What is the initial management for a patient with a thickened pericardium and right ventricular (RV) dysfunction?

Management of Thickened Pericardium with Right Ventricular Dysfunction

Immediate Diagnostic Confirmation

The priority is to confirm constrictive pericarditis through comprehensive imaging and hemodynamic assessment, as thickened pericardium with RV dysfunction strongly suggests this diagnosis and requires urgent evaluation for pericardiectomy. 1

Essential Diagnostic Workup

• Transthoracic echocardiography is the first-line imaging modality to assess RV dysfunction severity, chamber sizes, tricuspid valve function, and identify constrictive physiology including respiratory variation >25% across AV valves and ventricular interdependence 1, 2

• Cardiac catheterization must be performed to document the pathognomonic "dip-plateau" or "square root" sign in RV/LV pressure curves and equalization of end-diastolic pressures within 5 mmHg, which confirms constriction 1

• CT or cardiac MRI should be obtained to measure pericardial thickness (though 18% of surgically proven cases have normal thickness) and assess for calcification, which helps predict surgical complexity 1

• Assess RV systolic function using tricuspid annular plane systolic excursion (TAPSE <17 mm abnormal), RV fractional area change (FAC <40% abnormal), and tissue Doppler of the tricuspid annulus, as reduced RV systolic function indicates myocardial involvement and predicts worse outcomes 2, 3, 4

Critical Prognostic Assessment

• Evaluate for myocardial involvement as diseases causing constrictive pericarditis infiltrate the RV myocardium more than the LV, causing RV systolic dysfunction that persists after pericardiectomy and increases operative mortality 3, 4

• Identify high-risk features including moderate-to-severe tricuspid regurgitation (OR 28.8 for mortality), low cardiac index (OR 25.3 for mortality), pleural effusion (OR 16.2 for mortality), elevated GGT (OR 28.3 for ECMO need), and decreased serum protein (OR 24.7 for ECMO need) 5

• Coronary angiography is mandatory in all patients over 35 years and those with history of mediastinal irradiation regardless of age before proceeding to surgery 1

Initial Medical Management

Hemodynamic Optimization

• Diuretics are the cornerstone of initial therapy to reduce venous congestion, jugular venous distension, abdominal distension, and peripheral edema while awaiting definitive surgical intervention 1, 2

• Maintain adequate preload as aggressive diuresis can precipitate hemodynamic collapse; the goal is symptom relief without inducing hypotension 1

• Avoid aggressive afterload reduction as these patients are preload-dependent and vasodilators can worsen hypotension 1

Preparing for Surgery

• Optimize volume status carefully as both hypovolemia and volume overload are problematic—hypovolemia worsens low cardiac output while volume overload exacerbates RV dysfunction 6, 5

• Correct metabolic abnormalities including hypoalbuminemia and hepatic congestion, which predict need for mechanical support 5

• Plan for potential ECMO support in high-risk patients with severe RV dysfunction, significant tricuspid regurgitation, low cardiac index, or elevated liver enzymes, as 12% of patients require ECMO for post-pericardiectomy RV failure 5

Definitive Treatment: Pericardiectomy

Pericardiectomy should not be delayed once constrictive physiology is confirmed, as progression of myocardial fibrosis increases the risk of irreversible RV dysfunction and operative mortality. 3, 5

Surgical Considerations

• Complete pericardiectomy from phrenic nerve to phrenic nerve is the standard approach, though patients with pre-existing RV systolic dysfunction have higher perioperative risk 1, 5

• Elective pre- or intraoperative ECMO should be strongly considered in patients with severe RV dysfunction (TAPSE <17 mm), moderate-to-severe tricuspid regurgitation, cardiac index <2.0 L/min/m², or elevated GGT/low protein, as these predict post-operative RV failure 5

• Concomitant tricuspid valve repair may be necessary if significant structural tricuspid regurgitation is present, though functional TR may improve after pericardial decompression 5

Post-Pericardiectomy Management

• Anticipate transient RV failure from abrupt increase in venous return and myocardial atrophy from prolonged constriction; treat with inotropes (dobutamine or milrinone) and careful diuresis 6

• Immediate ECMO support should not be delayed if significant RV failure develops post-operatively, as early mechanical support improves survival 5

• Serial echocardiography is essential to monitor RV recovery, which typically occurs over weeks to months in patients without myocardial involvement 6

Important Caveats

• Normal pericardial thickness does not exclude constriction—18% of surgically proven cases have normal thickness, so pericardiectomy should not be denied based solely on imaging if hemodynamics confirm constriction 1

• Persistent RV dysfunction after pericardiectomy indicates irreversible myocardial fibrosis and portends poor long-term outcomes; these patients require ongoing heart failure management 3, 4

• Atrial fibrillation complicates diagnosis as respiratory variation in Doppler flows may be absent; hepatic vein flow reversal during expiration helps confirm constriction in this setting 1

• Radiation-induced and post-cardiotomy constrictive pericarditis carry significantly higher operative mortality (p=0.009 and p=0.049 respectively) and require heightened vigilance for post-operative complications 5