Constrictive Pericarditis: Symptoms, Types, Causes and Treatment

Constrictive pericarditis (CP) is a complex cardiac disorder where the pericardium—the fibrous sac surrounding the heart—becomes thickened, scarred, or calcified. This restricts the heart’s normal movement and filling, leading to troublesome symptoms and heart failure. Once considered a rare complication of infections like tuberculosis, CP is now increasingly recognized in people with a history of cardiac surgery, radiation, or idiopathic origins. Early recognition and intervention can transform outcomes for patients, making a clear understanding of the presentation, types, causes, and treatments essential for both clinicians and patients.

Symptoms of Constrictive Pericarditis

When the pericardium tightens around the heart, it leads to a set of symptoms that reflect impaired cardiac filling and right-sided heart failure. Recognizing these symptoms early can make a significant difference in patient outcomes.

Symptom	Description	Frequency/Prevalence	Source(s)
Dyspnea	Shortness of breath, especially on exertion	Very common (81-96%)	2 4 15
Peripheral Edema	Swelling of legs/ankles	Common (32-90%)	2 4 15
Raised JVP	Elevated jugular venous pressure	Very common (86-96%)	2 4
Ascites	Abdominal swelling due to fluid	Frequent (68%)	4
Hepatomegaly	Enlarged liver	Frequent (70-92%)	4
Kussmaul’s Sign	JVP rises with inspiration	Present in most cases (up to 96%)	4 15
Fatigue	General tiredness	Common (29-30%)	2 4
Pericardial Knock	Early diastolic heart sound	Occasional (56%)	4
Chest Pain	Discomfort in chest	Occasional (34%)	2

Table 1: Key Symptoms

Overview of Symptoms

The hallmark features of constrictive pericarditis are those of right-sided heart failure. This is because the stiff pericardium limits diastolic filling, leading to congestion in the venous system.

Common Presentations

• Dyspnea on exertion is nearly universal, as the heart’s inability to expand fully results in poor cardiac output during activity. Some patients experience breathlessness even at rest as the disease progresses 2 4.
• Peripheral edema (swelling of the legs and sometimes the abdomen) and ascites (fluid in the abdomen) are classic signs of elevated venous pressure 2 4.
• Raised jugular venous pressure (JVP) is a consistent clinical finding and may be further accentuated by Kussmaul’s sign—a paradoxical rise in JVP with inspiration, which is highly suggestive of CP 4 15.
• Hepatomegaly (enlarged liver) and pleural effusions (fluid around the lungs) may also be present, especially as the disease advances 4.

Less Common, But Notable Symptoms

• Pericardial knock is an early diastolic sound created by the abrupt cessation of ventricular filling, heard in about half of cases 4.
• Fatigue and muscle wasting may occur in advanced disease, reflecting chronic low cardiac output and poor nutrition 4.
• Chest pain is less common but can be present, particularly if there is an underlying inflammatory process 2.

Clinical Signs

Physical exam findings are vital for raising suspicion. In addition to the symptoms above:

• Pulsus paradoxus (a drop in blood pressure during inspiration) can occur in some cases 4.
• Distant heart sounds may be heard due to the thickened, rigid pericardium 4.

Early recognition of this symptom constellation—especially in patients with risk factors—can prompt timely diagnosis and improve outcomes.

Types of Constrictive Pericarditis

Constrictive pericarditis is not a one-size-fits-all diagnosis. It exists in several forms, each with unique features, presentations, and implications for management.

Type	Key Feature(s)	Distinctive Aspect	Source(s)
Chronic (Classic)	Progressive fibrosis/calcification	Most common, slow onset	3 4 15
Subacute/Transient	Inflammation may resolve	May improve with Rx	13 18 19
Effusive-Constrictive	Effusion with constriction	Both tamponade & constriction	8 10 15
Localized	Limited to a section of pericardium	Focal symptoms	15
Occult	Subtle, intermittent symptoms	Diagnosis challenging	15
Constriction with Normal Thickness	Absence of obvious thickening	Seen post-radiation/surgery	12 15

Table 2: Types of Constrictive Pericarditis

Chronic (Classic) Constrictive Pericarditis

• Most common form, resulting from long-standing fibrosis, scarring, and often calcification of the pericardium 3 4 15.
• Presents insidiously over months or years, with slowly progressive symptoms of right heart failure.
• Often associated with previous infections (like tuberculosis in certain regions), cardiac surgery, or idiopathic causes.

