Idiopathic Effusive-Constrictive Pericarditis in a Teenager
Alessandra Guiner, MD1,2 • Amanda Granato, BS3
1UT Southwestern Medical Center, Department of Pediatrics, Division of Emergency Medicine, Dallas, Texas
2Children’s Health Children’s Medical Center Dallas, Pediatric Emergency Medicine, Dallas, Texas
3Texas College of Osteopathic Medicine, Fort Worth, Texas
Guiner A, Granato A. Idiopathic effusive-constrictive pericarditis in a teenager. Consultant. 2021;61(12):e34-e37. doi:10.25270/con.2021.03.00022
Received November 6, 2020. Accepted December 21, 2020. Published online March 31, 2021.
The authors report no relevant financial relationships.
Alessandra Guiner, MD, Department of Pediatrics, Division of Emergency Medicine, 1935 Medical District Drive, MC: E2-03, Dallas, TX 75235 (email@example.com)
A 17-year-old Black woman with a medical history of sickle cell disease initially presented to her primary care provider (PCP) with a fever of 39.4 °C for 1 day and intermittent lightheadedness. She denied having a cough, difficulty breathing, chest pain, nausea, vomiting, or diarrhea. She also denied being in contact with sick people and reported that she was fully vaccinated.
The PCP reported that the patient appeared well. Laboratory tests to evaluate sickle cell fever and a chest radiography scan to evaluate for pneumonia were performed (Figure 1). The scan revealed a large, left-sided chest mass concerning for airway deviation, so the patient was referred immediately to a tertiary care pediatric facility for further evaluation.
Figure 1. The chest radiography scan showed a large, left-sided chest mass.
On arrival in the emergency department (ED), the patient appeared well, with a normal heart rate, blood pressure, respiratory rate, and oxygen saturation level. She had a normal physical examination, including regular heart rate and rhythm without murmurs, clear lungs, no acute respiratory distress or labored breathing, and no hepatosplenomegaly or significant jugular vein distention.
Differential diagnoses. Given these findings, the differential diagnoses considered were mediastinal mass, thymic mass, cardiac mass, thymic cyst, abscess, and pericardial effusion. About 50% of mediastinal masses are anterior, the most common being thymoma, teratoma, thyroid goiter, and lymphoma.1 In children, the most common cause of anterior mediastinal mass is lymphoma.2
A computed tomography (CT) scan with contrast was determined to be the best next step in helping to differentiate the possible diagnosis. While a magnetic resonance imaging test may have shown further detail, it can be more difficult to conduct because of limited availability and longer time in the scanner. There was concern regarding whether the patient could safely tolerate lying flat with the large chest mass. Mediastinal masses, depending on location, can cause airway obstruction and hemodynamic compromise from vessel compression.3 The patient reported she was able to lay flat at home, and her ability to lay flat without difficulty or experiencing a change in vital signs was tested in the ED before she was transferred for the CT scan to avoid complications in an area not ideal for airway management.
Diagnostic tests and imaging. The CT scan (Figure 2) showed a large, left-sided cystic chest mass measuring 14.3 × 20 × 18 cm within the anterior mediastinum and extending into the left hemithorax, with significant mass effect on the heart and mediastinum. There were no enhancing soft tissue components seen, and the structure surrounded the heart. Radiologic differential diagnoses were listed as likely thymic or pericardial cyst. Neoplasm or pericardial effusion could not be ruled out but would be atypical in presentation. The radiologists also noted several prominent lymph nodes in the upper abdomen, axilla, and retroperitoneal area, which were suspected to be inflamed, and a diffusely enlarged thyroid.
Figure 2. The chest CT scan conducted in the ED showed a large, left-sided chest mass with mediastinal deviation.
The CT scan did not show a conclusive diagnosis, so a stat electrocardiography (ECG) scan and point-of-care ultrasound (POCUS) of the chest were conducted in the ED to evaluate the patient’s heart. The ECG showed a low-voltage QRS with nonspecific T-wave changes (Figure 3), which are classic for a pericardial effusion.4 POCUS of the chest revealed a large pericardial effusion, after which a cardiologist was consulted to conduct an emergent echocardiography scan and to plan for intervention.
Figure 3. An initial EKG showed low-voltage QRS, which is consistent with large pericardial effusion.
On the initial echocardiography scan, a massive pericardial effusion with fluid extending in all directions, with the largest diameter being 11.7 cm, was seen. Echogenic evidence of tamponade physiology was noted with an underfilled left ventricle and early diastolic right ventricle collapse with compression of the right atrial cavity.
When the diagnosis was made and some of its common symptoms were explained, the patient retrospectively reported mild shortness of breath and dyspnea on exertion after climbing several sets of stairs the previous day, which quickly resolved, but otherwise had no orthopnea or respiratory concerns. She also reported intermittent lightheadedness but no syncope or fevers other than on the day of admission to the ED.
