrictive pericarditis, require a transesophageal echocardiogram (TEE) and a pericardial window?

Management of Suspected Constrictive Pericarditis with Moderate Pericardial Effusion in a 72-Year-Old with Smoldering Myeloma

This patient requires urgent pericardiocentesis with drain placement for the moderate pericardial effusion, followed by cardiac catheterization to confirm constrictive physiology, and likely will need pericardiectomy if chronic constriction is confirmed—TEE is not necessary for diagnosis, and a pericardial window is reserved for specific surgical indications rather than initial management of constriction. 1, 2

Immediate Diagnostic and Therapeutic Steps

Address the Pericardial Effusion First

• Perform urgent echocardiography-guided pericardiocentesis with drain placement for the moderate pericardial effusion, as this patient presents with severe symptoms (4+ pitting edema, shortness of breath) suggesting hemodynamic compromise. 2

• The drain should remain in place for 3-5 days to prevent reaccumulation. 2

• Send pericardial fluid for cytology, chemistry analysis, and microbiology to evaluate for malignant infiltration (plasma cell involvement from smoldering myeloma) versus other etiologies. 2, 3, 4

• TEE is not indicated for diagnosis of constrictive pericarditis or pericardial effusion—transthoracic echocardiography is the Class I recommendation for assessment, and TEE adds no diagnostic value in this clinical scenario. 1, 5

Confirm Constrictive Physiology

• Cardiac catheterization is required when non-invasive methods suggest but do not definitively confirm constriction, looking for the "dip and plateau" sign and equalization of diastolic pressures within 5 mmHg. 5, 1

• After pericardiocentesis, if right atrial pressure fails to fall by 50% or below 10 mmHg, this confirms effusive-constrictive pericarditis, which requires the same treatment approach as chronic constriction. 1

• CT or cardiac MRI should be obtained to evaluate pericardial thickness (>3mm), calcifications, and degree of inflammation via contrast enhancement—these help distinguish transient from chronic constriction. 5, 1

Determining Treatment Path: Transient vs. Chronic Constriction

Features Suggesting Chronic Disease (Requires Surgery)

• Cachexia, atrial fibrillation, hepatic dysfunction, and pericardial calcification indicate chronic disease mandating pericardiectomy. 1

• Your patient's severe 4+ pitting edema and shortness of breath suggest advanced hemodynamic compromise consistent with chronic constriction. 5

• Pericardial thickening of >3mm is absent in 18% of surgically proven constrictive pericarditis cases—normal pericardial thickness should not exclude the diagnosis or deny pericardiectomy when clinical and hemodynamic features indicate constriction. 5

Features Suggesting Transient Constriction (May Respond to Medical Therapy)

• Elevated CRP indicating active inflammation, pericardial contrast enhancement on CT/CMR, recent onset pericarditis with mild effusion, and hemodynamically stable presentation suggest transient constriction. 1

• A 2-3 month trial of anti-inflammatory therapy with loop diuretics and CT/CMR surveillance may be suitable for patients without evidence of chronic disease. 1

• However, your patient's severe symptoms (4+ edema, dyspnea) make a conservative trial less appropriate—symptomatic patients (NYHA class III-IV) require prompt surgical referral. 1

Surgical Decision-Making

When to Proceed Directly to Surgical Drainage

• A pericardial window is indicated for specific scenarios: aortic dissection with hemopericardium, penetrating cardiac trauma, purulent pericarditis, bleeding into the pericardium, subacute free wall rupture post-MI, failed pericardiocentesis, drainage output remaining high at 6-7 days post-pericardiocentesis, or recurrent tamponade. 2

• A pericardial window is NOT the primary treatment for constrictive pericarditis—pericardiectomy (complete removal of both parietal and visceral pericardium via midline sternotomy) is the definitive treatment for chronic permanent constrictive pericarditis. 1, 6

Pericardiectomy Indications

• Symptomatic patients with chronic constrictive pericarditis (NYHA class III-IV) require pericardiectomy, as medical therapy provides only temporary symptom relief and delaying surgery can lead to disease progression. 1

• Early pericardiectomy before development of myocardial atrophy or fibrosis improves survival. 1

• Operative mortality ranges from 6-12%, with poor outcomes predicted by prior mediastinal radiation, worse renal function, higher pulmonary artery systolic pressure, abnormal left ventricular systolic function, lower serum sodium, older age, and Child-Pugh score ≥7. 1

Special Considerations for Multiple Myeloma

• Neoplastic pericardial diseases, including multiple myeloma, carry an intermediate risk (2-5%) of progression to constrictive pericarditis. 7

• Pericardial involvement in multiple myeloma is caused by plasma cell infiltration, amyloidosis, infections, or bleeding abnormalities, usually at a late or terminal stage. 3

• Malignant pericardial effusion from myeloma infiltration is often fatal and unresponsive to chemotherapy—optimal treatment has not been established. 3, 4

• Uremic pericarditis from renal involvement is another consideration in multiple myeloma patients. 7

Critical Pitfalls to Avoid

• Do not delay pericardiocentesis in a symptomatic patient with moderate effusion—this provides both diagnostic fluid and therapeutic relief. 2

• Do not use vasodilators or diuretics before drainage if tamponade physiology is present—these are contraindicated. 2

• Do not rely on pulsus paradoxus alone for diagnosis, as it may be absent in atrial septal defect, severe aortic regurgitation, and regional tamponade. 2

• Do not order TEE for this indication—it adds no diagnostic value and delays appropriate management. 5, 1

• Do not confuse a pericardial window with pericardiectomy—they serve different purposes and are not interchangeable for treating constrictive pericarditis. 1, 2, 6