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Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia
J THoRAc
CARDIOVASC SURG
1987;94:518-20
Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95 %) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5 %) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.
B. Marino, M.D., R. Calabro, M.D.,* M. G. Gagliardi, M.D., M. Bevilacqua, M.D., L. Ballerini, M.D., and C. Marcelletti, M.D., Rome, Italy
h e knowledge of pulmonary arterial anatomy and blood supply is essential for palliative and corrective operations in patients with pulmonary atresia. When pulmonary atresia is associated with intact ventricular septum (IVS), the pulmonary arteries are confluent and a patent ductus arteriosus (PDA) is always present.!" On the contrary, when there is a ventricular septal defect (VSD) and the great arteries are normally related (tetralogy of Fallot with pulmonary atresia), pulmonary blood flow may be supplied by a PDA associated with confluent pulmonary arteries or by so-called major collateral arteries':" associated with hypoplastic, nonconfluent, or absent pulmonary arteries.l->" These two types of pulmonary blood supply only rarely can coexist in the same patient.5,9,16 Pulmonary atresia may also occur in a setting of complex congenital heart defects (i.e., single ventricle" or complete" or corrected'S" transposition of great From the Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu Hospital, Rome, Italy. Received for publication June 30, 1986. Accepted for publication Oct. 7, 1986. Address for reprints: Dr. Bruno Marino, Dipartimento MedicoChirurgico, di Cardiologia Pediatrica, Ospedale "Bambino Gesu", P. zza S. Onofrio, 4, 00165 Rorna, Italy. "Department of Cardiology Pausilipon Hospital, Naples, Italy.
518
arteries [TGA)), but there is substantially less information in the literature about the pulmonary arterial anatomy and pulmonary blood supply in this type of pulmonary atresia. 1, 17-20 Methods We reviewed the cineangiocardiograms of 155 consecutive patients with pulmonary atresia studied at the Bambino Gesu Hospital, Rome, Italy, and Pausilipon Hospital, Naples, Italy. The ages ranged from 1 day to 16 years. Each patient had a right or left ventricular angiocardiogram, or both, to confirm the diagnosis of pulmonary atresia and to reveal the intracardiac anatomy. Each patient also had an ascending aortogram or a balloon occlusion aortogram, or both,21,22 to demonstrate the blood supply to the lungs and the structure of the pulmonary arteries. Some patients required multiple selective injections into major collateral arteries and pulmonary venous wedge angiograms': 8, 9, 23, 24 to show the pulmonary arterial tree. Good radiologic display of the native blood supply and of the pulmonary arteries was essential for inclusion in this study. Patients Among 155 patients with pulmonary atresia studied in our institutions, 45 had pulmonary atresia and IVS,
Volume 94 Number 4 October 1987
70 pulmonary atresia and VSD (tetralogy of Fallot type), and 40 had pulmonary atresia and complex congenital heart disease. In this last group of patients, which is the subject of our report, single ventricle" (double-inlet ventricle or tricuspid atresia) was present in 21 patients (six with asplenia syndrome), TGA with VSDJ8.19 in 11 patients (three with asplenia), and congenitally corrected TGA with VSD in eight patients (all with situs solitus of the atria).':"
Results In 38 (95%) of the 40 patients with pulmonary atresia and complex congenital heart disease, the aortogram, with or without stop-flow technique, showed PDA and confluent pulmonary arteries; only two (5%) patients had an abnormal pulmonary arterial tree with nonconfluent pulmonary arteries. Of these, the first had atrial situs solitus, TGA with VSD, and pulmonary atresia; nonconfluent pulmonary arteries supplied the left lung by small collateral arteries and the right by a PDA. 16 The second patient had asplenia syndrome with single ventricle (common inlet ventricle), pulmonary atresia, nonconfluent pulmonary arteries, and multiple major collateral arteries to both lungs.
