Pleuroperitoneal Shunts for Refractory Chylothorax After Operation for Congenital Heart Disease Karen S. Rheuban, MD, Irving L. Kron, MD, Martha A. Carpenter, MD, Howard P. Gutgesell, MD, and Bradley M. Rodgers, MD Departments of Pediatrics and Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia
Between 1980 and 1990,lO of 12 children With a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitaneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal
shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.
P
after operation for congenital heart disease [5-71. Other investigators have questioned the effectiveness of pleuroperitoneal shunting in this subset of patients, who may have elevated right atrial pressures during the postoperative period [8]. Concern has been raised that elevated right atrial pressures transmitted to the peritoneal venous and lymphatic bed may impair absorption of shunted chyle. We report the successful use of the pleuroperitoneal shunt in patients after operation for congenital heart disease, even in the face of moderate elevations in right atrial pressure.
ersistent chylothorax is a recognized complication of operation for congenital heart disease and may occur secondary to trauma to the thoracic duct, caval obstruction, or elevated central venous pressures. Medical therapy (low-fat diet, supplementation with medium-chain triglycerides, intravenous alimentation, tube thoracostomy) has a high failure rate (>75%) and may be accompanied by respiratory compromise, lymphopenia, and protein deficiency [l,21. Reexpansion of the lung on the affected side serves both to relieve symptoms of respiratory distress and to tamponade the thoracic duct [l].Late reaccumulation of chylous effusions may occur, and prolonged hospitalization (often exceeding 6 weeks) may be necessary before successful resolution of the chylothorax. Traditional therapy for chylothorax that is refractory to tube drainage has included pleurodesis and thoracotomy with ligation of the thoracic duct. Anatomic variants limit the success of thoracic duct ligation in many cases, and reaccumulation of chylous effusions may occur after apparently successful thoracic duct ligation [3]. In 1982, Weese and Schouten [4] introduced the use of the pleuroperitoneal shunt as treatment for malignant pleural effusions in adults. Since 1983, we have used pleuroperitoneal shunts as surgical treatment for refractory chylothorax in children, and have previously reported our preliminary experience with this technique, including 6 children with chylothorax Accepted for publication July 17, 1991. Presented at the Fortieth Annual Scientific Session of the American College of Cardiology, Atlanta, GA, March 5, 1991. Address reprint requests to Dr Rheuban, Department of Pediatrics, University of Virginia Health Sciences Center, Box 386, Charlottesville, VA 22908.
0 1992 by The Society of Thoracic Surgeons
(Ann Thoruc Surg 1992;53:85-7)
Material and Methods We reviewed the records of 879 patients with congenital heart disease who underwent cardiac operation at the University of Virginia between June 1980 and June 1990. During this period of time, a symptomatic chylothorax developed in 12 patients (1.3%)after operation for congenital heart disease. The diagnosis of chylothorax was made when a pleural effusion developed that was milky in appearance with an elevated triglyceride level or a lymphocyte coutlt greater than 90%, or if a serous pleural effusion with the same chemical and cellular characteristics developed in a nonfed patient. Age; diagnosis; operative procedure; volume, onset, and duration of pleural drainage; and right atrial pressure at cardiac catheterizatiotl before pletlroperitoneal shunting are indicated in Table 1. In all patients, respiratory compromise was initially treated with tube thoracostomy. Nutritional support was maintained by intravenous alimentation or a low fat diet. When pleural drainage failed to diminish after a period of at least 10 days, patients underwent placement of a pleuroperitoneal shunt. OO03-4975/92/$3.50
86
RHEUBAN ET AL PLEUROl'ERlTONEAL SHUNTS
Ann Thorac Surg 1992;53:857
Table 1 . Characteristics of Patients Requiring Pleuroperitoneal Shunts Patient No. 1 2
3 4 5 6 7
8 9 10
RA Onset of Effusion Daily Volume Pressure Days to Days to Months to Operation Age (mo) (days postop) (mag) (mm Hg) Shunt Discharge Removal
Diagnosis d-TGA Pulmonary atresia AV canal Single ventricle Pulmonary atresia d-TGA Tetralogy, residual VSD d-TGA d-TGA d-TGA
Median AV = atrioventricular;
9
29
...
4
10
...
9 42
2
22 23
...
