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-specific symptoms that usually mimic the manifestations of other conditions such as restrictive cardiomyopathy, tricuspid insufficiency, 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 inflow variation and hepatic vein flow 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 benefit 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, sodium-water 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-inflammation, 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 define exact indications, required dose and response predictors in regard of albumin use, in order that patients gain the maximum benefit 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 significance.[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%.[16–18] Moreover, the Child-Pugh score of 7 or higher, preoperative early diastolic mitral inflow velocity of 71 cm/s or higher and diabetes mellitus have recently been identified 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.