Discussion The differences in embryology, anatomical features, and pulmonary blood supply between pulmonary atresia with IVS and with VSD (tetralogy of Fallot type) has been extensively investigated in the literature.':" Pulmonary atresia with IVS is always associated with PDA and confluent, almost normal pulmonary arteries, 1-4 whereas in pulmonary atresia with VSD the pulmonary blood supply and the structure of the pulmonary arteries are not uniform: Some patients have PDA and confluent pulmonary arteries; others have major collateral arteries with hypoplastic, nonconfluent, or absent pulmonary arteries. IS. 16 The prevalence of major collateral arteries and absent PDA is high in pulmonary atresia with VSD and ranges between 35% and 50% of cases.':" The pattern of pulmonary blood supply in complex cases of pulmonary atresia had occasionally been reported in the literature I. 17-20 but has never been systematically studied and has received little attention compared with other types of pulmonary atresia. In our series of patients with complex forms of pulmonary atresia, we found that only two patients (5%) had nonconfluent pulmonary arteries and atypical pulmonary blood supply; the other 38 patients had PDA and confluent pulmonary arteries. This percentage of pulmonary arterial abnormalities is significantly lower than that in the tetralogy of Fallot type of pulmonary atresia>" and is similar to that
Complex congenital heart disease 5 1 9
Table I Authors Elliot et al. 17* (1964) Ruttenberg et al." (1964) Berry et aI.' (1974) Allwork et al. 2ot (1976) Van Praagh et al. 1 (1976) Marcelletti et al. 18:j: (1976) Thiene et alY:j: (1977) Marino et al. (1987)
Pulmonary atresia. complex forms
MeA
6 9
6 9
o o
29 6
25 6
o
28
27
13
12
7
7
98 40
92 38
4
o 6 (6.1%) 2 (5%)
Legend: PDA. Patent ductus arteriosus. MeA. Major collateral arteries. ·Only single ventricle with pulmonary atresia. tOnly complete transposition of the great arteries with pulmonary atresia. :j:Only transposition of the great arteries.
reported in previous studies in the same patients with complex malformations. I. 7.11.17-20 A comprehensive review of the published reports of pulmonary atresia and complex congenital heart defects I. 7.11.17-20 has yielded 98 patients, of whom only six, (6.1%) had major collateral arteries and absent PDA; the remainder had PDA and confluent pulmonary arteries (Table I). Knowledge of these data is useful to plan cardiac catheterization before the corrective operation. On the other hand, with two-dimensional echocardiographic visualization of the intracardiac anatomy and pulmonary arterial confluence, plus knowledge of the uniformity of pulmonary blood supply and the low incidence of pulmonary arterial abnormalities, emergency palliative operations in the neonatal period may be performed in this group of patients without invasive study, as reported for pulmonary atresia and IVS. 2s
1.
2.
3. 4.
REFERENCES Van Praagh R, Ando M, Van Praagh S, et al. Pulmonary atresia: anatomicconsideration. In: Kidd BSL, RoweRD, eds. The child with congenital heart disease after surgery. New York: Futura Publishing Co., 1976:103-34. Kutsche LM, Van Mierop LHS. Pulmonaryatresia with and without ventricular septal defect: a different etiology and pathogenesis for the atresia in the 2 types? Am J Cardiol 1983;51 :932-5. Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979;41:281-8. Freedom RM. The morphologic variations of pulmonary
The Journal of Thoracic and Cardiovascular Surgery
520 Marino et aJ.
atresia with intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983;4:183-8. 5. Jefferson K, Rees S, Somerville J. Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 1972;34:418-
15. Liao PK, Edwards W, Julsrud PR, Puga FJ, Danielson
Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation
GK, Feldt RH. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coli Cardiol 1985;6:1343-50. Frescura C, Talenti E, Pellegrino PA, Mazzucco A, Faggian G, Thiene G. Coexistence of ductal and systemic-pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Am J Cardiol 1984;53:348-9. Elliott LP, Anderson RC, Edwards JE. The common cardiac ventricle with transposition of the great vessels. Br Heart J 1964;26:289-301. Marcelletti C, Mair DD, McGoon DC, Wallace RB, Danielson GK. Complete repair of transposition of the great arteries with pulmonary atresia. J THORAC CARDIOVASC SURG 1976;72:215-20. Ruttenberg HD, Neufeld HN, Lucas RV, et al: Syndrome of congenital cardiac disease with asplenia. Am J Cardiol 1964:13:387-406. Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol 1976;38:920-3. Herraiz 1M, Bermudez Canete R, Gazzaniga M, et al. Occlusion aortography: evaluation of our results with this technique. First World Congress of Paediatric Cardiology [Abstracts]. London: 1980:174. Castaneda A, Zuniga W, Bass JI, Lock JE. Selective opacification of arteries with balloon occlusion angiography. Radiology 1981;138:727-9. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC. Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Pediatr Radi-
1979;60: 1066-74. 13. Thiene G, Frescura C, Bortolotti U, Del Maschio A,
011978;128:133-43. 24. Momma K, Takao A, Ando M, et al. Juxtaductal left
Valente ML. The systemic-pulmonary circulation in pulmonary atresia with ventricular septal defect: concept of reciprocal development of the fourth and sixth aortic arches. Am Heart Journal 1981;101:339-44. 14. Lacina SJ, Hamilton WT, Thilenius 00, Bharati S, Lev M, Arcilla RA. Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction. Pediatr Cardiol 1983;4:5-11.
