Ann Thorac Surg 2004;77:1069-1070
© 2004 The Society of Thoracic Surgeons
Case report
Congenital pericardial defect associated with ruptured type a aortic dissection
Naruto Matsuda, MDa*,
Akira Marumoto, MDa,
Hideki Nakashima, MDa,
Yoshinobu Nakamura, MDa,
Satoshi Kamihira, MDa,
Shingo Ishiguro, MDa,
Shigetsugu Ohgi, MDa
a Division of Cardiovascular Surgery, Department of Surgery, Tottori University School of Medicine, Tottori, Japan
Accepted for publication April 11, 2003.
* Address reprint requests to Dr Matsuda, Division of Cardiovascular Surgery, Tottori University School of Medicine, 36-1 Nishi-cho, Yonago, Tottori 683-8504, Japan
e-mail: amc-matsud{at}clubaa.com
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Abstract
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Congenital pericardial defect is a rare and little-known anomaly. Here we describe the unique clinical presentation of a 64-year-old man with partial defect of the left pericardium associated with ruptured acute type A aortic dissection manifesting massive left hemothorax. In this patient, the pericardial defect played the role of a pericardial draining window, which incidentally prevented the heart from cardiac tamponade. Emergent surgery was successfully performed with a prosthetic graft replacement.
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Introduction
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In acute type A aortic dissection, cardiac tamponade, which causes deterioration of hemodynamic function by means of cardiac compression in the pericardium and limitation of ventricular filling, is a critical situation associated with poor outcome [1, 2]. Therefore, the presence of an intact pericardium may be important in ruptured aortic dissection. We report an unusual clinical presentation of a case of ruptured acute type A aortic dissection in combination with congenital defect of the left pericardium, through which the hemopericardium was spontaneously drained into the left pleural space.
A 64-year-old man was referred to our department with acute onset of chest pain radiating to the posterior neck. His blood pressure was 90/62 mm Hg in both arms, pulse was 80, and respiratory rate was 16. Chest x-ray showed marked widening of the mediastinal shadow and left pleural fluid accumulation. Computed tomography (CT) showed an ascending aortic dissection toward the aortic arch with minimal effusion in the pericardial cavity (Fig 1). Therefore, the diagnosis was distal aortic arch rupture into the left pleural space associated with acute Stanford type A aortic dissection. An emergency operation was performed.
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Fig 1. Computed tomography of the chest showing aortic dissection of the ascending aorta with massive left hemothorax (top). Note the minimal fluid accumulation in the pericardial cavity (bottom).
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After the establishment of extracorporeal circulation with femoral arterial perfusion and femoral venous drainage, median sternotomy was performed. When the pericardium was opened, clotted blood was seen around the aortic root and the rupture site was found at the ascending aorta. We also found a 3.0 x 2.5-cm defect in the left pericardium near the left pulmonary artery (Fig 2), and an anomaly in the aortic arch, which is the common origin of carotid arteries. Intraoperative exploration revealed an intimal tear at the small curvature of the aortic arch but no evidence of extravasation at the distal aortic arch. Prosthetic graft replacement of the ascending aorta and total aortic arch, under deep hypothermic circulatory arrest, was successful. We opened the left pleura and removed a massive amount of clotted blood from the left chest cavity without reconstructing the deficit of the pericardium because of the minimal possibility of cardiac displacement. The postoperative course was uneventful and, on the 46th postoperative day, he was discharged from the hospital in stable condition.
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Fig 2. Intraoperative photo demonstrating the round-shaped defect of the left pericardium (white arrow). (Ao = ascending aorta; PA = pulmonary artery.)
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Comment
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Congenital pericardial defect is an uncommon anomaly. Most pericardial defects (86%) occur on the left side and result from premature atrophy of the left duct of Cuvier during embryologic development. In 30% of instances, congenital pericardial absence is associated with cardiovascular or pulmonary congenital anomalies (eg, atrial septal defect, patent ductus arteriosus, pulmonary sequestration, bronchogenic cyst, diaphragmatic defects) [3]. Indeed, an anomaly of the aortic arch vessels was seen in our case. In most cases, the anomaly had been found incidentally without any significant clinical symptoms. However, several cases have reported life-threatening complications due to incarceration or torsion of the left appendage through the defect [4, 5].
Acute type A aortic dissection sometimes leads to fatal rupture of the ascending aorta into the pericardium, resulting in cardiac tamponade. Previous studies have found that preoperative hemodynamic shock due to severe cardiac tamponade is an independent risk factor for increased in-hospital mortality [1, 2]. In our patient, although the aortic arch dissection extended retrograde toward the aortic root and ruptured into the pericardial cavity, the hemopericardium spontaneously drained into the left pleural space through the defect of the left pericardium (Fig 2). Fortunately, our patient survived, but one can speculate that this unique combination of ascending aortic rupture and congenital pericardial defect might result in excessive bleeding into the pleural space equivalent to direct extravasation due to free aortic rupture. Furthermore, considering the potential risk that pericardiocentesis could destabilize such patients in the case of cardiac tamponade [6], the absence of pericardium might be a vital disadvantage in this situation.
In summary, we need to take account of congenital pericardial defect for accurate preoperative diagnosis, and better understand the pathophysiology underlying massive hemothorax associated with type A aortic dissection.
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Acknowledgments
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The authors thank Tim Wiltshire for reviewing the English of this manuscript.
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References
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Abstract
Introduction
