Pediatr Cardiol 12:186-188, 1991
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991
Successful Modified Left Pneumonectomy
Fontan
Procedure
in a n A d o l e s c e n t
After
Delores Danilowicz, Alan Langsner, and Stephen B. Colvin D e p a r t m e n t s o f Pediatrics and Cardiovascular Surgery, N e w York University Medical Center, N e w York, N e w York, U S A
S U M M A R Y . We performed a Waterston's anastomosis on a patient with complicated tricuspid atresia when she was two months o f age. At age 14 years she required left p n e u m o n e c t o m y for massive, idiopathic hemoptysis. Four m o n t h s after the p n e u m o n e c t o m y we substituted a modified Fontan anastomosis for the Waterston anastomosis. Restudy when she was 17 years of age s h o w e d continued satisfactory function. K E Y W O R D S : Tricuspid atresia - - Fontan procedure - - Waterston anastomosis - - H e m o p t y sis - - P n e u m o n e c t o m y
The Fontan procedure and modifications thereof have been used since the early 1970s as a definitive procedure for patients with tricupsid atresia, other forms of hypoplastic right heart syndrome, and various types of single ventricle [1, 3-5]. Original guidelines for the operation included age, anatomy, physiology, prior surgery, etc. [2]. As the procedure was increasingly done, many of these initial guidelines were found to be less than absolute. At the present time, the major factors which appear to influence the results of the Fontan procedure include the following: size and integrity of the pulmonary vascular bed, pulmonary vascular resistance, ventricular function, and atrioventricular valve function. We present herein an adolescent who had a modified Fontan performed using the right pulmonary artery following a left pneumonectomy for severe hemoptysis.
Case Report A 17-year-old black w o m a n was restudied 3 years after placem e n t of a modified F o n t a n a n a s t o m o s i s b e t w e e n the superior v e n a caval/right atrial j u n c t i o n and the right p u l m o n a r y artery. She was noted to be cyanotic at birth and catheterization revealed tricupsid atresia, severe p u l m o n a r y stenosis, and both atrial and ventricular septal defects. A W a t e r s t o n a n a s t o m o s i s
Address offprint requests to: Delores Danilowicz, Tisch University Hospital, R o o m 576, 560 First A v e n u e , N e w York, N Y 10016, U S A .
was performed at the age of 2 m o n t h s with a good rise in oxygen saturation and moderate congestive heart failure. Subacute bacterial endocarditis occurred on two occasions at 9 and 10 years of age, both successfully treated without clinical deterioration. A positive tuberculin c o n v e r s i o n was treated with isoniazid for 1 year. By the age of 14 years, she was moderately limited in physical activity and r e m a i n e d on digoxin and diuretics. She then had her first episode of moderate h e m o p t y s i s and was transferred to Tisch Hospital for further evaluation and treatment. A cardiac catheterization done at that time (Table 1) revealed that the ventricular septal defect had closed and there was functional pulmonary atresia, as well as anatomic tricuspid atresia. The Waterston s h u n t was open to the right lung with moderate pulmonary hypertension; the p u l m o n a r y vascular resistance was at the upper limits of normal and the atrial defect was nonrestrictive. No left p u l m o n a r y artery could be found. This was thought to be due to probable kinking at the site of the W a t e r s t o n anastomosis followed s u b s e q u e n t l y by loss of perfusion to the left pulmonary artery from the heart w h e n the ventricular defect closed. Ventricular function was good and there was no atrioventricular valve regurgitation. While in the hospital for further observation, she had a m a s s i v e h e m o p t y s i s and was taken to the operating room. A clot was found in the left main b r o n c h u s and when r e m o v e d , there was fresh bleeding which could not be localized or controlled. A left p n e u m o n e c t o m y was therefore performed and her postoperative course was unremarkable. Bronchiectasis probably related to the tuberculosis was thought to be the cause for the h e m o p t y s i s , since no markedly dilated bronchial collaterals were found. The left upper lobe was markedly fibrotic as was a s e g m e n t of the left lower lobe. There was no active tubercular infection. The right lung, fed by the Waterston anastomosis, c o n t i n u e d at risk for the d e v e l o p m e n t of pulmonary hypertensive c h a n g e s and the patient was readmitted 4 m o n t h s after the p n e u m o n e c t o m y for a modified F o n t a n procedure. At operation, a c o m m u n i c a t i o n was fashioned between the right pulmonary artery and the superior v e n a caval/right atrial junction. No pericardial or prosthetic material was used. The atrial defect was