Subacute or Transient Constrictive Pericarditis

• Characterized by more active inflammation of the pericardium.
• Some patients, especially those identified early or with evidence of ongoing inflammation on imaging, may improve with anti-inflammatory therapy and avoid surgery 13 18 19.
• Early recognition is crucial, as this form has the potential for reversibility.

Effusive-Constrictive Pericarditis

• A rare hybrid syndrome where patients have both a significant pericardial effusion (fluid) and constriction caused by the visceral pericardium 8 10 15.
• Presents with features of both tamponade (compression by fluid) and constriction.
• Diagnosis requires both imaging and hemodynamic assessment; some cases resolve with medical therapy, but many require surgery.

Localized and Occult Forms

• Localized constriction affects only a specific portion of the pericardium, which can make diagnosis and management more challenging 15.
• Occult constrictive pericarditis is characterized by subtle or intermittent symptoms, sometimes only evident under stress or with detailed hemodynamic evaluation 15.

Constrictive Pericarditis with Normal Pericardial Thickness

• Not all cases show obvious thickening or calcification on imaging.
• This variant is especially associated with prior radiation or cardiac surgery, complicating diagnosis 12 15.

Causes of Constrictive Pericarditis

Understanding the underlying causes of constrictive pericarditis is crucial, as they can influence prognosis and guide management. The spectrum of causes has shifted over time, especially in developed countries.

Cause	Prevalence / Risk Group	Notes	Source(s)
Idiopathic/Viral	Most common (42-61%)	Exact cause unknown	1 6 9 16 18
Post-Cardiac Surgery	11-29% (rising in developed world)	Often noneffusive	2 6 9 15 16
Post-Radiation	2-31% (variable by region)	Long latency	6 9 12 16 17
Tuberculosis	Leading cause in developing regions	30-90% in some areas	1 3 7 14 15
Bacterial (Purulent)	Rare, but high risk if present	Poor prognosis	7 14
Connective Tissue Disease	2-7%	SLE, RA, others	4 6 7
Neoplastic/Metastatic	3-5%	Malignancy-related	6 7
Uremia	2-4%	Chronic kidney disease	6 9
Other (Sarcoid, Trauma)	<1%	Rare	6

Table 3: Causes of Constrictive Pericarditis

Idiopathic and Viral

• Idiopathic (unknown cause) is the leading etiology in most modern series, especially in developed countries 1 6 9 16 18.
• Many of these cases are likely due to prior unnoticed viral infections of the pericardium.

Post-Cardiac Surgery

• The incidence of post-surgical CP is rising, now accounting for up to a quarter of cases 2 6 9 15 16.
• It may develop months to years after procedures, sometimes without significant pericardial effusion 2 6.

Post-Radiation

• Radiation therapy to the chest (e.g., for lymphoma or breast cancer) is a well-documented risk factor 6 9 12 16 17.
• CP may appear many years (even a decade or more) after treatment 6.

Tuberculosis

• Still the primary cause in many developing countries and in immunocompromised or immigrant populations 1 3 14 15.
• Up to 90% of patients undergoing pericardiectomy for CP in some African centers have tuberculosis as the underlying cause 14.

Other Causes

• Purulent (bacterial) pericarditis carries a high risk for developing CP if not treated promptly 7 14.
• Connective tissue diseases such as lupus (SLE) and rheumatoid arthritis can result in chronic pericardial inflammation and scarring 4 6 7.
• Neoplastic involvement (cancer spread to the pericardium) and uremia (chronic kidney disease) are less common but recognized causes 6 7 9.

