- Email: [email protected]
Pulmonary Atresia with Ventricular Septal Defect
Pulmonary Atresia with Ventricular Septal Defect* selection of Patients for Systemic-ta-Pulmonary Artery Shunt Based on Echocardiography Bruno Marino, M.D.; Luciano Ibsquini, M.D.; lbolo Guccione, M.D.; Salvatore Giannico, M.D.; Maurizio Bevilacqua, M.D.; and Carlo Marcelletu, M.D.
From January 1987 to December 1988, in 22 infants with PAVSD, the diagnostic results obtained with echocardiography (twcHlimensiooal, Doppler, and color) were prospectively compared to the angiocardiographic findings. We classified into group 1 patients with conftuent and goodsized pulmonary (~3 mm) arteries, single ductus arteriosus, and normal pulmonary venous connections ("favorable pattern"). The other patients with PAVSD were classified into group 2 ("unfavorable pattern"). The intracardiac anatomy, the morphology of the pulmonary arteries, and the pattern of pulmonary blood supply and pulmonary venous connection were correctly identified with echocardiography in all but one patient, who was erroneously considered to be in group 2. No false-positive of the "favorable pattern" (group 1) was detected. Echocardiog-
raphy is an effective tool in infants with PAVSD, in order to discriminate cases with "favorable" and "unfavorable" patterns of pulmonary arteries, pulmonary blood supply, and pulmonary veins. The first group with the "favorable pattern" may be considered for systemic-to-pulmonary shunt surgery without angiocardiography. Based on tbis experience from January to December 1989, four patients with this "favorable pattern" underwent a successful systemic-to-pulmonary artery shunt with an echocardiographic diagnosis alone. (Chut 1991; 99:158-61)
artery morphology, the blood supT heply pulmonary to the lungs, and the pulmonary venous
angiocardiography. The age ranged from 1 to 78 days (mean, 19 days); 12 patients were boys and 10 girls. The detailed diagnoses are reported in 'Iable 1.
connections are variable in children with PAVSD. In fact, in about 50 percent of these patients, the pulmonary arteries are nonconfluent, and the pulmonary circulation is supplied by MCA. 1-5 Palliative operations in cyanotic infants have been recently reported on the basis of 2DDE,6.7 but the selection of patients tends to exclude the subjects with PAVSD because of their variability of pulmonary artery morphology and pulmonary blood supply. We prospectively studied with 2DDE and angiocardiography 22 consecutive infants with PAVSD in order to assess the reliability of the noninvasive method to identify (1) the intracardiac anatomy and segmental combination, (2) the pulmonary artery anatomy, (3) the pattern of blood supply to the lungs, and (4) the pulmonary venous connections. MATERIALS AND METHODS
From January 1987 to December 1988, some 22 consecutive infants with PAVSD were admitted to our department. The prospective study included 2DDE and cardiac catheterization with
-From the Department of Pediatric Cardiology and Cardiac Surgery. Bambino Gesu Hospital. Research Institute. Rome, Italy. Manuscript received March 19; revision accepted June 6. Reprint requests: Dr. Marirw. Pzza S Onofrio 4, Rome. Italy 00165
158
=
PAVSD pulmonary atresia with ventricular septal defect; MeA = major collateral arteries; 2DDE = two-dimensional and Dopplerechocardiography
Echocardiography
