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A congenital absence of the pericardium diagnosed by echocardiography
International Journal of Cardiology 118 (2007) e89 – e91 www.elsevier.com/locate/ijcard
Letter to the Editor
A congenital absence of the pericardium diagnosed by echocardiography Dainari Nakashima ⁎, Ayumi Nakaboh, Tomohiro Shinozuka, Koji Hasegawa, Atsushi Nakagawa, Shigeyuki Kojima, Hidefumi Hamada First Department of Internal Medicine, Nissay Hospital, Japan Received 1 December 2006; accepted 1 January 2007 Available online 3 April 2007
Abstract A 29-year-old man visited our office after experiencing palpitations at night. The electrocardiogram revealed premature ventricular contractions (PVCs), and he was admitted to our hospital for further tests. The echocardiography revealed paradoxical motion of the interventricular septum. After considering results from a previous cardiac catheter test, we suspected that the patient may have a congenital absence of the pericardium. A CT scan and magnetic resonance imaging were inconclusive, but through echocardiography and ECG performed during three positional changes we diagnosed a congenital absence of the pericardium. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Congenital absence of the pericardium; Echocardiography in position changing
Congenital absence of the pericardium is a rare hypoplastic defect of the pericardium and visceral pleura. Patients with this defect usually have a good prognosis, but some may experience chest pain and arrythmia resulting from impaction and ischemia, possibly leading to sudden death in rare cases. Generally, the condition is found incidentally during a thoracotomy and thoracoscopy; however, when attempting to diagnose this defect, CT scan, magnetic resonance imaging (MRI) and thoracoscopy are commonly used. In this case report we diagnosed a congenital absence of the pericardium noninvasively using echocardiography. A 29-year-old man visited our office for heart palpitations at night. His past medical family history was unremarkable. Since the electrocardiography (ECG) revealed premature ventricular contractions (PVC), he visited our hospital for further tests and examination. When an echocardiography demonstrated paradoxical motion of the interventricular
⁎ Corresponding author. Present address: First Department of Internal Medicine, Nissay Hospital, 6-3-8 Itachibori Nishi-ku, Osaka City, Osaka 550-0012, Japan. Tel.: +81 6 6543 3581; fax: +81 6 6532 6482. E-mail address: [email protected] (D. Nakashima). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.01.036
septum, he was admitted to our hospital. Differential diagnoses included tricuspid valve regurgitation, Ebstein's disease, pulmonary hypertension, pulmonary valve regurgitation, anteroseptal myocardial infarction, atrial septal defect, pericarditis constrictiva, right ventricular pacing, left bundle branch block, postpericardiotomy and left hand absence of the pericardium. Considering his history, which included a cardiac catheter test (coronary angiography, left ventriculogram and blood sampling), we were highly suspicious of a congenital absence of the pericardium. A CT scan and MRI revealed a fat defect on the left side of the heart, suggesting a congenital pericardial defect, but the results were not definitive. Next we performed echocardiography and an ECG in the dorsal decubitus position (Figs. 1a, 2a), the left lateral decubitus position (Figs. 1b, 2b), and the right lateral decubitus position (Figs. 1c, 2c) respectively. During echocardiography in the right lateral decubitus position, the paradoxical motion of the interventricular septum disappeared (Fig. 1), indicating that the deflection towards the left side of the heart improved while the patient was lying on his right side. In addition, the ECG revealed a max R wave shift in leads towards the left side while in the left lateral decubitus position. This suggested
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D. Nakashima et al. / International Journal of Cardiology 118 (2007) e89–e91
Fig. 1. Echocardiograms obtained with the patient in the dorsal decubitus position (a), the left lateral decubitus position (b), and the right lateral decubitus position (c). Paradoxical motion of the interventricular septum was marked in the left lateral decubitus but disappeared in the right lateral decubitus position. This indicated that the deflection to the left of the heart was improved when the patient was lying on his right side.
that the heart dropped towards the left side of the body while the patient was lying on his left side. Therefore we diagnosed him as having a congenital absence of the pericardium. Although seeing PVCs on his original ECG was the reason for performing further tests, they did not occur during the
ECGs performed in different positions. In this case, the relationship between the patient's PVCs and his congenital absence of the pericardium is unknown. Diagnosis of a congenital absence of the pericardium is generally confirmed by means of a CT scan [1], MRI [2] and/
Fig. 2. Electrocardiographic (ECG) strips recorded with the patient in the dorsal decubitus position (a), the left lateral decubitus position (b), and the right lateral decubitus position (c). The max R wave can be seen in lead V5 in the dorsal and right lateral decubitus positions, whereas the max R wave in the left lateral decubitus position is seen in lead V6 (⁎). This suggests that the heart dropped down through the defect towards the left side of the chest when the patient was lying on his left side.
