**8. Treatment**

Surgical pericardiectomy is the treatment of choice of CP with acceptable outcomes in the long-term [25–28]. Nonetheless, diuretic therapy is the first-line treatment of CP, often started even before proper diagnosis is obtained, and permits initially to control mild symptoms and reduce venous congestion. Unfortunately, as CP progresses, patients become refractory to diuretics and maintain an adequate cardiac output with compensatory sinus tachycardia. This is the reason why beta-adrenergic blockers, verapamil, and diltiazem should be avoided. In case of high-rate supraventricular arrhythmias, digoxin is the negative chronotropic agent of choice. In the specific subset of patients developing CP early (<3 months) after cardiac surgery, treatment with nonsteroidal anti-inflammatory agents, colchicine, and steroids for at least 3 months has been proposed. Predictors of success of this strategy are increased biomarkers of systemic inflammation (hsCRP) and evidence of significant pericardial inflammation visualized as intense delayed enhancement on MRI [29, 30]. Nonetheless, surgical pericardiectomy should not be delayed in case of failure of initial anti-inflammatory strategy as earlier surgery is associated with improved outcomes. In patients without ongoing acute pericardial inflammation or with long-standing symptoms, surgical pericardiectomy is the first-line treatment. Different surgical approaches have been described (on- versus off-pump, median sternotomy versus mini-invasive thoracotomy) without a clear benefit of one of them. Complete pericardiectomy, defined as extensive excision of pericardium up to superficial epicardium, if involved, anteriorly between the 2 phrenic nerves and from the great arteries superiorly to the diaphragm inferiorly, posteriorly between the left phrenic nerve to the left pulmonary veins,

#### **Figure 6.**

*Surgical approach to constrictive pericarditis. Complete pericardiectomy with extensive excision of pericardium anteriorly between the 2 phrenic nerves and from the great arteries superiorly to the diaphragm inferiorly (left panel) and posteriorly between the left phrenic nerve to the left pulmonary veins is considered the gold standard for treatment of CP. When extensive calcification is encountered, a less invasive approach with multiple transverse and longitudinal incisions up to the epicardial layer ("waffle" procedure) with the help of a dedicated ultrasonic scalpel (\*) is considered a valid alternative (right panel).*

including the diaphragmatic wall of left ventricle, is highly recommended. In case of severe calcification, it can be associated with ultrasound or laser debridement. Moreover, a less invasive approach with multiple transverse and longitudinal incisions on the epicardial layer (*"waffle"procedure*) has been proposed in patients with extensive calcific involvement of visceral pericardium and epicardium (**Figure 6**) [31, 32]. Despite long-standing experience in this procedure, pericardiectomy has a relatively high perioperative mortality rate (2–20%) associated with frequently reported low cardiac output. Predictors of poor perioperative outcomes are post-radiation CP, comorbidities (COPD, renal insufficiency, coronary artery disease, etc.), prior cardiac surgery, significant cardiac involvement (reduced LV systolic function, myocardial fibrosis/atrophy, severe tricuspid regurgitation), cardiopulmonary bypass, and poor functional status (New York Heart Association (NYHA) stage IV symptoms) [26]. Therefore, safety concerns about post-operative complications explain current indication to manage conservatively elderly healthy subjects, most of all in the presence of mild constriction, with pericardiectomy as a second-line therapy in case of progression. Similarly, patients at higher operative risk like elderly patients with severe symptoms and comorbidities are considered at prohibitively high risk, whereas radiation-induced CP is considered a relative contraindication to surgery.

### **9. Prognosis**

Pericardiectomy, if performed early after diagnosis, is usually associated with acceptable quality of life. Symptomatic relief (associated with diastolic function recovery in up to 50% of cases) usually occurs immediately after surgery or, only in a small proportion of patients, after few months [27]. Long-term survival rates, unfortunately, remain moderately acceptable, despite surgical advances in the last decades, as reported in a meta-analysis of patients submitted to pericardiectomy [2] in which pooled all-cause 1-year and 5-year mortality rates after pericardiectomy were 17.4% and 32.7%, respectively. Interestingly, patients enrolled after 2000 had higher 1-year and 5-year all-cause mortality rates compared with before 2000 (19.8% vs. 10%, p = 0.01, and 49.4% versus 20%, p < 0.001, respectively). This possibly reflects the shift that occurred in the last decades toward more complex and recurrent etiologies of CP like cardiac surgery or mediastinal radiotherapy. As a matter of fact, patients with CP secondary to cardiac surgery have significantly higher risk of all-cause mortality after pericardiectomy when compared with patients with idiopathic etiology (HR: 2.15; 95% CI: 1.21 to 3.61, p = 0.01), with even worse outcomes when CP secondary to radiotherapy is compared with idiopathic etiology (HR: 3.21; 95% CI: 1.56 to 6.50, p < 0.01) [2]. Finally, pericardiectomy performed in patients with CP secondary to tuberculosis, the most common etiology observed in developing countries, has been recently reported to have similar outcomes with respect to other etiologies, although with more technical complexity in terms of increased operative time, more blood loss, and prolonged ICU and hospital stay [3].

### **10. Conclusion**

Diagnosis of CP in the context of patients with signs and symptoms of heart failure can be challenging, and frequently, distinguishing it from RCM can be difficult. Firstly, thinking about CP when evaluating patients with diastolic dysfunction, most

### *Constrictive Pericarditis DOI: http://dx.doi.org/10.5772/intechopen.109793*

of all after cardiac surgery or mediastinal radiotherapy, is crucial to recall in our minds all the characteristics of CP and make it possible to address the correct diagnosis. CP should also be suspected when ventricular filling restrictions are observed few months after a tuberculosis infection, taking into account that tuberculosis is the first cause of CP in countries of sub-Saharan Africa and few countries of Asia.

Secondly, the use of multimodality imaging is the cornerstone for the diagnosis of CP and the evaluation of the extension of the disease and, finally, to guiding surgical treatment. In doubtful cases, we should not hesitate to ask for invasive pressure assessment, safe and diagnostic in the majority of the cases. Finally, long-terms results of surgery in patients with chronic end-stage disease are poor, most of all when CP is secondary to previous cardiac surgery or radiation therapy, also with less invasive surgical strategies like*"waffle"procedure.* Consequently, new therapeutic strategies are strongly warranted. Meanwhile, an early diagnosis could make the difference in the natural history of the disease and, therefore, should be actively promoted.
