Tuberculous Constrictive Pericarditis: A Classical Case and Review

Chronic constrictive pericarditis (CCP) is a pericardial disease caused by restricted expansion of heart chambers by pericardial restraint. Concomitant pleural effusion may cause diagnostic difficulty regarding the causative role of pericardial constriction for dyspnoea and needs thorough diagnostic evaluation. We present a case of tubercular CCP requiring detailed imaging and hemodynamic assessment.

A 60-year-old male patient with history of treated tubercular pleuro-pericardial effusion 1.5 years prior presented with right heart failure symptoms for 5 months. Clinical evaluation revealed bipedal oedema, pulsus paradoxus, elevated jugular venous pressure (JVP) with sharp x and y descents, no cardiomegaly or murmur, and a distinctive pericardial knock. There was moderate right-sided pleural effusion and soft hepatomegaly with no ascites. The electrocardiogram (Fig. 1A) showed low-voltage complexes, and chest X-ray (Fig. 1B) confirmed moderate right-sided pleural effusion. Transthoracic echocardiogram showed septal bounce, ventricular interdependence with respirophasic variation (Fig. 1C) and pericardial thickening (Fig. 1D). There was significant change in mitral and tricuspid inflow velocity during respiration (Fig. 1E) and hepatic venous expiratory flow reversal (Fig. 1F). Annulus reversus was noted (Fig. 1G and H). Pleural fluid was transudative with negative work-up for tuberculosis. Catheterization data suggested constrictive physiology with an “M” pattern in right atrium pressure tracings (Fig. 2A), positive Kussmaul’s sign, ventricular discordance (Fig. 2B), and left ventricular rapid filling wave (LVRFW) >7 mm Hg (Fig. 2C and D). Computed tomography showed pericardial thickness of 5.2 mm (Fig. 2E). Coronary angiogram showed an “entrapped RCA sign” (Fig. 2F and G). Complete hemodynamic data are shown in the Supplementary Fig. 1 (in the online-only Data Supplement). The diagnosis of CCP secondary to tubercular pericarditis with right heart failure was made and the patient was scheduled for surgery.

Fig. 1
Electrocardiogram, chest x-ray, and echocardiogram findings of the index patient. A: 12-lead electrocardiogram showing low-voltage complexes. B: Chest X-ray showing right-sided pleural effusion. C: M-mode echocardiography demonstrates respirophasic septal shift and septal shudder. D: Echocardiography showing thickened pericardium (red arrow). E: Mitral inflow pulsed-wave Doppler shows >66% expiratory increase in E velocity. F: Hepatic vein pulsed-wave Doppler showing expiratory diastolic flow reversal (red arrow). G: Lateral mitral annulus e’ is decreased relative to the medial annulus (annulus reversus). H: Medial mitral annulus tissue Doppler demonstrates elevated early diastolic velocity (e’), despite increased filling pressure (annulus paradoxus).

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Fig. 2
Cardiac catheterization and computed tomogram finding of the index patient. A: Right atrial (RA) pressure trace showing elevated pressure with classical “M” or “W” pattern and inspiratory decrease <5 mm Hg. B: Pressure tracing showing marked decrease in PCWP-LV diastolic pressure gradient during inspiration (single arrow) compared to expiration (double arrow). C: Left ventricular pressure trace showing “dip and plateau” pattern or “square root sign” and LV rapid filling wave >7 mm Hg. D: Ventricular discordance with a systolic area index of 1.9. E: Cardiac CT showing thickened pericardium. F and G: Right coronary angiogram depicting the “entrapped RCA sign”. PCWP-LV, pulmonary capillary wedge pressure-left ventricle; CT, computed tomography; RCA, right coronary artery.

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Tuberculosis is the most common aetiology of CCP in India, followed by post-cardiotomy for cardiac surgeries [1]. Pericardial thickness >3 mm on echocardiogram is both sensitive and specific for thickened pericardium. A septal “bounce,” “shudder,” or “diastolic checking” translating into septal notch may be present (Fig. 1C) [2]. During inspiration, venous return and right ventricle (RV) preload increase. Pulmonary capillary wedge pressure (PCWP) decreases but left ventricular end diastolic (LVED) pressure does not (because of loss of transmission of intrathoracic pressure due to the stiff pericardium). In this situation, PCWP-LVED pressure difference drops, leading to reduced left ventricle (LV) filling (Fig. 2B). This results in RV volume increase and LV volume decrease during inspiration, leading to a shift of the inter-ventricular septum towards the LV. The converse occurs during expiration, and consequent pressure changes lead to expiratory hepatic venous diastolic flow reversal [3]. In CCP, lateral e’ decreases due to fibrotic tethering, leading to annulus reversus (Fig. 1G and H) [3]. However, E/e’ does not correlate well with LV filling pressure, as in myocardial disease, a condition known as annulus paradoxus [3]. Global longitudinal strain was not measured, and the “Hot-septum” sign indicating exaggerated medial segment motion could not be analysed [4].

Right atrial (RA) pressure is elevated (Fig. 2A). Prominent “y” descent in JVP or RA pressure is known as Friedrich’s sign and is caused by rapid emptying of the RA during early diastole [5]. Prominent “y” and “x” descents give rise to a classical “M” or “W” pattern (Fig. 2A). Elevation and equalisation of diastolic pressure (within 5 mm Hg) (Supplementary Fig. 1 in the online-only Data Supplement) in all cardiac chambers is the hallmark of CCP caused by equal pressure exerted by a fixed pericardium during diastole. Early diastolic filling is not affected but comes to a sudden halt by stiff pericardium, resulting in a “dip and plateau” or “square-root sign” in ventricular tracing (Fig. 2C). RV end diastolic pressure is higher, usually >1/3 of RV systolic pressure. Rapid filling in early diastole is reflected by a deep rapid filling wave. LVRFW >7 mm Hg has 93% sensitivity and 57% specificity for CCP (Fig. 2C). Absence of fall or rise in RA pressure during inspiration is called “Kussmaul’s sign” [6]. The systolic area index is defined as (RV area/LV area in inspiration)/(RV area/LV area in expiration) and increases significantly (Fig. 2D) for reasons explained earlier. The mid-segment of the right coronary artery (RCA) showed significantly reduced motion along with reduced coronary foreshortening, the “entrapped RCA sign” (Fig. 2F and G) [7].

The online-only Data Supplement is available with this article at https://doi.org/10.22468/cvia.2023.00031.

Supplementary Fig. 1

Cardiac catheterization data of the index patient.