Danilowicz et al.: Modified Fontan Procedure
187
Table 2. Catheterization data, 1989
Table 1. Catheterization data, 1986
Site
SVC IVC RA RPA LA LV Ao
Pressure (mmHg)
a = 10, v = 6, m = 5 45/15, m = 26 a = 7, v = 7, m = 3 110/8 110/60, m = 85
PBF = 6.8 L/me/min SBF = 2.6 L/mZ/min Q p / Q s = 2.6/1
Oxygen saturation (%)
Site
Pressure (mmHg)
57 65 60 88 84
RA RPA LV Ao
a = 17, v = 16, m = 12 a = 12, v = l l, m = 10 110/8 105/75, m = 90
Oxygen saturation (%)
65 92
CI = 4.3 L/m2lmin 89
PVR = 2.4 U SVR = 22 U
Ao, aorta; IVC, inferior vena cava; L A , left atrium; LV, left ventricle; m, mean; PBF, p u l m o n a r y blood flow; PVR, pulmonary vascular resistance; Q p / Q s , p u l m o n a r y - t o - s y s t e m i c blood flow ratio; RA, right atrium; RPA, right p u l m o n a r y artery; SBF, systemic blood flow; SVR, systemic vascular resistance.
closed and the W a t e r s t o n a n a s t o m o s i s was taken down. At the end of the operation, the right atrial pressure was 10-12 m m H g . Cardiac function was good and normal sinus r h y t h m resumed. Atrial flutter occurred in the early postoperative period and was controlled with digoxin and quinidine. There were no other problems. One m o n t h after discharge, she developed an upper respiratory infection with a cough and had a n o t h e r episode of hemoptysis, bringing up about 50 ml of bright red blood. She was immediately r e a d m i n e d to Tisch Hospital but no further bleeding occurred. B r o n c h o s c o p y s h o w e d a normal right bronchial tree. There was mild tracheitis with inflammation but no bleeding site was found. She was discharged on m a i n t e n a n c e digoxin and quinidine and she presently considers herself to be a s y m p t o m a tic. She was restudied prior to entering college. On examination, vital signs were normal with no clubbing, cyanosis, or edema. There was no organomegaly or ascites. A grade 2/6 systolic flow m u r m u r was present along the upper left sternal border with no radiation. Catheterization (Table 2) revealed a normal cardiac output with a right atrial m e a n of 12 m m H g and a right pulmonary arterial m e a n of 10 m m H g . There was no obstruction at the a n a s t o m o s i s and there was no shunt. The systemic circuit was normal with a left ventricular end-diastolic pressure of 8 m m H g , good contractility, and very minor mitral regurgitation.
Ao, aorta; C1, cardiac index; LV, left ventricle; m, m e a n ; R A , right atrium; RPA, right p u l m o n a r y artery.
omy seem to do less well than those with a left ventricle, this is not a contraindication for attempting the Fontan [7]. Significant atrioventricular valve regurgitation can be lessened by repair or replacement of the valve as long as ventricular function is adequate [3]. Hypoplasia of one pulmonary artery has also not prevented successful use of the Fontan procedure as long as pulmonary vascular resistance is normal [6]. In our patient, the presence of some degree of pulmonary hypertension, thought to be due to the shunt flow since the pulmonary vascular resistance was near normal, did not negatively influence the results of the operation, in spite of all the cardiac output going into only one lung. Although our patient may still be at risk to the late development of complications, she has remained problemfree since the early atrial flutter was controlled. Other patients, thought to be better candidates for a Fontan, have been plagued with recurrent effusions, edema, and protein-losing enteropathy, despite seemingly unobstructed, adequate operations [4, 5, 7]. The reasons for this ambiguity of clinical results still remain to be elucidated. Although arteriovenous fistula formation in the lungs may occur following the Fontan procedure as it has after a Glenn anastomosis, this has not yet been identified in our patient.
