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Ann Thorac Surg 2007;83:1552-1553
© 2007 The Society of Thoracic Surgeons

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Case Reports

Congenital Defects of the Pericardium

^a Department of Cardiac Surgery, Papworth Hospital, Cambridge, United Kingdom
^b Department of Anesthesia, Papworth Hospital, Cambridge, United Kingdom

Accepted for publication October 24, 2006.

^_* Address correspondence to Dr Large, Department of Cardiac Surgery, Papworth Hospital, Cambridge, CB3 8RE United Kingdom (Email: stephen.large{at}papworth.nhs.uk).

	Abstract

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Congenital defects of the pericardium are rare, but when they are reported they are frequently associated with other cardiac lesions. We describe a case of partial pericardial defect found incidentally at surgery for closure of an ostium primum atrial septal defect. Proposed mechanisms of pericardial defect development are discussed and we suggest that associations with congenital and acquired heart disease are mostly circumstantial.

	Introduction

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Congenital defects of the pericardium are rare. Southworth and Stevenson [1] reported a single case in 14,000 autopsies at Johns Hopkins. Fifteen cases of partial or complete absence of the pericardium were found over 40 years at the Mayo Clinic among 34,000 patients undergoing cardiovascular surgery (0.044%), none of which were diagnosed preoperatively [2]. These lesions are usually an unexpected finding during surgery or at postmortem; so it is unsurprising that as many as half of the patients have another structural cardiac anomaly. Pericardial defects have been described in conjunction with the most common congenital cardiac lesions including patent arterial duct, tetralogy of Fallot, and secundum and sinus venosus atrial septal defects [2–4]. This is the first reported case of a pericardial defect found with a primum atrial septal defect.

A 27-year-old man presented to his local hospital complaining of left-sided chest pain. He was otherwise asymptomatic and had been previously fit and well. On examination, he was in sinus rhythm with a prominent right ventricular impulse and auscultation revealed fixed splitting of the second heart sound and an ejection systolic murmur. Electrocardiography showed prominent P waves, right axis deviation, and partial right bundle branch block. A large primum atrial septal defect was demonstrated on transthoracic echocardiography. His pain resolved spontaneously and he was referred to our unit.

Transesophageal echocardiography confirmed a primum atrial septal defect unsuitable for percutaneous device closure and he was referred for surgery. At sternotomy, a partial absence of the left pericardium was found, allowing free communication between the heart and the left chest (Fig 1). The pericardium was intact anteriorly but did not extend beyond the reflection of the pericardium with the left pleura. The pericardium reappeared beyond the left pulmonary veins with the phrenic nerve visualized over the remnant. The 2-cm ostium primum defect was closed with a composite velour and autologous pericardial patch; the pericardial defect was left untreated. On routine follow-up in the outpatient clinic, the patient has been found to remain well.

View larger version (153K): [in this window] [in a new window]   Fig 1. Incidental finding of a partial left pericardial defect (arrow) with the left chest beyond; opening above due to breach of the pleura during sternotomy.

	Comment

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Pericardial defects arise from maldevelopment of the pleuropericardial membrane. Almost all of the 200 or so reported cases have occurred on the left. In three-quarters of these, the left pericardium was all but absent with the heart and lung occupying a common serous cavity; in the remainder, the pericardial and left pleural cavities were connected by a foramen of variable diameter forming a partial defect [1]. The most widely accepted hypothesis for the origin of these defects is persistence of the pleuropericardial channel due to premature atrophy of the left common cardial vein, the size of the defect determined by the timing of its degeneration [1]. Others have suggested that herniation of a lung bud or enlargement of the developing heart may impair closure of the pleuropericardial foramen. A recent article proposed that some defects may result from a traction-induced tear in the pleuropericardial membrane during embryogenesis rather than failure of the foramen to close [6].

