Ann Thorac Surg 2009;88:1546-1550. doi:10.1016/j.athoracsur.2009.08.003
© 2009 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Pericardiectomy for Pericarditis in the Pediatric Population
Jess L. Thompson, MDa,
Harold M. Burkhart, MDa,*,
Joseph A. Dearani, MDa,
Frank Cetta, MDb,
Jae K. Oh, MDb,
Hartzell V. Schaff, MDa
a Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota
b Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
Accepted for publication August 4, 2009.
* Address correspondence to Dr Burkhart, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (Email: burkhart.harold{at}mayo.edu).
Presented at the Poster Session of the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.
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Abstract
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Background: Pericarditis requiring pericardiectomy is uncommon in the pediatric population. The aim of this study is to characterize our experience with this subset of patients.
Methods: Between February 1978 and May 2008 pericardiectomy was performed on 27 pediatric patients (25 male). The indication for surgery was inflammatory pericarditis in 16 and constrictive pericarditis in 11. Mean age was 16.7 years (range, 3 to 21 years). Chest pain was the most common presenting complaint. Median duration of symptoms prior to operation was 1 year. Most patients had aggressive pharmacologic treatment prior to operation. Before pericardiectomy, 10 patients were hospitalized for treatment of symptoms, 15 underwent pericardiocentesis, and 3 had a prior partial pericardiectomy.
Results: Twenty-one patients underwent complete pericardiectomy, 3 a biventricular pericardiectomy, and 3 a completion pericardiectomy. Pathologic histology of all specimens was positive for pericarditis. Pericardial cultures were obtained in 13 cases with bacteria retrieved from only 2 specimens. Median length of stay was 7 days, and the majority had an uneventful postoperative course. The one early mortality was due to acute hepatic failure in a patient with radiation-induced heart disease, 155 days after operation. After median follow-up of 1 year, complete resolution of symptoms was achieved in 89% of patients.
Conclusions: In properly selected pediatric patients, complete pericardiectomy can be performed with good outcomes. Although the etiology of pericardial irritation is frequently elusive, resolution of symptoms can be expected in most patients. Confronted with medically refractory pericarditis, earlier consideration for pericardiectomy may be warranted.
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Introduction
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Pericarditis is the result of a wide variety of diseases, and chest pain is the most frequent initial complaint [1]. While chest pain is a common complaint in children, it is not common for pericarditis to be the etiology [2, 3]. The majority of cases of pericarditis are self-limited and respond to supportive care, treatment of the inciting etiology, and the administration of medications such as nonsteroidal antiinflammatory drugs (NSAIDs) and colchicines [4, 5].
Some cases of pericarditis are refractory to medical therapy and require more aggressive treatment. Reports in the pediatric and young adult literature describing pericarditis that requires an invasive procedure are sparse and generally limited to effusive pericarditis, which is amenable to either pericardiocentesis or a pericardial window [6–11]. Literature describing complete pericardiectomy in the pediatric population is also limited.
It is common for reports about pericarditis to coalesce all of the patients into one cohort and not distinguish those with constrictive physiology from those who do not. This has resulted in much confusion in the literature as regards the diagnostic algorithm and surgical management of pericarditis. We have found it helpful to categorize complicated cases of pericarditis as either constrictive or inflammatory. The aim of the present study was to examine our experience with pericardiectomy in the pediatric population.
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Material and Methods
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Between February 1978 and May 2008, 27 patients ages 21 years and less who underwent pericardiectomy for pericarditis at Mayo Clinic were identified. Patients who had a portion of the pericardium removed as a planned part of another operation were excluded. Constrictive pericarditis was defined as restricted ventricular filling and high atrial pressure due to a thickened and (or) calcified pericardium. In all cases, the diagnosis of constrictive pericarditis was made preoperatively using standard echocardiography criteria [12]. Inflammatory pericarditis was defined as a condition predominantly with recurrent chest pain but without significant hemodynamic abnormalities [13].
A "complete" pericardiectomy was defined as wide excision of the anterior pericardium between the two phrenic nerves and from the great arteries superiorly to the diaphragm inferiorly; the pericardium posterior to both phrenic nerves to the pulmonary veins, and finally the pericardium on the diaphragmatic surface of the ventricles. A "biventricular" pericardiectomy is similar to a complete pericardiectomy, with the exception that the pericardium around the right atrium is not removed. In both instances, the pericardium posterior to the pulmonary veins and left atrium remain intact.