The study was performed with a sector scanner (Hewlett-Packard 77020) using a 5.Q-MHz transducer with pulsed-wave Doppler and with a separate multifrequency continuous-wave Doppler transducer. A complete assessment was obtained in each patient; in particular. the morphology and the size of the pulmonary arteries, the pattern of the pulmonary blood Row; and the pulmonary venous connections were delineated according to previous reports by using suprasternal. parasternal,"" and subcostal" views. The last 12 cases were investigated also with color Doppler echocardiography. '4 Our 2DDE analysis was finalized to delineate the intracardiac anatomy and to recognize the patients with the following features: (1) confluence of the pulmonary arteries and diameter of each pulmonary artery 2:3 mm; (2) single ductus arteriosus as the only source of pulmonary blood Row;and (3) normal pulmonary venous connections. The patients with all of these characteristics were defined as having a "favorable pattern" (group 1). The children without one of the prevtously mentioned elements were included in the group with an "unfavorable pattern" (group 2).The criteria for this classification are summarized in 'Iable 2. Angiocardiography
The cardiac catheterization was performed within one to seven days after the 2DDE study. Each patient had a right or left ventricular injection (or both) to confirm the diagnosis of PAVSD and the intracardiac anatomy. All patients underwent aortography, with or without balloon occlusion," and three also had pulmonary venous wedge angiography" to demonstrate the blood supply to the PuJmonaryAtnlsia with VSO (Marino et aI)
Table I-Diagnostic Re8ults with 2VVE and Angiography in 22 Patients with PAVSV* Diagnosis
Case
1 2 3 4 5
Associated Malformation
Aorta anterior from RV
6 7 8
Tricuspid atresia R isomerism; AVC; aorta anterior from RV
Tricuspid atresia Tricuspid atresia CCTGA CCTGA R isomerism; AVC; aorta anterior from RV
21 22
Angio-
2DDE Group Group Group Group Group
2 1 1
graphic
Morphology ofPA HypoPA; MCA
1 1
Group 2 Group 1 Group 1 Group 1 GnlUpl
Group 2 Group 1 Group 2
Group 2 Group 1 Group 2
HypoPA; MCA
Group 2 Group 2
Group 2 Group 1
Group 2 Group 1 Group 2
Group 2 Group 1 Group 2
Group 1 Group 2 Group 2 Group 1 Group 1 Group 1 Group 2
Group 1 Group 2 Group 2 Group 1 Group 1 Group 1 Group 2
Group 1 Group 2
Group 1 Gnmp 2
NonconHuent; bilateral DA Hypo PA; MCA
DISCUSSION
Hypo PA; MCA
·AVC, Atrioventricular canal; CCTGA, congenitally corrected transposition of great arteries; DA, ductus arteriosus; PA, pulmonary artery; R, right; RV, right ventricle; and hypo, hypoplastic. lungs and the morphology of the pulmonary arteries and pulmonary veins. The results of 2DDE in terms of intracardiac diagnosis and classification in group 1 or 2 were compared with the findings of cathe terization and angiocardiography RESULTS
The 2DDE diagnosis ofPAVSD and the assessment of segmental combination and intracardiac anatomy Table 2-Criteria for Classification of Patients with PAVSV*
Data
Favorabl e Pattern (Group 1)
Conlluent; both >3mm Pulmonary blood How Single DA Pulmonary venous Normal connections Pulmonary arteries
·DA, Ductus arteriosus.
were confirmed at catheterization and angiocardiography in all infants. In all patients the cardiac catheterization and angiocardiography revealed the pulmonary artery and venous anatomy and the blood supply to the lungs. Using 2DDE, 11 cases were classified as the "favorable pattern" (group 1), and the subsequent angiography confirmed these data (Fig I and 2). Eleven cases were classified as "unfavorable patterns" (group 2) with 2DDE , and the angiocardiography confirmed this diagnosis in all but one patient. In this neonate (case 10) with PAVSD, situs inversus, and dextrocardia, the visualization of a single ductus arteriosus and normal pulmonary veins was correct using 2DDE, but the confluence of the pulmonary arteries was not demonstrated, and the case was not initially included in group 1. The angiocardiography confirmed the pattern of blood supply to the lungs and venous connections but showed confluent pulmonary arteries, and the definitive classification was in group I. In the last 12 cases investigated also with color Doppler echocardiography, 14 we did not encounter any diagnostic mistake.
