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Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries
CASE REPORTS
Palliative Repair of Aortic: Atresia Associated With Tricuspid Atresia and Transposition of the Great Arteries Yasuharu Imai, MD, Hiromi Kurosawa, MD, Tadashi Fujiwara, MD, Shinji Fukuchi, MD, Kouzou Matsuo, MD, Masaaki Kawada, MD, and Goro Ohtsuka, MD Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patent ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the
main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.
A
ical examination showed a pressure gradient of 12 mm Hg between upper and lower extremities, a grade 2/6 !;ystolic ejection murmur along the left sternal border, and moderate hepatomegaly. The electrocardiogram showed normal sinus rhythm (140 beatdmin), normal axis, left ventricular hypertrophy, and diminished right ventricular force. The chest roentgenogram showed cardiorn.egaly and increased pulmonary blood flow. At the age of 18 days, the infant was taken to the operating room. The gross anatomy was compatible with the preoperative diagnosis (Fig 2). Both the pulmonary arteries were dissected out and encircled with snares. Pump perfusion was instituted with a 10F arterial cannula through the patent ductus arteriosus and with a single venous cannula in the right atrium. The hypoplastic ascending aorta was detached from the main pulmonary artery, which was transected proximal to the bifurcation. The ductus was also divided at its proximal end, and the defect in the pulmonary artery was oversewn. The proximal orifice was patch-closed with glutaraldehyde-,treated equine pericardium. Then, the ascending aorta was divided and cardioplegia was induced. The proximal stump of the ascending aorta was cut open until the aortic sinus was partly exposed to make a wide anastomotic orifice and was implanted into the main pulmonary artery in an end-to-side fashion. Through the distal aortic stump, a 21F plastic needle was inserted to maintain cerebral circulation. The ductal tissue was entirely removed from the descending aorta, which was then enlarged with a semicircular patch of equine pericardium along with the coarctated segment during a 20-minute circulatory arrest of the lower body. The main pulmonary artery stump was brought up to the aortic arch by passing it under the pulmonary artery bifurcation, and wide anastomosis was made partly with an equine pericardial patch and partly
ortic atresia and hypoplastic aortic arch associated with tricuspid atresia is a rare combination of anomalies and has morphological and hemodynamic aspects similar to the hypoplastic left heart syndrome. Although various first-stage palliations for hypoplastic left heart syndrome have been performed, only a small number of the patients reach the second-stage repair, Fontan-type operation. In addition, the surgical results of the secondstage repair are still poor as the first-stage repair causes several problems mainly in the pulmonary arteries or in the aortic arch. Recently, we performed a successful first-stage repair on a neonate with a new approach under minimum circulatory arrest of the lower body. In the current report, our experience with this patient is summarized, and the advantages of this unique method of repair are discussed. A Japanese male infant was born after an uncomplicated full-term pregnancy on September 25, 1988, weighing 3.6 kg. Soon after birth, he was noted to be cyanotic. At the regional pediatric cardiac unit, the tentative echocardiographic diagnosis of tricuspid atresia with transposition of the great arteries, aortic atresia, hypoplastic aortic arch, coarctation of the aorta, and patent ductus arteriosus was made. An infusion of prostaglandin E, (0.03 pg . kg-' . min-') was started. Angiography by retrograde injection from the radial artery showed severe hypoplasia of the ascending aorta 1 mm in diameter, narrow aortic arch and coarctation of the aorta, and patent ductus arteriosus (Fig 1). At 13 days of age, he was referred for surgical repair to the Heart Institute of Japan, Tokyo. On admission, phys~
Accepted for publication Aug 23, 1990. Address reprint requests to Dr Imai, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawadacho, Shinjuku-ku, Tokyo 162, Japan.