Treatment. Given the tamponade physiology and airway deviation, the patient was admitted to the pediatric intensive care unit despite having normal vital signs. A pericardial drain was placed, and 2.6 L of pale-yellow transudative fluid was drained in the first several hours. Over the next week, the patient had continued drainage of significant amounts of fluid with fibrinous deposits, which clogged the drain and required multiple replacements. The patient initially received nonsteroidal anti-inflammatory drugs (NSAIDs) as primary treatment before being transitioned to colchicine for continued fluid output refractory to NSAIDs. She was later put on a trial of anakinra, an interleukin-1 receptor agonist (off-label use); however, anakinra was discontinued when she developed transaminitis, which resolved after medication cessation.
A subsequent echocardiography scan was conducted on hospital day 11, results of which showed a constrictive component to the pericarditis with a thickened pericardium, progressive right and left atrial dilation, and restrictive ventricular filling. Abnormal interventricular septal motion with flattening during diastole was noted with normal right ventricular size, and normal left ventricular function. A diagnosis of a rare condition called effusive-constrictive pericarditis, confirmed with cardiac catheterization, was ultimately made.
The patient developed pleural effusion in addition to the pericardial effusion and eventually required the placement of a pericardial window (pericardiectomy) and chest tubes to manage the volume of fluid drainage refractory to medication management. Before discharge, another echocardiography scan was performed that showed normal right and left ventricular size and systolic function, mild tricuspid valve insufficiency, and a residual small posterior pericardial effusion.
Discussion. Pericarditis is less common among the pediatric population than it is in adults, and there are limited studies dedicated to the topic. The first large multicenter report about idiopathic pediatric pericarditis had found that the incidence of idiopathic pericarditis was highest among adolescent boys.5 Pericarditis is inflammation of the pericardium that surrounds the heart and can be categorized as acute, chronic, recurrent, constrictive, effusive-constrictive, or effusion/tamponade.4 High-risk features for patients with acute pericarditis include fever, subacute course, cardiac tamponade, significant pericardial effusion, and immunocompromised status.6 Patients presenting with any of these high-risk features generally require in-patient management.
Effusive-constrictive pericarditis is a rare clinical syndrome with constrictive hemodynamics that persist after the pericardial effusion is drained.7 First described in 1971,8 the syndrome is produced by pericardial scarring and constriction associated with pericardial effusion, leading to continued elevated right atrial pressure despite normalized intrapericardial pressure. Effusive-constrictive pericarditis has many potential etiologies and, in fact, can be caused by any condition that can lead to pericarditis. It is most commonly idiopathic in developed countries and infectious (ie, tuberculosis) in developing countries.9-12 In a 2012 systematic review that included a total of 642 patients from 5 studies, the etiologies of effusive-constrictive pericarditis were idiopathic in 50% of patients and post-radiation in 8%; the remaining etiologies were infectious, connective tissue disease, or postsurgical.11 However, it should be noted that these studies were conducted in adults. The incidence of effusive-constrictive pericardial effusion as stated in the literature ranges from 2.4% to 14.8% of patients presenting with pericardial effusion13,14; however, there are limited data on pediatric patients presenting with this finding, as few case reports have been published.
In cases of pericardial effusion causing cardiac tamponade, classic physical examination findings include Beck’s Triad (ie, hypotension, distant heart sounds, and jugular vein distension),15,16 but cases can also include pulsus paradoxus, edema, tachypnea, and tachycardia.9 However, the clinical presentation is often varied, and many patients may not develop these symptoms until late in the disease process. They may instead report dyspnea on exertion, fatigue, peripheral edema, and chest pressure. Because of decreased cardiac output as the disease progresses, patients can also present with dizziness or altered mental status.17
Diagnostic evaluation of a patient with suspected pericarditis with pericardial effusion includes ECG, echocardiography scanning, and chest radiography scanning, as well as laboratory evaluation of inflammatory markers and myocardial injury markers.2 On ECG scans, pericardial effusion may also present with sinus tachycardia and low QRS voltage, the combination of which is concerning for cardiac tamponade. Echocardiography is indicated to definitively diagnose cardiac tamponade.4
Management of pericarditis is centered around controlling pain, reducing inflammation, and preventing recurrence.4 Treatment typically consists of high-dose NSAIDs (typically ibuprofen or aspirin) as the first-line treatment, with colchicine used as a second-line therapy. Following the resolution of symptoms, NSAID therapy is often gradually tapered in an effort to prevent recurrence.4
In general, medication therapy is sufficient for treating patients with pericarditis. However, in patients with significant pericardial effusion, cardiac tamponade, or constrictive pericarditis, pericardial catheter drainage may be required. In cases where significant drainage is appreciated for longer than several days and in patients who develop constrictive pericarditis, pericardiectomy may be considered.4 While this condition and pericardiectomy in the adult population has a mortality rate of up to 50%—likely because of the underlying etiology of radiation or surgery12—pericardiectomy in pediatric constrictive pericarditis has been found to be well tolerated with good outcomes, often with complete resolution of symptoms.18
Patient outcome. No definitive cause of the patient’s pericardial effusion was discovered. Hypothyroidism was diagnosed, and the patient was started on levothyroxine, 125 mcg, daily. However, the endocrinology and cardiology specialists determined the hyperthyroidism was not significant enough to have caused the effusion. Autoimmune disease, malignancy, and infectious etiologies were also explored, but all were found to have negative results. The patient has been followed clinically and with imaging, including echocardiography and CT, without any return of clinical findings.