D. Nakashima et al. / International Journal of Cardiology 118 (2007) e89–e91
or thoracoscopy [3]. Some reported cases were diagnosed by identifying heart displacement to the left during echocardiography [4]. It is also reported that about 10% of patients can be misdiagnosed when using only CT scan and MRI results [5]. Congenital absence of the pericardium is categorized as absence of the left side or right side, and as a complete or partial absence [5]. This case is probably a complete absence of the left side of the pericardium. We were able to diagnose the defect through echocardiography and ECGs by seeing the heart's movement from side to side within the chest while the patient was lying in different positions. Therefore, in this case, we were able to definitively diagnose a congenital absence of the pericardium through the noninvasive techniques of echocardiography and ECG during position changing. Although congenital absence of the pericardium generally has a good prognosis, some cases have complications which require operative treatment [5]. Echocardiography during position changing is useful for diagnosing congenital absence of the pericardium.
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References [1] Salem DN, Hymanson AS, Isner JM, Bankoff MS, Konstam MA. Congenital pericardial defect diagnosed by computed tomography. Cathet Cardiovasc Diagn 1985;11(1):75–9. [2] Shiavone W. Congenital absence of the left position of parietal pericardium demonstrated by nuclear magnetic resonance imaging. Am J Cardiol 1985;55:1439. [3] Kojima S, Nakamura T, Sugiyama S, et al. Cardiac displacement with a congenital complete left-sided pericardial defect in a patient with exertional angina pectoris—a case report. Angiology Jul-Aug 2004;55(4):445–9. [4] Connolly HM, Click RL, Shattenberg TT, Seward JB, Tajik AJ. Congenital absence of the pericardium: echocardiography as a diagnostic tool. J Am Soc Echocardiogr Jan-Feb 1995;8(1):87–92. [5] Abbas AE, Appleton CP, Liu PT, Sweeney JP. Congenital absence of the pericardium: case presentation and review of literature. Int J Cardiol Jan 2005;98(1):21–5.
Letter to the Editor
A congenital absence of the pericardium diagnosed by echocardiography Dainari Nakashima ⁎, Ayumi Nakaboh, Tomohiro Shinozuka, Koji Hasegawa, Atsushi Nakagawa, Shigeyuki Kojima, Hidefumi Hamada First Department of Internal Medicine, Nissay Hospital, Japan Received 1 December 2006; accepted 1 January 2007 Available online 3 April 2007
Abstract A 29-year-old man visited our office after experiencing palpitations at night. The electrocardiogram revealed premature ventricular contractions (PVCs), and he was admitted to our hospital for further tests. The echocardiography revealed paradoxical motion of the interventricular septum. After considering results from a previous cardiac catheter test, we suspected that the patient may have a congenital absence of the pericardium. A CT scan and magnetic resonance imaging were inconclusive, but through echocardiography and ECG performed during three positional changes we diagnosed a congenital absence of the pericardium. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Congenital absence of the pericardium; Echocardiography in position changing
Congenital absence of the pericardium is a rare hypoplastic defect of the pericardium and visceral pleura. Patients with this defect usually have a good prognosis, but some may experience chest pain and arrythmia resulting from impaction and ischemia, possibly leading to sudden death in rare cases. Generally, the condition is found incidentally during a thoracotomy and thoracoscopy; however, when attempting to diagnose this defect, CT scan, magnetic resonance imaging (MRI) and thoracoscopy are commonly used. In this case report we diagnosed a congenital absence of the pericardium noninvasively using echocardiography. A 29-year-old man visited our office for heart palpitations at night. His past medical family history was unremarkable. Since the electrocardiography (ECG) revealed premature ventricular contractions (PVC), he visited our hospital for further tests and examination. When an echocardiography demonstrated paradoxical motion of the interventricular
⁎ Corresponding author. Present address: First Department of Internal Medicine, Nissay Hospital, 6-3-8 Itachibori Nishi-ku, Osaka City, Osaka 550-0012, Japan. Tel.: +81 6 6543 3581; fax: +81 6 6532 6482. E-mail address: [email protected] (D. Nakashima). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.01.036