pulmonary artery obstruction in pulmonary atresia. Br Heart J 1986;55:39-44. 25. Marino B, Franceschini E, Ballerini L, Marcelletti C, Thiene G. Anatomical-echocardiographic correlations in pulmonary atresia with intact ventricular septum: use of subcostal cross-sectionalviews. Int J Cardiol 1986;11:I 039.
16.
27.
6. Edwards JE, McGoon DC. Absence of anatomic origin from heart of pulmonary arterial supply. Circulation 1973;42:393-8.
7. Berry BE, McGoon DC, Ritter DG, Davis GD. Absence of anatomic origin from heart of pulmonary arterial supply. J THORAC CARDIOVASC SURG 1974;68:119-25. 8. Macartney F, Deverall P, Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973;35:28-37. 9. Macartney FJ, Scott 0, Deverall PB. Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect including a case of persistent fifth aortic arch. Br Heart J
17.
18.
19.
20.
1974;36:1049-60. 10. Bharati S, Paul MH, Idriss FS, Potkin RT, Lev M. The
surgical anatomy of pulmonary atresia with ventricular septal defect: pseudotruncus. J THORAC CARDIOVASC SURG 1975;69:713-21. 11. Thiene G, Bortolotti U, Gallucci V, Valente ML, Dalla Volta S. Pulmonary atresia with ventricular septal defect: further anatomical observations. Br Heart J 1977; 39:1223-33. 12. Thiene G, Frescura C, Bini RM, Valente ML, Gallucci V.
21.
22.
23.
CARDIOVASC SURG
1987;94:518-20
Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95 %) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5 %) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.
B. Marino, M.D., R. Calabro, M.D.,* M. G. Gagliardi, M.D., M. Bevilacqua, M.D., L. Ballerini, M.D., and C. Marcelletti, M.D., Rome, Italy
h e knowledge of pulmonary arterial anatomy and blood supply is essential for palliative and corrective operations in patients with pulmonary atresia. When pulmonary atresia is associated with intact ventricular septum (IVS), the pulmonary arteries are confluent and a patent ductus arteriosus (PDA) is always present.!" On the contrary, when there is a ventricular septal defect (VSD) and the great arteries are normally related (tetralogy of Fallot with pulmonary atresia), pulmonary blood flow may be supplied by a PDA associated with confluent pulmonary arteries or by so-called major collateral arteries':" associated with hypoplastic, nonconfluent, or absent pulmonary arteries.l->" These two types of pulmonary blood supply only rarely can coexist in the same patient.5,9,16 Pulmonary atresia may also occur in a setting of complex congenital heart defects (i.e., single ventricle" or complete" or corrected'S" transposition of great From the Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu Hospital, Rome, Italy. Received for publication June 30, 1986. Accepted for publication Oct. 7, 1986. Address for reprints: Dr. Bruno Marino, Dipartimento MedicoChirurgico, di Cardiologia Pediatrica, Ospedale "Bambino Gesu", P. zza S. Onofrio, 4, 00165 Rorna, Italy. "Department of Cardiology Pausilipon Hospital, Naples, Italy.
