- Email: [email protected]
Pulmonary artery banding for persistent truncus arteriosus
T h e J o u r n a l of P E D I A T R I C S
557
Pulmonary artery banding for persistent truncus arterioms Report o / a case Persistent truneus arteriosus belongs to that group of congenital heart dejects [or which no surgical correction is" considered possible. Persistent truncus arteriosus with a greatly increased flow presents a problem analogous to large felt-to-right shunts. A 22-day-old prematurely born [emale (now 14 months old) underwent pulmonary artery banding o[ a type I persistent truncus arteriosus when medical management [ailed to control cardiac decompensation. Since persistent truncus arteriosus with low pulmonary blood flow has a better prognosis [or li[e, procedures to reduce pulmonary flow should be considered in selected cases.
Marshall B. Kreidberg, M.D.,* John H. Fisher, M.D., Frank G. DeLuca, M.D. Harvey L. Chernoff, M.D. BOSTON~ M A S S .
P ~ R s I s T E N T truncus arteriosus is considered to belong to that group of congenital heart defects for which no surgical correction is possible. We think, however, that in some cases, surgical palliation can be offered. A case is presented illustrating the application of the pulmonary artery banding procedure to persistent truncus arteriosus. Persistent truncus arteriosus presents in essentially four forms (Fig. 1). Of the three
From the Departments o[ Pediatrics and Surgery, Tu/ts University School o[ Medicine and the Departments of Cardiology and Surgery, The Boston Floating Hospital [or In[ants and Children. *Address, Pediatrle Cardiologist, The" Boston Floating Hospital, 20 Ash Street, Boston 1l, Mass.
types I, I I and I I I are most often associated with increased pulmonary flow. In type IV, the pulmonary arteries are absent and there is a markedly reduced pulmonary flow. The outlook is best in this latter type. The prognosis is poorest in type I where the pulmonary flow is persistently high. From a physiologic standpoint, the patient with persistent truncus arteriosus of the high flow type presents with much the same problem as large left-to-right shunts such as those seen in large ventricular septal defects. REPORT
OF C A S E
A 22-day-old prematurely born female (birth weight: 1,930 grams) was admitted for evaluation and control of intractable failure. On physical examination pulse was 150 and respirations
558
I<2reidberg et al.
Fig. 1. Major anatomic types of persistent truncus arteriosus. 1, Short pulmonary trunk and ascending aorta arise from truncus. 2, Both right and left puhnonary arteries arise directly from truncus, their orifices close to each other. 3, Pulmonary arteries arise independently from the right and left lateral wails of the truncus. 4, Neither main, right or left pulmonary arteries present, lungs supplied solely by bronchial arteries arising from the aorta, (Modified from Collett and Edwards. 1)
Fig. 2. Explanation in text.
April 1964
45 with subcostal and intercostal retractions. Rales were heard bilaterally. There was a Grade 3-4/6 low-pitched systolic murmur over the lower left sternal border radiating to the apex and a Grade 3 / 6 higher-pitched systolic murmur in the second left intercostal space radiating to the neck and back. T h e ~liver was enlarged 5 cm below the right costal margin with a rounded edge. The electrocardiogram showed right atrial hypertrophy and combined ventricular hypertrophy. Initial x-rays showed bilateral puhnonary infiltrates, marked increase in pulmonary vascular markings and cardiomegaly. Antibiotics and anticongestive therapy were instituted shortly after admission. Despite the vigorous medical regimen, the cardiac failure could not be controlled. Clinically the infant was thought to have a large left-to-right shunt, probably a ventricular septaI defect, possibly with an associated patent ductus arteriosus. Cardiac catheterization with selective cinecardiography was performed w i t h the hopes of finding a surgically correctable lesion or a lesion amenable to palliative surgery. The combined study was interpreted as showing a large, high, interventricular septal defect with right ventricular and pulmonary artery hypertension (Table I ) . Pulmonary flow was estimated as being 3 times the systemic flow. PuImonary artery banding was recommended. At surgery, there was a mild thrill over the right ventricular Outflow tract; There arose from the heart a single vessel which, 2.0 cm. from the heart, divided into an aortic trunk and a single 1.0 cm. long main pulmonary arterial vessel. The latter divided into right and left pulmonary arteries, the right coursing behind the truncus. The aortic arch curved to the right. The main pulmonary artery was dissected free and banded with a braided cotton umbilical tape. Right ventricular and pulmonary arteriaI pressures distal to the banding were monitored simultaneously with the use of 19 gauge needles (Fig. 2). Pressures in the right ventricle and pulmonary artery prior to constriction of the band were as described at catheterization. The band was tightened until a mean pulmonary a~cterial pressure of 30 mm. Hg was achieved. There was a minimal dilatation of the heart and slowing, of heart rate following the tightening of the band. These changes resolved spontaneously. The tape was ligated with 5-0 silk and fixed to the adventitia with several 6-0 silk sutures. Monitoring of the pressures was continued during the fixation of the tape to be sure that there was no slipping during the time the tape was ligated.