0 1991 by The Society of Thoracic Surgeons
(Ann Thorac Surg 1991;51:Li46-8)
0003-4975/91/$3.50
CASE REPORT Ih4AI ET AL MODIFIED NORWOOD PROCEDURE
Ann Thorac Surg 1991;51:646-6
647
Fig 1 . Preoperative angiography by retrograde radial arterial injection showing severe hypoplasia of ascending aorta (As. Ao) and aortic arch. (It. IMA = left internal mammary artery.)
with an orifice in the transverse arch using 6-0 and 7-0 absorbable sutures. After full-flow perfusion was resumed, the right atrium was opened and arterial septectomy was performed. A polytetrafluoroethylene tube 5 mm in diameter was bridged between the main pulmonary artery and the right ventricle (Fig 3). On completion of intracardiac procedure, arterial oxygen tension ranged from 32 to 35 mm Hg on an inspired oxygen fraction between 0.30 and 0.60. The postoperative course was uneventful, and he was weaned off the respirator on the 10th postoperative day and was discharged from the hospital with an arterial oxygen tension of 38 mm Hg. At 14 months of age, postoperative catheterization showed a pulmonary artery index of 180, no pressure gradient across the reconstructed arch, good
coronary perfusion, mild neoaortic regurgitation, and an arterial oxygen tension of 31 mm Hg. To encourage further growth of pulmonary artery, a modified left BlalockTaussig shunt was performed. He is currently waiting for a modified Fontan procedure.
Comment After Norwood's initial report of successful first-stage palliation of hypoplastic left heart syndrome [l], modifications of the Norwood procedure for various lesions were proposed to overcome the early and late complications [2, 31. Poor coronary perfusion resulting from distortion of the ascending aorta is a fatal complication. When a conduit or a homograft was used to achieve Fig 2 . lntraoperative view showing severe hypoplasia of the ascending aorta and dilated main pulmonary artery (A) before operation and (B)after completion of operation.
648
CASE REPORT IMAI ET AL MODIFIED NORWOOD PROCEDURE
Ann Thorac Surg 1991;51:646-8
Fig 3 . Operative procedure and findings.
continuity between the proximal main pulmonary artery and the aorta, minimal distortion at the most proximal point of the ascending aorta can result in poor coronary perfusion (31. As presented here, the end-to-side anastomosis of ascending aorta just above the sinus of Valsalva can establish a "single" coronary system and can prevent its kinking or distortion. Resection of the rest of the ascending aorta makes it possible to mobilize the aortic arch and to perform anastomosis between the main pulmonary artery and the aorta without fearing kinking or distortion of hypoplastic aortic root even without a conduit and with minimal materials. Suitability for a subsequent Fontan procedure is dependent on growth and on development of the branch pulmonary arteries centrally and pulmonary arterioles peripherally [4].The transfer of the central pulmonary artery to a position anterior to the main pulmonary artery, which is connected to the aortic arch, can avoid compression of the central pulmonary artery by the main pulmonary artery. Furthermore, it allows us to put a shunt on the center of the central pulmonary artery. Right BlalockTaussig shunt was reported to result in a tenting of the right pulmonary artery and uneven growth of the central pulmonary artery. In addition, this procedure is advantageous for future use of the right ventricle as in Bjork's modification of Fontan procedure in a subsequent correction, as there is a usable right ventricle in this particular instance.
The shunt between the right ventricle and the pulmonary artery has different blood flow characteristics from the aortopulmonary shunt. It has only systolic flow demonstrated by postoperative Doppler study in this case, whereas continuous flow is the rule in aortopulmonary shunt. As a result, the total amount of flow should be smaller in the shunt between the right ventricle and the pulmonary artery than the conventional Blalock-Taussig shunt. Compared with the conventional shunt, therefore, it is possible to use a graft with a larger diameter for the shunt between the right ventricle and the pulmonary artery, which is advantageous for prevention of late obstruction.
References 1. Nonvood WI, Lang P, Castaneda AR, Cambell DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1981;82:511-9. 2. Sade RM,Fyfe D, Alpert CC. Hypoplastic left heart Syndrome: a simplified palliative operation. Ann Thorac Surg 1987;43: 309-12. 3. Jonas RA, Lang P, Hansen D, Hickey P, Castaneda AR. First stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size. J Thorac Cardiovasc Surg 1986;92:&13. 4. Pigott JD, Murphy KD, Barbar G , Nonvood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 1988;45:122-8.