518
arteries [TGA)), but there is substantially less information in the literature about the pulmonary arterial anatomy and pulmonary blood supply in this type of pulmonary atresia. 1, 17-20 Methods We reviewed the cineangiocardiograms of 155 consecutive patients with pulmonary atresia studied at the Bambino Gesu Hospital, Rome, Italy, and Pausilipon Hospital, Naples, Italy. The ages ranged from 1 day to 16 years. Each patient had a right or left ventricular angiocardiogram, or both, to confirm the diagnosis of pulmonary atresia and to reveal the intracardiac anatomy. Each patient also had an ascending aortogram or a balloon occlusion aortogram, or both,21,22 to demonstrate the blood supply to the lungs and the structure of the pulmonary arteries. Some patients required multiple selective injections into major collateral arteries and pulmonary venous wedge angiograms': 8, 9, 23, 24 to show the pulmonary arterial tree. Good radiologic display of the native blood supply and of the pulmonary arteries was essential for inclusion in this study. Patients Among 155 patients with pulmonary atresia studied in our institutions, 45 had pulmonary atresia and IVS,
Volume 94 Number 4 October 1987
70 pulmonary atresia and VSD (tetralogy of Fallot type), and 40 had pulmonary atresia and complex congenital heart disease. In this last group of patients, which is the subject of our report, single ventricle" (double-inlet ventricle or tricuspid atresia) was present in 21 patients (six with asplenia syndrome), TGA with VSDJ8.19 in 11 patients (three with asplenia), and congenitally corrected TGA with VSD in eight patients (all with situs solitus of the atria).':"
Results In 38 (95%) of the 40 patients with pulmonary atresia and complex congenital heart disease, the aortogram, with or without stop-flow technique, showed PDA and confluent pulmonary arteries; only two (5%) patients had an abnormal pulmonary arterial tree with nonconfluent pulmonary arteries. Of these, the first had atrial situs solitus, TGA with VSD, and pulmonary atresia; nonconfluent pulmonary arteries supplied the left lung by small collateral arteries and the right by a PDA. 16 The second patient had asplenia syndrome with single ventricle (common inlet ventricle), pulmonary atresia, nonconfluent pulmonary arteries, and multiple major collateral arteries to both lungs.
Discussion The differences in embryology, anatomical features, and pulmonary blood supply between pulmonary atresia with IVS and with VSD (tetralogy of Fallot type) has been extensively investigated in the literature.':" Pulmonary atresia with IVS is always associated with PDA and confluent, almost normal pulmonary arteries, 1-4 whereas in pulmonary atresia with VSD the pulmonary blood supply and the structure of the pulmonary arteries are not uniform: Some patients have PDA and confluent pulmonary arteries; others have major collateral arteries with hypoplastic, nonconfluent, or absent pulmonary arteries. IS. 16 The prevalence of major collateral arteries and absent PDA is high in pulmonary atresia with VSD and ranges between 35% and 50% of cases.':" The pattern of pulmonary blood supply in complex cases of pulmonary atresia had occasionally been reported in the literature I. 17-20 but has never been systematically studied and has received little attention compared with other types of pulmonary atresia. In our series of patients with complex forms of pulmonary atresia, we found that only two patients (5%) had nonconfluent pulmonary arteries and atypical pulmonary blood supply; the other 38 patients had PDA and confluent pulmonary arteries. This percentage of pulmonary arterial abnormalities is significantly lower than that in the tetralogy of Fallot type of pulmonary atresia>" and is similar to that
Complex congenital heart disease 5 1 9
Table I Authors Elliot et al. 17* (1964) Ruttenberg et al." (1964) Berry et aI.' (1974) Allwork et al. 2ot (1976) Van Praagh et al. 1 (1976) Marcelletti et al. 18:j: (1976) Thiene et alY:j: (1977) Marino et al. (1987)
Pulmonary atresia. complex forms
MeA
6 9
6 9
o o
29 6
25 6
o
28
27
13
12
7
7
98 40
92 38
4
o 6 (6.1%) 2 (5%)
Legend: PDA. Patent ductus arteriosus. MeA. Major collateral arteries. ·Only single ventricle with pulmonary atresia. tOnly complete transposition of the great arteries with pulmonary atresia. :j:Only transposition of the great arteries.
reported in previous studies in the same patients with complex malformations. I. 7.11.17-20 A comprehensive review of the published reports of pulmonary atresia and complex congenital heart defects I. 7.11.17-20 has yielded 98 patients, of whom only six, (6.1%) had major collateral arteries and absent PDA; the remainder had PDA and confluent pulmonary arteries (Table I). Knowledge of these data is useful to plan cardiac catheterization before the corrective operation. On the other hand, with two-dimensional echocardiographic visualization of the intracardiac anatomy and pulmonary arterial confluence, plus knowledge of the uniformity of pulmonary blood supply and the low incidence of pulmonary arterial abnormalities, emergency palliative operations in the neonatal period may be performed in this group of patients without invasive study, as reported for pulmonary atresia and IVS. 2s
1.
2.
3. 4.
