Aim: To report a case of “end-stage” constrictive pericarditis with clinical manifestations such as ascites mimicking as cirrhosis of liver. Introduction: In “End-stage” constrictive pericarditis, the etiology remains unknown in majority of cases and inflammation plays a central role in its development. It has been readily confused with cirrhosis of liver in which there may be ascites, but venous pressure is normal, the neck veins are not engorged and cardiac enlargement is frequent in other causes of heart failure. Case Report: A 67 years old male presented with sudden onset of tachycardia. Clinical examination revealed right-sided heart failure, “Egg-shell” calcification in Chest X-ray and echocardiographic features of pericardial constriction such as septal bounce and dynamic respiratory changes in mitral inflow velocity. The patient was advised medical measures. Conclusion: When clinical signs of right heart failure become unresponsive to increased doses of diuretics, constrictive pericarditis is more likely the underlying disease since severe, right-sided failure develops in very advanced, the “end-stage” of the disease.
The normal pericardium is a fibroelastic sac enveloping the heart and consists of two layers. The visceral pericardium (serous pericardium) is a single layer of mesothelial cells contiguous with epicardium and a tough, fibrous layer as a parietal pericardium. When the pericardium limits the heart’s ability to function normally either due to accumulation of fluid (pericardial effusion) or scarred and inelastic (constriction), the pericardial compression syndromes may occur.
The constrictive pericarditis is typically chronic, but variants including acute, subacute, transient, occult and end-stage may occur. Historically, the eponym “Pick’s disease” was given to constrictive pericarditis with ascites and hepatomegaly [
A 67 years old male was admitted with sudden onset of palpitations in the emergency room. ECG revealed tachycardia with a heart rate of 150 bpm as in
Review of literature
In 1669, Lower described the clinical effects of interference of cardiac diastole by a constricting fibrous pericardium. In 1756, Morgagni contributed to the understanding of pathophysiology of constrictive pericarditis. In 1828, Lancisi described the characteristic syndrome of constrictive pericarditis and in 1842 [
Etiopathogenesis
Constrictive pericarditis is most commonly caused by conditions or events that cause inflammation to develop around the heart. Inflammatory process of the pericardium typically causes pain and fluid accumulation and more chronically results in fibrosis and calcification of pericardium with pericardial constriction, the process that inhibit diastolic filling of the heart. The most common antecedents are idiopathic and tuberculosis. The tuberculosis accounted for 49% of cases of constrictive pericarditis in a series reported in 1962 [
Constrictive pericarditis can occur after many pericardial disease processes. All causes of pericarditis can lead to subsequent constriction [
The causes of constrictive pericarditis are shown in
Hemodynamic changes
The normal pericardium can stretch to accommodate the physiological changes in cardiac volume. In constrictive pericarditis, the visceral and parietal pericar-
Most common | Less common |
---|---|
Idiopathic―42% to 49% [ | Other infections |
Mediastinal irradiation (5 - 10 years duration) | Neoplasms―5% - 17% (lung―33%, breast―25%, adenocarcinoma of intestine [ |
Following Cardiac surgery―11% to 37% (post-pericardiectomy―10% to 40%, previous cardiac surgery―0.3%) | Connective tissue disorders (rheumatoid Arthritis, systemic lupus erythematosis, Scleroderma) |
Radiotherapy―9% to 31% | Drugs (procainamide, hydralazine, methysergide) |
Post-infectious―3% to 6% | Trauma |
Tuberculosis Viral infections (coxsackie virus A and B, Adenovirus, echovirus) Pyogenic infections | Hereditary―Mulibrey nanism (Mu―muscle, Li―liver, br―brain, ey―eyes, nanism―dwarf) in Finland and United states [ |
dium are fibrosed and fused together [
In the heart with a normal pericardium, inspiration causes a decrease in intrathoracic pressure, which is reflected in the cardiac chambers as decrease in intracardiac pressures simultaneously and there is no change in the driving pressure from the lungs across the pulmonary veins into the left atrium and across the mitral valve into the left ventricle. There is some increase in the filling of the right ventricle because of enhanced venous return, but filling of the left ventricle is unaffected throughout the cardiac cycle. In patients with constrictive pericarditis, the rigid pericardium does not allow the decrease in intrathoracic pressure to be transmitted to the left -sided chambers and there is a lower driving force from the lungs into the left side of the heart and the left ventricle becomes underfilled with a reciprocal increase in the filling of right ventricle and therefore a septal shift occurs [
Once the ventricular diastolic filling reaches the limitations of the pericardial restraint, the pressure and volume in the cavity rise, filling ceases, and congestion occurs [
In isolated constrictive pericarditis, the myocardium is unaffected and therefore the systolic function and early diastolic filling are normal. In the mixed form (constrictive―restrictive―mainly due to radiation―induced, post cardiac surgery), atrophy of myocardial cells and fibrosis may develop during long-term compression by the pericardium. Both the irritation of heart by the actual process involving the myocardium and the constricting effect of left heart chambers on the right ventricle and right atrium result in atrial arrhythmias such as atrial fibrillation as shown in
High venous pressure |
---|
Diminution of blood pressure |
Paradoxical pulse |
Atrial fibrillation |
Ascites |
Low cardiac output (cardiac index ≤ 1.2 L/m2/minute) |
Pseudocirrhosis |
Pedal edema |
Diagnostic Methods
Radiological
The plain radiograph is frequently abnormal in patients with hemodynamically significant constrictive pericarditis [
Calcification of pericardium on chest X-ray strongly suggests constrictive pericarditis in patients with features of heart failure ( especially right heart failure) and it is more obvious in regions where the normal fat is found, namely in atrioventricular and interventricular grooves. A localized form of constriction in the mid ventricular segments as a result of localized severe calcification resembl-
ing a “napkin” ring shape is termed as “napkin-ring” constrictive pericarditis [
1) The calcium can be thin and linear, and appears as “egg-shell calcification” around the margins of the heart (tortoise shell like) as shown in
2) The calcification also can appear as thick, shaggy, amorphous and historically believed to be specific for tuberculous pericarditis.
