Pediatric pericardiectomy—a narrative review

Abstract

Background and Objective

Pediatric pericardiectomies are rare operations used to treat recurrent, purulent, or constrictive pericarditis. Given the lack of pediatric-specific guidelines, we conducted a review to summarize the current literature on pediatric pericardiectomies including: etiology, diagnosis, timing of operation, surgical methods, and outcomes.

Methods

We conducted a PubMed literature review including articles in English from 2000–2025. To ensure completeness, we utilized a systematic search with “pediatric” and “pericardiectomy” as medical subject heading or text words. We identified 92 articles and included 58 relevant publications.

Key Content and Findings

The most common etiology of pericarditis in pediatric patients requiring a pericardiectomy is idiopathic. The second most common etiology is prior cardiac surgery in the United States and bacterial infections in India. The majority of pediatric patients (i.e., >80%) treated with a pericardiectomy present with dyspnea, chest pain, or heart failure symptoms. Transthoracic echocardiogram (TTE) is the first line imaging test. Adult guidelines for the treatment of pericarditis can be utilized to inform individualized care decisions, including timing of operation, for pediatric patients potentially requiring a pericardiectomy. Specifically for constrictive pericarditis, pericardiectomy is the mainstay treatment for chronic constriction. In addition, there is concern that delaying surgery in cases of constrictive pericarditis could result in deteriorating patient status and irreversible myocardial damage. Most pediatric pericardiectomies (~90%) are performed without the use of cardiopulmonary bypass. Complete pericardiectomies are more common than partial pericardiectomies, and a median sternotomy is the most utilized approach. Among patients in the United States, the mortality rate is ~2% (1/45, range 0–4%) compared to ~11% (8/70, range 0–22%) in lower-resourced countries. The increased mortality rate in lower-resourced countries could be due to the predominance of tubercular etiology or delayed patient presentation, emphasizing the potential importance of identifying patients requiring pericardiectomy and intervening early.

Conclusions

Pediatric pericardiectomies are rare and generally safe operations, especially in the United States. There is a need to further investigate and delineate outcomes in relation to timing for pediatric pericardiectomies.

Introduction

Pediatric pericarditis is a rare disease (1,2). Among the general population of developed countries, the annual incidence of acute pericarditis is estimated at 28 per 100,000 (3). Pericarditis is less common among children (2). There is limited data concerning the incidences of pericarditis specifically in the pediatric population (2,4). An analysis of the Pediatric Health Information System (PHIS) database of 38 United States (US) hospitals identified 11,364 hospitalizations from 2007–2012 (~2,000 per year) involving the disease classification code of pericarditis and pericardial effusion (4).

The pathophysiological mechanism of pericarditis begins with an insult to the pericardium (e.g., mechanical injury secondary to surgery, radiation, infection, or autoimmune reaction). In response to the initial insult there is increased vascular permeability, fibroblast proliferation, and granulation tissue formation (5,6). Some of these patients progress to recurrent inflammation and chronic pericardial thickening and constriction.

While generally a self-limited disease, there are three major potential complications: recurrent pericarditis, purulent pericarditis, and constrictive pericarditis. Approximately a third of patients with acute pericarditis will develop recurrent pericarditis (7). Patients with pericarditis caused by a bacterial infection can develop life-threatening purulent pericarditis, with pus accumulating in the pericardial space (8,9). Constrictive pericarditis can occur if pericardial inflammation results in thickening of the pericardium and adhesion of the pericardium to the myocardium causing diastolic dysfunction (10,11).