Unfavorable Pattern (Group 2) NonconHuent or absent or hypoplastic (>3 mm) MCA; bilateral DA Abnormal or obstructed (or both)
The complete diagnosis and surgical treatment of infants with PAVSD is still a challenge for pediatric cardiologists and cardiac surgeons. The cases with MCA and discontinuity of pulmonary arteries (group 2) require several surgical procedures, including uni focalization of the pulmonary artery tree, MCA ligation, or systemic-to-pulmonary artery shunt before complete correction.F:" Conversely, in patients with confluent pulmonary arteries and single ductus arteriosus (group 1), surgical treatment may consist of a simple systemic-to-pulmonary shunt followed by complete correction or in a definitive correction as the initial procedure.P In cases with "right isomerism," an anomaly of the pulmonary venous connections frequently associated may complicate the surgical approach." The conventional method for diagnosis and surgical indication in this malformation is cardiac catheterization and angiocardiography.I-5.1~22 Recent reports of palliative shunts being performed on the basis of 2DDE in patients with reduced pulmonary blood flow tend to exclude the cases ofPAVSD because ofthe nonpredictive pattern of the pulmonary arteries, pulmonary veins, and blood supply.5,'This prospective study correctly identified with 2DDE the pattern of intracardiac anatomy and of the pulmonary circulation in infants with PAVSD; we obtained an adequate correlation between 2DDE and angiocardiographic data. In particular, the intracardiac anatomy was correctly assessed in all cases by 2DDE, and we recognized with this method all ten patients with an "unfavorable pattern" of the pulmonary arterial tree (group 2) and II of 12 children with the "favorable CHEST I 99 I 1 I JANUARY, 1991
159
1. Two-dimensio nal ec hoca rdiogram s in 1 with I'A\'5 D . (111'1" "'): Ri ~hl oblique subxiphokl view s howi n~ pulm ona ry aln',ia (white "rmw). snbaor lic ventricular st'plal tie red , confluent pulmonary arte ries (1'). and morph ology of right pulmonary arler> (black: " rmu·s). Il {lmcer): Left obliqu e subxiphoid view s h owi n~ inl raca rdiae anatomy anti morph oloJ.:y on .·rt pulm onary ar tery (black "r", II~') . A. Aorla: L\: lert ventr icle; HA, right alrinm: anti H\( rizht ve ntricle , F I(: U Il E
~ron p
A o
FIGURE 2. Croup 1 with PAVSO. A (left): Two-dimensional echocardiogram. Suprasternal view showing tortuous ductus arteriosus (0) with acute angle with descending aorta (A). P, Pulmonary artery; and LA, left atrium. B (right): Aortogram in same patient, which confirmed morphology of pulmonary blood supply.
180
Pulmonary AInIsla wtlh VSO (Marino et 81)
pattern" (group 1) without false-positives. Our single diagnostic error occurred in a patient with situs inversus and dextrocardia. At present, 2DDE is not able to diagnose any single pattern of pulmonary circulation in children with PAVSD as much as angiocardiography does; however, we suggest that in patients with PAVSD, a precise 2DDE analysis improved by color Doppler echocardiography':' can allow a discrimination between cases with confluent and goodsized (2:3 mm) pulmonary arteries, single ductus arteriosus, and normal pulmonary venous connections (group 1) and cases with an "unfavorable pattern" of pulmonary arterial circulation. The patients in group 1 may be considered good candidates for a palliative procedure without further invasive study, as suggested for other malformations with reduced pulmonary blood flOW. 6,7 On the contrary, patients in group 2 still require cardiac catheterization and angiocardiography to assess the pattern of pulmonary circulation and to choose the best surgical option. Based on our experience and in agreement with a very recent report;" in our department during the period from January 1989 to March 1990, five patients with PAVSD and a "favorable pattern" underwent a successful