Value of Transesophageal Echocardiography in the Diagnosis of Compressive, Atypically Located Pericardial Cysts Francesco Antonini-Canterin, MD, Rita Piazza, MD, Luigi Ascione, MD, Daniela Pavan, MD, and Gian Luigi Nicolosi, MD, Pordenone and Napoli, Italy
Pericardial cysts are not common and rarely cause symptoms. We report 2 cases of atypically located pericardial cysts with hemodynamic compromise because of the direct compression of the pulmonary veins and the right pulmonary artery. In the first case, transesophageal echocardiography (TEE) disclosed a round cystic mass compressing the posterior wall of the right pulmonary artery, with blood flow reduction in the right lung. Because of
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ericardial cysts are uncommon intrathoracic lesions, typically located in the cardiophrenic angles at radiography, usually detected by chance, and associated with an excellent long-term prognosis.1 However, a complicated clinical course of pericardial cysts has been occasionally reported, and Freedman et al2-5 recently described a case of sudden death after exercise stress testing in a patient with a large pericardial cyst. We report 2 cases of atypically located pericardial cysts with hemodynamic compromise because of direct compression of the pulmonary veins and the right pulmonary artery. Such lesions were correctly diagnosed by transesophageal echocardiography (TEE) and favorably treated surgically. CASE REPORTS Case 1 A 29-year-old previously healthy man was admitted to our institution with a 1-week history of retrosternal chest pain From the Divisione di Cardiologia, ARC, Azienda Ospedaliera S. Maria degli Angeli, Pordenone, Italy; and the Divisione di Cardiologia, Ospedale S. Maria di Loreto (L.A.), Napoli, Italy. Reprint requests: Francesco Antonini-Canterin, MD, Divisione di Cardiologia, ARC., Via Montereale 24, 33170 Pordenone, Italy (E-mail:
[email protected]). Copyright © 2002 by the American Society of Echocardiography. 0894-7317/2002/$35.00 + 0 27/4/117537 doi:10.1067/mje.2002.117537
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the symptoms, the pericardial cyst was drained and the patient had an uneventful 1-year follow-up. In the second case, a large cystic mass compressing the left atrium and the pulmonary vein outflow was clearly shown by TEE in a patient with severe respiratory distress, cyanosis, and low blood pressure. A left ventricular thoracotomy was performed with the drainage of 500 mL serous fluid from the cyst. (J Am Soc Echocardiogr 2002;15:192-4.)
and dry cough in the recumbent position. Physical examination revealed normal heart and lung findings; blood pressure and heart rate were 140/80 mm Hg and 90 bpm, respectively. The electrocardiogram showed normal sinus rhythm and incomplete right bundle branch block. A 2dimensional transthoracic echocardiography revealed normal left ventricular chamber size and function, a mildly enlarged right ventricular chamber, and a small pericardial effusion. An asymmetrical blood pulmonary flow distribution, with a reduced flow in the right lung, was present on the chest radiograph, and the finding was confirmed by a perfusion lung scintigraphy. The subsequent pulmonary angiography showed a slow filling of contrast agent, without intraluminal defects. We decided to perform a TEE, which disclosed a 5 × 4.7-cm round hypodense mass compressing the posterior wall of the right pulmonary artery in the upper transesophageal horizontal view (Figure 1).A computed tomographic scanning confirmed the presence of a round (5 × 5 cm) low-density pericardial mass, with a thin capsule, adjacent to the posterior wall of the right pulmonary artery. On the basis of these data, a diagnosis of pericardial cyst with atypical location was made, and surgery was undertaken because of patient symptoms. Surgery confirmed the diagnosis of pericardial cyst, which was drained after right ventricular thoracotomy. The pathologic examination showed the presence of fibroadipose tissue with rare hemorrhagic areas and no sign of malignancy. The patient had an uneventful clinical course after surgery and during a 1-year follow-up.
Journal of the American Society of Echocardiography Volume 15 Number 2
Figure 1 Upper esophageal horizontal scan showing a round hypodense mass compressing posterior wall of right pulmonary artery.
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Figure 2 Midesophageal longitudinal scan disclosing a round mass surrounded by a thick membrane compressing left atrium.
Case 2 A 36-year-old man with a history of hypertension was admitted to our hospital emergency department because of sudden onset of dyspnea at rest. Fifteen days before his admission, he began to have shortness of breath on exertion.At the emergency department, he was in severe respiratory distress with cyanosis and low blood pressure. The electrocardiogram showed sinus rhythm with no evidence of QRS complex and ST-T segment abnormalities. Because of hypoxemia and hemodynamic deterioration, the patient was intubated and given mechanical ventilation. A 2-dimensional transthoracic echocardiogram revealed a normal aortic valve, mild billowing of mitral leaflets into the left atrium, normal left ventricular chamber size and function, and an enlarged right ventricular chamber. In a subcostal 4-chamber view, color flow imaging showed turbulent flow at the junction between the left atrium and pulmonary veins; a pulsed wave Doppler study of pulmonary veins was not performed, because the alignment was too poor to correctly assess pulmonary flow dynamics. A TEE study was then performed and midesophageal scans disclosed a round 7 × 6-cm lowdensity mass, surrounded by a thick membrane, compressing the left atrium (Figure 2). Pulsed wave Doppler showed turbulent flow at the junction between left atrium and left pulmonary veins consistent with pulmonary flow obstruction (Figure 3). The diagnosis of pericardial cyst was confirmed by a chest computed tomographic scan, and the patient underwent left ventricular thoracotomy with the drainage of 500 mL serous fluid from the cyst. The clinical course after surgery was complicated, with transient acute renal failure treated with dialytic therapy.