Pericardial defects have been described in patients with a range of congenital and acquired heart conditions. As the associations are broad and infrequent, it is unlikely that most of these defects are related to the pathogenic mechanisms of other congenital heart disease. We believe that the finding of pericardial defects in these patients predominately reflects the greater frequency of operative interventions and postmortem examinations, illustrated by the chance finding in our case. However, pericardial defects may form part of a spectrum of abnormal coelomic development affecting the diaphragm, sternum, and anterior abdominal wall, including ectopia cordis and the rare pentalogy of Cantrell. The latter is believed to be due to segmental failure of lateral mesoderm migration and is associated with congenital heart defects, particularly ventricular septal defect and tetralogy of Fallot [7].

Most pericardial defects are asymptomatic and are an unexpected finding at surgery or postmortem, although a partial defect may have an acute presentation. Chest pain, dyspnea, dysrhythmias, syncope, and even sudden death due to cardiac chamber herniation have been reported, and may require prompt diagnosis and emergent intervention [2]. Chronic pain may result from traction on cardiac structures in the absence of the pericardium or even compression of the left anterior descending coronary artery by the rim of a partial defect [8]. In our patient, the defect was small and unlikely to explain the episode of nonspecific chest pain, although its origin remains unclear. Pericardial defects have been diagnosed noninvasively by plain chest roentgenograms, computed tomography, and magnetic resonance imaging [8]. With the widespread use of advanced imaging, nonoperative diagnosis may redefine the prevalence of this condition.

Although the pericardium has several functions, including fixation of the heart in the mediastinum, its integrity is not essential. Asymptomatic complete absence of the pericardium and small defects present no hazard to the patient and require no intervention. Moderate-sized defects are rarely of clinical significance and despite the potential for cardiac chamber herniation, most authors advise leaving incidental defects untreated; in cases diagnosed at surgery for other lesions, subsequent adhesions are likely to restrict cardiac mobility. Therefore intervention should be reserved for symptomatic defects, either primary closure or longitudinal pericardotomy for partial defects, or pericardioplasty for absent left pericardium [2, 8]. We advocate enlarging partial defects to relieve tension on the pericardial rim and prevent restrictive herniation. Direct incision decreases the risk of patch suture injury to the phrenic nerve, which usually tracks the anterior free edge of the defect but may follow an aberrant course [6].

	Acknowledgments

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We thank Rana Sayeed for his advice on this article.

	References

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1. Southworth H, Stevenson CS. Congenital defects of the pericardium Arch Intern Med 1938;61:223-240.[Abstract/Free Full Text]
2. van Son JAM, Danielson GK, Schaff HV, Mullany CJ, Julsrud PR, Breen JF. Congenital partial and complete absence of the pericardium Mayo Clin Proc 1993;68:743-747.[Medline]
3. Nasser WK, Helmen C, Tavel ME, Feigenbaum H, Fisch C. Congenital absence of the left pericardiumClinical, electrocardiographic, radiographic, hemodynamic and angiographic findings in six cases. Circulation 1970;41:469-478.[Abstract/Free Full Text]
4. Skalski J, Wites M, Haponiuk I, et al. A congenital defect of the pericardium Thorac Cardiovasc Surg 1999;47:401-404.[Medline]
5. Sadler TW. Langman’s Medical Embryology. 9th ed.. Baltimore, MD: Lippincott Williams & Wilkins; 2004. pp. 216-217.
6. Kaneko Y, Okabe H, Nagata N. Complete left pericardial defect with dual passage of the phrenic nerve: a challenge to the widely accepted embryogenic theory Pediatr Cardiol 1998;19:414-417.[Medline]
7. Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium and heart: a case report and review of the syndrome Pediatrics 1972;50:778-792.[Abstract/Free Full Text]
8. Gatzoulis MA, Munk M-D, Merchant N, Van Arsdell GS, McCrindle BW, Webb GD. Isolated congenital absence of the pericardium: clinical presentation, diagnosis and management Ann Thorac Surg 2000;69:1209-1215.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Nigel E. Drury Ravi J. De Silva Roger M.O. Hall Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Drury, N. E. Articles by Large, S. R. Search for Related Content PubMed PubMed Citation Articles by Drury, N. E. Articles by Large, S. R. Related Collections Congenital - acyanotic Pericardium