palliative systemic-to-pulmonary artery shunt with 2DDE diagnosis alone. In the same period, five further patients underwent cardiac catheterization and angiocardiography that confirmed the 2DDE diagnosis of PAVSD and an "unfavorable pattern." ACKNOWLEDGMENTS: We thank Ms. Orietta Castellacci for assistance in the preparation of the manuscript and Mr. Giuseppe Bolla for technical assistance. REFERENCES 1 Macartney F, Deverall P, Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973; 35:28-37 2 Macartney FJ, Scott 0, Deverall PB. Haemodynamic and anatomic characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect including a case of persistent fifth aortic arch. Br Heart J 1974; 36:1049-60 3 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 4 Thiene G, Bortolotti V, Gallucci Y, Valente ML, Dalla Volta S, Pulmonary atresia with ventricular septal defect: further anatomical observations. Br Heart J 1977; 39:1223-33 5 Thiene G, Frescura C, Bini RM, Valente ML, Gallucci V. Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation 1979; 60:1066-74 6 Veda K, Nojima K, Saito A, Nakono H, Yokota M, Muraoka R. Modified Blalock-Taussig shunt operation without cardiac catheterization: two-dimensional echocardiographic preoperative assessment in cyanotic infants. Am J Cardiol 1984; 54:1296-99 7 Marino B, Corno A, Pasquini L, Guccione P, Carta MG, Ballerini L, et al, Indication for systemic to pulmonary artery shunts
guided by two-dimensional and Doppler echocardiography: criteria for patients selection, Ann Thorac Surg 1987; 44:495-98 8 Snider AR, Enderlein MA, Teitel DF, Juster RP. Two-dimensional echocardiographic determination of aortic and pulmonary artery size from infancy to adulthood in normal subjects. Am J Cardiel 1984; 53:218-23 9 Gutgesell Hp, Hutha JC, Cohen MH, Latson LA. Two-dimensional echocardiographic assessment of pulmonary artery and aortic arch anatomy in cyanotic infants. J Am Coli Cardioll984; 4:1242-48 10 Sahn DJ, Allen HD. Real-time cross-sectional echocardlographic imaging and measurements of the patent ductus arteriosus in infants and children. Circulation 1978; 58:343-54 11 Smallhorn JF, Hutha JC, Anderson RH, Macartney FJ. Suprasternal cross-sectional echocardiography in assessment of patent ductus arteriosus. Br Heart J 1982; 48:321-30 12 Smallhorn FJ, Gow R, Olley PM, Freedom RM, Swyer PR, Perlman M, et al. Combined noninvasive assessment of the patent ductus arteriosus in the preterm infant before and after indomethacin treatment. Am J Cardiel 1984; 54:1300-04 13 Marino B, Ballerini L, Marcelletti C, Piva R, Pasquini L, Zacche C, et al. Right oblique subxiphoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease. Am J Cardiol 1984; 54:1064-68 14 Smyllie JH, Sutherland GR, Keeton BR. The value of Doppler color How mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect. J Am Coli Cardiol 1989; 14/7:1759-65 15 Castaneda-Zunigo \v, Bass JI, Lock JE. Selective opacification of arteries with balloon occlusion angiography. Radiology 1981; 138:727-29 16 Freedom RM, Pongiglione G, Williams WG, Trusler GA, Moes CAF, Rowe RD, Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients. Am J Cardiol 1983; 51:936-39 17 Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC. Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Pediatr Radiol 1978; 128: 133-43 18 Momma K, Takao A, Ando M, Nakazawa M, Satomi G, Imai Y, et al. JuxtaductaI left pulmonary artery obstruction in pulmonary atresia. Br Heart J 1986; 55:39-44 19 Liao PK, Edwards \v, Jursland PR, Puga FJ, Danielson GK, Feldt RH. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coli Cardiol 1985; 6:1343-50 20 Shimakazi Y, Maehara T, Blackstone EH, Kirklin J\v, Bargeron LM. The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia: a quantitative cineangiographic study. J Thorne Cardiovasc Surg 1988; 95:1048-58 21 Millikan JS, Puga FJ, Danielson GK, Schaff HY, Jursland PR, Mair DD. Staged surgical repair of pulmonary atresia, ventricular septal defect, and hypoplastic, confluent pulmonary arteries. J Thorac Cardiovasc Surg 1986; 91:818-25 22 Olin CL, Ritter DG, McGoon DC, Wallace RB, Danielson GK. Pulmonary atresia: surgical considerations and results in 103 patients undergoing definitive repair. Circulation 1976; 54:3540 23 Di Donato R, di Carlo D, Squitieri C, Rossi E, Ammirati A, Marino B, et al, Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorne Surg 1987; 44:35-9
