Incidental finding of congenital pericardial and ...

Radiography xxx (2015) 1e4

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Case report

Incidental finding of congenital pericardial and mediastinal pleural defect by pneumothorax in an adult Y. Sugiura a, *, Y. Matsusaka b, E. Nemoto a, T. Hashizume a, S. Kaseda a a

Department of Pulmonary and Thoracic Surgery, National Hospital Organization, Kanagawa National Hospital, 666-1 Ochiai, Hadano, Kanagawa 257-8585, Japan b Department of Radioloy, Keio University, School of Medicine, 35 Shinjuku-ku, Tokyo 160-8582, Japan

a r t i c l e i n f o

a b s t r a c t

Article history: Received 15 September 2014 Received in revised form 21 November 2014 Accepted 21 December 2014 Available online xxx

Introduction: Congenital pericardial defect (CPD) is an uncommon anomaly. If once cardiac herniation occurs, it threatens life. We report a case of left-sided pneumothorax with consequent protrusion of the heart into left thoracic cavity through not only a large CPD but also congenital pleuropericardium window. Case presentation: A 67-year-old man presenting with sudden-onset left-sided chest pain and slight dyspnea was referred to our hospital. Chest X-ray showed a left lung collapse, and also revealed a pneumopericardium along the right border of the ascending aorta. Subsequent computed tomography (CT) scan revealed that the heart was displaced into the left hemithorax. Thus, we diagnosed the patient with pneumothorax and a defect of the pericardial and mediastinal pleurae. Subsequently, a chest tube was inserted into the left thoracic cavity, and the collapsed lung was promptly inflated. The cardiac position was reinstated within mediastinum as evidenced by follow-up CT scan. The QRS axis on his electrocardiogram (ECG) was altered from 52 to 73 . Together with the cardiac relocation evidenced by the QRS axis shift on ECG and findings of CT, we determined that there was a low potential for complications and opted against surgical repair. Discussion: When the CPD is sufficiently large, surgical intervention is not necessary. The size of the CPD can be assessed not only by CT findings, but the alteration of the QRS axis on ECG also provides useful information whether cardiac herniation can be resolved by the inflated lung. © 2014 The College of Radiographers. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords: Pericardial defect Pleuropericardium window Pneumothorax

Introduction Congenital pericardial defect (CPD) is an uncommon anomaly with a reported prevalence of approximately 0.002%e0.004%.1,2 Rarely, some patients present with chest pain and arrhythmia because of cardiac herniation.3,4 Cardiac herniation is a lifethreatening condition, occurs more frequently with partial defects than with complete absence.5 Tabakin et al. reported the CPD case with pneumothorax in 1965, which was diagnosed by the chest X-ray looking for air appearing abnormally under the right-sided pericardium on a left lateral decubitus view.6,7 We report a case of left-sided

* Corresponding author. E-mail addresses: [email protected] (Y. Sugiura), [email protected] (Y. Matsusaka), [email protected] (E. Nemoto), [email protected] (T. Hashizume), [email protected] (S. Kaseda).

pneumothorax with consequent protrusion of the heart into left thoracic cavity through not only a large CPD but also congenital pleuropericardium window (CPPW), diagnosed by utilizing computed tomography (CT) and electrocardiogram (ECG). Herein, we describe the case management and a literature review. Case report A 67-year-old man with sudden-onset left-sided chest pain and slight dyspnea was referred to our hospital because of the diagnosis of left pneumothorax in April 2014. He had history of a heart murmur detected 20 years earlier and was diagnosed with aortic and mitral stenosis by echocardiography in family care physician. He was a heavy smoker (three packs/day for 45 years), had never undergone cardiac surgery, and had never injured his chest. Chest X-ray on admission showed a collapsed left lung in the lower lung field and an unidentified linear double contour

http://dx.doi.org/10.1016/j.radi.2014.12.010 1078-8174/© 2014 The College of Radiographers. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/ licenses/by-nc-nd/4.0/).

Please cite this article in press as: Sugiura Y, et al., Incidental finding of congenital pericardial and mediastinal pleural defect by pneumothorax in an adult, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2014.12.010

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Y. Sugiura et al. / Radiography xxx (2015) 1e4

Figure 1. Postero-anterior chest X-ray showed a left pneumothorax (black arrowheads), and lucency between the silhouette of the right side of heart and the right lung (black arrows).

