Pericardial diseases are uncommon and, consequently, are sometimes not diagnosed due to a lack of clinical suspicion. This results in missed opportunities for treatment. With recent advances in understanding the pathophysiology of pericardial diseases, alongside the development of multimodal imaging techniques for diagnosis and updated treatment strategies, an up-to-date overview is essential, particularly focusing on constrictive pericarditis (CP).
Given the intraoperative and postoperative risks of surgical procedures, the challenge for surgeons and the medical team is to determine the appropriate patient, timing, and treatment.
CP is a reversible cause of heart failure, yet it is difficult to diagnose and requires a high level of suspicion. CP can follow any pericardial pathology, with the primary etiologies being idiopathic or viral (42-61%); post-cardiac surgery (11-37%); post-radiotherapy, mainly in cases of Hodgkin’s lymphoma and breast cancer (2-31%); connective tissue disease (3-7%); and post-infection, such as tuberculous pericarditis (3-15%). Other causes, including malignancy, sarcoidosis, uremic pericarditis, asbestosis, trauma, or drug-induced origins, are rare (<10%).
Patients with CP primarily exhibit symptoms of right heart failure, such as ascites, peripheral edema, and elevated jugular venous pressure, although dyspnea and pleural effusion may also occur.
Traditionally, cardiac catheterization has been the reference test for diagnosis. However, currently, multimodal imaging (echocardiography, computed tomography, magnetic resonance imaging, and PET-CT) provides equally valuable findings for both diagnosis and follow-up, constituting non-invasive alternatives. Indeed, classic signs, such as pericardial calcification, are no longer required to support the hemodynamic diagnosis, as it suffices to demonstrate inflammatory activity and/or significant thickening resulting from chronic inflammation.
The presentation of CP can range from simple pericardial inflammation, which typically resolves with anti-inflammatory agents, colchicine, and/or steroids (subacute or transient pericarditis), to chronic effusive, constrictive, or recurrent pericarditis, which may require pericardiectomy.
When? Since 2015, the European Society of Cardiology (ESC) Working Group on the Diagnosis and Management of Pericardial Diseases, endorsed by the European Association for Cardiothoracic Surgery (EACTS), has published the following recommendations:
- Pericardiectomy is recommended in patients with chronic CP and New York Heart Association (NYHA) functional class III or IV symptoms.
- Pericardiectomy may be considered in patients with refractory recurrent pericarditis (RP).
- Pericardiectomy is recommended in patients with partial pericardial agenesis resulting in cardiac herniation and hemodynamic compromise.
- Pericardiectomy is rarely performed in cases of recurrent pericardial effusions, even in the presence of loculated effusions, or when a biopsy is needed, due to advances in surgical pericardial window techniques, including minimally invasive approaches.
Who? Patient selection should include a risk assessment. While specific estimation systems are not available, commonly used scores such as STS-PROM and EuroSCORE II may be applied. However, integrating scales like the MELD-XI, which assesses both renal and hepatic dysfunction in relation to systemic congestion, is valuable. A careful evaluation of patients with end-stage renal disease and/or advanced liver disease, with a Child-Pugh score of B or C (>7 points) or MELD-XI score of 13.7-30.6, can help in identifying cases of futility or high surgical risk. In patients with “end-stage” CP who present with cachexia, malnutrition, hypoalbuminemia due to protein-losing enteropathy, cardiac cirrhosis, and low cardiac output, surgery is not indicated. This presentation is common in patients with post-radiation CP.
Regarding etiology, idiopathic pericarditis has the best prognosis, followed by post-surgical and post-radiation pericarditis. In a study involving 601 patients, the overall in-hospital mortality was 6%, with 1.1% in idiopathic cases, 9.7% in post-surgical cases, and 27% in post-radiation cases. The 5-, 10-, and 20-year survival rates were 87%, 73%, and 30%, respectively. Patients with idiopathic disease had a survival rate of over 80% at 5-7 years, while those with post-radiation CP showed survival rates of 53.4% and 32.1% at 5 and 10 years, respectively.
How? Radical pericardiectomy via median sternotomy is currently preferred. Traditionally, anterior pericardium resection from one phrenic nerve to the contralateral phrenic nerve was performed, also releasing the pericardial ring around the superior vena cava (SVC), ascending aorta, and pulmonary artery. However, leaving the pericardium over the left ventricle (LV), on the diaphragmatic surface, or on the posterior pericardium has been associated with cases of recurrent constrictive physiology, where hemodynamic analysis reveals a more “postcapillary” than “precapillary” profile. This has led to recommendations to extend resection to the lateral LV, diaphragmatic pericardium, and posterior pericardium whenever possible. In this way, complete resection avoids any residual pericardial band that could cause residual constriction, while carefully managing the phrenic nerve pedicles to prevent iatrogenic injury.
