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Ann Thorac Surg 2001;72:193-196
© 2001 The Society of Thoracic Surgeons

Original article: cardiovascular

Chylopericardium after cardiac operations in children

Rosalie M. Campbell, MB, BChb, Lee N. Benson, MDa,c, William W. Williams, MDa,d, Ian Adatia, MB, ChBa,b,c a Division of Cardiology and Cardiovascular Surgery, Toronto Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
b Department of Critical Care Medicine, Toronto Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
c Department of Paediatrics, Toronto Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
d Department of Surgery, Toronto Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada

Accepted for publication March 1, 2001.

Address reprint requests to Dr Adatia, Critical Care Medicine and Cardiology, Hospital For Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada
e-mail: ian.adatia{at}sickkids.on.ca


   Abstract
Top
 Abstract
Introduction
 Material and methods
 Results
 Comment
 References
 
Background. Chylopericardium is a rare complication after operation for congenital heart disease. The incidence and clinical outcomes in a large cohort of surgical patients are unknown.

Methods. We retrospectively reviewed the clinical records spanning more than 12 years in a single institution of 16 children with chylopericardium after cardiac operation.

Results. We identified 16 patients with chylopericardium between 1985 and 1997. Chylopericardium was isolated in 7 patients. Twelve patients required pericardial drainage. Patients with isolated chylopericardium presented late and were treated initially as having postpericardiotomy syndrome. Three patients underwent thoracic duct ligation. There were two late deaths unrelated to the chylothorax. Associated diagnoses were internal jugular vein thrombosis and recurrent pulmonary vein obstruction (1 of 16 patients), an associated syndrome but not Turner or Noonan (10 of 16), superior cavopulmonary or total cavopulmonary anastomosis (7 of 16), atrioventricular septal defect repair (5 of 16), and repair of tetralogy of Fallot (2 of 16).

Conclusions. Percutaneous drainage to relieve tamponade together with a low-fat or medium-chain triglyceride diet results in resolution in most cases of postoperative chylopericardium. If a pericardial effusion enlarges, fails to clear on aspirin therapy, or presents late after hospital discharge, diagnostic pericardial tap and a low-fat diet are indicated.


   Introduction
Top
 Abstract
 Introduction
Material and methods
 Results
 Comment
 References
 
Chylopericardium is a rare complication after operation for congenital heart disease. Although first described in 1966 [1], subsequent reports have been few and restricted to descriptions of one to three cases [1–6] The incidence in a large cohort of surgical patients is unknown. Therefore, we present our experience over 12 years of 16 children with symptomatic chylopericardium complicating recovery from cardiac operation.


   Material and methods
Top
 Abstract
 Introduction
 Material and methods
Results
 Comment
 References
 
We reviewed the records of all children undergoing pericardiocentesis for symptomatic or diagnostic evaluation of pericardial effusion, as well as those with chylous pleural effusion after cardiac operations. We diagnosed chylopericardium if the aspirate was milky and sterile with a high yield of chylomicrons, triglycerides, and lymphocytes on biochemical analysis. If pericardiocentesis was not performed, chylopericardium was assumed if there was a concomitant chylothorax with pleural drainage of chyle and the pericardial effusion diminished with institution of a low-fat diet or a medium-chain triglyceride formula. Pericardial effusion was suspected clinically or from increased cardiac silhouette on chest roentgenogram with echocardiographic confirmation in all cases. Percutaneous pericardiocentesis was performed as reported previously [7].


   Results
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 Abstract
 Introduction
 Material and methods
 Results
Comment
 References
 
We identified 16 patients (7 boys and 9 girls) aged between 3 months and 7 years (median 10 months) with chylopericardium between 1985 and 1997 (Table 1). The incidence of chylopericardium after cardiac operation was 0.22% during the 12 years. Chylopericardium accounted for 15% of symptomatic postoperative pericardial effusions and 15% of patients with an associated chylous pleural effusion.


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  		Table 1. Postoperative Chylopericardium Details

 
Symptomatic presentation occurred a median of 23 days after cardiac operations (range 7 days to 8 weeks). Seven patients presented late, after initial hospital discharge, with pericardial tamponade. Patients with isolated chylopericardium (7 of 16 cases, not associated with a chylous pleural effusion) tended to present later (median 17 days versus 14.5 days). The diagnosis of chylopericardium was delayed in 8 patients until an empiric course of aspirin failed to clear the effusion.

