Ann Thorac Surg 2005;79:e7-e8
© 2005 The Society of Thoracic Surgeons
Case report
Hemoptysis From Collateral Arteries 12 Years After a Fontan-Type Operation
Kenji Suda, MDa,*,
Masahiko Matsumura, MDa,
Akira Sano, MDb,
Shinichiro Yoshimura, MDc,
Tetsuko Ishii, MDd
a Division of Pediatric Cardiology, Department of Pediatrics, Tenri, Japan
b Department of Radiology, Tenri Hospital, Tenri, Japan
c Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
d Department of Pediatric Cardiology, Tokyo Women's Medical University, Tokyo, Japan
Accepted for publication August 10, 2004.
* Address reprint requests to Dr Suda, Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-Machi, Kurume City, 830-0011, Japan; (E-mail: kensuda{at}tenriyorozu-hp.or.jp).
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Abstract
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A 20-year-old patient who had a single ventricle and pulmonary stenosis presented with recurrent hemoptysis 12 years after a Fontan-type operation. He was referred to us because of unsuccessful treatment with a tentative diagnosis of lung tuberculosis for 3 months. He had been relatively well for the prior 12 years, although he had attacks of paroxysmal supraventricular tachycardia and had underwent successful catheter ablation 3 years ago. Aortography revealed multiple collateral arteries as a cause of hemoptysis and coil embolization of these collateral arteries successfully stopped the hemoptysis. This case illustrates that collateral arteries may stay open or develop, and these collateral arteries can become a source of hemoptysis long after a Fontan-type operation.
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Introduction
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Many cyanotic patients with single ventricular physiology have been palliated by a Fontan-type operation. In these patients, multiple aortopulmonary collateral arteries frequently develop and can cause serious problem just after a Fontan-type operation [1, 2]. However, it is still uncommon to see late complications of these collateral arteries long after a Fontan-type operation. We describe a patient who suffered from hemoptysis due to aortopulmonary collateral arteries 12 years after a Fontan-type operation.
A 20-year-old patient visited our hospital because of recurrent hemoptysis despite 3 months of unsuccessful treatment with anti-tuberculous medication in a local hospital. He did have a positive sputum test with Gaffky scale class 2, although it was found to be false positive later on.
His medical history included two surgical interventions and one catheter intervention. Soon after birth he was noted to have cyanosis and at the age of 2 he underwent cardiac catheterization that revealed a left-sided inferior caval vein, right-sided left atrium, single right ventricle with rudimentary chamber, complete atrioventricular septal defect, malposition of the great arteries, and pulmonary stenosis (asplenia syndrome with isomeric right appendages), and he underwent a modified left Blalock-Taussig shunt. At 5 years of age, he underwent a second cardiac catheterization that revealed a well developed pulmonary vasculature (pulmonary arterial index = 301 mm/M2), low pulmonary vascular resistance (1.2 Wood U·M2), but relatively high pulmonary arterial pressure (mean pulmonary pressure = 20 mm Hg), and his parents did not want him to have a Fontan-type operation at that time. However, repeat cardiac catheterization at 6 years of age revealed a decreased mean pulmonary arterial pressure of 12 mm Hg, good pulmonary vasculature, and a low pulmonary vascular resistance. He underwent a Fontan-type operation at 8 years of age. Operative procedure included oblique partition of the right-sided left atrium, direct anastomosis of the right-sided atrial appendage to the right pulmonary artery, and direct anastomosis of the left pulmonary artery to the left superior vena cava. Although he was extubated the next day, his postoperative course was complicated by 24 days of left pleural effusion and chylothorax that was successfully treated by diuretics and diet therapy. Cardiac catheterization 35 days after this operation revealed an acceptably low mean pulmonary arterial pressure of 17 mm Hg and an arterial oxygen saturation of 93%. He was relatively well for 2 years until he had paroxysmal supraventricular tachycardia that was controlled by digoxin, disopyramide, and warfarin. The tachycardia had a heart rate of 220 to 240 beats per minute and occurred once every several months and usually lasted < 2 hours. Although it induced hypoperfusion indicated by a pale face, it did not induce syncope and was sometimes stopped by breath holding. Ten years after the Fontan-type operation, this paroxysmal supraventricular tachycardia had become more frequent (once a day for 2 to 3 minutes), and he underwent successful catheter radiofrequency ablation for this arrhythmia. Since then he had been well for 3 years until he had hemoptysis develop.
When he presented to us, he had suffered from seven episodes of recurrent hemoptysis, and all of them were documented in the right lower lobe on chest roentgenogram (Fig 1). According to his specific medical history, we assumed that this hemoptysis was caused by the remaining aortopulmonary collateral arteries and brought him to the catheterization laboratory. Indeed, an angiogram showed major aortopulmonary collateral arteries supplying the right lower lobe (Fig 2). He underwent successful coil embolization of these collateral arteries, and the hemoptysis stopped. After coil embolization he remained well without hemoptysis for 2 years. The last cardiac catheterization 13 years after a Fontan-type operation revealed an acceptably low mean pulmonary pressure of 12 mm Hg and a high arterial oxygen saturation of 96%.
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Fig 2. Aortography showed multiple aortopulmonary collateral arteries (arrows) supplying the right lower lobe as a cause of recurrent hemoptysis 12 years after a Fontan-type operation. Note the right pulmonary artery (arrowhead) visualized by collateral arteries.
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Comment
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Our patient illustrates that even long after a Fontan-type operation, patients who had cyanotic congenital heart disease have a risk of hemoptysis from aortopulmonary collateral arteries. Although it has been reported that aortopulmonary collateral arteries lead to perioperative problems of a Fontan-type operation, such as heart failure or prolonged pleural effusion [1, 2], it is very rare to see these collateral arteries lead to hemoptysis long after the operation.
Little is known about the fate of these collateral arteries after a Fontan-type operation. Although small collateral arteries must have regressed with improved oxygenation as in patients with bronchopulmonary dysplasia [3], it is questionable whether certain sized collateral arteries regress with time. In this patient, the fact that his postoperative course was complicated by prolonged pleural effusion suggests that he probably had these collateral arteries at the time of the Fontan-type operation. On the other hand, supraventricular tachycardia seen in this specific patient might have some impact on the development of these collateral arteries after a Fontan-type operation. More detailed studies on the fate and development of collateral arteries long after a Fontan-type operation are required.
Although there is still controversy about whether we should universally close aortopulmonary collateral arteries before a Fontan-type operation [4], we certainly need to keep in mind that these aortopulmonary collateral arteries can cause hemoptysis long after a Fontan-type operation.
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References
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- Kanter KR, Vincent RN, Raviele AA. Importance of acquired systemic-to-pulmonary collaterals in the Fontan operation Ann Thorac Surg 1999;68:969-974.[Abstract/Free Full Text]
- Kaulitz R, Ziemer G, Paul T, Peuster M, Bertram H, Hausdorf G. Fontan-type procedures: residual lesions and late interventions Ann Thorac Surg 2002;74(3):778-785.[Abstract/Free Full Text]
- Acherman RJ, Siassi B, Pratti-Madrid G, et al. Systemic to pulmonary collaterals in very low birth weight infants: color Doppler detection of systemic to pulmonary connections during neonatal and early infancy period Pediatrics 2000;105:528-532.[Abstract/Free Full Text]
- Bradley SM. Management of aortopulmonary collateral arteries in Fontan patients: routine occlusion is not warranted Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:55-67.[Medline]
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Abstract
Introduction
