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Ann Thorac Surg 1995;59:1570-1571
© 1995 The Society of Thoracic Surgeons

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Case Report

Heart Transplantation for Chyloptysis After Fontan Operation

Victorian Paediatric Cardiac Surgical Unit and Department of Cardiology, Royal Children's Hospital, Melbourne, Australia

Accepted for publication October 28, 1994.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
Chyloptysis is a rare clinical problem, fewer than 10 patients having been reported in the literature. We report a patient with intractable chyloptysis associated with a Fontan procedure, who was palliated by heart transplantation.

	Introduction

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Chyloptysis is a term coined by Kinmonth [1] to describe the expectoration of chyle. He postulated two possible mechanisms: retrograde flow of chyle through the bronchopulmonary lymphatics with fistula formation into the bronchial tree, and the coexistence of a bronchopleural fistula and a chylous pleural effusion. The elevated venous pressures typically seen after the Fontan procedure theoretically might favor retrograde flow of chyle.

Late Fontan failure can occur in a number of modes, including ventricular dysfunction, protein-losing enteropathy, arrhythmia, and increasing pulmonary vascular resistance. Transplantation may be the only therapeutic option (for otherwise suitable candidates). Herein, we report another indication for transplantation after Fontan operation: refractory chyloptysis, presumably caused by elevated systemic venous pressure.LINE SPACE

The patient was cyanosed from birth, with situs solitus, atrioventricular concordance, tricuspid stenosis, aorta from a small right ventricle, ventricular septal defect, and pulmonary atresia. She underwent neonatal balloon atrial septostomy followed by right modified Blalock-Taussig shunt, with good weight gain and normal development for 2 years. Subsequently, increased cyanosis and dyspnea on exertion developed.

At age 31 months, she underwent a modified Fontan procedure (Kreutzer). Her preoperative pulmonary artery and left atrial pressures were 8 and 6 mm Hg, respectively. Immediately after the procedure, the mean right atrial pressure was 12 to 15 mm Hg, the mean left atrial pressure 6 to 9 mm Hg, and the hemoglobin saturation 99%. Postoperatively she had transient atrioventricular dissociation. She was discharged on the eighth postoperative day on a regimen of diuretics.

She was readmitted three times for recurrent right pleural effusions in the following 18 months. Biochemical analysis of the fluid on each occasion showed low protein, cholesterol, and triglyceride levels, ruling out chylothorax. Twenty months postoperatively she had a right surgical pleurodesis, and thereafter had no further accumulation of pleural fluid.

She improved considerably after Fontan operation, but remained in a predominantly atrioventricular junctional rhythm, with a rate of 80 to 125 beats/min, alternating with periods of a slower sinus rhythm. Fifteen months after the Fontan repair fatigue on minimal effort, anorexia, and intermittent abdominal pain developed. A catheter study 19 months postoperatively showed retrograde P waves coincident with the QRS complex, with ``cannon'' waves of 14 mm Hg in the wedge trace. The corresponding ``a'' wave pressure in the right atrium was 24 mm Hg. Mean left atrial pressure was 10 mm Hg, and mean distal pulmonary arterial pressure was 16 mm Hg. There was no pressure gradient between the distal pulmonary arterial branches and the right atrium, and angiography showed a well-formed pulmonary arterial branching pattern. Ventriculography showed good systolic function and no atrioventricular valve regurgitation. Left ventricular end-diastolic pressure was 10 mm Hg. Sotalol therapy was begun 20 months postoperatively, with a drop in atrioventricular junctional rate, but no symptomatic improvement.

Beginning 29 months postoperatively, a chronic cough developed, productive of sterile, milky, tenacious sputum. Sputum production increased and microscopic examination showed numerous fat-laden macrophages as well as free fat droplets, consistent with the expectoration of chyle. Her anorexia and fatigue worsened, and her activities became restricted. An exercise-responsive atrial pacemaker was inserted 47 months postoperatively in conjunction with sotalol therapy, again without symptomatic improvement.

A repeat catheter study 50 months postoperatively showed mean left atrial pressure of 10 mm Hg, mean right atrial pressure of 20 mm Hg, cardiac index 1.2 L/min, and pulmonary resistance 8 Wood units falling to 4 Wood units with inhalation of nitric oxide and 60% oxygen. Ventricular systolic function remained good. Because of her New York Heart Association functional class III–IV symptoms and the unavailability of any other therapeutic option, she was placed on the heart transplant list.

Orthotopic heart transplantation was performed 52 months after the Fontan operation. Upon reopening of the sternum, there was copious drainage of lymph from the entire upper mediastinum, which ceased during cardiopulmonary bypass and after implantation of the donor heart. Postoperatively, the patient was maintained on cyclosporin A and azathioprine. During the first few weeks she still had a cough productive of thick white sputum, causing intermittent left lung collapse. She required three bronchoscopic examinations within the first 10 days to aid lung reexpansion. At each examination, thick, adherent bronchial casts were aspirated. Histologic sections of the aspirated material showed a fibrinous exudate containing lymphocytes, suggestive of chyle. This was confirmed by lipid staining, which showed large collections of fat droplets within macrophages, as well as abundant free neutral fat. She was placed on a medium-chain triglyceride diet for 6 weeks, with resolution of the problem.