A potential treatment for pediatric patients with recurrent pericarditis, purulent pericarditis, or constrictive pericarditis is a pericardiectomy (1). Pediatric pericardiectomies are not commonly performed. For example, Shakti et al. found that 11 of 543 (2%) pediatric patients with viral or idiopathic pericarditis underwent pericardiectomies (4). In addition, there are no pediatric specific guidelines for when to perform a pericardiectomy nor how best to perform one (2). Given the lack of guidelines, we conducted a review to describe: the etiologies of pediatric pericarditis treated with a pericardiectomy; how to diagnose pediatric pericarditis; when to perform a pericardiectomy; how pericardiectomies are performed; and outcomes of pericardiectomies. We present this article in accordance with the Narrative Review reporting checklist (available at https://jtd.amegroups.com/article/view/10.21037/jtd-2025-aw-2033/rc).

Methods

We conducted a PubMed search on September 30^th, 2025. We searched the term “Pediatric pericardiectomy”. To ensure completeness of our search, we also conducted a systematic search with “pediatric” as a medical subject heading or text word and “pericardiectomy” as a medical subject heading or text word. Our search yielded 92 publications. Following abstract screening, 21 articles that did not study pericardiectomies, were non-human studies, did not include pediatric patients, or were not in English were excluded. We retrieved 70 publications which were reviewed in full, and 58 articles were included. Table 1 summarizes our search strategy and Figure 1 provides a flow diagram of study selection.

Table 1. Search strategy summary.

Items	Specification
Date of search	September 30th 2025
Database searched	PubMed
Search terms	“Pediatric pericardiectomy”, “pediatric” & “pericardiectomy” as medical subject heading or text word
Timeframe	2000–2025
Inclusion and exclusion criteria	Inclusion: PubMed indexed publications focused on pediatric patients
	Exclusion: non-English publications, books, publications not describing pericardiectomies, adult case studies
Selection process	Initial selection performed by N.M. Full-text review and consensus conducted jointly by N.M. and R.M.

Figure 1.

Flow diagram of study selection.

As pediatric pericarditis is a rare disease, and pericardiectomy a rare treatment, the literature on pediatric pericardiectomies is limited. Due to this limitation, we included all relevant publications, including case reports, in our narrative review.

Findings

Etiology of pediatric pericarditis treated with a pericardiectomy

The most common etiology of pericarditis in pediatric patients treated with pericardiectomies is idiopathic. The second most common etiology varies by region and includes prior cardiac surgery in the US and bacterial infections in India (12,13). In a US case series (N=27), the causes of pericarditis were idiopathic (37%), prior cardiac surgery (30%), infection (22%), autoimmune disease (7%), and prior chest radiation (7%) (12). Following cardiac surgery, patients can develop post pericardiotomy syndrome, an autoimmune inflammatory reaction of the pericardium that usually occurs within weeks after cardiac surgery (14)_. Post pericardiotomy syndrome is a poorly understood condition that has also been reported at 4 and 9 months post-operation (15,16).

In an Indian case series from 2002–2011 (N=27), the most common causes of pericarditis were idiopathic (59%), non-tuberculosis bacterial infection (22%), and tuberculosis infection (19%) (13). Historically, in lower-resourced countries, tuberculosis was the most common cause of constrictive pericarditis requiring a pericardiectomy (17). Pericarditis occurs in 1–4% of patients with pulmonary tuberculosis infections and can also occur following non-tuberculosis bacterial pneumonias (18,19).

Additional reported etiologies of pediatric pericarditis requiring a pericardiectomy include genetic disorders [e.g., Mulibrey nanism, camptodactyly-arthropathy-coxa vara-pericarditis (CACP), Myhre syndrome, Familial Mediterranean fever] and cancers (e.g., anaplastic large cell lymphoma, teratoma). Table 2 summaries the published pediatric pericarditis case reports and their corresponding etiologies.

Table 2. Reported case study etiologies of pericarditis requiring pericardiectomies (see Appendix 1 for full list of included studies).