REFERENCES Van Praagh R, Ando M, Van Praagh S, et al. Pulmonary atresia: anatomicconsideration. In: Kidd BSL, RoweRD, eds. The child with congenital heart disease after surgery. New York: Futura Publishing Co., 1976:103-34. Kutsche LM, Van Mierop LHS. Pulmonaryatresia with and without ventricular septal defect: a different etiology and pathogenesis for the atresia in the 2 types? Am J Cardiol 1983;51 :932-5. Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact ventricular septum. Br Heart J 1979;41:281-8. Freedom RM. The morphologic variations of pulmonary
The Journal of Thoracic and Cardiovascular Surgery
520 Marino et aJ.
atresia with intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983;4:183-8. 5. Jefferson K, Rees S, Somerville J. Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 1972;34:418-
15. Liao PK, Edwards W, Julsrud PR, Puga FJ, Danielson
Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation
GK, Feldt RH. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coli Cardiol 1985;6:1343-50. Frescura C, Talenti E, Pellegrino PA, Mazzucco A, Faggian G, Thiene G. Coexistence of ductal and systemic-pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Am J Cardiol 1984;53:348-9. Elliott LP, Anderson RC, Edwards JE. The common cardiac ventricle with transposition of the great vessels. Br Heart J 1964;26:289-301. Marcelletti C, Mair DD, McGoon DC, Wallace RB, Danielson GK. Complete repair of transposition of the great arteries with pulmonary atresia. J THORAC CARDIOVASC SURG 1976;72:215-20. Ruttenberg HD, Neufeld HN, Lucas RV, et al: Syndrome of congenital cardiac disease with asplenia. Am J Cardiol 1964:13:387-406. Allwork SP, Bentall HH, Becker AE, et al. Congenitally corrected transposition of the great arteries: morphologic study of 32 cases. Am J Cardiol 1976;38:920-3. Herraiz 1M, Bermudez Canete R, Gazzaniga M, et al. Occlusion aortography: evaluation of our results with this technique. First World Congress of Paediatric Cardiology [Abstracts]. London: 1980:174. Castaneda A, Zuniga W, Bass JI, Lock JE. Selective opacification of arteries with balloon occlusion angiography. Radiology 1981;138:727-9. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC. Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Pediatr Radi-
1979;60: 1066-74. 13. Thiene G, Frescura C, Bortolotti U, Del Maschio A,
011978;128:133-43. 24. Momma K, Takao A, Ando M, et al. Juxtaductal left
Valente ML. The systemic-pulmonary circulation in pulmonary atresia with ventricular septal defect: concept of reciprocal development of the fourth and sixth aortic arches. Am Heart Journal 1981;101:339-44. 14. Lacina SJ, Hamilton WT, Thilenius 00, Bharati S, Lev M, Arcilla RA. Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction. Pediatr Cardiol 1983;4:5-11.
pulmonary artery obstruction in pulmonary atresia. Br Heart J 1986;55:39-44. 25. Marino B, Franceschini E, Ballerini L, Marcelletti C, Thiene G. Anatomical-echocardiographic correlations in pulmonary atresia with intact ventricular septum: use of subcostal cross-sectionalviews. Int J Cardiol 1986;11:I 039.
16.
27.
6. Edwards JE, McGoon DC. Absence of anatomic origin from heart of pulmonary arterial supply. Circulation 1973;42:393-8.
7. Berry BE, McGoon DC, Ritter DG, Davis GD. Absence of anatomic origin from heart of pulmonary arterial supply. J THORAC CARDIOVASC SURG 1974;68:119-25. 8. Macartney F, Deverall P, Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973;35:28-37. 9. Macartney FJ, Scott 0, Deverall PB. Haemodynamic and anatomical characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect including a case of persistent fifth aortic arch. Br Heart J
17.
18.
19.
20.
1974;36:1049-60. 10. Bharati S, Paul MH, Idriss FS, Potkin RT, Lev M. The
surgical anatomy of pulmonary atresia with ventricular septal defect: pseudotruncus. J THORAC CARDIOVASC SURG 1975;69:713-21. 11. Thiene G, Bortolotti U, Gallucci V, Valente ML, Dalla Volta S. Pulmonary atresia with ventricular septal defect: further anatomical observations. Br Heart J 1977; 39:1223-33. 12. Thiene G, Frescura C, Bini RM, Valente ML, Gallucci V.
21.
22.
23.