Discussion References Although the Fontan procedure does not result in an anatomically normal heart, it does restore nearnormal physiology with obliteration of the right-toleft shunt. The original limitations suggested for the procedure [2] have been much modified with children under 4 years, as well as adults now having the operation. Dysrhythmias per se do not appear to be an absolute contraindication [3]. Although patients with a single ventricle of the right ventricular anat-
1. F o n t a n F, B a u d e t E (1971) Surgical repair of tricuspid atresia. Thorax 26:240-248 2. F o n t a n F, C h o u s s a t A, B r o m AG, C h a u v e A, Deville C, Castro-Celo A (1977) Repair of tricuspid a t r e s i a - - S u r g i c a l considerations and results. In: A n d e r s o n R H , Shinebourne E A (eds) Paediatric Cardiology. Churchill Livingstone, Edinburgh, pp 567-580 3. Gale A W , D a n i e l s o n G K , M c G o o n DC, Mair DD (1979) Modified F o n t a n operation for univentricular heart and compli-
188
cated congenital lesions. J Thorac Cardiovasc Surg 78:831838 4. Gale AW, Danielson GK, McGoon DC, Wallace RB, Mair DD (1980) Fontan procedure for tricuspid atresia. Circulation 62:91-96 5. Girod DA, Fontan F, Deville C, Ottenkamp J, Choussat A (1987) Long-term results of the Fontan operation for tricuspid atresia. Circulation 75:605-610
Pediatric Cardiology Vol. 12, No. 3, 1991
6. Sade RM, Riopel DA, Taylor AB (1980) Orthoterminally corrective operation in the presence of severe hypoplasia of a pulmonary artery. J. Thorac Cardiovasc Surg 80:424-426 7. Stephanelli G, Kirklin JW, Naftel DC, Blackstone EH, Pacirico AD, Kirklin JW, Soto B, Bargeron LM (1984) Early and intermediate-term (10 years) results of surgery for univentricular atrioventricular connection ("single ventricle"). Am J Cardiol 54:811-821
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991
Successful Modified Left Pneumonectomy
Fontan
Procedure
in a n A d o l e s c e n t
After
Delores Danilowicz, Alan Langsner, and Stephen B. Colvin D e p a r t m e n t s o f Pediatrics and Cardiovascular Surgery, N e w York University Medical Center, N e w York, N e w York, U S A
S U M M A R Y . We performed a Waterston's anastomosis on a patient with complicated tricuspid atresia when she was two months o f age. At age 14 years she required left p n e u m o n e c t o m y for massive, idiopathic hemoptysis. Four m o n t h s after the p n e u m o n e c t o m y we substituted a modified Fontan anastomosis for the Waterston anastomosis. Restudy when she was 17 years of age s h o w e d continued satisfactory function. K E Y W O R D S : Tricuspid atresia - - Fontan procedure - - Waterston anastomosis - - H e m o p t y sis - - P n e u m o n e c t o m y
The Fontan procedure and modifications thereof have been used since the early 1970s as a definitive procedure for patients with tricupsid atresia, other forms of hypoplastic right heart syndrome, and various types of single ventricle [1, 3-5]. Original guidelines for the operation included age, anatomy, physiology, prior surgery, etc. [2]. As the procedure was increasingly done, many of these initial guidelines were found to be less than absolute. At the present time, the major factors which appear to influence the results of the Fontan procedure include the following: size and integrity of the pulmonary vascular bed, pulmonary vascular resistance, ventricular function, and atrioventricular valve function. We present herein an adolescent who had a modified Fontan performed using the right pulmonary artery following a left pneumonectomy for severe hemoptysis.