Occasionally, extensive calcification involving the interventricular septum [
Thickening of pericardium occurs heterogeneously with some areas thicker than other (thinnest over the left ventricle (0.7 - 1.2 mm). A thickened pericardium (>4 mm) on its own does not indicate constrictive pericarditis [
carditis is almost always associated with pericardial thickening and mostly, the pericardial thickening is >3 mm by the time the patient becomes symptomatic and >6 mm when the patient is clinically in heart failure.
Two-dimensional Echocardiography
The thickened, constricting pericardium affects the posterior left ventricle (pericardial thickness is 8 mm) more than the posterior left atrium, which then expands at a more acute angle respected to the LV wall [
M-mode findings
In constrictive pericarditis, the LV posterior wall rapidly expands posteriorly during early diastole, followed by abrupt cessation of such movement during mid and late diastole, which corresponds to abrupt termination of rapid ventricular filling [
Doppler Echocardiography
Doppler echocardiographic findings include:
1) Prominent, usually >25% increase in initial E velocity during expiration and decrease during inspiration as shown in
Patients with restrictive cardiomyopathy (an infiltrative process that leads to myocardial stiffening) may not show any respiratory variation in mitral inflow velocity as in
Atrial fibrillation may complicate the interpretation of respiratory variation of Doppler velocities, but respiratory variation can still be appreciated regardless of cardiac cycle length.
2) The
Cardiac catheterization
Cardiac catheterization can yield classic findings of constrictive pericarditis, but these findings are also present in restrictive cardiomyopathy which include an increase and equalization of end-diastolic pressures in all four cardiac chambers, a “dip (or drop) and plateau” pattern (square root sign) in the ventricular pressure curves as demonstrated by Hansen, et al. [
proximately equal on both sides in simultaneous recordings, a fluid bolus should theoretically increase LVEDP above RVEDP in restrictive cardiomyopathy [
Management
The treatment of chronic constrictive pericarditis is very much discouraging, but both medical and surgical treatments have been improved greatly.
Medical therapy
The constriction may be transient or reversible. In these conditions, the constriction is due to inflammation (acute inflammatory pericarditis) and non-ste- roidal anti-inflammatory drugs (NSAIDs) are the most frequent treatment. Follow-up studies showing resolution within 2 months to 2 years (usually responding in an average of 8 weeks) and typically effective in idiopathic cases.
In chronic constrictive pericarditis, the efforts are made to keep the systemic congestion under control. Loop diuretics (torsemide if bowel edema is suspected or intravenous furosemide), thiazides and aldosterone antagonists (especially if ascites is present) may be helpful. However, the overzealous use of diuretics is not recommended and may lead to sudden death [
Surgical therapy
The surgical treatment of chronic constrictive pericarditis was first recommended by Delorme in 1898 and some years later, the first pericardiectomy for constrictive pericarditis was performed by Franz Volhard collaborates with Viktor Schmieden in 1923 and in United States by Churchill in 1929. Decreased cardiac output resulting from a chronic constrictive process may require surgical intervention.
Pericardial resection (or pericardial stripping) is a surgical procedure where the entire pericardium is pealed away from the heart, is a delicate time-consuming procedure, somewhat hazardous and it is different from Brauer’s operation (freeing the heart from thoracic cage). It was the custom to approach the heart anteriorly and to free the right heart chambers and if the whole heart is affected or elevated pulmonary pressure, it would be wise to decorticate the left heart chambers posteriorly first. In patients with heavy calcification penetrating the myocardium, the pericardium could be resected in patches and some islands of epicardium and pericardium were left intact with multiple turtle shell incisions.
It is difficult to predict preoperatively which patients are likely to respond to total pericardiectomy. Therefore, recommendations of surgery should be done cautiously in patients with mild, very advanced disease, mixed constrictive-restrictive disease, myocardial dysfunction, significant renal dysfunction and radiation induced constriction since the prognosis after pericardiectomy was reported to be poor in these disorders [
Waffle procedure
Constrictive pericarditis is somewhat associated with constrictive epicarditis especially in Japanese people. Constrictive epicardial thickness might leads to repeat surgery in some cases. Attempts are made to decorticate the white, fibrous, thickened layer of epicardium over the ventricles. The waffle procedure performed by incising the tight, fibrotic epicardium in a crosshatched manner releases the epicardial constriction [
Amniotic membrane patches (amniotic stem cell therapy)
Current treatment focused on targeting inflammation cental to this disease process and has shown overall positive outcomes [
Outcome
The prognosis varies after pericardiectomy according to the etiology of constrictive pericarditis [
Preoperative clinical status such as older age, pulmonary hypertension with concomitant myocardial dysfunction, multi-organ failure, atrial fibrillation and high mitral inflow E velocity in Doppler study [
Case analysis
This case was presented with sudden onset of tachycardia with features of right heart failure. Imaging studies showed an “egg-shell” calcification of pericardium, dynamic respiratory changes of mitral inflow velocity and a plethoric inferior vena cava suggesting a constrictive physiology. High venous pressure as “engorged neck vein”, atrial fibrillation, ascites and pedal edema is the manifestations of this “end-stage” disease.
Cardiac catheterization is no longer performed to diagnose constrictive pericarditis [
Muthiah R. (2017) “End-Stage” Constrictive Pericarditis―A Case Report. Case Reports in Clinical Medicine, 6, 255-273. https://doi.org/10.4236/crcm.2017.610029