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Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
Tex Heart Inst J. 2006; 33(3): 404–405.
PMCID: PMC1592288

Nontraumatic Pericardiophrenic Defect with Tamponade

Raymond F. Stainback, MD, Section Editor
Department of Adult Cardiology, Texas Heart Institute and St. Luke's Episcopal Hospital, 6624 Fannin Street, Suite 2480, Houston, TX 77030

A 2-year-old boy with multiple congenital anomalies, including bilateral club feet, arthrogryposis, and multilevel aortic coarctation and aneurysms, presented with acute abdominal pain, vomiting, abdominal distention, and lethargy. An unusual intraluminal air collection projecting over the left heart was seen on the chest radiograph. Echocardiography showed a structurally normal heart with a small concentric pericardial effusion and an echogenic mass posterior to the left ventricle (Fig. 1). There were echo-free spaces and cavitations evident within the mass. In addition, the left atrium was compressed, and there was respiratory variability of mitral valve inflow seen on spectral Doppler echocardiography, which suggested borderline tamponade. Urgent computed tomography of the chest and abdomen confirmed the presence of dilated loops of bowel within the pericardial space (Fig. 2). On computed tomography, there was a diaphragmatic defect posterior to the left lobe of the liver, rather than anterior, as would be expected with a Morgagni hernia.

figure 30FF1
Fig. 1 Four-chamber echocardiographic view shows concentric pericardial effusion with an echogenic mass posterior to the left ventricle.
figure 30FF2
Fig. 2 An oblique coronal reconstruction of a computed tomogram shows the pericardial effusion with dilated loops of bowel within the pericardial space.

The patient was subsequently taken to the operating room, where he was noted to have a dilated transverse colon and several loops of small intestine herniating through a diaphragmatic defect that was anterior and just to the left of the esophageal hiatus. Inspection of the bowel revealed no areas of frank ischemia or necrosis. The bowel was easily reduced through the diaphragmatic defect, and the defect was closed primarily with pledgeted sutures.

In this patient, the diagnosis of distal aortic coarctation with multiple regions of aortic narrowing in combination with aortic aneurysms was suspected antenatally. At several days of age, he underwent diagnostic cardiac catheterization, which confirmed severe coarctation of the upper abdominal aorta just below the level of the diaphragm, with multiple aneurysmal dilations distal to the coarctation. At 3 weeks of age, he underwent an uncomplicated interventional cardiac catheterization, during which 5 overlapping intravascular stents were placed in the abdominal aorta. The most proximal stent was in the distal thoracic aorta at the level of the diaphragm.


Most cases of herniation into the pericardial space are related to traumatic pericardiophrenic rupture.1 In our patient, it is difficult to exclude the possibility that the defect was related to the deployment of the intravascular stents. However, this seems unlikely, given that the intravascular stents were distant from the defect in the pericardium.

Other complications can occur with diaphragmatic defects, such as herniation of the liver into the pericardium as a result of a congenital defect in the central tendon of the diaphragm.2 In addition, a deficiency of the diaphragmatic pericardium is associated with pentalogy of Cantrell, which includes a variety of midline defects.3 It is likely that our patient had a rare, isolated congenital defect in the inferior pericardial space at the level of the diaphragm that allowed herniation of the abdominal contents into the pericardium. This anatomic defect appears to be unrelated to his other congenital malformations.


Address for reprints: Jack C. Salerno, MD, University of Washington School of Medicine, Children's Hospital and Regional Medical Center, Pediatric Cardiology W-4841, 4800 Sand Pointe Way NE, Seattle, WA 98105 E-mail: gro.snerdlihcelttaes@onrelas.kcaj


1. Clark DE, Wiles CS 3rd, Lim MK, Dunham CM, Rodriguez A. Traumatic rupture of the pericardium. Surgery 1983;93: 495–503. [PubMed]
2. Anandaraja S, Kumar A, Agarwala S, Gulati GS, Bal CS, Kothari SS. Liver herniation into the pericardium: an unusual cause of massive pericardial effusion with intrapericardial mass in a neonate. Pediatr Cardiol 2005;26:862–5. [PubMed]
3. Westra SJ, Foglia R, Smith JB, Boechat MI. Omphalocele associated with intrapericardial diaphragmatic hernia. Pediatr Radiol 1991;21:525–6. [PubMed]

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