A retrospective review of the patients' medical records was performed to gather clinical characteristics, details of the pericardiectomy, and postoperative outcome. Results described in the manuscript are for the entire cohort, while the tables separate the patients based on whether the pericarditis was constrictive or inflammatory. The study protocol was approved by the Institutional Review Board of the Mayo Clinic.
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Results
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During the study period, 27 patients were identified as having undergone a pericardiectomy. Sixteen patients had inflammatory pericarditis and 11 had constrictive physiology. The clinical characteristics are represented in Table 1. Interestingly, 24 (89%) of the patients were male. The mean age at the time of pericardiectomy was 16.7 ± 4.2 years (range, 3 to 21 years). The incidence of pericardiectomy has been increasing.
The presenting symptoms are depicted in Table 2. Chest pain was the presenting symptom in 14 (52%) patients; while 7 (26%) patients complained of either shortness of breath or dyspnea (2 were diagnosed with pneumonia at the initial presentation). Miscellaneous presentations included lower extremity swelling, ascites, fever, and one case found incidentally when the patient was undergoing a routine postoperative echocardiogram (ECHO). For the entire cohort, the median symptomatic duration before pericardiectomy was 13 months (range, 0.2 to 66.5 months).
The causes of pericarditis were multifactorial (Table 3). Ten (37%) of the patients had indeterminate causes of pericarditis, and were classified as idiopathic. Other putative etiologies of pericarditis included prior pericardial-cardiac surgery (29.6%), infection (22%), autoimmune (7%), and prior radiation to the chest (7%). For the 7 patients with prior cardiac surgery, the median time between the preceding pericardiotomy and the development of symptoms of pericarditis was 53 months (range, 0.4 to 192.7 months).
Various imaging methods were employed to help secure the diagnosis of pericarditis prior to pericardiectomy (Table 4). All of the patients, with one exception, underwent two-dimensional and Doppler ECHO examination in a standard fashion. Computed tomography (CT) scan was obtained in 13 patients; 10 of which were done after the year 2001. Eight patients had cardiac catheterization prior to pericardiectomy, and the three that were preformed after the year 2000 were done so by referring institutions. Only 6 magnetic resonance images (MRI) were performed; 5 being done after 2001.
Twenty-five patients received a trial of medical therapy prior to pericardiectomy (Table 5). Importantly, the average number of medications taken in order to treat pericarditis at the time of presentation for pericardiectomy was 2.2. Fourteen patients had been given at least one course of steroids and the median length of steroid use prior to pericardiectomy was 38 months. Five patients had received enough steroids to have developed cushingoid features at the time of pericardiectomy. Colchicine had been taken by only 7 patients. Of note, both patients who did not receive preoperative medical therapy were operated on prior to 1975.
Occasionally, therapy escalated to more than treatment with medication. Eleven patients required hospitalization at some point in order to treat the pericarditis; six of whom required at least 2 in-patient stays. Fluid in the pericardial space was removed by pericardiocentesis in 14 and a pericardial window in 3. Thoracentesis was performed in 3 patients.
A complete pericardiectomy was performed in 24 of the 27 patients. Three patients with constrictive physiology underwent a "biventricular" pericardiectomy, and all 3 of these were approached using a left anterolateral thoracotomy. Cardiopulmonary bypass was used in 8 patients with a mean bypass time of 51 minutes. The only concomitant procedure was closure of an atrial septal defect. Generally, the pericardium of patients undergoing pericardiectomy for constrictive physiology was rigid, tended to be thickened, and was more adherent to the epicardium. In contrast, gross inspection of the pericardium in the inflammatory group revealed an edematous pericardium that was frequently erythematous and thickened. Stigmata of acute inflammation were seen microscopically in 9 patients with constriction while the other 2 had both acute and chronic inflammatory changes. In patients with inflammatory pericarditis, 12 had microscopic evidence of acute inflammation and 4 had evidence of both acute and chronic pericarditis. Interestingly, microscopic calcifications were only observed in 2 patients of each group. The average pericardial thickness was 5 ± 2.5 mm. Gram stain and culture were obtained on 10 specimens. Gram positive cocci were visible on 2 of the Gram stains, and cultures isolated Proprionibacteria from one specimen and Corynebacterium from the other.
