Tuberculosis Effusive-Constrictive Pericarditis

Abstract

Background

Effusive-constrictive pericarditis is a rare pericardial syndrome characterized by the coexistence of constrictive physiology and pericardial effusion. Tuberculosis remains a leading cause of pericardial disease in endemic regions, posing significant diagnostic challenges.

Case Summary

A 67-year-old man from Brazil presented with progressive dyspnea, low-grade fever, and signs of right-sided heart failure. Imaging demonstrated constrictive physiology and progressive pericardial calcification. He developed cardiac tamponade requiring surgical pericardiectomy. Analysis of the pericardial fluid revealed a purulent effusion with elevated adenosine deaminase levels (200 U/L), consistent with tuberculous etiology. Despite medical and surgical management, the patient deteriorated owing to refractory shock.

Discussion

This case illustrates the complex interplay between effusion and pericardial constriction and underscores the diagnostic utility of imaging and biochemical markers such as adenosine deaminase in tuberculosis-endemic settings.

Take-Home Messages

Tuberculosis remains a leading cause of effusive-constrictive pericarditis in endemic regions. Early recognition is critical to guide timely and appropriate therapy.

Visual Summary

Visual Summary.

Tuberculous Effusive-Constrictive Pericarditis: A Diagnostic and Therapeutic Challenge

History of Presentation

A 67-year-old Brazilian man presented with progressive dyspnea over several weeks, accompanied by low-grade nocturnal fevers, unintentional weight loss, and generalized fatigue. On physical examination upon arrival, he was afebrile and in sinus tachycardia at 116 beats/min, with a normal blood pressure. Jugular venous pressure was elevated, with paradoxical inspiratory distension consistent with Kussmaul sign. Cardiopulmonary auscultation revealed decreased breath sounds at the lung bases. Abdominal examination demonstrated moderate ascites, and bilateral lower extremity edema was noted.

Take-Home Messages

• •Tuberculosis remains a leading cause of effusive-constrictive pericarditis in endemic regions.
• •Early recognition of effusive-constrictive pericarditis is critical to guide timely and appropriate therapy.
• •Echocardiography is essential in identifying constrictive physiology.
• •An adenosine deaminase level of ≥40 U/L in pericardial fluid strongly supports tuberculous pericarditis.

Past Medical History

The patient had a history of paraplegia after a spinal cord injury sustained in a motor vehicle accident in 2016. His comorbidities included a chronic sacral pressure ulcer and poorly controlled type 2 diabetes mellitus. He had prior hospitalizations for infected sacral ulcers in 2020 and 2023, during which chest computed tomography was performed as part of the evaluation for infectious foci.

Differential Diagnosis

The patient's presentation was initially suggestive of right-sided heart failure. The presence of low-grade fever raised the possibility of an infectious or inflammatory etiology. Infective endocarditis with secondary heart failure was considered as a potential diagnosis. The presence of Kussmaul sign however, pointed toward pericardial pathology, and both constrictive pericarditis and effusive-constrictive pericarditis (ECP) were included in the differential diagnosis. Pulmonary thromboembolism with right ventricular dysfunction and pulmonary hypertension were also considered. The insidious nature of the symptoms, combined with fever and unintentional weight loss in a tuberculosis-endemic region, also raised suspicion for tuberculous pericarditis.

Investigations

Initial blood tests revealed elevated C-reactive protein (62 mg/dL). Transthoracic echocardiography revealed findings consistent with constrictive physiology, including dilated inferior vena cava with reduced inspiratory collapse, septal bounce, and respiratory variation in mitral and tricuspid inflow velocities (Figure 1). Chest computed tomography scans obtained during prior hospitalizations for unrelated reasons demonstrated progressive pericardial thickening and calcification. The current scan demonstrated marked pericardial calcification and thickening, with new loculated gas collection adjacent to the pericardium and bilateral pleural effusions (Figure 2).

Figure 1.

Transthoracic Echocardiography Demonstrating Constrictive Physiology

Pulsed-wave Doppler imaging of (Top) mitral and (Bottom) tricuspid inflow velocities demonstrating constrictive physiology. There is marked respirophasic variation, with a 36% decrease in mitral inflow and a 43% increase in tricuspid inflow during inspiration (arrows).

Figure 2.

Serial Chest Computed Tomography Demonstrate Progressive Pericardial Thickening and Calcification

(A) May 2020: mild pericardial thickening with early calcification. (B) March 2023: increased pericardial calcification with further thickening. (C) June 2024: marked pericardial calcification and thickening, with new loculated gas collection adjacent to the pericardium and bilateral pleural effusions.