CHEST I 99 I 1 I JANUARY, 1991
161
From January 1987 to December 1988, in 22 infants with PAVSD, the diagnostic results obtained with echocardiography (twcHlimensiooal, Doppler, and color) were prospectively compared to the angiocardiographic findings. We classified into group 1 patients with conftuent and goodsized pulmonary (~3 mm) arteries, single ductus arteriosus, and normal pulmonary venous connections ("favorable pattern"). The other patients with PAVSD were classified into group 2 ("unfavorable pattern"). The intracardiac anatomy, the morphology of the pulmonary arteries, and the pattern of pulmonary blood supply and pulmonary venous connection were correctly identified with echocardiography in all but one patient, who was erroneously considered to be in group 2. No false-positive of the "favorable pattern" (group 1) was detected. Echocardiog-
raphy is an effective tool in infants with PAVSD, in order to discriminate cases with "favorable" and "unfavorable" patterns of pulmonary arteries, pulmonary blood supply, and pulmonary veins. The first group with the "favorable pattern" may be considered for systemic-to-pulmonary shunt surgery without angiocardiography. Based on tbis experience from January to December 1989, four patients with this "favorable pattern" underwent a successful systemic-to-pulmonary artery shunt with an echocardiographic diagnosis alone. (Chut 1991; 99:158-61)
artery morphology, the blood supT heply pulmonary to the lungs, and the pulmonary venous
angiocardiography. The age ranged from 1 to 78 days (mean, 19 days); 12 patients were boys and 10 girls. The detailed diagnoses are reported in 'Iable 1.
connections are variable in children with PAVSD. In fact, in about 50 percent of these patients, the pulmonary arteries are nonconfluent, and the pulmonary circulation is supplied by MCA. 1-5 Palliative operations in cyanotic infants have been recently reported on the basis of 2DDE,6.7 but the selection of patients tends to exclude the subjects with PAVSD because of their variability of pulmonary artery morphology and pulmonary blood supply. We prospectively studied with 2DDE and angiocardiography 22 consecutive infants with PAVSD in order to assess the reliability of the noninvasive method to identify (1) the intracardiac anatomy and segmental combination, (2) the pulmonary artery anatomy, (3) the pattern of blood supply to the lungs, and (4) the pulmonary venous connections. MATERIALS AND METHODS
From January 1987 to December 1988, some 22 consecutive infants with PAVSD were admitted to our department. The prospective study included 2DDE and cardiac catheterization with
-From the Department of Pediatric Cardiology and Cardiac Surgery. Bambino Gesu Hospital. Research Institute. Rome, Italy. Manuscript received March 19; revision accepted June 6. Reprint requests: Dr. Marirw. Pzza S Onofrio 4, Rome. Italy 00165
158
=
PAVSD pulmonary atresia with ventricular septal defect; MeA = major collateral arteries; 2DDE = two-dimensional and Dopplerechocardiography
Echocardiography