In some patients, pericardium removal alone may not be sufficient as the epicardium is thickened and fibrotic (epicarditis), contributing to constriction. In these cases, it is also essential to remove the epicardium (visceral pericardium) to relieve constriction. This represents a significant surgical challenge, as complete removal may not be feasible in all cases. However, a “chessboard” or “tortoise shell” technique, in which the fibrotic layer is divided into multiple areas to allow heart expansion, can be applied. This technique is mainly described for tuberculous pericarditis, where epicardial calcification infiltration is present.
Despite being far from a surgery without cardiopulmonary bypass (CPB), which must be kept on standby for potential bleeding complications, CPB is used in 40%-63% of cases in various series, and many specialized centers recommend its use to facilitate access to lateral pericardium areas that would not be accessible without CPB. Moreover, with the classic approach, it is even more critical to relieve the epicardial constriction on the ventricles rather than the atria alone. Cardiac arrest may be necessary to assist dissection of the lateral LV surface when adhesions are firm, and the epicardial surface is fragile, or when concomitant procedures are required.
Evaluation of the mitral and tricuspid valves is essential. Surgical planning should account for the potential need for mitral and tricuspid repair. Even mild disease can progress after the removal of the “external annuloplasty” effect of the pericardium, as well as due to the hemodynamic changes resulting from the relief of constriction and recovery of cardiac output. This is particularly important in severe chronic CP, which can lead to annular dilation and worsening of valvular regurgitation postoperatively. Another mechanism of tricuspid regurgitation involves right ventricular (RV) dysfunction, which can stem from multifactorial causes such as surgical manipulation, hyperflow from the release of caval venous rings, and myocardial damage due to chronic inflammation. Worsening tricuspid regurgitation after pericardiectomy occurs in half of the cases and is associated with decreased survival. In fact, prophylactic annuloplasty should be considered in patients with moderate or greater preoperative tricuspid or mitral regurgitation.
The intraoperative and early postoperative management principles are “dry and tight,” meaning that positive fluid balances are kept to an absolute minimum, avoiding situations that lead to pulmonary hypertension (e.g., vasoconstrictors and desaturation), with enhanced diuretic therapy through sequential nephron blockade (loop diuretics, potassium-sparing agents, and SGLT2 inhibitors, with or without thiazide diuretics, especially chlorthalidone), avoiding bradycardia with temporary pacemaker support if necessary, considering dobutamine support according to RV response, and limiting volume expansion generally to blood products and albumin.
COMMENTARY:
There are currently no guidelines from the American College of Cardiology/American Heart Association for managing pericardial diseases. According to ESC recommendations, surgical treatment with pericardiectomy is the only definitive treatment for chronic CP.
The 2015 ESC/EACTS guidelines recommend resecting “as much as possible” of the pericardium while avoiding cardiopulmonary bypass, using it only in cases of uncontrollable bleeding. This guidance is somewhat subjective, as there is no consistent way to determine intraoperatively when “enough” pericardium has been resected. In fact, as previously noted, expert groups advocate for extended resection, including the use of CPB if necessary to achieve it.
Therefore, the current recommendation is to perform pericardiectomy via median sternotomy, considering the use of cardiopulmonary bypass for a more aggressive resection, as partial resections can lead to recurrence. The use of CPB has the drawback of a higher incidence of bleeding, which must be taken into account during the intervention. In cases of postoperative heart failure, early consideration for support with ECMO or oxy-RVAD may be warranted.
Ultimately, one of the limiting factors in decision-making is the stage of disease progression at the time of diagnosis, which may be late, resulting in missed treatment opportunities and compromised patient prognosis. Currently, we have non-invasive multimodal imaging methods that enable early diagnosis, changing prognosis and improving patients’ quality of life.
REFERENCE:
Al-Kazaz M, Klein AL, Oh JK, Crestanello JA, Cremer PC, Tong MZ, et al. Pericardial Diseases and Best Practices for Pericardiectomy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2024 Aug 6;84(6):561-580. doi: 10.1016/j.jacc.2024.05.048.
