Idiopathic Effusive Constrictive Pericarditis Accompanying Multiple Complications: A Case Report

Effusive constrictive pericarditis (ECP) is a unique clinical syndrome that is characterized by the coexistence of pericardial effusion and constrictive pericardium. The etiology of ECP usually contains tuberculosis, idiopathic, and neoplastic causes. The early diagnosis, treatment strategy and prognostic predictor of ECP still remain a big problem nowadays due to the sophisticated clinical situations. Case Presentation We here report a rare case of idiopathic ECP with thickened adherent visceral pericardium and normal parietal pericardium, accompanying multiple complications mainly comprising severe tricuspid regurgitation, hypoproteinemia, and proximal deep venous thrombosis. The patient was referred for radical pericardiectomy successfully, but the long-term prognosis may be unfavorable. This case aims to provide some clinical experience of such situation in which the clinician should weight the benets and the risks to a particular patient.


Introduction
Effusive constrictive pericarditis (ECP) has been increasingly recognized by clinicians as a unique clinical syndrome that is characterized by the coexistence of pericardial effusion and constrictive pericardium. The etiology of ECP in developing countries usually contains tuberculosis, idiopathic, and neoplastic causes. The clinical presentations are variable depending on the course of the disease, chest pain, fatigue, dyspnea, and leg edema are the most frequent complaints. [1,2] Since the most distinct pathophysiological feature of ECP is the persistently elevated right atrial pressure after pericardial effusion drainage, patients might even present with symptoms of constriction or diastolic heart failure for a long time following pericardiocentesis. [3] Typically, the inner visceral pericardium consists of a monolayer mesothelial cells that cover the surface of the heart supported by brous and adipose tissues. [4] In ECP, brosis leading to thickened adhesions of both parietal pericardium and visceral pericardium with associated pericardial uid are commonly observed. However, the visceral pericardium that is responsible for the constrictive component is not typically thickened or calci ed of this process. [5] In review of the past case reports, no ECP patient with normal parietal pericardium has been described. Here, we report a rare case of idiopathic ECP with thickened adherent visceral pericardium and normal parietal pericardium, manifesting as long-term right heart failure and accompanying many other complications.

Case Report
A 23-year-old man was admitted to our hospital with a 7-year history of bilateral leg edema and intermittent shortness of breath. Pericardiocentesis was performed 7 years ago at the local hospital due to massive pericardial effusion. About 7 months ago, the symptoms of bilateral leg edema and abdominal distension reappeared and the echocardiography showed massive pericardial effusion, suggesting effusive pericarditis. Therefore, pericardiocentesis was performed again and oral diuretics were prescribed for him to abate the discomforts. Over the past 1 month, he presented with symptoms of progressive right heart failure, pleural effusion and seroperitoneum, due to fatigue. The echocardiography suggested constrictive pericarditis. Thoracentesis was conducted at the local hospital due to the ineffective drug therapy.
Physical examination on admission showed that heart rate of 91 bpm, abnormal heart sounds, and pulse de cit; blood pressure of 89/60 mmHg; kussmaul respiration; obvious distension of jugular vein, severe bilateral leg edema, abdominal distension and hepatomegaly. The central venous pressure (CVP) was of 330 mmH2O. Laboratory ndings included severe hypoproteinemia with albumin of 27.5 g/L and globulin of 17 g/L; hypokalemia with serum potassium of 2.38 mmol/L; N-terminal portion of proBNP (NT-proBNP) was of 1267 pg/mL. The chest X-ray showed straightened cardiac silhouette of both sides (shown in Fig. 1). The echocardiography revealed respirophasic septal shift (Supplemental video 1), mild pericardial effusion, severe tricuspid regurgitation (Supplemental video 2), bi-atrial enlargement (the left atrium was 54 mm × 57 mm; the right atrium was 43 mm × 56 mm), stroke volume was of 58 ml; dilated inferior vena cava (27 mm) with disappeared respiratory-induced collapse. Cervical vascular ultrasound showed thrombosis of bilateral internal jugular vein and left brachiocephalic vein (shown in Fig. 2).
Computed tomography (CT) indicated pericardial calci cation and hepatomegaly (shown in Fig. 3). Consequently, the patient was diagnosed as ECP, New York Heart Association (NYHA) class IV, and therefore referred for radical pericardiectomy. No signi cant change of the parietal pericardium was observed during the procedure, and the pericardial cavity was smooth with about 50 ml clear pericardial effusion. Additionally, the visceral pericardium was thickened to 1.5 mm and closely adherent to the heart surface, resulting in a constrictive band like an "eggshell" to wrap the heart, which severely in uenced the diastolic and systolic functions. Finally, the thickened visceral layer was successfully removed and the CVP was immediately reduced to 190 mmH2O (shown in Fig. 4). The pathologic examination revealed brosis suggesting idiopathic pericarditis (shown in Fig. 5). The patient was meanwhile prescribed with hydrochlorothiazide, frusemide, spironolactone, benazepril, digoxin, rivaroxaban, albumin infusion and other symptomatic treatment to alleviate his symptoms of heart failure. The degree of tricuspid regurgitation decreased gradually (shown in Fig. 6) and no symptoms of heart failure were reported during the follow-up period.