The postoperative course was unremarkable for the majority of the patients. Twelve patients required blood transfusion, and an average of 1.4 ± 1.7 units was transfused. One patient required reoperation for postoperative bleeding. Another patient had temporary hemiparesis of the left diaphragm that resolved prior to going home. The one early mortality occurred 155 days after operation. The patient was a 17 year old male who had undergone prior radiation therapy to the chest for non-Hodgkin's lymphoma. Preoperative investigations including ECHO, cardiac catheterization, and CT scan demonstrated a combination of constrictive and restrictive physiology. Postoperatively he developed fulminant hepatic failure and succumbed. The median length of stay for the remaining patients was 6 days (range, 4 to 39 days). Median length of follow-up of 1 year revealed complete resolution of symptoms in 24 (89%) patients. Two patients, one in each group, continued to have intermittent chest pain. The NSAIDs effectively controlled symptoms in one patient, while a combination of NSAIDs and steroids were necessary for symptom relief in the other patient.
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Comment
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The present study examines our experience of pericardiectomy in the pediatric population. Based on echocardiography [14], we are able to categorize patients preoperatively as to whether they have constrictive or inflammatory pericarditis. Currently, a MRI or CT scan is obtained on almost all patients to assist in the evaluation of the pericardium looking for signs of inflammation, thickening, or calcification. At our institution, cardiac catheterization is not performed routinely unless the echocardiographic findings are nondiagnostic.
The preoperative determination of constrictive physiology is important in planning the operative approach. Patients with inflammatory pericarditis require a complete pericardiectomy or risk recurrent pericarditis. This must be performed through a median sternotomy so that the pericardium around the right atrium, inaccessible from the left chest, can be removed. Although the majority of patients with constriction underwent a complete pericardiectomy all that is required is a biventricular pericardiectomy, which can be accomplished through the left chest [15].
At the time of pericardiectomy, most patients were taking at least 2 medications to treat pericarditis symptoms. Only 7 were taking colchicine, but 14 were taking steroids. Complete pericardiectomy was performed in 21 patients, biventricular pericardiectomy in 3, and completion pericardiectomy in 3. Postoperatively there were few major morbidities and a single mortality. Complete resolution of symptoms occurred in greater than 90% of patients.
The true burden of disease caused by pericarditis in children is not known. Of 107 pediatric cardiology consultations for chest pain at a tertiary academic hospital, 5 (4.7%) were due to pericarditis [16]. This is similar to the adult population where pericarditis has accounted for 5% of adults presenting to the emergency room with chest pain [17]. Pericarditis was also a rare cause of chest pain in the report by Selbst and colleagues [2] (1 case of confirmed pericarditis in 407 consults for chest pain). It is important to note, however, that the etiology of chest pain in 21% of their patients was classified as idiopathic. The etiology for chest pain may be classified as idiopathic when patients lack constitutional symptoms consistent with pericarditis; which has been shown to occur in the pediatric age group [18]. Subclinical pericarditis has also been observed in the adult literature, where the prevalence of pericarditis has been observed to be 1% in autopsy studies [19].
Frequently, the natural history of pericarditis is benign and the management is largely supportive [5]. Unfortunately, inflammatory pericarditis is a common complication after an episode of acute pericarditis; occurring in 8% to 80% of cases [20]. The clinical manifestations during a relapse are similar to the first episode, but the symptoms are usually less intense [21]. In cases of inflammatory pericarditis, aspirin and NSAIDs are administered to relieve chest pain and are effective in approximately 85% to 90% of cases [22, 23]. Administration of colchicine reduces symptoms in patients with recurrent pericarditis, and may prevent future relapses [4]. Colchicine has been shown to be effective in the adolescent age group [24]. Administration of corticosteroids is very effective at quickly controlling the symptoms of acute pericarditis, but their use may lead to inflammatory pericarditis. The current study found that at the time of pericardiectomy most patients were taking at least 2 medications in order to control the symptoms of pericarditis. Surprisingly, only 8 of the 27 patients in this study received colchicine. Five of the 8 were patients operated on after 2004, which may be a reflection of recent awareness about the benefits of colchicine. Fifty-two percent of the patients in the present study were using steroid at the time of pericardiectomy, 5 of whom had developed cushingoid features.