septum, he was admitted to our hospital. Differential diagnoses included tricuspid valve regurgitation, Ebstein's disease, pulmonary hypertension, pulmonary valve regurgitation, anteroseptal myocardial infarction, atrial septal defect, pericarditis constrictiva, right ventricular pacing, left bundle branch block, postpericardiotomy and left hand absence of the pericardium. Considering his history, which included a cardiac catheter test (coronary angiography, left ventriculogram and blood sampling), we were highly suspicious of a congenital absence of the pericardium. A CT scan and MRI revealed a fat defect on the left side of the heart, suggesting a congenital pericardial defect, but the results were not definitive. Next we performed echocardiography and an ECG in the dorsal decubitus position (Figs. 1a, 2a), the left lateral decubitus position (Figs. 1b, 2b), and the right lateral decubitus position (Figs. 1c, 2c) respectively. During echocardiography in the right lateral decubitus position, the paradoxical motion of the interventricular septum disappeared (Fig. 1), indicating that the deflection towards the left side of the heart improved while the patient was lying on his right side. In addition, the ECG revealed a max R wave shift in leads towards the left side while in the left lateral decubitus position. This suggested
e90
D. Nakashima et al. / International Journal of Cardiology 118 (2007) e89–e91
Fig. 1. Echocardiograms obtained with the patient in the dorsal decubitus position (a), the left lateral decubitus position (b), and the right lateral decubitus position (c). Paradoxical motion of the interventricular septum was marked in the left lateral decubitus but disappeared in the right lateral decubitus position. This indicated that the deflection to the left of the heart was improved when the patient was lying on his right side.
that the heart dropped towards the left side of the body while the patient was lying on his left side. Therefore we diagnosed him as having a congenital absence of the pericardium. Although seeing PVCs on his original ECG was the reason for performing further tests, they did not occur during the
ECGs performed in different positions. In this case, the relationship between the patient's PVCs and his congenital absence of the pericardium is unknown. Diagnosis of a congenital absence of the pericardium is generally confirmed by means of a CT scan [1], MRI [2] and/
Fig. 2. Electrocardiographic (ECG) strips recorded with the patient in the dorsal decubitus position (a), the left lateral decubitus position (b), and the right lateral decubitus position (c). The max R wave can be seen in lead V5 in the dorsal and right lateral decubitus positions, whereas the max R wave in the left lateral decubitus position is seen in lead V6 (⁎). This suggests that the heart dropped down through the defect towards the left side of the chest when the patient was lying on his left side.
D. Nakashima et al. / International Journal of Cardiology 118 (2007) e89–e91
or thoracoscopy [3]. Some reported cases were diagnosed by identifying heart displacement to the left during echocardiography [4]. It is also reported that about 10% of patients can be misdiagnosed when using only CT scan and MRI results [5]. Congenital absence of the pericardium is categorized as absence of the left side or right side, and as a complete or partial absence [5]. This case is probably a complete absence of the left side of the pericardium. We were able to diagnose the defect through echocardiography and ECGs by seeing the heart's movement from side to side within the chest while the patient was lying in different positions. Therefore, in this case, we were able to definitively diagnose a congenital absence of the pericardium through the noninvasive techniques of echocardiography and ECG during position changing. Although congenital absence of the pericardium generally has a good prognosis, some cases have complications which require operative treatment [5]. Echocardiography during position changing is useful for diagnosing congenital absence of the pericardium.
e91
References [1] Salem DN, Hymanson AS, Isner JM, Bankoff MS, Konstam MA. Congenital pericardial defect diagnosed by computed tomography. Cathet Cardiovasc Diagn 1985;11(1):75–9. [2] Shiavone W. Congenital absence of the left position of parietal pericardium demonstrated by nuclear magnetic resonance imaging. Am J Cardiol 1985;55:1439. [3] Kojima S, Nakamura T, Sugiyama S, et al. Cardiac displacement with a congenital complete left-sided pericardial defect in a patient with exertional angina pectoris—a case report. Angiology Jul-Aug 2004;55(4):445–9. [4] Connolly HM, Click RL, Shattenberg TT, Seward JB, Tajik AJ. Congenital absence of the pericardium: echocardiography as a diagnostic tool. J Am Soc Echocardiogr Jan-Feb 1995;8(1):87–92. [5] Abbas AE, Appleton CP, Liu PT, Sweeney JP. Congenital absence of the pericardium: case presentation and review of literature. Int J Cardiol Jan 2005;98(1):21–5.