along the cardiac border of the left mediastinal pleura (Fig. 1). The ECG showed sinus tachycardia (100 bpm) with a 52 QRS axis. CT scan showed that the heart protruded through the CPD into the left hemithorax (Fig. 2(c)). Furthermore, CT also showed the air in the transverse sinus between the aorta root and pulmonary artery trunk (Fig. 2(a)), and a left-sided defect of the mediastinal pleura, that is pleuropericardium window (Fig. 2(b)). The liner double contour in the chest X-ray was identified as pneumopericardium (Figs. 1 and 2(d)). Although the cardiac position shifted considerably, the patient was hemodynamically stable. A chest tube was inserted into the left thoracic cavity to manage the pneumothorax, which promptly inflated the collapsed lung and the heart was elevated back into the mediastinum (Fig. 3). After lung inflation, the QRS axis was changed to 73 . On the third day, the air leak stopped and the tube was removed. Discussion The present case report demonstrates two important instructive points. First, the potential risk caused by pneumothorax in patients with CPD. Second, how to manage a case of CPD with pneumothorax. CPD rarely becomes symptomatic.2 However, if strangulation of cardiac structures occurs, it can become life-threatening, which causes myocardial infarction,8 arrhythmia,9 angina,10 syncope,11 and sudden death.12 Incarcerated cardiac herniation occurs

more often with small rather than large defect.13e15 When a pneumothorax occurs in a patient with large CPD and the left lung cannot buttress the heart, the heart cannot be maintained within the mediastinum. In this case, the QRS axis shifted from 52 to 73 after lung inflation. CPD is a developmental defect that results from faulty partitioning of the pleuropericardiac cavity during the 5th week of development.16 Associated congenital abnormalities have been reported in patients with congenital defects of the pericardium, including cardiac abnormality, chest and abdominal anterior wall abnormality.15 In the present case, aortic and mitral stenosis was presented. The CPD as a hernia orifice was so large that the inflated lung could reduce the cardiac position into the mediastinum in the present case. Therefore, evaluation of the defect size is crucial. It is unclear whether CT or magnetic resonance imaging (MRI) are the most appropriate for assessing the size of CPD. Montaudon et al. reported two cases with CPD diagnosed by MRI and emphasized that MRI was the more reliable complementary modality because of its ability to image the heart in any plane.17 On the other hand, Abbas et al. insisted that CT was a powerful diagnostic tool for evaluating the pericardium and associated abnormalities because of its excellent spatial resolution.14 Based on the findings of CT and the changes in the ECG (pre- and post-inflation), we determined that the CPD was sufficiently large for the heart to pass through freely.



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Figure 2. (aec) Axial slices of computed tomography using lung window settings. (a) Free air in the transverse sinus and the space between the right lung and the right side of aorta (Ao). PA indicates main trunk of pulmonary artery and the asterisk shows the transverse sinus. (b) The anterior mediastinal pleura on the left side was obviously deficient (white arrow head). (c) The cardiac position shifted into the left thorax. (d) The coronal slice demonstrated free air trapped at the superior margin of pericardial space just above the aortic root (white arrow). The black dotted line indicates part of the left residual mediastinal pleura.

Figure 3. The cardiac position was elevated by the inflated lung after the chest tube was inserted.

Patients with complete CPD typically require no intervention, unless complications occur.15 However, in the cases of small CPDs which the inflated lung cannot push the heart back to the mediastinum from the thoracic cavity, surgical closure or enlargement may be required to alleviate complications such as herniation and entrapment of the cardiac chamber.18 It is controversial which is superior, pericardiectomy or pericardioplasty.19 In this case, we considered that the CPD did not require surgical intervention, because of the absence of complications associated with the herniation that followed the left pneumothorax and the correction of the cardiac position after lung re-inflation. Therefore, the size of the CPD can be assessed not only by CT findings, but the alteration of the QRS axis on ECG also provides useful information whether cardiac herniation can be resolved by the inflated lung. Moreover, Montaudon et al. recommended that MRI should be used to investigate whether partial or total absence of the pericardium, and thoracoscopy may represent the ultimate diagnostic tool.17 In conclusion, diagnosis of CPD with utilizing multimodality including MRI may contribute to judge whether surgical intervention is necessary or not.15,17e19


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Conflict of interest statement The author and all co-authors (YS, EN, TH, and SK) declare no conflicts of interest. Role of the funding source The author and all co-authors did not have any funding support. References 1. Van Son JA, Danielson GK, Schaff HV, Mullany CJ, Julsrud PR, Breen JF. Congenital partial and complete absence of the pericardium. Mayo Clin Proc 1993;68:743e7. 2. Maisch B, Seferovic PM, Ristic AD, Erbel R, Rienmuller R, Adler Y, et al. Guidelines on the diagnosis and management of pericardial diseases executive summary; the task force on the diagnosis and management of pericardial diseases of the European society of cardiology. Eur Heart J 2004;25:587e610. 3. Garnier F, Eicher JC, Philip JL, Lalande A, Bieber H, Voute MF, et al. Congenital complete absence of the left pericardium: a rare cause of chest pain or pseudoright heart overload. Clin Cardiol 2010;33:E52e7. 4. Steinberg C, Pelletier MJ, Champagne J. Complete absence of precordial R waves due to absence of left-sided pericardium. Ann Noninvasive Electrocardiol 2012;17:401e4. 5. Steinberg C, Pelletier MJ, Perron J, Kumar A, Champagne J. Sudden cardiac arrest due to subtotal absence of left-sided pericardium e case report and review of the literature. Congenit Heart Dis 2013;8:E92e8. 6. Tabakin BS, Hanson JS, Tampas JP, Caldwell EJ. Congenital absence of the left pericardium. Am J Roentgenol Radium Ther Nucl Med 1965;94:122e8.

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