Changing Trends

• The etiological spectrum has shifted from predominantly infectious (especially TB) to more iatrogenic (post-surgical, post-radiation) and idiopathic in developed countries 12 15.
• However, in regions with high rates of tuberculosis or limited healthcare resources, TB remains the top cause 14 15.

Treatment of Constrictive Pericarditis

Timely and effective management can transform the prognosis for patients with constrictive pericarditis. Treatment strategies depend on the underlying cause, disease stage, and patient comorbidities.

Treatment	Indication	Key Points	Source(s)
Pericardiectomy	Chronic/symptomatic cases	Only definitive cure	1 2 3 16 17 18 19
Medical (Diuretics)	Symptom relief in stable pts	Not curative; bridge	1 15 19
Anti-inflammatory	Subacute/inflammatory cases	May reverse early disease	13 18 19
Steroids	Early post-surgical/inflammatory	Temporary benefit	2 19
Pericardiocentesis	Effusive-constrictive/tamponade	Diagnostic & therapeutic	8 10

Table 4: Treatment Approaches

Pericardiectomy

• Surgical removal of the pericardium is the only curative therapy for chronic, symptomatic constrictive pericarditis 1 2 3 16 17 18 19.
• Outcomes are best when surgery is performed before advanced heart failure develops; early intervention improves survival and functional status 16.
• Operative mortality ranges from 5% to 14%, influenced by etiology (worst in post-radiation cases) and preoperative condition (higher NYHA class, comorbidities) 2 6 9 14 16 17.
• In experienced centers, most survivors experience dramatic improvement in symptoms and quality of life 16 17.

Medical Management

• Diuretics can help control symptoms of fluid overload but do not address the underlying constriction 1 15 19.
• Anti-inflammatory therapy (NSAIDs, colchicine, steroids) is particularly valuable in subacute or transient forms, where ongoing inflammation is present 13 18 19.
  • Some patients with early disease may be spared surgery if the pericardium is still pliable and inflammation can be reversed.
• Steroids may provide temporary benefit, especially after cardiac surgery, but are not a long-term solution for established constriction 2 19.

Treatment of Effusive-Constrictive Pericarditis

• Pericardiocentesis is performed to relieve tamponade in effusive-constrictive cases 8 10.
• Persistent constriction after fluid removal often requires surgery, but some idiopathic cases may resolve spontaneously 8.

Prognosis and Outcomes

• Prognosis depends on underlying cause and disease severity at presentation.
  • Idiopathic cases have the best long-term survival (5-year survival >80%) 9 17.
  • Post-radiation and post-surgical cases have higher risks, especially if surgery is delayed or comorbidities are present 9 16 17.
• Early diagnosis and referral to specialized centers improve outcomes 16 17.

Conclusion

Constrictive pericarditis is a challenging but potentially curable cause of heart failure. Prompt recognition, accurate diagnosis, and timely intervention are essential for optimal patient outcomes.

Key points covered in this article:

• Symptoms: Most patients present with signs of right-sided heart failure such as dyspnea, edema, raised JVP, ascites, and hepatomegaly. Early symptoms can be subtle, so clinical suspicion is crucial.
• Types: CP includes classic chronic, subacute/transient, effusive-constrictive, localized, and forms with normal pericardial thickness—each with unique clinical and diagnostic features.
• Causes: The most frequent causes are idiopathic/viral, post-surgical, post-radiation, and tuberculosis (especially in developing countries), with other causes including bacterial, connective tissue disease, and neoplastic involvement.
• Treatment: While diuretics and anti-inflammatory agents can manage symptoms or, rarely, reverse early disease, pericardiectomy remains the only definitive treatment for established, symptomatic CP.

Early identification and referral for specialist evaluation are crucial, as surgery is more successful before the onset of severe heart failure or comorbidities. With modern diagnostic tools and surgical expertise, many patients with constrictive pericarditis can look forward to significant improvement in their quality of life.