Etiology	Age (years)	Number of cases
Bacterial non-mycobacterium tuberculosis	0–10	9
Camptodactyly-arthropathy-coxa vara-pericarditis	2–13	6
Idiopathic	3–17	4
Viral: non-coronavirus disease 19	2–13	4
Mulibrey nanism	3–12	3
Bacterial mycobacterium tuberculosis	4–12	2
Viral: coronavirus disease 19	7–13	2
Anaplastic large cell lymphoma	11	1
Arthrogryposis multiplex	10	1
Chronic appendicitis	14	1
Familial Mediterranean fever	8	1
Intrapericardial cactus spine penetration	5	1
Myhre syndrome	11	1
Post pericardiotomy syndrome	9	1
Rosai-Dorfman disease	6	1

Overview of rare and genetic diseases associated with pediatric pericarditis treated with pericardiectomies

CACP is caused by an autosomal recessive genetic defect in the proteoglycan 4 gene that encodes for lubricin, which helps lubricate synovial joints. The deficiency in lubricin results in progressive cartilage destruction and a characteristic flexion deformity of the proximal interphalangeal finger joints. Six to thirty percent of patients with CACP develop pericarditis. The disease is more common in patients with consanguineous parents (20-23). It is hypothesized that lubricin also serves as an anti-adhesive between the visceral and parietal pericardium (21,24).

Familial Mediterranean fever is generally an autosomal recessive disease involving the pyrin gene and resulting in higher levels of acute-phase reactants and recurrent inappropriate fevers. This inappropriate inflammatory reaction can result in pericarditis (25).

Mulibrey nanism is another autosomal recessive disease. It is caused by a mutation in the TRIM37 gene encoding for the peroxisomal ubiquitin ligase TRIM37 protein. Patients with Mulibrey nanism can develop pericardial constriction and myocardial diastolic dysfunction (26-28).

Myhre syndrome is caused by a mutation in the SMAD4 gene (29). The SMAD4 protein is involved in multiple signaling pathways including transforming-growth-factor-B, bone-morphogenetic-protein, and activin. Patients with Myhre syndrome often have short stature, mild intellectual disability, craniofacial abnormalities, and cardiovascular complications. Among patients with Myhre syndrome, 63% have congenital heart defects, 17% have pericardial disease, and 9% have restrictive cardiomyopathy (30).

There is also a case study of constrictive pericarditis secondary to Rosai-Dorfman disease, a histiocytic disorder of unknown etiology associated with lymphadenopathy (31).

Diagnosis

The diagnosis of pericarditis requires meeting two of four diagnostic criteria: (I) chest pain that worsens upon inspiration and when supine; (II) ST-segment elevation and PR deviation on electrocardiogram; (III) pericardial friction rub on auscultation; and (IV) a pericardial effusion (6). The majority (i.e., >80%) of pediatric patients requiring a pericardiectomy present with either dyspnea, chest pain, or congestive heart failure symptoms (12,13). Interestingly, among pediatric patients, the classic findings specific to adult pericarditis, such as friction rub and muffled heart sounds, are rare. A meta-analysis of 123 cases of pediatric pericarditis due to tuberculosis found that only 11% had muffled heart sounds and only 11% had a friction rub (18).

The presenting signs of pediatric pericarditis can be subtle, and as patients can rapidly deteriorate, it is important to identify the signs quickly (8,18,32). Peters et al. report a case where protein-loss-enteropathy was the only principal sign of constrictive pericarditis (21). Bansal et al. report the case of a previously health 16-month-old female with an insidious presentation of purulent pericarditis who developed cardiac tamponade two days after presentation to the emergency room with sepsis (32). Additional presenting symptoms included arthralgia, abdominal distension, facial edema, fever, and hepatomegaly (20,21,25,31,33-36).

Initial chest X-rays in patients who will require a pericardiectomy can show enlarged cardiac silhouettes, pleural effusions, and calcifications (37-40). Once pericarditis is suspected, transthoracic echocardiogram (TTE) is the first line imaging test. TTE can confirm the diagnosis and assess severity (2,12). TTE can demonstrate ventricular enlargement and function (41). A “septal bounce” secondary to constrictive pericarditis and pleural effusions can also be appreciated on TTE (42-46).