Figure 3 Pulsed wave Doppler of left upper pulmonary vein showing turbulent flow at junction with left atrium consistent with pulmonary flow obstruction.
DISCUSSION Pericardial cysts are uncommon intrathoracic developmental abnormalities, occurring in approximately 1 of 100 000 persons.6 In the largest series reported by Feigin et al,1 they were typically located on chest radiograph, at the right costophrenic angle (70%) or at the left costophrenic angle (22%), whereas the remaining 8% were found above and remote from the diaphragma, along the upper mediastinum, hila, or left ventricular cardiac border. Such lesions are usually asymptomatic, or may rarely cause chest pain because of cyst torsion, and should be managed conservatively, because the long-term prognosis is favorable.7 However, the natural history of pericardial cyst
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is not completely known, and there are some reports describing a complicated clinical course.2-4 Freedman et al5 reported a case of a 44-year-old apparently healthy man who died suddenly after an exercise stress test, despite advanced cardiac life support. Postmortem examination revealed that the coronary arteries were normal in origin and distribution and totally free of atherosclerotic disease or thrombosis. A large cystic mass, located above the atria, anterior to the superior vena cava and posterior to the aorta and pulmonary artery, was noted. The cyst was intimately associated with the sinoatrial and the atrioventricular nodes, and for this reason, the authors suggested that the pericardial cyst, with its inflammatory process involving the conduction system, was responsible for the patient’s death. More recently, Okubo et al4 reported a successful needle aspiration of a pericardial cyst compressing the right ventricular chamber in a patient in cardiogenic shock. Transthoracic echocardiography can play an important role in the diagnosis of pericardial cyst, allowing the view of a spherical cystic echo-free space, contiguous to the heart.8-9 Sometimes, however, echocardiographic findings may be misleading and a differential diagnosis includes, in addition to pericardial cyst, cardiac aneurysm or pseudoaneurysm and cystic tumor. In such cases, the diagnosis is possible by means of cardiac computed tomographic scanning.10 To the best of our knowledge, this is the first report in which a life-threatening compression of pulmonary vessels by pericardial cysts was diagnosed by TEE. In the first case,TEE disclosed a round cystic mass compressing the posterior wall of the right pulmonary artery, reducing the blood flow in the right lung. In the second case, a large cystic mass compressing the left atrium and the outflow of the pulmonary veins was clearly shown by midesophageal horizontal and vertical planes. TEE has gained widespread acceptance for the evaluation of posterior structures, because of the proximity of high-frequency transducers to the left atrium.11 In our 2 cases, pericardial cysts were located remote from the diaphrag-
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ma, along the upper and posterior mediastinum, so that the direct compressive mechanism of pericardial cysts was clearly elucidated by TEE. The combination of TEE and computed tomography clearly defined the relation between these masses and the pericardium. In conclusion, pericardial cysts may determine a life-threatening condition in which the direct pulmonary vessel compression is the most important factor. TEE provides an accurate and reliable diagnostic tool for recognition of such lesions, allowing a rapid and appropriate therapeutic strategy. REFERENCES 1. Feigin DS, Fenoglio JJ, Mc Allister HA, Madewell JE. Pericardial cysts: a radiologic-pathologic correlation and review. Radiology 1977;125:15-20. 2. Bandeira FC, de Sà VPO, Moriguti JC, Rodriguez AJ, Jurca MC, Oswaldo C, et al. Cardiac tamponade: an unusual complication of pericardial cyst. J Am Soc Echocardiogr 1996;9:108-12. 3. Borges AC, Gellert K, Dietel M, Baumann G, Witt C. Acute right-sided heart failure because of hemorrhage into a pericardial cyst. Ann Thoracic Surg 1997;63:845-7. 4. Okubo K, Chino M, Fuse J, Yo S, Nishimura F. Life saving needle aspiration of a cardiac-compressing pericardial cysts. Am J Cardiol 2000;85:521. 5. Freedman CS, Parsons SR, Aquino TI, Hamilton WP. Sudden death after a stress test in a patient with a large pericardial cyst. Am Heart J 1994;127:946-50. 6. Roober P, Maisin J, Lacquet A. Congenital pleural pericardial cysts. Thorax 1963;18:146-50. 7. Stoller JK, Shaw C, Mattay RA. Enlarging, atypically located pericardial cyst: recent experience and literature review. Chest 1986;3:402-6. 8. Pezzano A, Belloni A, Faletra F, Binaghi G, Colli A, Rovelli F. Value of two-dimensional echocardiography in the diagnosis of pericardial cysts. Eur Heart J 1983;4:238-46. 9. Hynes JK, Tajik AJ, Osborn MJ, Orszulak TA, Seward JB. Two-dimensional echocardiographic diagnosis of pericardial cyst. Mayo Clin Proc 1983;58:60-3. 10. Moncada R, Baliga K, Moguillansky SJ, Subramanian R, Demos TC, Lozada C, et al. CT diagnosis of congenital intrapericardial masses. J Comput Assist Tomogr 1985;9:56-9. 11. Seward JB, Khanderia BK, Oh JK, Abel MD, Hughes RW Jr, Edwards WD, et al. Transesophageal echocardiography: technique, anatomic correlation, implementation and clinical application. Mayo Clin Proc 1988;63:649-80.