The study was performed with a sector scanner (Hewlett-Packard 77020) using a 5.Q-MHz transducer with pulsed-wave Doppler and with a separate multifrequency continuous-wave Doppler transducer. A complete assessment was obtained in each patient; in particular. the morphology and the size of the pulmonary arteries, the pattern of the pulmonary blood Row; and the pulmonary venous connections were delineated according to previous reports by using suprasternal. parasternal,"" and subcostal" views. The last 12 cases were investigated also with color Doppler echocardiography. '4 Our 2DDE analysis was finalized to delineate the intracardiac anatomy and to recognize the patients with the following features: (1) confluence of the pulmonary arteries and diameter of each pulmonary artery 2:3 mm; (2) single ductus arteriosus as the only source of pulmonary blood Row;and (3) normal pulmonary venous connections. The patients with all of these characteristics were defined as having a "favorable pattern" (group 1). The children without one of the prevtously mentioned elements were included in the group with an "unfavorable pattern" (group 2).The criteria for this classification are summarized in 'Iable 2. Angiocardiography
The cardiac catheterization was performed within one to seven days after the 2DDE study. Each patient had a right or left ventricular injection (or both) to confirm the diagnosis of PAVSD and the intracardiac anatomy. All patients underwent aortography, with or without balloon occlusion," and three also had pulmonary venous wedge angiography" to demonstrate the blood supply to the PuJmonaryAtnlsia with VSO (Marino et aI)
Table I-Diagnostic Re8ults with 2VVE and Angiography in 22 Patients with PAVSV* Diagnosis
Case
1 2 3 4 5
Associated Malformation
Aorta anterior from RV
6 7 8
Tricuspid atresia R isomerism; AVC; aorta anterior from RV
Tricuspid atresia Tricuspid atresia CCTGA CCTGA R isomerism; AVC; aorta anterior from RV
21 22
Angio-
2DDE Group Group Group Group Group
2 1 1
graphic
Morphology ofPA HypoPA; MCA
1 1
Group 2 Group 1 Group 1 Group 1 GnlUpl
Group 2 Group 1 Group 2
Group 2 Group 1 Group 2
HypoPA; MCA
Group 2 Group 2
Group 2 Group 1
Group 2 Group 1 Group 2
Group 2 Group 1 Group 2
Group 1 Group 2 Group 2 Group 1 Group 1 Group 1 Group 2
Group 1 Group 2 Group 2 Group 1 Group 1 Group 1 Group 2
Group 1 Group 2
Group 1 Gnmp 2
NonconHuent; bilateral DA Hypo PA; MCA
DISCUSSION
Hypo PA; MCA
·AVC, Atrioventricular canal; CCTGA, congenitally corrected transposition of great arteries; DA, ductus arteriosus; PA, pulmonary artery; R, right; RV, right ventricle; and hypo, hypoplastic. lungs and the morphology of the pulmonary arteries and pulmonary veins. The results of 2DDE in terms of intracardiac diagnosis and classification in group 1 or 2 were compared with the findings of cathe terization and angiocardiography RESULTS
The 2DDE diagnosis ofPAVSD and the assessment of segmental combination and intracardiac anatomy Table 2-Criteria for Classification of Patients with PAVSV*
Data
Favorabl e Pattern (Group 1)
Conlluent; both >3mm Pulmonary blood How Single DA Pulmonary venous Normal connections Pulmonary arteries
·DA, Ductus arteriosus.
were confirmed at catheterization and angiocardiography in all infants. In all patients the cardiac catheterization and angiocardiography revealed the pulmonary artery and venous anatomy and the blood supply to the lungs. Using 2DDE, 11 cases were classified as the "favorable pattern" (group 1), and the subsequent angiography confirmed these data (Fig I and 2). Eleven cases were classified as "unfavorable patterns" (group 2) with 2DDE , and the angiocardiography confirmed this diagnosis in all but one patient. In this neonate (case 10) with PAVSD, situs inversus, and dextrocardia, the visualization of a single ductus arteriosus and normal pulmonary veins was correct using 2DDE, but the confluence of the pulmonary arteries was not demonstrated, and the case was not initially included in group 1. The angiocardiography confirmed the pattern of blood supply to the lungs and venous connections but showed confluent pulmonary arteries, and the definitive classification was in group I. In the last 12 cases investigated also with color Doppler echocardiography, 14 we did not encounter any diagnostic mistake.