Volume 64 Number 4
The postoperative period was stormy. During the first 2 postoperative days, the murmurs described changed to a Grade 4/6 harsh systolic murmur over the entire precordium and a Grade 3/6 harsh diastolic murmur along the left sternal border. The patient had several episodes of pneumonitis, the initial bouts, no doubt, extensions of the pneumonitis present prior to operation. The initial postoperative pulmonary problem was complicated by empyema which was slow in resolving. Episodes of aspiration pneumonitis occurred. Anemia was a moderate problem requiring packed blood cell transfusions. Despite the pneumonitis, empyema, and anemia during the postoperative period, the cardiac decompensation was controlled medically whereas preoperatively medical management was not successful. The patient began a slow but steady weight gain. At the time of the writing of this article, the patient was 14 months of age. Length was 43.7 cm. and weight was 5,500 grams. She was able to sit without support and to navigate herself across the floor on her abdomen. There was n6 tachypnea or tachycardia. There was right ventricular heave, a 4-plus systolic thrill, and a systolic-diastolic murmur heard over the entire precordium loudest along the upper left sternal border. The second heart sound was masked by the murmur. The liver edge was sharp and could be felt 2 cm. below the right costal margin. The splenic tip was palpable. Peripheral pulses were equal and of good quality bilaterally. The patient is presently being maintained on digoxin with no evidence of decompensatlon. She has withstood a bout of otitis media with fever up to 104 ~ and a one week illness with an influenzal-like syndrome without evidence of cardiac decompensation. COMMENT I t has been stated by H o n i c k a n d colleagues ~ t h a t in their ~xperience b a n d i n g of the p u l m o n a r y artery in truneus arteriosus is not helpful. Armer, DeOliveira, a n d Lurie 3 in an abstract m e n t i o n plication of the pulm o n a r y artery in persistent truncus arteriosus b u t give no follow-up. O c h s n e r a n d Cooley *-5 m e n t i o n b a n d i n g in a p a t i e n t with truncus arteriosus w h o d i d well i m m e d i a t e l y postoperatively b u t also offer no follow-up. O u r p a t i e n t who was b a n d e d at a p p r o x i m a t e l y 4 weeks of age has been followed now for
Pulmonary artery banding
559
T a b l e I. C a t h e t e r i z a t i o n d a t a
SVC RA RV LPA LA LV FA
Oxygen % SatuContent ration Pressure (ram. Hg) 7.1 47.3 8.1 54.0 2.7 ~ 9.9 66.0 88.5/0 10.6 70.7 88.5/40.5 (57.0 ~) 12.9 86.2 9.0 * 13.4 89.1 106.5/0 15.0 100.0t 106.5/40.5 (61.5 ~ )
~Mean. "~100 per
cent
oxygen inhalation.