Eleven of the 27 patients in the present study had constrictive physiology. In the adult population, constrictive pericarditis is a debilitating condition and frequently presents with signs and symptoms consistent with refractory congestive heart failure. Although pericardiectomy is the only accepted curative treatment for constrictive pericarditis [25–27] pericardiectomy performed in the setting of constriction in the adult population carries with it a substantial mortality rate [28, 29]. Also, it has been shown that patients with abnormal left ventricular contractility and relaxation properties before surgery incur a higher operative mortality and poorer long-term outcome after surgery [12]. In the present study it was interesting to find that the majority of patients had either New York Hear Association (NYHA) class I or II symptoms. The 2 patients with class III symptoms did have constrictive physiology. The ejection fractions (EF) were also relatively preserved; with a mean EF in the constriction group slightly less at 0.54 ± 0.0 5 compared with the 0.59 ± 0.0.5 of the inflammatory group. We hypothesize that preserved left ventricular function may be the reason why the two groups had similar preoperative NYHA classifications. Preoperative use of heart failure medications did not seem to be more common in the constrictive versus the inflammatory group. There was only one mortality (inflammatory group), and relatively few postoperative morbidities in either group. These results may be due to a superior preoperative physiologic state and reserve of children and young adults undergoing pericardiectomy compared with the adult population. This would suggest that early pericardiectomy should be recommended when constrictive pericarditis is diagnosed.
The present study demonstrated the efficacy of pericardiectomy at relieving the symptoms associated with inflammatory or constrictive pericarditis. For cases of inflammatory pericarditis we feel it imperative to remove all of the pericardium that could serve as a nidus for future relapses, and for that reason a complete pericardiectomy must be performed. Although a small area of pericardium remains posterior to the left atrium, this does not appear to result in recurrences. A complete pericardiectomy is accomplished through a median sternotomy. By comparison, constrictive physiology is a problem primarily of the ventricles and can be alleviated by decorticating both the right and left ventricles with a biventricular pericardiectomy. This can be accomplished through the left chest or median sternotomy. Cardiopulmonary bypass may be used selectively and is required if hemodynamic instability is encountered as the heart is manipulated in order to remove all areas of the pericardium. Extra caution should be used when attempting an off-pump pericardiectomy through a median sternotomy in the setting of constriction. Decortication of the right ventricle first can result in the loss of ventricular interdependence. Because the left ventricle is still surrounded by constricting pericardium, inspiratory augmented blood flow to the right ventricle may overwhelm the capacity of the left ventricle and result in pulmonary edema.
In our series, one patient suffered temporary phrenic nerve palsy. In either constriction or inflammatory pericarditis identification of the phrenic nerves can be difficult. This is particularly true of the left phrenic nerve. One strategy we have employed when visualization of either nerve is compromised is to locate the nerve using a nerve stimulator. The entire course of the nerve can then be outlined using a sterile marking pen. At the end of the case, the nerve stimulator is used again to verify that both phrenic nerves are intact. If a nerve stimulator is to be used, however, communication with members of the anesthesiology team is imperative because short-acting muscle relaxants are required.
In conclusion, literature about pericarditis requiring pericardiectomy in the pediatric age group is scarce. The etiology of pericarditis is heterogeneous, but many cases remain idiopathic. In this age demographic those with constrictive pericarditis do not appear to present much differently than those with inflammatory pericarditis. Most patients have symptoms for approximately one year prior to pericardiectomy being performed. During this time, most patients undergo aggressive pharmacotherapy, including taking steroids. Almost half will require hospitalization and undergo an invasive procedure in order to control the symptoms and sequelae of pericarditis. Complete pericardiectomy through a median sternotomy should be performed on cases of inflammatory pericarditis. While the majority of cases with constrictive physiology undergo a complete pericardiectomy, biventricular pericardiectomy is a reasonable alternative. Pericardiectomy can be accomplished safely and resolution of symptoms can be expected. Earlier referral for consideration of pericardiectomy should occur in cases of constrictive pericarditis and may be appropriate for inflammatory pericarditis that is refractory to medical treatment.
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Abstract
Introduction