Palliative Repair of Aortic: Atresia Associated With Tricuspid Atresia and Transposition of the Great Arteries Yasuharu Imai, MD, Hiromi Kurosawa, MD, Tadashi Fujiwara, MD, Shinji Fukuchi, MD, Kouzou Matsuo, MD, Masaaki Kawada, MD, and Goro Ohtsuka, MD Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, Tokyo, Japan
Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patent ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the
main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.
A
ical examination showed a pressure gradient of 12 mm Hg between upper and lower extremities, a grade 2/6 !;ystolic ejection murmur along the left sternal border, and moderate hepatomegaly. The electrocardiogram showed normal sinus rhythm (140 beatdmin), normal axis, left ventricular hypertrophy, and diminished right ventricular force. The chest roentgenogram showed cardiorn.egaly and increased pulmonary blood flow. At the age of 18 days, the infant was taken to the operating room. The gross anatomy was compatible with the preoperative diagnosis (Fig 2). Both the pulmonary arteries were dissected out and encircled with snares. Pump perfusion was instituted with a 10F arterial cannula through the patent ductus arteriosus and with a single venous cannula in the right atrium. The hypoplastic ascending aorta was detached from the main pulmonary artery, which was transected proximal to the bifurcation. The ductus was also divided at its proximal end, and the defect in the pulmonary artery was oversewn. The proximal orifice was patch-closed with glutaraldehyde-,treated equine pericardium. Then, the ascending aorta was divided and cardioplegia was induced. The proximal stump of the ascending aorta was cut open until the aortic sinus was partly exposed to make a wide anastomotic orifice and was implanted into the main pulmonary artery in an end-to-side fashion. Through the distal aortic stump, a 21F plastic needle was inserted to maintain cerebral circulation. The ductal tissue was entirely removed from the descending aorta, which was then enlarged with a semicircular patch of equine pericardium along with the coarctated segment during a 20-minute circulatory arrest of the lower body. The main pulmonary artery stump was brought up to the aortic arch by passing it under the pulmonary artery bifurcation, and wide anastomosis was made partly with an equine pericardial patch and partly
ortic atresia and hypoplastic aortic arch associated with tricuspid atresia is a rare combination of anomalies and has morphological and hemodynamic aspects similar to the hypoplastic left heart syndrome. Although various first-stage palliations for hypoplastic left heart syndrome have been performed, only a small number of the patients reach the second-stage repair, Fontan-type operation. In addition, the surgical results of the secondstage repair are still poor as the first-stage repair causes several problems mainly in the pulmonary arteries or in the aortic arch. Recently, we performed a successful first-stage repair on a neonate with a new approach under minimum circulatory arrest of the lower body. In the current report, our experience with this patient is summarized, and the advantages of this unique method of repair are discussed. A Japanese male infant was born after an uncomplicated full-term pregnancy on September 25, 1988, weighing 3.6 kg. Soon after birth, he was noted to be cyanotic. At the regional pediatric cardiac unit, the tentative echocardiographic diagnosis of tricuspid atresia with transposition of the great arteries, aortic atresia, hypoplastic aortic arch, coarctation of the aorta, and patent ductus arteriosus was made. An infusion of prostaglandin E, (0.03 pg . kg-' . min-') was started. Angiography by retrograde injection from the radial artery showed severe hypoplasia of the ascending aorta 1 mm in diameter, narrow aortic arch and coarctation of the aorta, and patent ductus arteriosus (Fig 1). At 13 days of age, he was referred for surgical repair to the Heart Institute of Japan, Tokyo. On admission, phys~
Accepted for publication Aug 23, 1990. Address reprint requests to Dr Imai, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College, 8-1 Kawadacho, Shinjuku-ku, Tokyo 162, Japan.