1
Fontan
17
43
27
Senning Repair
12 19
3 2
17 90
Senning Senning Revision of Mustard
8 18 5
3 6 1
10.5
3.5
19.5
Senning Central shunt Repair Fontan
8 0.25
d-TGA = dextrotransposition of the great arteries;
Results A symptomatic chylothorax developed in 12 patients 1 to 43 days (median, 3.5 days) postoperatively. A chylous pleural and pericardial effusion did not develop in 1 patient (patient 5) until 6 weeks after an uncomplicated Fontan operation. Medical therapy was successful in 2 patients (17%)within 2 weeks of the onset of the effusion. Chylous effusions necessitating surgical therapy persisted in 10 children (83%),who subsequently underwent placement of a pleuroperitoneal shunt 11 to 57 days (median, 19.5 days) after the onset of the effusion and 14 to 64 days (median, 30.5 days) after the initial operation. The procedure was successful in draining the pleural effusion in 9 of 10 patients (90%) but failed to relieve the effusion in 1 patient who had undergone repair of tetralogy of Fallot 57 days before shunt placement. This patient's right atrial pressure was 25 mm Hg at postoperative cardiac catheterization. He had a large residual ventricular septal defect and ultimately died of sepsis and low cardiac output after reclosure of his ventricular septal defect. Of the 9 surviving patients, right atrial pressure measurements were documented at postoperative cardiac catheterization in 4 patients and ranged from 10 to 16 mm Hg (median, 13.5 mm Hg). Of the 5 patients with transposition of the great arteries, cardiac catheterization was performed in 3 and showed mild baffle obstruction (mean gradient from superior vena cava to right atrium was 4 mm Hg). Discharge from the hospital occurred 6 to 49 days (median, 15 days) after placement of the pleuroperitoneal shunt. The frequency of manual pumping was gradually decreased over a period of months, and in 8 of the 9 successful cases, the shunt was removed electively
11 11
11 41
15 13
20 14
8 7
21
6
4
16 25
18 57
49 Died
...
6
...
16 13
10 12
28 31 27
15 15 10
3 10 4
15
19.5
15
7
RA = right atrial;
15
5 Died
9
VSD = ventricular septd defect.
2.5 to 10 months (median, 7 months) after placement without reaccumulation of effusion. The 1 patient whose shunt was not removed died of extreme hypoxemia secondary to pulmonary artery hypoplasia 4 months after the central shunt and 1 month after placement of a modified Blalock-Taussigshunt. She had no pleural effusion before her death, and the shunt had not been pumped in weeks.
Comment Pleuroperitoneal shunting offers an effective means of reducing respiratory symptoms in patients with chylothorax after operation for congenital heart disease while preventing the extreme protein, lipid, and lymphocyte depletion that accompanies tube thoracostomy. The only hemodynamic contraindication is a markedly elevated right atrial pressure. Pleuroperitoneal shunting was effective in 4 patients with a moderately elevated right atrial pressure but not in 1 patient with a right atrial pressure of 25 mm Hg. We postulate, as have others, that markedly elevated right atrial pressures transmitted to the venous and lymphatic bed of the peritoneal space may impair absorption of shunted pleural fluid [8]. Pleuroperitoneal shunting is not a substitute for postoperative hemodynamic investigation or, if necessary, reoperation. However, if there are no simple medical or surgical options, placement of a pleuroperitoneal shunt is preferable to thoracic duct ligation, pleurodesis, or prolonged chest tube drainage. We now recommend early intervention (after 7 to 10 days) in patients with serious chylous effusions that fail to regress with medical therapy.
Ann Thorac Surg 19!92;53857
References 1. Higgins CB, Mulder DG. Chylothorax after surgery for congenital heart disease. J Thorac Cardiovasc Surg 1971;61:411-8. 2. Puntis JWL, Roberts KD, Handy D. How should chylothorax be managed? Arch Dis Child 1987;62:593-6. 3. Robinson CLN. The management of chylothorax. Ann Thorac Surg 1985;39:90-5. 4. Weese JL, Schouten JT. Pleural peritoneal shunts for treatment of malignant pleural effusions. Surg Gynecol Obstet 1982;154: 391-2. 5. Azizkhan RG, Canfield J, Alford BA, Rodgers BM. Pleuroperi-
RHEUBAN ET AL PLEUROPERITONEAL SHUNTS
87
toneal shunts in the management of neonatal chylothorax. J Pediatr Surg 1983;18:842-50. Milsom JW, Kron IL, Rheuban KS, Rodgers BM. Chylothorax: an assessment of current surgical management. J Thorac Cardiovasc Surg 1985;89221-7. Murphy MC, Newman BM, Rodgers BM. Pleuroperitoneal shunts in the management of persistent chylothorax. Ann Thorac Surg 1989;48:195-200. Sade RM, Wiles HB. Pleuroperitoneal shunt for persistent pleural drainage after Fontan procedure. J Thorac Cardiovasc Surg 1990;100:6213.
Notice From the American Board of Thoracic Surgery "l-te part 1 (written) exmination will be held at the WattTxt On FebruDallas Fort ary 7, 1993. The closing date for registration is August 1, 1992. To be for the part II examination, a candidate must have successfully completed the part I (written) examination.