Case Report A 17-year-old black w o m a n was restudied 3 years after placem e n t of a modified F o n t a n a n a s t o m o s i s b e t w e e n the superior v e n a caval/right atrial j u n c t i o n and the right p u l m o n a r y artery. She was noted to be cyanotic at birth and catheterization revealed tricupsid atresia, severe p u l m o n a r y stenosis, and both atrial and ventricular septal defects. A W a t e r s t o n a n a s t o m o s i s
Address offprint requests to: Delores Danilowicz, Tisch University Hospital, R o o m 576, 560 First A v e n u e , N e w York, N Y 10016, U S A .
was performed at the age of 2 m o n t h s with a good rise in oxygen saturation and moderate congestive heart failure. Subacute bacterial endocarditis occurred on two occasions at 9 and 10 years of age, both successfully treated without clinical deterioration. A positive tuberculin c o n v e r s i o n was treated with isoniazid for 1 year. By the age of 14 years, she was moderately limited in physical activity and r e m a i n e d on digoxin and diuretics. She then had her first episode of moderate h e m o p t y s i s and was transferred to Tisch Hospital for further evaluation and treatment. A cardiac catheterization done at that time (Table 1) revealed that the ventricular septal defect had closed and there was functional pulmonary atresia, as well as anatomic tricuspid atresia. The Waterston s h u n t was open to the right lung with moderate pulmonary hypertension; the p u l m o n a r y vascular resistance was at the upper limits of normal and the atrial defect was nonrestrictive. No left p u l m o n a r y artery could be found. This was thought to be due to probable kinking at the site of the W a t e r s t o n anastomosis followed s u b s e q u e n t l y by loss of perfusion to the left pulmonary artery from the heart w h e n the ventricular defect closed. Ventricular function was good and there was no atrioventricular valve regurgitation. While in the hospital for further observation, she had a m a s s i v e h e m o p t y s i s and was taken to the operating room. A clot was found in the left main b r o n c h u s and when r e m o v e d , there was fresh bleeding which could not be localized or controlled. A left p n e u m o n e c t o m y was therefore performed and her postoperative course was unremarkable. Bronchiectasis probably related to the tuberculosis was thought to be the cause for the h e m o p t y s i s , since no markedly dilated bronchial collaterals were found. The left upper lobe was markedly fibrotic as was a s e g m e n t of the left lower lobe. There was no active tubercular infection. The right lung, fed by the Waterston anastomosis, c o n t i n u e d at risk for the d e v e l o p m e n t of pulmonary hypertensive c h a n g e s and the patient was readmitted 4 m o n t h s after the p n e u m o n e c t o m y for a modified F o n t a n procedure. At operation, a c o m m u n i c a t i o n was fashioned between the right pulmonary artery and the superior v e n a caval/right atrial junction. No pericardial or prosthetic material was used. The atrial defect was
Danilowicz et al.: Modified Fontan Procedure
187
Table 2. Catheterization data, 1989
Table 1. Catheterization data, 1986
Site
SVC IVC RA RPA LA LV Ao
Pressure (mmHg)
a = 10, v = 6, m = 5 45/15, m = 26 a = 7, v = 7, m = 3 110/8 110/60, m = 85
PBF = 6.8 L/me/min SBF = 2.6 L/mZ/min Q p / Q s = 2.6/1
Oxygen saturation (%)
Site
Pressure (mmHg)
57 65 60 88 84
RA RPA LV Ao
a = 17, v = 16, m = 12 a = 12, v = l l, m = 10 110/8 105/75, m = 90
Oxygen saturation (%)
65 92
CI = 4.3 L/m2lmin 89
PVR = 2.4 U SVR = 22 U
Ao, aorta; IVC, inferior vena cava; L A , left atrium; LV, left ventricle; m, mean; PBF, p u l m o n a r y blood flow; PVR, pulmonary vascular resistance; Q p / Q s , p u l m o n a r y - t o - s y s t e m i c blood flow ratio; RA, right atrium; RPA, right p u l m o n a r y artery; SBF, systemic blood flow; SVR, systemic vascular resistance.