Management (Medical/Interventions)

The patient developed hemodynamic instability owing to tamponade and underwent urgent pericardiectomy. Surgical drainage yielded 150 mL of purulent fluid. Cultures and stains were negative, but high adenosine deaminase (ADA) levels were detected (200 U/L). Tuberculous ECP was diagnosed, consistent with residence in a high-prevalence tuberculosis region. The anatomopathological analysis confirmed constrictive pericarditis, characterized by chronic suppurative inflammation with polymorphonuclear cells, debris, and dystrophic calcification (Figure 3).

Figure 3.

Histopathology of the Pericardium (Hematoxylin and Eosin Stain, High Magnification) Demonstrating Chronic Inflammatory Infiltrate and Fibrotic Thickening

Pericardial tissue demonstrating (1) dystrophic calcification, (2) chronic suppurative inflammation with polymorphonuclear infiltrates and cellular debris, and (3) focal recent hemorrhage possibly related to recent instrumentation.

Outcome and Follow-Up

Antituberculosis therapy was initiated, and the patient showed initial clinical improvement. However, despite aggressive medical management, including antibiotics and hemodynamic support, the patient's condition deteriorated, culminating in refractory shock.

Discussion

ECP is a rare and often under-recognized pericardial syndrome, characterized by the coexistence of pericardial effusion and constrictive physiology.¹ The hallmark of ECP is the persistence of elevated right atrial pressure after pericardial fluid drainage, reflecting ongoing constraint from the visceral pericardium. Clinical manifestations include jugular venous distention, abdominal swelling, lower extremity edema, pericardial friction rub, pulsus paradoxus, and Kussmaul sign.² Echocardiographic findings such as septal bounce, dilated inferior vena cava, and transmitral and transtricuspid Doppler recordings showing respiratory variation in ventricular inflows are key diagnostic clues. While invasive hemodynamic assessment has historically been the standard for diagnosing constrictive physiology, noninvasive multimodality imaging, particularly echocardiography and cardiac magnetic resonance, has become central to its detection and is now recommended in contemporary guidelines for the diagnosis and management of pericardial disease.3, 4, 5, 6, 7

Tuberculosis is a leading cause of pericardial disease worldwide, particularly in low- and middle-income countries. Tuberculous pericarditis may present as an effusive, constrictive, or mixed effusive-constrictive form. The inflammatory response induced by Mycobacterium tuberculosis can cause progressive thickening and fibrosis of the pericardium, particularly the visceral layer, leading to constriction. In this context, elevated ADA levels in the pericardial fluid serve as a sensitive and specific diagnostic marker, particularly when microbiological tests are negative.⁸ A pericardial ADA level ≥40 U/L has been shown to have a sensitivity of 88% and specificity of 83% in tuberculosis-endemic areas.² Timely recognition of tuberculous etiology is critical, given its high associated morbidity and mortality.

In the present case, the patient's initial symptoms were nonspecific, but progressive signs of constriction and imaging findings consistent with pericardial calcification raised concern for a chronic inflammatory process. The onset of cardiac tamponade prompted surgical intervention, which revealed a purulent effusion. The markedly elevated ADA level (200 U/L) in a region endemic to tuberculosis, along with histopathological evidence of chronic suppurative pericarditis, confirmed the diagnosis of tuberculous ECP. Diagnosing cardiac tuberculosis can be challenging given the low sensitivity of detecting M tuberculosis in pericardial fluid.⁹ In this context, a diagnostic algorithm can be a valuable clinical tool (Figure 4). This case underscores the importance of maintaining a high index of suspicion for tuberculosis and highlights the need for early diagnostic work-up in patients presenting with overlapping features of effusive and constrictive pericardial disease.

Figure 4.

Diagnostic and Management Algorithm for Cardiovascular Tuberculosis

ADA = adenosine deaminase; CT = Computed tomography; CT = computed tomography; CVP = central venous pressure; CXR = chest X-ray; ECG = electrocardiogram; INF = interferon; MRI = magnetic resonance imaging; PCR = polymerase chain reaction; PET = positron emission tomography.

The management of ECP includes treatment of the underlying etiology, such as appropriate antimicrobial therapy in cases of infection. In patients with clinical or imaging evidence of ongoing pericardial inflammation, anti-inflammatory agents may be considered. Pericardiectomy, which entails removal of the thickened and inflamed pericardium, can be technically challenging and is generally reserved for patients with persistent constrictive physiology, particularly in the setting of chronic disease characterized by pericardial calcification or advanced systemic congestion.^1,3,7

Conclusions

ECP is a diagnostic challenge, particularly in tuberculosis-endemic areas. High clinical suspicion, early imaging, and use of biomarkers such as ADA are critical for prompt diagnosis and management.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.