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J Thorac Cardiovasc Surg 1995;109:805-807
© 1995 Mosby, Inc.

BRIEF COMMUNICATIONS

Congenital total absence of the pericardium: Case report of a 72-year-old man and review of the literature

Ayman J. Hammoudeh, MD, Michael E. Kelly, MD, FACC, Haroutune Mekhjian, MD, FACS, FACC


Paterson and South Orange, N.J.

From the Departments of Cardiology and Cardiothoracic Surgery, Saint Joseph's Hospital and Medical Center, Paterson, N.J., and Seton Hall University School of Graduate Medical Education, South Orange, N.J.

Congenital absence of the pericardium (CAP) is rarely encountered during clinical practice. Most patients remain free of symptoms, and the diagnosis is made intraoperatively or at postmortem examination. Go 1 Some cases have been diagnosed by various imaging techniques, ranging from plain x-ray films to nuclear magnetic resonance. We report the case of a man with coronary artery disease (CAD) who underwent stress thallium scan. The scintigraphic images showed abnormal left ventricular (LV) orientation and levorotation. Intraoperatively, CAP was diagnosed.

CASE REPORT

This 72-year-old man with hypertension reported episodic chest pain, dyspnea, and palpitations for about 20 years. Coronary angiography and cardiac catheterization in May 1993 revealed triple-vessel CAD and mild aortic stenosis. Because the major coronary lesions were mostly distal in location, medical management was undertaken. Chest pain recurred 2 months later and stress thallium scan was recommended. Preexercise examination revealed a blood pressure of 140/60 and a heart rate of 58 beats/min, and the cardiac apex was shifted to the left with an ejection systolic murmur at the left sternal border. The electrocardiogram (Fig. 1) revealed sinus bradycardia, right axis deviation, left posterior hemiblock, and QRS complex clockwise rotation. The patient exercised for 6 minutes (modified Bruce protocol) and achieved a highest exercise level of 1.7 mph at 5% grade for 3 minutes during which he had angina. The blood pressure dropped from 170/70 to 150/60 before he reached 85% of age-predicted target heart rate. No electrocardiographic changes were noted. Thallium-201 was injected 45 seconds before exercise termination and imaging was done immediately and 4 hours later in the anterior and 40- and 60-degree left anterior oblique views. The scintigraphic images showed cardiac levorotation and abnormal LV orientation with no increased lung uptake or LV dilation. Delayed tracer uptake was noted in the anterolateral wall.



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  		Fig. 1. Twelve-lead electrocardiogram from patient illustrating sinus bradycardia, right axis deviation, and clockwise rotation of QRS complexes in precordial leads.

 
On the basis of the poor effort tolerance, angina, and failure to sustain blood pressure the patient was referred for coronary artery bypass grafting. Intraoperatively, the heart was displaced to the left hemithorax with total absence of the pericardium. There was severe CAD with a left main coronary artery ostial lesion and a heavily calcified aortic valve with sclerotic leaflets and anulus. A large lingula and right lung accessory lobe were noted. Triple saphenous vein graft bypass and aortic valve replacement with a Carpentier-Edwards porcine valve (Baxter Healthcare Corp., Santa Ana, Calif.) were done.

DISCUSSION

Four decades after Columbus reported the first case of CAP in 1559, Go 1 Ellis, Leeds, and Himmelstein, Go 2 in 1959, described the roentgenologic sign of left-sided pericardial absence. CAP takes place in the eighth embryonic week when premature atrophy of the left common cardiac vein (Cuvier's duct) compromises the blood supply to the pleuropericardial membrane with subsequent formation of various degrees of CAP. Go 1 Complete or almost complete CAP is more commonthan partial pericardial defects. Go 3 The loss of adequate cardiac support may explain the chest pain and palpitations. Shortness of breath and syncope are other potential symptoms. Go Go 4,5 Although partial pericardial defects are potentially lethal because of myocardial herniation and incarceration in the young, Go 6 total absence of the pericardium can remain asymptomatic and can be compatible with long life. To our knowledge, CAP was reported only in one patient (77 years old) older than ours. Go 7

Patients with absent left pericardium have a leftward cardiac apex shift with systolic murmur The electrocardiogram typically shows the same findings seen in our patient. Go 8 On chest x-ray film, the heartis shifted to the left hemithorax with a large pulmonary trunk. Go 9 The old diagnostic procedure of inflicting a left-sided pneumothorax to outline the pericardium Go 10 is now obsolete because of the availability of echocardiography, computed tomographic scanning, and nuclear magnetic resonance imaging. Go Go Go 9,11,12 CAP is associated with other cardiac and pulmonary anomalies in about 30% of the cases.

CAP should be suspected when myocardial perfusion scintigraphy shows abnormal cardiac position and LV orientation.

Footnotes

J THORAC CARDIOVASC SURG 1995;109:805-7 Back

References

  1. Saito R, Hotta F. Congenital pericardial defect associated with cardiac incarceration: case report. Am Heart J 1980;100:866-70.[Medline]
  2. Ellis K, Leeds NE, Himmelstein A. Congenital deficiencies in parietal pericardium: a review of two new cases including successful diagnosis by plain roentgenography. Am J Roentgenol 1959;82:125-37.
  3. Southworth H, Stevenson CS. Congenital defects of the pericardium. Arch Intern Med 1938;61:223-9.[Abstract/Free Full Text]
  4. Pernot C, Hoeffel JC, Frisch HR, Brauer B. Partial left pericardial defect with herniation of the left atrial appendage. Thorax 1972;27:246-50.[Abstract/Free Full Text]
  5. Robin E, Ganguli SN, Fowler MS. Strangulation of the left atrial appendage through a congenital partial pericardial defect. Chest 1975;67:354-5.[Abstract/Free Full Text]
  6. Altman CA, Ettedgui JA, Wozney P, Beerman LB. Noninvasive diagnostic features of partial absence of the pericardium. Am J Cardiol 1989;63:1536-7.[Medline]
  7. Glancy DL, Sanders CV, Porta A. Posterior chest wall pulsation in congenital complete absence of the left pericardium. Chest 1974;65:564-6.[Abstract/Free Full Text]
  8. Kansal S, Roitman D, Sheffield LT. Two-dimensional echocardiography of congenital absence of pericardium. Am Heart J 1985;109:912-5.[Medline]
  9. Schiavone WA, O'Donnell JK. Congenital absence of the left portion of parietal pericardium demonstrated by nuclear magnetic resonance imaging. Am J Cardiol 1985;55:1439-40.[Medline]
  10. Glover LB, Barcia A, Reeves TJ. Congenital absence of the pericardium: a review of the literature with demonstration of a previously unreported fluoroscopic finding. Am J Roentgenol 1969;106:542-9.[Abstract/Free Full Text]
  11. Nasser WK, Helman C, Tavel ME, Feigenbaum H, Fisch C. Congenital absence of the left pericardium: clinical, electrocardiographic, radiographic, hemodynamic and angiographic findings in six cases. Circulation 1970;41:469-78.[Abstract/Free Full Text]
  12. Burrows PE, Smallhorn JS, Trusler GA, Daneman A, Moes CAF, Rowe RD. Partial absence of the left parietal pericardium with herniation of the left atrial appendage: diagnosis by cross-sectional echocardiography and contrast-enhanced computed tomography. Pediatr Cardiol 1987;8:205-8.[Medline]




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