closed and the W a t e r s t o n a n a s t o m o s i s was taken down. At the end of the operation, the right atrial pressure was 10-12 m m H g . Cardiac function was good and normal sinus r h y t h m resumed. Atrial flutter occurred in the early postoperative period and was controlled with digoxin and quinidine. There were no other problems. One m o n t h after discharge, she developed an upper respiratory infection with a cough and had a n o t h e r episode of hemoptysis, bringing up about 50 ml of bright red blood. She was immediately r e a d m i n e d to Tisch Hospital but no further bleeding occurred. B r o n c h o s c o p y s h o w e d a normal right bronchial tree. There was mild tracheitis with inflammation but no bleeding site was found. She was discharged on m a i n t e n a n c e digoxin and quinidine and she presently considers herself to be a s y m p t o m a tic. She was restudied prior to entering college. On examination, vital signs were normal with no clubbing, cyanosis, or edema. There was no organomegaly or ascites. A grade 2/6 systolic flow m u r m u r was present along the upper left sternal border with no radiation. Catheterization (Table 2) revealed a normal cardiac output with a right atrial m e a n of 12 m m H g and a right pulmonary arterial m e a n of 10 m m H g . There was no obstruction at the a n a s t o m o s i s and there was no shunt. The systemic circuit was normal with a left ventricular end-diastolic pressure of 8 m m H g , good contractility, and very minor mitral regurgitation.
Ao, aorta; C1, cardiac index; LV, left ventricle; m, m e a n ; R A , right atrium; RPA, right p u l m o n a r y artery.
omy seem to do less well than those with a left ventricle, this is not a contraindication for attempting the Fontan [7]. Significant atrioventricular valve regurgitation can be lessened by repair or replacement of the valve as long as ventricular function is adequate [3]. Hypoplasia of one pulmonary artery has also not prevented successful use of the Fontan procedure as long as pulmonary vascular resistance is normal [6]. In our patient, the presence of some degree of pulmonary hypertension, thought to be due to the shunt flow since the pulmonary vascular resistance was near normal, did not negatively influence the results of the operation, in spite of all the cardiac output going into only one lung. Although our patient may still be at risk to the late development of complications, she has remained problemfree since the early atrial flutter was controlled. Other patients, thought to be better candidates for a Fontan, have been plagued with recurrent effusions, edema, and protein-losing enteropathy, despite seemingly unobstructed, adequate operations [4, 5, 7]. The reasons for this ambiguity of clinical results still remain to be elucidated. Although arteriovenous fistula formation in the lungs may occur following the Fontan procedure as it has after a Glenn anastomosis, this has not yet been identified in our patient.
Discussion References Although the Fontan procedure does not result in an anatomically normal heart, it does restore nearnormal physiology with obliteration of the right-toleft shunt. The original limitations suggested for the procedure [2] have been much modified with children under 4 years, as well as adults now having the operation. Dysrhythmias per se do not appear to be an absolute contraindication [3]. Although patients with a single ventricle of the right ventricular anat-
1. F o n t a n F, B a u d e t E (1971) Surgical repair of tricuspid atresia. Thorax 26:240-248 2. F o n t a n F, C h o u s s a t A, B r o m AG, C h a u v e A, Deville C, Castro-Celo A (1977) Repair of tricuspid a t r e s i a - - S u r g i c a l considerations and results. In: A n d e r s o n R H , Shinebourne E A (eds) Paediatric Cardiology. Churchill Livingstone, Edinburgh, pp 567-580 3. Gale A W , D a n i e l s o n G K , M c G o o n DC, Mair DD (1979) Modified F o n t a n operation for univentricular heart and compli-
188
cated congenital lesions. J Thorac Cardiovasc Surg 78:831838 4. Gale AW, Danielson GK, McGoon DC, Wallace RB, Mair DD (1980) Fontan procedure for tricuspid atresia. Circulation 62:91-96 5. Girod DA, Fontan F, Deville C, Ottenkamp J, Choussat A (1987) Long-term results of the Fontan operation for tricuspid atresia. Circulation 75:605-610
Pediatric Cardiology Vol. 12, No. 3, 1991
6. Sade RM, Riopel DA, Taylor AB (1980) Orthoterminally corrective operation in the presence of severe hypoplasia of a pulmonary artery. J. Thorac Cardiovasc Surg 80:424-426 7. Stephanelli G, Kirklin JW, Naftel DC, Blackstone EH, Pacirico AD, Kirklin JW, Soto B, Bargeron LM (1984) Early and intermediate-term (10 years) results of surgery for univentricular atrioventricular connection ("single ventricle"). Am J Cardiol 54:811-821