A candidate applying for admission to the certifying examination must fulfill all the requirements of the board in force at the time the application is received. Please address all communications to the American Board of Thoracic SurgeVt One Rotary Center, Suite 803, Evanston, IL 60201.
Between 1980 and 1990,lO of 12 children With a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitaneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal
shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.
P
after operation for congenital heart disease [5-71. Other investigators have questioned the effectiveness of pleuroperitoneal shunting in this subset of patients, who may have elevated right atrial pressures during the postoperative period [8]. Concern has been raised that elevated right atrial pressures transmitted to the peritoneal venous and lymphatic bed may impair absorption of shunted chyle. We report the successful use of the pleuroperitoneal shunt in patients after operation for congenital heart disease, even in the face of moderate elevations in right atrial pressure.
ersistent chylothorax is a recognized complication of operation for congenital heart disease and may occur secondary to trauma to the thoracic duct, caval obstruction, or elevated central venous pressures. Medical therapy (low-fat diet, supplementation with medium-chain triglycerides, intravenous alimentation, tube thoracostomy) has a high failure rate (>75%) and may be accompanied by respiratory compromise, lymphopenia, and protein deficiency [l,21. Reexpansion of the lung on the affected side serves both to relieve symptoms of respiratory distress and to tamponade the thoracic duct [l].Late reaccumulation of chylous effusions may occur, and prolonged hospitalization (often exceeding 6 weeks) may be necessary before successful resolution of the chylothorax. Traditional therapy for chylothorax that is refractory to tube drainage has included pleurodesis and thoracotomy with ligation of the thoracic duct. Anatomic variants limit the success of thoracic duct ligation in many cases, and reaccumulation of chylous effusions may occur after apparently successful thoracic duct ligation [3]. In 1982, Weese and Schouten [4] introduced the use of the pleuroperitoneal shunt as treatment for malignant pleural effusions in adults. Since 1983, we have used pleuroperitoneal shunts as surgical treatment for refractory chylothorax in children, and have previously reported our preliminary experience with this technique, including 6 children with chylothorax Accepted for publication July 17, 1991. Presented at the Fortieth Annual Scientific Session of the American College of Cardiology, Atlanta, GA, March 5, 1991. Address reprint requests to Dr Rheuban, Department of Pediatrics, University of Virginia Health Sciences Center, Box 386, Charlottesville, VA 22908.
0 1992 by The Society of Thoracic Surgeons
(Ann Thoruc Surg 1992;53:85-7)
Material and Methods We reviewed the records of 879 patients with congenital heart disease who underwent cardiac operation at the University of Virginia between June 1980 and June 1990. During this period of time, a symptomatic chylothorax developed in 12 patients (1.3%)after operation for congenital heart disease. The diagnosis of chylothorax was made when a pleural effusion developed that was milky in appearance with an elevated triglyceride level or a lymphocyte coutlt greater than 90%, or if a serous pleural effusion with the same chemical and cellular characteristics developed in a nonfed patient. Age; diagnosis; operative procedure; volume, onset, and duration of pleural drainage; and right atrial pressure at cardiac catheterizatiotl before pletlroperitoneal shunting are indicated in Table 1. In all patients, respiratory compromise was initially treated with tube thoracostomy. Nutritional support was maintained by intravenous alimentation or a low fat diet. When pleural drainage failed to diminish after a period of at least 10 days, patients underwent placement of a pleuroperitoneal shunt. OO03-4975/92/$3.50
86
RHEUBAN ET AL PLEUROl'ERlTONEAL SHUNTS
Ann Thorac Surg 1992;53:857
Table 1 . Characteristics of Patients Requiring Pleuroperitoneal Shunts Patient No. 1 2
3 4 5 6 7
8 9 10
RA Onset of Effusion Daily Volume Pressure Days to Days to Months to Operation Age (mo) (days postop) (mag) (mm Hg) Shunt Discharge Removal
Diagnosis d-TGA Pulmonary atresia AV canal Single ventricle Pulmonary atresia d-TGA Tetralogy, residual VSD d-TGA d-TGA d-TGA
Median AV = atrioventricular;
9
29
...
4
10
...
9 42
2
22 23
...