Chylopericardium occurred frequently after superior cavopulmonary or total cavopulmonary anastomosis (7 of 16 patients), atrioventricular septal defect repair (5 of 16), and repair of tetralogy of Fallot (2 of 16). The median postoperative central venous mean pressure was 13 mm Hg (range 5 to 20 mm Hg). No patient had more than mild atrioventricular valve regurgitation and 1 patient (number 12) had tricuspid valve stenosis.

Ten of the 16 patients (63%) had an associated syndrome (7 with Trisomy 21, 1 with VATER syndrome (vertebral, anal, tracheal, esophageal, and renal anomalies), 1 with Goldenhar syndrome, and 1 with chromosome 22q-). No patient had Noonan or Turner syndrome [8]. One patient (number 7) had internal jugular vein thrombosis and recurrent pulmonary vein stenosis, which were considered contributory to the prolonged chylous drainage.

Twelve percutaneous pericardiocenteses were performed in 11 patients. The initial tap yielded a median fluid volume of 12 mL/kg (range 6 to 23 mL/kg), and drainage continued for a median of 7 days (range 2 to 49 days). Surgical drainage with creation of a pericardial window was required twice: in 1 patient as an emergency for impending cardiac tamponade before percutaneous pericardiocentesis was performed routinely and in a second patient, after relief of tamponade by percutaneous pericardiocentesis, at the time of thoracic duct ligation for chronic chylothorax. Four patients did not require pericardial drainage as the effusion regressed with pleural drainage and dietary therapy.

On diagnosis, treatment was dietary with either a medium-chain triglyceride formula or a low-fat diet for 6 weeks. Three patients underwent thoracic duct ligation: 2 for chronic pleural drainage and 1 for chronic chylous pleuropericardial drainage (patient 7 in Table 1). Chylous drainage persisted for 35, 25, and 3 days after thoracic duct ligation in patients 7, 9, and 15 in Table 1.

The lowest lymphocyte count was 1.59 x 109 (median) with a range of 5.44 x 109 to 0.12 x 109. Three patients had serious infections with positive blood cultures and lymphocyte counts of 1.26 x 109, 0.12 x 109, and 1.94 x 109, respectively (patients 1, 7, and 15).

Median length of stay in hospital was 31 days (range 13 to 92 days) and the median weight loss (calculated as (preoperative weight - lowest weight)/preoperative weight) was 4.0% (range 0% to 15.1%).

All patients survived initial hospitalization and were discharged home with two late deaths unrelated to the chylothorax.


   Comment
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
References
 
Thus far, there are 23 cases of chylopericardium, reported in 19 publications, complicating cardiac operation in children [2, 3, 9, 10]. Our report documents 16 cases of chylopericardium in children after cardiac operation, representing an incidence of 0.22% over 12 years. Although chylopericardium remains a rare complication of cardiac operation, review of our cases illustrates useful points in the diagnosis and management.

Refractory cases of chylopericardium, in contrast to chylous pleural drainage, were unusual in our series, with only 1 patient requiring thoracic duct ligation for prolonged pericardial drainage, although 2 patients underwent thoracic duct ligation for associated persistent chylous pleural drainage. Our data suggest that chylopericardium can be managed successfully by percutaneous pericardiocentesis or a low-fat or medium-chain triglyceride diet in most cases. However, treatment of patients with prolonged drainage by pericardial-peritoneal shunting has been described [11]. Alternative treatment regimes include discontinuing enteral feeds and instituting total parenteral nutrition.

Prolonged drainage has been associated with persistently elevated pulmonary venous or upper limb systemic venous pressure. As others have noted, thrombus in the upper body great veins may be responsible for persistent drainage by either direct obstruction of the thoracic duct [12] or elevated systemic venous pressure transmitted back into the lymphatic channels [5].

Isolated chylopericardium is difficult to diagnose and may be mistaken for the postpericardiotomy syndrome, with further delay in diagnosis while a trial of aspirin therapy is instituted. Indeed, we treated 8 patients with aspirin until pericardiocentesis or the appearance of pleural chyle defined the diagnostic error. Late presentation after hospital discharge occurred in 43% of cases and would support the prudence of early echocardiography for at-risk patients.

The etiology of the chylous collection in the pericardial space after operations to correct congenital heart disease is controversial especially when it complicates extrapericardial operation. A rich double layer of lymphatics surround the pericardial space, with a high turnover of fluid from within the pericardial sac, and there is a rich network of lymphatic interconnections [13]. The association with elevated systemic venous pressures [14] suggests that back pressure sufficient to overwhelm the lymphatic valves within the major mediastinal lymphatics may be transmitted to smaller lymphatics within the pericardial space that may have been transected during operation and result in chyle accumulation. The marked variation in the terminal portion of the thoracic duct predisposes it to injury during mediastinal or thoracic surgical procedures and, together with inadvertent pericardial opening, may be an additional explanation for chylopericardium after extrapericardial operation as seen in one case in this series and noted by others [15].