While a TTE is often sufficient, computed tomography (CT) and cardiac magnetic resonance imaging (CMRI) can provide additional information (e.g., pericardial thickness, signs of inflammation, calcifications, masses, and foreign bodies) (18,33,47). CMRI is of particular value in evaluating the extent of pericardial inflammation (6). Such cross-sectional imaging also helps in surgical planning. Cardiac catheterization can show elevated and equal right atrial pressure, left pulmonary capillary wedge pressure, and ventricular end-diastolic pressures, confirming the diagnosis of constrictive pericarditis (42,48,49). Cardiac catheterization can be particularly useful in differentiating constrictive pericarditis from restrictive cardiomyopathy (48).

When to perform a pericardiectomy

There are no pediatric-specific guidelines for pericarditis treatment (2). However, the adult European Society of Cardiology (ESC) guidelines can inform individualized care decisions for pediatric patients who may require a pericardiectomy. The ESC guidelines recommend pericardiectomy as a fourth-line treatment for recurrent pericarditis (38). First-line treatment includes aspirin, colchicine, and limited physical activity. Second-line treatment includes low-dose corticosteroids, and third-line treatment options are intravenous immunoglobulin (IVIG), anakinra, or azathioprine (38). An analysis of the PHIS database found a 10% readmission rate among patients with idiopathic or viral pericarditis (4).

For purulent pericarditis, the ESC guidelines state that a pericardiectomy should be considered for cases of dense adhesions, thick effusions, recurrent tamponade, and purulent pericarditis that progresses to constriction (38). The literature is unclear on if/when a pericardiectomy is needed. For patients with pericardial effusions, percutaneous balloon pericardiotomy can be considered prior to surgery. However, one study (N=11 patients) found a 45% reintervention rate, with 27% of patients ultimately requiring surgery (50). A case series of three Israeli patients (14–36 months old) with purulent pericarditis were successfully treated with echocardiography guided pericardiocentesis and repetitive treatment with intrapericardial urokinase (e.g., 25,000 U twice daily for three days) (1). Conversely, an older Indian case series from 1972–2003 of pediatric patients with purulent pericarditis found a 9% mortality (2/22) among patients treated with a pericardiectomy vs. an 80% mortality (12/15) among patients treated with repeat pericardiocentesis or sub-xiphoid tube drainage (P<0.01) (51). Finally, two case studies describe successful pericardiectomies performed for pericardial effusions that persisted despite treatment with pericardial drainage and pericardial window creation (32,52).

For constrictive pericarditis, the ESC guidelines state that surgery is the mainstay of therapy in cases of chronic permanent constriction (38). As worsening New York Heart Association (NYHA) class is associated with worse surgical outcomes, some practitioners argue that a diagnosis of constrictive pericarditis is a sufficient indication for pericardiectomy (13). There is a concern that delaying surgery could result in a deterioration of patient status and irreversible myocardial damage, as chronic constriction can cause myocardial atrophy (53,54). The myocardial degeneration could be caused by impaired myocardial microcirculation due to insufficient diastolic relaxation (16). Unfortunately, no study has been conducted to compare outcomes for early vs. delayed pericardiectomy in pediatric patients with constrictive pericarditis.

Methods for conducting the surgery

A variety of surgical approaches are utilized for pericardiectomies with no clearly superior approach. In an adult cohort of 513 patients undergoing isolated pericardiectomies, 81% underwent a median sternotomy, 14% a left thoracotomy, and 5% a clamshell. There were no differences in outcomes between the three approaches utilized (55). Thompson et al. (N=27) utilized a median sternotomy for 24 pediatric patients (89%) who underwent a complete pericardiectomy and a left anterolateral thoracotomy for three pediatric patient who underwent biventricular pericardiectomy (12). Talwar et al. utilized a sternotomy in 18/27 pediatric cases (67%) and a left anterolateral thoracotomy in the remaining nine pediatric cases (33%) (13). Another pediatric case series reports 22 partial anterior pericardiectomies utilizing a left anterolateral thoracotomy through the 5^th intercostal space (51). Finally, a thoracoscopic approach was utilized in another pediatric case series (N=21) (56). The minimally invasive approach could be beneficial for patients who are high risk for open surgery (57). The authors prefer a median sternotomy to perform this operation. This allows excellent exposure to all parts of the heart, easy access to cannulate for cardiopulmonary bypass, if required, and also allows better access to manage complications, such as control of bleeding.