- Tomiyama N, Honda O, Tsubamoto M, et al. Anterior mediastinal tumors: diagnostic accuracy of CT and MRI. Eur J Radiol. 2009;69(2):280-288. https://doi.org/10.1016/j.ejrad.2007.10.002
- Ranganath SH, Lee EY, Restrepo R, Eisenberg RL. Mediastinal masses in children. AJR Am J Roentgenol. 2012;198(3):W197-W216. https://doi.org/10.2214/AJR.11.7027
- Gropper M, Eriksson L, Fleisher L, Wiener-Kronish J, Cohen N, Leslie, eds. Miller’s Anesthesia. 9th ed. Elsevier; 2019.
- Adler Y, Charron P. The 2015 ESC guidelines on the diagnosis and management of pericardial diseases. Eur Heart J. 2015;36(42):2873-2885. https://doi.org/10.1093/eurheartj/ehv479
- Shakti D, Hehn R, Gauvreau K, Sundel RP, Newburger JW. Idiopathic pericarditis and pericardial effusion in children: contemporary epidemiology and management. J Am Heart Assoc. 2014;3(6):e001483. https://doi.org/10.1161/JAHA.114.001483
- Imazio M, Cecchi E, Demichelis B, et al. Indicators of poor prognosis of acute pericarditis. Circulation. 2007;115(21):2739-2744. https://doi.org/10.1161/CIRCULATIONAHA.106.662114
- Syed FF, Ntsekhe M, Mayosi BM, Oh JK. Effusive-constrictive pericarditis. Heart Fail Rev. 2013;18(3):277-287. https://doi.org/10.1007/s10741-012-9308-0
- Hancock EW. Subacute effusive-constrictive pericarditis. Circulation. 1971;43(2):183-192. https://doi.org/10.1161/01.cir.43.2.183
- Troughton RW, Asher CR, Klein AL. Pericarditis. Lancet. 2004;363(9410):717-727. https://doi.org/10.1016/S0140-6736(04)15648-1
- Sagristà-Sauleda J, Angel J, Sánchez A, Permanyer-Miralda G, Soler-Soler J. Effusive-constrictive pericarditis. N Engl J Med. 2004;350(5):469-475. https://doi.org/10.1056/NEJMoa035630
- Spodick DH. Acute cardiac tamponade. N Engl J Med. 2003;349(7):684-690. https://doi.org/10.1056/NEJMra022643
- Yacoub M, Quintanilla Rodriguez BS, Mahajan K. Constrictive-effusive pericarditis. In: StatPearls. StatPearls Publishing; December 8, 2020. https://www.ncbi.nlm.nih.gov/books/NBK519579/
- Ntsekhe M, Shey Wiysonge C, Commerford PJ, Mayosi BM. The prevalence and outcome of effusive constrictive pericarditis: a systematic review of the literature. Cardiovasc J Afr. 2012;23(5):281-285. https://doi.org/10.5830/CVJA-2011-072
- Kim KH, Miranda WR, Sinak LJ, et al. Effusive-constrictive pericarditis after pericardiocentesis: incidence, associated findings, and natural history. JACC Cardiovasc Imaging. 2018;11(4):534-541. https://doi.org/10.1016/j.jcmg.2017.06.017
- Beck CS. Two cardiac compression triads. JAMA. 1935;104(9):714-716. https://doi.org/10.1001/jama.1935.02760090018005
- Sternbach G. Claude Beck: cardiac compression triads. J Emerg Med. 1988;6(5):417-419. https://doi.org/10.1016/0736-4679(88)90017-0
- Stashko E, Meer JM. Cardiac tamponade. In: StatPearls [Internet]. StatPearls Publishing; 2021. https://www.ncbi.nlm.nih.gov/books/NBK431090/
- Thompson JL, Burkhart HM, Dearani JA, Cetta F, Oh JK, Schaff HV. Pericardiectomy for pericarditis in the pediatric population. Ann Thorac Surg. 2009;88(5):1546-1550. https://doi.org/10.1016/j.athoracsur.2009.08.003