As far as we are aware, chylopericardium has not been associated particularly with VATER, Goldenhar, or CATCH 22 (Cardiac defects, abnormal facies, thymus, cleft palate, hypocalcemia) syndromes. However, congenital chylothorax is more common in Trisomy 21 and increased lymphatic permeability due to congenital lymphatic dysplasia may explain our finding that 45% of patients with chylopericardium had Down syndrome. Chylothorax with lymphatic dysplasia is also a feature of Noonan and Turner syndromes [16].

In summary, we have described the clinical features and treatment of 16 patients with postoperative chylopericardium. Percutaneous drainage to relieve tamponade together with a low-fat or medium-chain triglyceride diet results in resolution in most cases. If a pericardial effusion enlarges, fails to clear on aspirin therapy, or presents late after hospital discharge, diagnostic pericardial tap is indicated to avoid confusion with the postpericardiotomy syndrome.


   References
Top
 Abstract
 Introduction
 Material and methods
 Results
 Comment
 References
 

  1. Louhimo I, Kekomäki M, Pasila M, Sulamaa H. Chylothorax and chylopericardium as a problem in pediatric surgery. Eur J Ped Surg 1966;Suppl:19–28.
  2. Rusca M., Spaggiari L., Carbognani P., Cattelani L., Medici D., Bobbio P. Late spontaneous chylopericardium following complex cardiac surgery. J Cardiovasc Surg 1995;36:175-176.[Medline]
  3. Denfield S.W., Rodriguez A., Miller-Hance W.C., et al. Management of postoperative chylopericardium in childhood. Am J Cardiol 1989;63:1416-1418.[Medline]
  4. Pugliese P., Santi C., Eufrate S. Isolated chylopericardium after successful correction of total anomalous pulmonary venous drainage. J Cardiovasc Surg 1984;25:75-77.[Medline]
  5. Thomas C.S., McGoon D.C. Isolated massive chylopericardium following cardiopulmonary bypass. J Thorac Cardiovasc Surg 1971;61:945-948.[Medline]
  6. Pollard W.M., Schuchmann G.F., Bowen T.E. Isolated chylopericardium after cardiac operations. J Thorac Cardiovasc Surg 1981;81:943-946.[Abstract]
  7. Zahn E.M., Houde C., Benson L., Freedom R.M. Percutaneous pericardial catheter drainage in childhood. Am J Cardiol 1992;70:678-680.[Medline]
  8. Chan D.K.L., Ho N.K. Noonan syndrome with spontaneous chylothorax at birth. Aust Paediatr J 1989;25:296-298.[Medline]
  9. Nguyen D.M., Shum-Tim D., Dobell A.R.C., Tchervenkov C.I. The management of chylothorax/chylopericardium following pediatric cardiac surgery: a 10- year experience. J Card Surg 1995;10:302-308.[Medline]
  10. Skarsgard E.D., Filler R.M., Superina R.A. Postpericardiotomy syndrome and chylopericardium: two unusual complications after aortopexy for tracheomalacia. J Pediatr Surg 1994;29:1534-1536.[Medline]
  11. Chan B.B.K., Murphy M.C., Rodgers B.M. Management of chylopericardium. J Pediatr Surg 1990;25:1185-1189.[Medline]
  12. Hinckley M.E. Thoracic-duct thrombosis with fatal chylothorax caused by a long venous catheter. N Engl J Med 1969;280:95-96.
  13. Eliskova M., Eliska O., Miller A.J. The lymphatic drainage of the parietal pericardium in man. Lymphology 1995;28:208-217.[Medline]
  14. Pedra S.R.F., Pedra C.A.C., Fontes V.F., Santana M.V.T., Paulista P.P. Chylothorax after surgery for congenital heart disease in children. Cardiol Young 1998;8:199-204.
  15. Greenfield J., Gottlieb M.I. Variations in the terminal portion of the human thoracic duct. Arch Surg 1956;73:955-959.[Abstract/Free Full Text]
  16. Lanning P., Similä S., Suramo I., Paavilainen T. Lymphatic abnormalities in Noonan’s syndrome. Pediatr Radiol 1978;7:106-109.[Medline]



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