Bypass is not commonly utilized for pediatric pericardiectomies. Of the 38 case studies we reviewed, bypass was only utilized in three (16,41,58). Bypass was not utilized in any of the 27 cases reported by Talwar et al. (13). Thompson et al. utilized bypass for 8/27 operations (30%), mean bypass time of 51 minutes (12). Bypass was selectively used in patients with hemodynamic instability (12). Of particular concern, decortication of the right ventricle before the left ventricle can result in pulmonary edema as the left ventricle is still surrounded by constricting pericardium and increased blood flow to the right ventricle may overwhelm the left ventricle (12). This is easier stated than done in reality. With situs position of the heart, access to the lateral aspects of the pericardium, overlying the left ventricle, are harder to access than the more anteriorly covered right ventricle. Bypass is more frequently utilized for adults undergoing pericardiectomy. In an adult cohort of 513 patients undergoing isolated pericardiectomy [1993–2013], 40% of operations utilized cardiopulmonary bypass. In univariate analysis bypass was associated with increased late (i.e., >30 days post-operative) mortality [hazard ratio (HR) 1.51, P=0.03] but the difference was not statistically significant in multivariate analysis potentially reflecting the increased use of bypass in sicker patients (55). While pediatric on-pump pericardiectomies are uncommon, it is prudent to have a cardiopulmonary bypass machine and perfusionist on stand-by in case significant bleeding occurs (e.g., accidently lacerating the right atrium) (11,59).

Most pericardiectomies are complete rather than partial. A complete pericardiectomy involves removing the pericardium between the phrenic nerves, anteriorly from the great arteries to the diaphragm, and posteriorly from the pulmonary veins to the diaphragm, leaving only the pericardium posterior to the pulmonary veins and left atrium (12). A meta-analysis of adult patients undergoing pericardiectomy for constrictive pericarditis (N=2,114) found that a total pericardiectomy was utilized in 86% of cases (54). Talwar et al. (N=27) utilized a complete pericardiectomy in 74% of cases and elected to perform a partial pericardiectomy in patients with severe adhesions and direct myocardial involvement, as it was deemed safer (13). Thompson et al. (N=27) utilized a complete pericardiectomy in 89% of cases (12). Complete pericardiectomies are of particular value to patients with inflammatory pericarditis as a partial pericardiectomy risks recurrent inflammation (12). A complete pericardiectomy is mostly easily achieved through a median sternotomy, however Sudeep et al. report a complete pericardiectomy via an anterior thoracotomy (12,60). Anterior pericardiectomy is more commonly utilized for patients with purulent pericarditis (51,56).

In challenging pericardiectomies, where the outer rind is easily removed but the inner layer is adherent to the epicardium, the waffle procedure can be utilized to produce non-connected islands of constricting epicardium (42,55). This procedure involves scoring the pericardium in a grid-like fashion, using a scalpel, to effectively relieve the constriction. The heart can be seen expanding in real time. Care must be taken while performing such an operation to not injure the epicardial coronary vessels. Additionally, the non-decompressed heart (i.e., not being on bypass), facilitates performing this procedure.