a p p r o x i m a t e l y one year. A l t h o u g h her develo p m e n t has been slow with m o t o r function ranging between the seven and ninth m o n t h levels, she is nevertheless alert a n d shows no signs of m e n t a l retardation. SUMMARY
A case of type I persistent truncus arteriosus is presented in w h i c h p u l m o n a r y artery b a n d i n g was employed because of failure of medical t h e r a p y to control decompensation. T h e i m m e d i a t e p o s t b a n d i n g results and a one year follow-up are given. W e think t h a t in selected cases of high p u l m o n a r y flow truncus arteriosus, reduction of the p u l m o n a r y blood flow will alter the h e m o d y n a m i c s sufficiently to convert some of these patients from a status of c a r d i a c failure (unresponsive to medical therapy) to a status of cardiac compensation. REFERENCES
1. Collett, R. W., and Edwards, J. E.: Persistent truncus arteriosus: A classification according to anatomic types., Surg. Clin. North America 29: 1245, 1949. 2. Honick, G. L., Cayler, G. G., Williams, G. R., and Jaques, W. E.: Incomplete transposition of the great vessels (Taussig-Bing cOmplex), Am. J. Cardiol. 10: 166, 1962. 3. Armer, R. M., DeOliveira, P. F., and Lurie, P. R.: True truncus arteriosus: Review of seventeen cases and report of surgery in seven patients, Abstracts of 34th Scientific Sessions, Circulation 24: 878, 1961. 4. Ochsner, J. L., Cooley, D. A., McNamara, D. G., and Kline, A.: Surgical treatment of cardiovascular anomalies in 300 infants younger than one year of age, J. Thoracic and Cardiovasc. Surg. 43: 182, 1962. 5. Cooley, D. A., Berman, S., and Santibanez-
5 60
Kreidberg et al.
Woolrich, F. A.: Surgery in the newborn for congenital cardiovascular lesions: Report of 400 consecutive operations, J. A. M. A. 9: 912, 1962. 6. Smith, G. W., Thompson, W. M., Jr., Dammann, J. F., Jr., and Muller, W. H., Jr.: Use of the pulmonary-artery banding procedure in treating type II truncus arteriosus, Circulation 28: 807, 1963.
April 1964
ADDENDUM Since this report was written, an abstract was presented by Smith, Thompson, Dammann, and Muller in which they describe use of bilateral simultaneous Muller-Dammann procedures for the amelioration of type II truncus arteriosus defect, s
557
Pulmonary artery banding for persistent truncus arterioms Report o / a case Persistent truneus arteriosus belongs to that group of congenital heart dejects [or which no surgical correction is" considered possible. Persistent truncus arteriosus with a greatly increased flow presents a problem analogous to large felt-to-right shunts. A 22-day-old prematurely born [emale (now 14 months old) underwent pulmonary artery banding o[ a type I persistent truncus arteriosus when medical management [ailed to control cardiac decompensation. Since persistent truncus arteriosus with low pulmonary blood flow has a better prognosis [or li[e, procedures to reduce pulmonary flow should be considered in selected cases.
Marshall B. Kreidberg, M.D.,* John H. Fisher, M.D., Frank G. DeLuca, M.D. Harvey L. Chernoff, M.D. BOSTON~ M A S S .
P ~ R s I s T E N T truncus arteriosus is considered to belong to that group of congenital heart defects for which no surgical correction is possible. We think, however, that in some cases, surgical palliation can be offered. A case is presented illustrating the application of the pulmonary artery banding procedure to persistent truncus arteriosus. Persistent truncus arteriosus presents in essentially four forms (Fig. 1). Of the three
From the Departments o[ Pediatrics and Surgery, Tu/ts University School o[ Medicine and the Departments of Cardiology and Surgery, The Boston Floating Hospital [or In[ants and Children. *Address, Pediatrle Cardiologist, The" Boston Floating Hospital, 20 Ash Street, Boston 1l, Mass.
types I, I I and I I I are most often associated with increased pulmonary flow. In type IV, the pulmonary arteries are absent and there is a markedly reduced pulmonary flow. The outlook is best in this latter type. The prognosis is poorest in type I where the pulmonary flow is persistently high. From a physiologic standpoint, the patient with persistent truncus arteriosus of the high flow type presents with much the same problem as large left-to-right shunts such as those seen in large ventricular septal defects. REPORT
OF C A S E
A 22-day-old prematurely born female (birth weight: 1,930 grams) was admitted for evaluation and control of intractable failure. On physical examination pulse was 150 and respirations
558
I<2reidberg et al.