At recent follow-up, 6 months after transplantation, she remains well, has a normal exercise tolerance, has been eating a normal diet for 4 months, and has had no further chyloptysis.

	Comment

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Chyloptysis is a rare clinical condition, protean in its manifestations. Some patients cough up large amounts of sputum containing bronchial casts, whereas others experience nocturnal dyspnea and an asthma-like picture. The wheezing in such patients is related to the partial obstruction of smaller airways by plugs of chylous material. The casts seen in this syndrome are quite distinctive, being yellowish and containing large amounts of free neutral fat and fat-laden macrophages [2]. Chylous reflux into the lungs usually is associated with concurrent reflux into the pleura, causing a chylothorax. In this situation, the chylothorax focuses attention on an abnormal lymph flow pattern [3]. Pulmonary interstitial fluid normally enters the lymphatic vessels at the level of the alveolar ducts. It drains centrally toward the hila, peripherally over the surface of the lungs into the interlobar lymphatics, and finally into the bronchomediastinal trunks that empty into the subclavian and internal jugular veins, or into the thoracic duct. Lymphatic valves regulate the flow unidirectionally. Therefore, under normal conditions the lymphatic vessels in the lung do not contain chyle [4].

In our patient, increased venous pressure in the context of a ``failing'' Fontan circulation may have rendered the valves incompetent. Seepage of chyle into the alveolar spaces could have caused bronchial cast formation [5]. Although injury of major lymphatic channels at the time of the original Fontan procedure is another possible explanation, we do not believe this to be the case as our patient did not have chylothorax or chyloptysis in the period immediately after the Fontan operation.

Increased venous pressure was shown by Blalock and colleagues [6] to cause chylothorax in experimental animal models. In our patient, both the chronically elevated venous pressure and the low cardiac output were likely to have been causative factors. In humans, chylothorax has been reported as a complication of thrombosis of the superior vena cava or its major tributaries [7], and as a consequence of baffle obstruction after Mustard repair for transposition of the great arteries [8, 9]. In this latter context, Syamasundar and Wilson [8] have described its successful treatment by balloon angioplasty. Without relief of the venous obstruction, the lungs themselves become lymphangiectatic and this contributes to the long-term morbidity [10].

In summary, we present a case of chyloptysis after Fontan operation that was treated successfully by heart transplantation.

	Footnotes

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Address reprint requests to Dr Karl, Victorian Paediatric Cardiac Surgical Unit, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3052, Australia.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Kinmonth JS. The lymphatics: surgery, lymphography and diseases of the chyle and lymph systems. 2nd ed. Baltimore: Edward Arnold, 1982:251–60.
2. Davies GM. Lymph casts of the bronchi. Br J Dis Chest 1967;61:45–9.[Medline]
3. Maier HC. Chylous reflux in the lungs and pleura. Thorax 1968;23:281–96.[Abstract/Free Full Text]
4. Valentine VG, Raffin TA. The management of chylothorax. Chest 1992;102:586–91.[Free Full Text]
5. Sanders JS, Rosenow EC III, Piehler JM, Gloviczki P, Brown LR. Chyloptysis (chylous sputum) due to thoracic lymphangiectasis with successful surgical correction. Arch Intern Med 1988;148:1465–6.[Abstract/Free Full Text]
6. Blalock A, Cunningham RS, Robinson CS. Experimental production of chylothorax by occlusion of the superior vena cava. Ann Surg 1936;104:359–64.[Medline]
7. Kramer SS, Taylor GA, Garfinkel DJ, Simmons MA. Lethal chylothoraces due to superior caval thrombosis in infants. AJR 1981;137:559–63.[Abstract/Free Full Text]
8. Syamasundar PR, Wilson AD. Chylothorax, an unusual complication of baffle obstruction following Mustard operation: successful treatment with balloon angioplasty. Am Heart J 1992;125:244–8.
9. Cumming GR, Ferguson CC. Obstruction of superior vena cava after the Mustard procedure for TGA conservative management of chylothorax. J Thorac Cardiovasc Surg 1975;70:242–7.[Abstract]
10. Warren WH, Altman JS, Gregory SA. Chylothorax secondary to the obstruction of the superior vena cava: a complication of Le Veen shunt. Thorax 1990;45:978–9.[Abstract/Free Full Text]

This article has been cited by other articles:

				K. G. Lim, E. C. Rosenow III, B. Staats, C. Couture, and T. I. Morgenthaler Chyloptysis in Adults: Presentation, Recognition, and Differential Diagnosis Chest, January 1, 2004; 125(1): 336 - 340. [Abstract] [Full Text] [PDF]

This Article Abstract 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): Tom R. Karl Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Robotin, M. C. Articles by Karl, T. R. Search for Related Content PubMed PubMed Citation Articles by Robotin, M. C. Articles by Karl, T. R.