Unfavorable Pattern (Group 2) NonconHuent or absent or hypoplastic (>3 mm) MCA; bilateral DA Abnormal or obstructed (or both)
The complete diagnosis and surgical treatment of infants with PAVSD is still a challenge for pediatric cardiologists and cardiac surgeons. The cases with MCA and discontinuity of pulmonary arteries (group 2) require several surgical procedures, including uni focalization of the pulmonary artery tree, MCA ligation, or systemic-to-pulmonary artery shunt before complete correction.F:" Conversely, in patients with confluent pulmonary arteries and single ductus arteriosus (group 1), surgical treatment may consist of a simple systemic-to-pulmonary shunt followed by complete correction or in a definitive correction as the initial procedure.P In cases with "right isomerism," an anomaly of the pulmonary venous connections frequently associated may complicate the surgical approach." The conventional method for diagnosis and surgical indication in this malformation is cardiac catheterization and angiocardiography.I-5.1~22 Recent reports of palliative shunts being performed on the basis of 2DDE in patients with reduced pulmonary blood flow tend to exclude the cases ofPAVSD because ofthe nonpredictive pattern of the pulmonary arteries, pulmonary veins, and blood supply.5,'This prospective study correctly identified with 2DDE the pattern of intracardiac anatomy and of the pulmonary circulation in infants with PAVSD; we obtained an adequate correlation between 2DDE and angiocardiographic data. In particular, the intracardiac anatomy was correctly assessed in all cases by 2DDE, and we recognized with this method all ten patients with an "unfavorable pattern" of the pulmonary arterial tree (group 2) and II of 12 children with the "favorable CHEST I 99 I 1 I JANUARY, 1991
159
1. Two-dimensio nal ec hoca rdiogram s in 1 with I'A\'5 D . (111'1" "'): Ri ~hl oblique subxiphokl view s howi n~ pulm ona ry aln',ia (white "rmw). snbaor lic ventricular st'plal tie red , confluent pulmonary arte ries (1'). and morph ology of right pulmonary arler> (black: " rmu·s). Il {lmcer): Left obliqu e subxiphoid view s h owi n~ inl raca rdiae anatomy anti morph oloJ.:y on .·rt pulm onary ar tery (black "r", II~') . A. Aorla: L\: lert ventr icle; HA, right alrinm: anti H\( rizht ve ntricle , F I(: U Il E
~ron p
A o
FIGURE 2. Croup 1 with PAVSO. A (left): Two-dimensional echocardiogram. Suprasternal view showing tortuous ductus arteriosus (0) with acute angle with descending aorta (A). P, Pulmonary artery; and LA, left atrium. B (right): Aortogram in same patient, which confirmed morphology of pulmonary blood supply.
180
Pulmonary AInIsla wtlh VSO (Marino et 81)
pattern" (group 1) without false-positives. Our single diagnostic error occurred in a patient with situs inversus and dextrocardia. At present, 2DDE is not able to diagnose any single pattern of pulmonary circulation in children with PAVSD as much as angiocardiography does; however, we suggest that in patients with PAVSD, a precise 2DDE analysis improved by color Doppler echocardiography':' can allow a discrimination between cases with confluent and goodsized (2:3 mm) pulmonary arteries, single ductus arteriosus, and normal pulmonary venous connections (group 1) and cases with an "unfavorable pattern" of pulmonary arterial circulation. The patients in group 1 may be considered good candidates for a palliative procedure without further invasive study, as suggested for other malformations with reduced pulmonary blood flOW. 6,7 On the contrary, patients in group 2 still require cardiac catheterization and angiocardiography to assess the pattern of pulmonary circulation and to choose the best surgical option. Based on our experience and in agreement with a very recent report;" in our department during the period from January 1989 to March 1990, five patients with PAVSD and a "favorable pattern" underwent a successful palliative systemic-to-pulmonary artery shunt with 2DDE diagnosis alone. In the same period, five further patients underwent cardiac catheterization and angiocardiography that confirmed the 2DDE diagnosis of PAVSD and an "unfavorable pattern." ACKNOWLEDGMENTS: We thank Ms. Orietta Castellacci for assistance in the preparation of the manuscript