Fig. 1. Major anatomic types of persistent truncus arteriosus. 1, Short pulmonary trunk and ascending aorta arise from truncus. 2, Both right and left puhnonary arteries arise directly from truncus, their orifices close to each other. 3, Pulmonary arteries arise independently from the right and left lateral wails of the truncus. 4, Neither main, right or left pulmonary arteries present, lungs supplied solely by bronchial arteries arising from the aorta, (Modified from Collett and Edwards. 1)
Fig. 2. Explanation in text.
April 1964
45 with subcostal and intercostal retractions. Rales were heard bilaterally. There was a Grade 3-4/6 low-pitched systolic murmur over the lower left sternal border radiating to the apex and a Grade 3 / 6 higher-pitched systolic murmur in the second left intercostal space radiating to the neck and back. T h e ~liver was enlarged 5 cm below the right costal margin with a rounded edge. The electrocardiogram showed right atrial hypertrophy and combined ventricular hypertrophy. Initial x-rays showed bilateral puhnonary infiltrates, marked increase in pulmonary vascular markings and cardiomegaly. Antibiotics and anticongestive therapy were instituted shortly after admission. Despite the vigorous medical regimen, the cardiac failure could not be controlled. Clinically the infant was thought to have a large left-to-right shunt, probably a ventricular septaI defect, possibly with an associated patent ductus arteriosus. Cardiac catheterization with selective cinecardiography was performed w i t h the hopes of finding a surgically correctable lesion or a lesion amenable to palliative surgery. The combined study was interpreted as showing a large, high, interventricular septal defect with right ventricular and pulmonary artery hypertension (Table I ) . Pulmonary flow was estimated as being 3 times the systemic flow. PuImonary artery banding was recommended. At surgery, there was a mild thrill over the right ventricular Outflow tract; There arose from the heart a single vessel which, 2.0 cm. from the heart, divided into an aortic trunk and a single 1.0 cm. long main pulmonary arterial vessel. The latter divided into right and left pulmonary arteries, the right coursing behind the truncus. The aortic arch curved to the right. The main pulmonary artery was dissected free and banded with a braided cotton umbilical tape. Right ventricular and pulmonary arteriaI pressures distal to the banding were monitored simultaneously with the use of 19 gauge needles (Fig. 2). Pressures in the right ventricle and pulmonary artery prior to constriction of the band were as described at catheterization. The band was tightened until a mean pulmonary a~cterial pressure of 30 mm. Hg was achieved. There was a minimal dilatation of the heart and slowing, of heart rate following the tightening of the band. These changes resolved spontaneously. The tape was ligated with 5-0 silk and fixed to the adventitia with several 6-0 silk sutures. Monitoring of the pressures was continued during the fixation of the tape to be sure that there was no slipping during the time the tape was ligated.
