Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging

INTERESTING CASE REPORT
Year
: 2019  |  Volume : 3  |  Issue : 1  |  Page : 23--26

Right-sided constrictive pericarditis: A rare variant of an uncommon disease


Mehul Popatlal Oswal, Chandrashekhar Kashinath Ponde 
 Department of Cardiology, P. D. Hinduja National Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Correspondence Address:
Mehul Popatlal Oswal
Chandan Farm, Bhigwan Road, Baramati, Pune - 413 102, Maharashtra
India

Abstract

Constrictive pericarditis (CP) is characterized by thick pericardial fibrosis and calcification that causes progressively impaired diastolic filling of the heart with associated symptoms of heart failure. Rarely, it can also present as localized CP and depending on the CP location, clinical presentation can be variable, including compression and obstruction of right ventricular inflow tract, coronary obstruction, or pulmonary stenosis. We report a rare case of isolated constriction of the right side of the heart due to loculated tuberculous pericardial effusion.



How to cite this article:
Oswal MP, Ponde CK. Right-sided constrictive pericarditis: A rare variant of an uncommon disease.J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:23-26


How to cite this URL:
Oswal MP, Ponde CK. Right-sided constrictive pericarditis: A rare variant of an uncommon disease. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2019 Oct 23 ];3:23-26
Available from: http://www.jiaecho.org/text.asp?2019/3/1/23/254257


Full Text

 Introduction



Constrictive pericarditis (CP) is an end stage of an inflammatory process involving the pericardium resulting in fibrosis, calcification, and adhesions of the parietal and visceral pericardium. Causes can be idiopathic, infectious, inflammatory, neoplastic, postsurgical, or radiation injury. The most common cause in developing countries is likely to be related to tuberculosis. CP is characterized by uniform restriction of ventricular filling and manifests with symptoms and signs of right heart failure. Rarely, it can present with localized constriction resulting in right ventricular (RV) or left ventricular (LV) inflow obstruction. We report a rare case of isolated right-sided CP due to loculated tubercular pericardial effusion compressing RV free wall.

 Clinical Presentation



A 60-year-old female with no comorbidities presented with 4-month history of dyspnea on exertion, abdominal distension, bilateral lower limb swelling, and easy fatiguability. She was diagnosed to have tuberculous pericardial effusion in outside hospital 3 months back and was started on Category I antitubercular treatment and steroids. One month later, on follow-up echocardiogram, she developed loculated pericardial effusion along RV free wall of which approximately 60 cc was tapped. She remained asymptomatic for 2 months only to present again with the same complaints this time to our hospital.

Physical examination revealed tachycardia, normal blood pressure, raised jugular venous pressure, and bilateral pitting edema. There were no other gross signs of heart failure and pericardial knock or rub could not be heard. Laboratory investigations were unremarkable. Electrocardiograph showed low-voltage pattern with nonspecific ST-T changes. Chest X-ray showed cardiomegaly [Figure 1]. M-mode -two-dimensional echocardiography [Figure 2] showed septal notching of interventricular septum (IVS). Apical four-chamber view [Figure 3] revealed septal bounce and an echolucent space along RV free wall. Flow propagation velocity was raised [Figure 4]. Doppler studies showed mitral inflow respiratory variation of 16% and tricuspid inflow respiratory variation of 45% [Figure 5] and [Figure 6]. Tissue Doppler imaging showed a medial mitral annular velocity of 6 cm/s and lateral mitral annular velocity of 12 cm/s [Figure 7] and [Figure 8]. Hepatic vein Doppler studies revealed expiratory diastolic reversal [Figure 9]. Inferior vena cava (IVC) was dilated and plethoric [Figure 10]. All the above findings were consistent with constrictive physiology features localized to the right side, whereas the left heart was free from the disease. A high-resolution computed tomography chest revealed loculated pericardial effusion measuring 7 cm × 5.5 cm × 2 cm with mild-enhancing pericardial thickening (5 mm) along the right heart border which is highly specific for pericardial constriction [Figure 11]. The patient was advised to undergo cardiac catheterization followed by surgery but was lost to follow-up later.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}{Figure 7}{Figure 8}{Figure 9}{Figure 10}{Figure 11}