Outcomes

For adults, pericardiectomies are high risk procedures with a reported perioperative morality rate of 6–16% (17,59). Adult patients who underwent pericardiectomy due to post-cardiac surgery or radiation-induced pericarditis had significantly higher mortality than those with idiopathic etiologies (HR 2.15, P=0.01 for post-cardiac surgery; HR 3.21, P≤0.01 for post-radiation) (54). The reported perioperative mortality rate for pediatric patients is lower. Specifically for patients in the United States, no perioperative mortalities were reported in the case series by Thompson et al. (N=27), the PHIS analysis of pediatric patients treated with a pericardiectomy (n=11), nor the 7 US pericardiectomy case reports (Table 3) (4,12,30,32,37,42,61-63). One patient in the Thompson et al.’s case series with radiation-induced heart disease died of acute hepatic failure on post-operative day 155 (12). It is worth noting that prolonged post-operative pleural drainage is common and often requires extended hospitalization.

Table 3. United States pediatric pericardiectomy mortality (4,12,30,32,37,42,61-63).

Author	Years	Patients (n)	Mortalities (n)	Mortality rate (%)
Thompson	1978–2008	27	1 (postoperative day 155)	4
Shakti	2007–2012	11	0	0
US case reports	2006–2023	7	0	0
Total	–	45	1	2

Pediatric patients undergoing pericardiectomies in lower-resourced countries may have worse outcomes when compared to patients in the United States (Table 4). The potentially higher mortality in lower-resourced countries could be driven by delayed patient presentation and tubercular etiology. In an Indian cohort, Talwar et al. found that worsening NYHA class was associated with an increased risk of hospital death (0% mortality for NYHA class II, 18% NYHA class III, and 50% NYHA class IV, P=0.02) (13). Nineteen of 27 (70%) patients in the Talwar et al. cohort were in NYHA class III/IV compared to 7% in the US case series by Thompson et al. (12,13).

Table 4. Pediatric pericardiectomy mortality in lower-resourced countries (13,51,56).

Author (country)	Years	Patients (n)	Mortalities (n)	Mortality rate (%)
Talwar (India)	2002–2011	27	6	22
Hayavadana (India)	1972–2003	22	2	9
Liem (Vietnam)	2001–2005	21	0	0
Total	–	70	8	11

Further suggesting the importance of early intervention, Kumpf et al. report of a 12-year-old boy with Mulberry nanism who continued to suffer from right ventricular failure post pericardiectomy and died 2 months following the operation. The patient’s family refused surgery when he presented at 6 years old with constrictive pericarditis and protein loss enteropathy (41). Among adults with constrictive pericarditis undergoing pericardiectomies (N=99), operation <6 months since symptom onset was associated with lower hospital mortality [odds ratio (OR) 0.11, P=0.054] and hepatomegaly was associated with higher mortality (OR 13.4, P=0.029) (11). As hepatomegaly can result from severe right heart failure, this data suggests early surgical intervention in cases of constrictive pericarditis could be warranted.

Limitations and future directions

A limitation of our review is that we only included articles in English. Furthermore, to maintain a clinical focus, we only utilized the PubMed database.

Moving forward, further research into pediatric pericardiectomy is required. Of particular importance are the following questions: (I) how often are pediatric pericardiectomies performed; (II) when should pediatric pericardiectomies be performed instead of alternative medical/surgical interventions; and (III) what are the long-term outcomes of complete vs. partial pericardiectomies? Database studies (e.g., utilizing The Society of Thoracic Surgeons National Database) can elucidate some of these questions. The contemporary incidence, etiologies, mortality, length of stay and complications need to be better delineated.

Conclusions

A pericardiectomy is a surgical intervention for the treatment of select cases of pediatric pericarditis and constriction. The etiology of pediatric diagnoses treated with a pericardiectomy varies and includes idiopathic, bacterial, genetic, constrictive and post pericardiotomy syndrome. The majority of children who undergo a pericardiectomy present with dyspnea, chest-pain, or other symptoms of heart failure. Most pediatric pericardiectomies are performed via a median sternotomy, are off-pump, and remove the entire pericardium. There is a need to develop pediatric-specific guidelines for when and how to perform a pericardiectomy, especially since patients who undergo surgery earlier may have superior outcomes.

Supplementary

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Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.