Volume 64 Number 4
The postoperative period was stormy. During the first 2 postoperative days, the murmurs described changed to a Grade 4/6 harsh systolic murmur over the entire precordium and a Grade 3/6 harsh diastolic murmur along the left sternal border. The patient had several episodes of pneumonitis, the initial bouts, no doubt, extensions of the pneumonitis present prior to operation. The initial postoperative pulmonary problem was complicated by empyema which was slow in resolving. Episodes of aspiration pneumonitis occurred. Anemia was a moderate problem requiring packed blood cell transfusions. Despite the pneumonitis, empyema, and anemia during the postoperative period, the cardiac decompensation was controlled medically whereas preoperatively medical management was not successful. The patient began a slow but steady weight gain. At the time of the writing of this article, the patient was 14 months of age. Length was 43.7 cm. and weight was 5,500 grams. She was able to sit without support and to navigate herself across the floor on her abdomen. There was n6 tachypnea or tachycardia. There was right ventricular heave, a 4-plus systolic thrill, and a systolic-diastolic murmur heard over the entire precordium loudest along the upper left sternal border. The second heart sound was masked by the murmur. The liver edge was sharp and could be felt 2 cm. below the right costal margin. The splenic tip was palpable. Peripheral pulses were equal and of good quality bilaterally. The patient is presently being maintained on digoxin with no evidence of decompensatlon. She has withstood a bout of otitis media with fever up to 104 ~ and a one week illness with an influenzal-like syndrome without evidence of cardiac decompensation. COMMENT I t has been stated by H o n i c k a n d colleagues ~ t h a t in their ~xperience b a n d i n g of the p u l m o n a r y artery in truneus arteriosus is not helpful. Armer, DeOliveira, a n d Lurie 3 in an abstract m e n t i o n plication of the pulm o n a r y artery in persistent truncus arteriosus b u t give no follow-up. O c h s n e r a n d Cooley *-5 m e n t i o n b a n d i n g in a p a t i e n t with truncus arteriosus w h o d i d well i m m e d i a t e l y postoperatively b u t also offer no follow-up. O u r p a t i e n t who was b a n d e d at a p p r o x i m a t e l y 4 weeks of age has been followed now for
Pulmonary artery banding
559
T a b l e I. C a t h e t e r i z a t i o n d a t a
SVC RA RV LPA LA LV FA
Oxygen % SatuContent ration Pressure (ram. Hg) 7.1 47.3 8.1 54.0 2.7 ~ 9.9 66.0 88.5/0 10.6 70.7 88.5/40.5 (57.0 ~) 12.9 86.2 9.0 * 13.4 89.1 106.5/0 15.0 100.0t 106.5/40.5 (61.5 ~ )
~Mean. "~100 per
cent
oxygen inhalation.
a p p r o x i m a t e l y one year. A l t h o u g h her develo p m e n t has been slow with m o t o r function ranging between the seven and ninth m o n t h levels, she is nevertheless alert a n d shows no signs of m e n t a l retardation. SUMMARY
A case of type I persistent truncus arteriosus is presented in w h i c h p u l m o n a r y artery b a n d i n g was employed because of failure of medical t h e r a p y to control decompensation. T h e i m m e d i a t e p o s t b a n d i n g results and a one year follow-up are given. W e think t h a t in selected cases of high p u l m o n a r y flow truncus arteriosus, reduction of the p u l m o n a r y blood flow will alter the h e m o d y n a m i c s sufficiently to convert some of these patients from a status of c a r d i a c failure (unresponsive to medical therapy) to a status of cardiac compensation. REFERENCES
1. Collett, R. W., and Edwards, J. E.: Persistent truncus arteriosus: A classification according to anatomic types., Surg. Clin. North America 29: 1245, 1949. 2. Honick, G. L., Cayler, G. G., Williams, G. R., and Jaques, W. E.: Incomplete transposition of the great vessels (Taussig-Bing cOmplex), Am. J. Cardiol. 10: 166, 1962. 3. Armer, R. M., DeOliveira, P. F., and Lurie, P. R.: True truncus arteriosus: Review of seventeen cases and report of surgery in seven patients, Abstracts of 34th Scientific Sessions, Circulation 24: 878, 1961. 4. Ochsner, J. L., Cooley, D. A., McNamara, D. G., and Kline, A.: Surgical treatment of cardiovascular anomalies in 300 infants younger than one year of age, J. Thoracic and Cardiovasc. Surg. 43: 182, 1962. 5. Cooley, D. A., Berman, S., and Santibanez-
5 60
Kreidberg et al.
Woolrich, F. A.: Surgery in the newborn for congenital cardiovascular lesions: Report of 400 consecutive operations, J. A. M. A. 9: 912, 1962. 6. Smith, G. W., Thompson, W. M., Jr., Dammann, J. F., Jr., and Muller, W. H., Jr.: Use of the pulmonary-artery banding procedure in treating type II truncus arteriosus, Circulation 28: 807, 1963.
April 1964
ADDENDUM Since this report was written, an abstract was presented by Smith, Thompson, Dammann, and Muller in which they describe use of bilateral simultaneous Muller-Dammann procedures for the amelioration of type II truncus arteriosus defect, s