 Discussion



CP occurs when a fibrotic, thickened, and adherent pericardium restricts diastolic filling of the heart. It usually begins with an initial episode of acute pericarditis, characterized by fibrin deposition, often with a pericardial effusion. This then slowly progresses to a subacute stage of organization and resorption of the effusion, followed by a chronic stage consisting of fibrous scarring and thickening of the pericardium with obliteration of the pericardial space.[1] CP usually is a symmetrical scarring process that produces uniform restriction of the filling of all heart chambers. Rare cases of strictly localized pericardial thickening have been reported, including constricting bands in the atrioventricular groove or in the RV outflow and inflow tract and vena cava.[2],[3]

We believe this is one of the rare case report of CP localized solely to the right ventricle that impaired the diastolic filling of the same ventricle due to compressive effect of loculated tuberculous pericardial effusion.[4],[5]

Fibrotic thickened pericardium prevents transmission of intrathoracic respiratory pressure changes to cardiac chambers. These changes in pressure continue to be transmitted to pulmonary circulation. Inspiratory drop in intrathoracic pressure is transmitted to pulmonary veins but not to the left side of the heart. Pulmonary vein to left atrial pressure gradient is reduced which reduces transmitral inflow. Inspiratory decrease in LV filling allows increase in RV filling and shift of IVS to the left. Opposite occurs in expiration. These changes result in exaggerated respiratory variation in mitral and tricuspid inflow and in LV and RV systolic and diastolic pressure and volume known as ventricular interdependence.

Diagnosis of this patient was based on echocardiographic parameters of septal notching on M mode, increased inspiratory tricuspid inflow velocity, expiratory hepatic vein flow diastolic reversal, and IVC plethora which suggests features of the right-sided constriction. Absence of annulus paradoxus and normal respiratory variation in mitral inflow velocity suggests that the left heart is free from the disease.

Tuberculosis is responsible for approximately 70% of cases of large pericardial effusion and most cases of CP in developing countries. Tuberculous pericarditis is a dangerous disease with a mortality of 17%–40%; constriction occurs in a similar proportion of cases after tuberculous pericardial effusion. Pericardial constriction may be the first presentation of tuberculous pericarditis,[6],[7] often with no history to indicate a prior effusive stage. Clinical features vary according to the duration and severity of constriction and persistence of pericardial inflammation.[8] Constriction may also be regional, resulting in atypical features.[9] Global constriction manifests first through the right-sided dysfunction resulting in systemic venous congestion that can be complicated by cardiac cirrhosis and protein-losing enteropathy; left-sided symptoms are those of pulmonary congestion and subsequently of LV output failure and failure of organ perfusion.

Early diagnosis and institution of appropriate therapy are critical to prevent mortality. A probable or presumed diagnosis is based on proof of tuberculosis elsewhere in a patient with otherwise unexplained pericarditis, a lymphocytic pericardial exudate with elevated biomarkers of tuberculosis infection, and/or appropriate response to a trial of antitubercular treatment. Treatment consists of four drug treatment HRZE, i.e., isoniazid (H), rifampicin (R), pyrazinamide (Z), and ethambutol (E) for 2 months followed by two drugs HR for 4 months. Role of adjunctive corticosteroids in reducing mortality of pericardial constriction is uncertain. Surgical resection of the pericardium is indicated for those with calcific CP or with persistent signs of constriction after a 6–8 weeks trial of antitubercular treatment in patients with noncalcific CP.

 Conclusion



CP is an uncommon, but potentially curable disease and can have atypical presentation. Isolated constriction should be considered while evaluating a case of undiagnosed right heart failure in developing countries where tuberculosis is still the most common cause of constrictive pericarditis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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