Discussion
This patient is a rare case of idiopathic ECP with thickened adherent visceral pericardium and normal parietal pericardium, presenting with symptoms of right heart failure and accompanying many other complications, which confused the diagnosis, treatment and prognosis.
The early diagnosis of ECP remains to be a huge challenge due to the non-speci c symptoms that usually mimic the manifestations of other conditions such as restrictive cardiomyopathy, tricuspid insu ciency, heart failure, and cirrhosis. [6] Therefore, auxiliary tools are necessary to make an accurate diagnosis. Although invasive cardiac catheterization assessing the alterations of right atrial pressure and intrapericardial pressure pre-and post-pericardiocentesis remains the gold standard for the diagnosis of ECP, echocardiography is currently critical and more convenient in evaluation of pericardial disease. [7] The key echo-Doppler hallmark of ECP patient is respirophasic interventricular septal shift due to dissociation of intracardiac and intrathoracic pressures and ventricular interdependence, which can be observed in our patient. Other features of ECP include a higher mean medial mitral e' velocity, a higher prevalence of mitral in ow variation and hepatic vein ow reversal. [5] Many clinical trials have demonstrated that tricuspid regurgitation tremendously complicates ECP and preoperative higher grade tricuspid regurgitation is frequently associated with increased postoperative mortality. [8] Additionally, tricuspid regurgitation scarcely improves with pericardiectomy alone and valve repair has little bene t on late survival. Therefore, simultaneous tricuspid valve repair should be considered to reduce symptoms only if it does not increase the operative risk. [9,10] As for this patient, we considered that tricuspid valvuloplasty would increase the immediate risk of the operation, only pericardiectomy was performed. The tricuspid regurgitation gradually decreased to mild-moderate volume and no symptoms of heart failure were reported during the postoperative follow-up.
Albumin infusion (1-2 g/kg) was applied for this patient due to his severe hypoalbuminemia, sodiumwater retention, massive pleural effusion, seroperitoneum, and postoperative drainage, until the bad situation had improved. Albumin has many crucial physiological functions including volume expansion, anti-oxidation, anti-in ammation, and endothelial protection, which seems to be an ideal treatment solution for critically ill patients. [11] However, the use of albumin is still controversial. Despite that safety of albumin use has been proven, albumin infusion is expensive and has little impact on the survival rate.
[12] Accordingly, more efforts are urgently needed to de ne exact indications, required dose and response predictors in regard of albumin use, in order that patients gain the maximum bene t from its administration.
To date, proximal deep venous thrombosis (DVT) complicating ECP remains an extremely rare scenario.
Such patients are at high risk of developing further thromboembolic complications including pulmonary embolism (PE), intracardiac thrombi, thrombus incarceration of tricuspid valve, and even heart failure, especially during the procedure. Therefore, ultrasound-guided central venous catheterization to prevent these immediate complications is of great signi cance. [13,14] Besides, antithrombotic therapy to prevent further thromboembolic complications is of equal importance. According to the antithrombotic guideline, in patients with proximal DVT or PE, long-term (3 months) anticoagulant therapy such as dabigatran, rivaroxaban is recommended. Aspirin is also suggested to prevent recurrent venous thromboembolism (VTE) after anticoagulant therapy. [15] Central venous catheter placement was performed for our patient in prophylaxis of perioperative thromboembolic complications and gave rivaroxaban (10 mg/d) to aspirin 100 mg/d after being discharged from the hospital for three months to prevent further thromboembolic complications.
The long-term survival post-pericardiectomy depends on many factors especially the underlying etiology and the overall physical condition which mainly incorporates NYHA class III-IV, radiation history, age, serum sodium, serum albumin and serum creatinine levels. Patients with idiopathic ECP usually has the best in-hospital and long-term prognosis, with 7-year Kaplan-Meier survival of 88%. Preoperative NYHA class III-IV has been proved as a robust independent predictor of late death, with long-term survival below 50%. [16][17][18] Moreover, the Child-Pugh score of 7 or higher, preoperative early diastolic mitral in ow velocity of 71 cm/s or higher and diabetes mellitus have recently been identi ed as independent prognostic factors of poor prognosis post radical pericardiectomy. [19,20] Despite that our patient was diagnosed as idiopathic ECP, he was in NYHA class IV, Child-Pugh score was 11 and with multiple complications, therefore, the prognosis may be unfavorable.

Conclusion
ECP is becoming an increasingly recognized clinical syndrome by clinicians. The early diagnosis, treatment strategy and prognostic predictor of ECP still remain a big problem nowadays due to the sophisticated clinical situations. Our report demonstrates a rare case of ECP with multiple complications in order to provide some experience in such situation in which the clinician should weight the bene ts and the risks to a particular patient.