and Mr. Giuseppe Bolla for technical assistance. REFERENCES 1 Macartney F, Deverall P, Scott O. Haemodynamic characteristics of systemic arterial blood supply to the lungs. Br Heart J 1973; 35:28-37 2 Macartney FJ, Scott 0, Deverall PB. Haemodynamic and anatomic characteristics of pulmonary blood supply in pulmonary atresia with ventricular septal defect including a case of persistent fifth aortic arch. Br Heart J 1974; 36:1049-60 3 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 4 Thiene G, Bortolotti V, Gallucci Y, Valente ML, Dalla Volta S, Pulmonary atresia with ventricular septal defect: further anatomical observations. Br Heart J 1977; 39:1223-33 5 Thiene G, Frescura C, Bini RM, Valente ML, Gallucci V. Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation 1979; 60:1066-74 6 Veda K, Nojima K, Saito A, Nakono H, Yokota M, Muraoka R. Modified Blalock-Taussig shunt operation without cardiac catheterization: two-dimensional echocardiographic preoperative assessment in cyanotic infants. Am J Cardiol 1984; 54:1296-99 7 Marino B, Corno A, Pasquini L, Guccione P, Carta MG, Ballerini L, et al, Indication for systemic to pulmonary artery shunts
guided by two-dimensional and Doppler echocardiography: criteria for patients selection, Ann Thorac Surg 1987; 44:495-98 8 Snider AR, Enderlein MA, Teitel DF, Juster RP. Two-dimensional echocardiographic determination of aortic and pulmonary artery size from infancy to adulthood in normal subjects. Am J Cardiel 1984; 53:218-23 9 Gutgesell Hp, Hutha JC, Cohen MH, Latson LA. Two-dimensional echocardiographic assessment of pulmonary artery and aortic arch anatomy in cyanotic infants. J Am Coli Cardioll984; 4:1242-48 10 Sahn DJ, Allen HD. Real-time cross-sectional echocardlographic imaging and measurements of the patent ductus arteriosus in infants and children. Circulation 1978; 58:343-54 11 Smallhorn JF, Hutha JC, Anderson RH, Macartney FJ. Suprasternal cross-sectional echocardiography in assessment of patent ductus arteriosus. Br Heart J 1982; 48:321-30 12 Smallhorn FJ, Gow R, Olley PM, Freedom RM, Swyer PR, Perlman M, et al. Combined noninvasive assessment of the patent ductus arteriosus in the preterm infant before and after indomethacin treatment. Am J Cardiel 1984; 54:1300-04 13 Marino B, Ballerini L, Marcelletti C, Piva R, Pasquini L, Zacche C, et al. Right oblique subxiphoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease. Am J Cardiol 1984; 54:1064-68 14 Smyllie JH, Sutherland GR, Keeton BR. The value of Doppler color How mapping in determining pulmonary blood supply in infants with pulmonary atresia with ventricular septal defect. J Am Coli Cardiol 1989; 14/7:1759-65 15 Castaneda-Zunigo \v, Bass JI, Lock JE. Selective opacification of arteries with balloon occlusion angiography. Radiology 1981; 138:727-29 16 Freedom RM, Pongiglione G, Williams WG, Trusler GA, Moes CAF, Rowe RD, Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients. Am J Cardiol 1983; 51:936-39 17 Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC. Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates. Pediatr Radiol 1978; 128: 133-43 18 Momma K, Takao A, Ando M, Nakazawa M, Satomi G, Imai Y, et al. JuxtaductaI left pulmonary artery obstruction in pulmonary atresia. Br Heart J 1986; 55:39-44 19 Liao PK, Edwards \v, Jursland PR, Puga FJ, Danielson GK, Feldt RH. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. J Am Coli Cardiol 1985; 6:1343-50 20 Shimakazi Y, Maehara T, Blackstone EH, Kirklin J\v, Bargeron LM. The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia: a quantitative cineangiographic study. J Thorne Cardiovasc Surg 1988; 95:1048-58 21 Millikan JS, Puga FJ, Danielson GK, Schaff HY, Jursland PR, Mair DD. Staged surgical repair of pulmonary atresia, ventricular septal defect, and hypoplastic, confluent pulmonary arteries. J Thorac Cardiovasc Surg 1986; 91:818-25 22 Olin CL, Ritter DG, McGoon DC, Wallace RB, Danielson GK. Pulmonary atresia: surgical considerations and results in 103 patients undergoing definitive repair. Circulation 1976; 54:3540 23 Di Donato R, di Carlo D, Squitieri C, Rossi E, Ammirati A, Marino B, et al, Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy. Ann Thorne Surg 1987; 44:35-9
CHEST I 99 I 1 I JANUARY, 1991
161