1
Fontan
17
43
27
Senning Repair
12 19
3 2
17 90
Senning Senning Revision of Mustard
8 18 5
3 6 1
10.5
3.5
19.5
Senning Central shunt Repair Fontan
8 0.25
d-TGA = dextrotransposition of the great arteries;
Results A symptomatic chylothorax developed in 12 patients 1 to 43 days (median, 3.5 days) postoperatively. A chylous pleural and pericardial effusion did not develop in 1 patient (patient 5) until 6 weeks after an uncomplicated Fontan operation. Medical therapy was successful in 2 patients (17%)within 2 weeks of the onset of the effusion. Chylous effusions necessitating surgical therapy persisted in 10 children (83%),who subsequently underwent placement of a pleuroperitoneal shunt 11 to 57 days (median, 19.5 days) after the onset of the effusion and 14 to 64 days (median, 30.5 days) after the initial operation. The procedure was successful in draining the pleural effusion in 9 of 10 patients (90%) but failed to relieve the effusion in 1 patient who had undergone repair of tetralogy of Fallot 57 days before shunt placement. This patient's right atrial pressure was 25 mm Hg at postoperative cardiac catheterization. He had a large residual ventricular septal defect and ultimately died of sepsis and low cardiac output after reclosure of his ventricular septal defect. Of the 9 surviving patients, right atrial pressure measurements were documented at postoperative cardiac catheterization in 4 patients and ranged from 10 to 16 mm Hg (median, 13.5 mm Hg). Of the 5 patients with transposition of the great arteries, cardiac catheterization was performed in 3 and showed mild baffle obstruction (mean gradient from superior vena cava to right atrium was 4 mm Hg). Discharge from the hospital occurred 6 to 49 days (median, 15 days) after placement of the pleuroperitoneal shunt. The frequency of manual pumping was gradually decreased over a period of months, and in 8 of the 9 successful cases, the shunt was removed electively
11 11
11 41
15 13
20 14
8 7
21
6
4
16 25
18 57
49 Died
...
6
...
16 13
10 12
28 31 27
15 15 10
3 10 4
15
19.5
15
7
RA = right atrial;
15
5 Died
9
VSD = ventricular septd defect.
2.5 to 10 months (median, 7 months) after placement without reaccumulation of effusion. The 1 patient whose shunt was not removed died of extreme hypoxemia secondary to pulmonary artery hypoplasia 4 months after the central shunt and 1 month after placement of a modified Blalock-Taussigshunt. She had no pleural effusion before her death, and the shunt had not been pumped in weeks.
Comment Pleuroperitoneal shunting offers an effective means of reducing respiratory symptoms in patients with chylothorax after operation for congenital heart disease while preventing the extreme protein, lipid, and lymphocyte depletion that accompanies tube thoracostomy. The only hemodynamic contraindication is a markedly elevated right atrial pressure. Pleuroperitoneal shunting was effective in 4 patients with a moderately elevated right atrial pressure but not in 1 patient with a right atrial pressure of 25 mm Hg. We postulate, as have others, that markedly elevated right atrial pressures transmitted to the venous and lymphatic bed of the peritoneal space may impair absorption of shunted pleural fluid [8]. Pleuroperitoneal shunting is not a substitute for postoperative hemodynamic investigation or, if necessary, reoperation. However, if there are no simple medical or surgical options, placement of a pleuroperitoneal shunt is preferable to thoracic duct ligation, pleurodesis, or prolonged chest tube drainage. We now recommend early intervention (after 7 to 10 days) in patients with serious chylous effusions that fail to regress with medical therapy.
Ann Thorac Surg 19!92;53857
References 1. Higgins CB, Mulder DG. Chylothorax after surgery for congenital heart disease. J Thorac Cardiovasc Surg 1971;61:411-8. 2. Puntis JWL, Roberts KD, Handy D. How should chylothorax be managed? Arch Dis Child 1987;62:593-6. 3. Robinson CLN. The management of chylothorax. Ann Thorac Surg 1985;39:90-5. 4. Weese JL, Schouten JT. Pleural peritoneal shunts for treatment of malignant pleural effusions. Surg Gynecol Obstet 1982;154: 391-2. 5. Azizkhan RG, Canfield J, Alford BA, Rodgers BM. Pleuroperi-
RHEUBAN ET AL PLEUROPERITONEAL SHUNTS
87
toneal shunts in the management of neonatal chylothorax. J Pediatr Surg 1983;18:842-50. Milsom JW, Kron IL, Rheuban KS, Rodgers BM. Chylothorax: an assessment of current surgical management. J Thorac Cardiovasc Surg 1985;89221-7. Murphy MC, Newman BM, Rodgers BM. Pleuroperitoneal shunts in the management of persistent chylothorax. Ann Thorac Surg 1989;48:195-200. Sade RM, Wiles HB. Pleuroperitoneal shunt for persistent pleural drainage after Fontan procedure. J Thorac Cardiovasc Surg 1990;100:6213.
Notice From the American Board of Thoracic Surgery "l-te part 1 (written) exmination will be held at the WattTxt On FebruDallas Fort ary 7, 1993. The closing date for registration is August 1, 1992. To be for the part II examination, a candidate must have successfully completed the part I (written) examination.
A candidate applying for admission to the certifying examination must fulfill all the requirements of the board in force at the time the application is received. Please address all communications to the American Board of Thoracic SurgeVt One Rotary Center, Suite 803, Evanston, IL 60201.