0 1991 by The Society of Thoracic Surgeons
(Ann Thorac Surg 1991;51:Li46-8)
0003-4975/91/$3.50
CASE REPORT Ih4AI ET AL MODIFIED NORWOOD PROCEDURE
Ann Thorac Surg 1991;51:646-6
647
Fig 1 . Preoperative angiography by retrograde radial arterial injection showing severe hypoplasia of ascending aorta (As. Ao) and aortic arch. (It. IMA = left internal mammary artery.)
with an orifice in the transverse arch using 6-0 and 7-0 absorbable sutures. After full-flow perfusion was resumed, the right atrium was opened and arterial septectomy was performed. A polytetrafluoroethylene tube 5 mm in diameter was bridged between the main pulmonary artery and the right ventricle (Fig 3). On completion of intracardiac procedure, arterial oxygen tension ranged from 32 to 35 mm Hg on an inspired oxygen fraction between 0.30 and 0.60. The postoperative course was uneventful, and he was weaned off the respirator on the 10th postoperative day and was discharged from the hospital with an arterial oxygen tension of 38 mm Hg. At 14 months of age, postoperative catheterization showed a pulmonary artery index of 180, no pressure gradient across the reconstructed arch, good
coronary perfusion, mild neoaortic regurgitation, and an arterial oxygen tension of 31 mm Hg. To encourage further growth of pulmonary artery, a modified left BlalockTaussig shunt was performed. He is currently waiting for a modified Fontan procedure.
Comment After Norwood's initial report of successful first-stage palliation of hypoplastic left heart syndrome [l], modifications of the Norwood procedure for various lesions were proposed to overcome the early and late complications [2, 31. Poor coronary perfusion resulting from distortion of the ascending aorta is a fatal complication. When a conduit or a homograft was used to achieve Fig 2 . lntraoperative view showing severe hypoplasia of the ascending aorta and dilated main pulmonary artery (A) before operation and (B)after completion of operation.
648
CASE REPORT IMAI ET AL MODIFIED NORWOOD PROCEDURE
Ann Thorac Surg 1991;51:646-8
Fig 3 . Operative procedure and findings.
continuity between the proximal main pulmonary artery and the aorta, minimal distortion at the most proximal point of the ascending aorta can result in poor coronary perfusion (31. As presented here, the end-to-side anastomosis of ascending aorta just above the sinus of Valsalva can establish a "single" coronary system and can prevent its kinking or distortion. Resection of the rest of the ascending aorta makes it possible to mobilize the aortic arch and to perform anastomosis between the main pulmonary artery and the aorta without fearing kinking or distortion of hypoplastic aortic root even without a conduit and with minimal materials. Suitability for a subsequent Fontan procedure is dependent on growth and on development of the branch pulmonary arteries centrally and pulmonary arterioles peripherally [4].The transfer of the central pulmonary artery to a position anterior to the main pulmonary artery, which is connected to the aortic arch, can avoid compression of the central pulmonary artery by the main pulmonary artery. Furthermore, it allows us to put a shunt on the center of the central pulmonary artery. Right BlalockTaussig shunt was reported to result in a tenting of the right pulmonary artery and uneven growth of the central pulmonary artery. In addition, this procedure is advantageous for future use of the right ventricle as in Bjork's modification of Fontan procedure in a subsequent correction, as there is a usable right ventricle in this particular instance.
The shunt between the right ventricle and the pulmonary artery has different blood flow characteristics from the aortopulmonary shunt. It has only systolic flow demonstrated by postoperative Doppler study in this case, whereas continuous flow is the rule in aortopulmonary shunt. As a result, the total amount of flow should be smaller in the shunt between the right ventricle and the pulmonary artery than the conventional Blalock-Taussig shunt. Compared with the conventional shunt, therefore, it is possible to use a graft with a larger diameter for the shunt between the right ventricle and the pulmonary artery, which is advantageous for prevention of late obstruction.
References 1. Nonvood WI, Lang P, Castaneda AR, Cambell DN. Experience with operations for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1981;82:511-9. 2. Sade RM,Fyfe D, Alpert CC. Hypoplastic left heart Syndrome: a simplified palliative operation. Ann Thorac Surg 1987;43: 309-12. 3. Jonas RA, Lang P, Hansen D, Hickey P, Castaneda AR. First stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size. J Thorac Cardiovasc Surg 1986;92:&13. 4. Pigott JD, Murphy KD, Barbar G , Nonvood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 1988;45:122-8.