An Unusual Complication of Three-Patch Repair of Supravalvar Aortic Stenosis
2014; Elsevier BV; Volume: 98; Issue: 4 Linguagem: Inglês
10.1016/j.athoracsur.2013.11.076
ISSN1552-6259
AutoresRalph E. Delius, Henry L. Walters, Igor Bondarenko,
Tópico(s)Aortic Disease and Treatment Approaches
ResumoThree-patch repair of supravalvar aortic stenosis is a widely accepted surgical approach for this congenital heart lesion. We describe an unusual complication of this approach, which resulted in ischemia in the left anterior coronary artery distribution. Subtle oversizing of the left sinus of Valsalva patch led to kinking of the origin of the left anterior descending artery; the circumflex artery was not affected. Sinus of Valsalva reconstruction and reimplantation of the left coronary button restored normal coronary perfusion. Three-patch repair of supravalvar aortic stenosis is a widely accepted surgical approach for this congenital heart lesion. We describe an unusual complication of this approach, which resulted in ischemia in the left anterior coronary artery distribution. Subtle oversizing of the left sinus of Valsalva patch led to kinking of the origin of the left anterior descending artery; the circumflex artery was not affected. Sinus of Valsalva reconstruction and reimplantation of the left coronary button restored normal coronary perfusion. Multiple techniques for repair of supravalvar aortic stenosis (SVAS) have been described. The most commonly used techniques include a simple onlay patch, the Doty two-sinus technique, and variations that involve reconstructing all three sinuses, including the Brom and Meyer techniques [1Doty D.B. Polansky D.B. Jenson C.B. Supravalvular aortic stenosis repair by extended aortoplasty.J Thorac Cardiovasc Surg. 1977; 74: 362-371PubMed Google Scholar, 2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 3Kavarana M.N. Riley M. Sood V. et al.Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique.Ann Thorac Surg. 2012; 93: 1274-1279Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar]. Data definitively supporting one technique over another have been elusive, but most groups have adopted some form of three-sinus reconstruction in the belief that this type of repair is more symmetric and may offer some degree of hemodynamic benefit [4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. The present case describes an unusual complication of a three-patch repair of SVAS.A 4-month-old 6-kilogram infant with Williams syndrome, SVAS, and supravalvar pulmonary stenosis was referred for surgical repair. Preoperative catheterization demonstrated a 60 mm Hg gradient across the pulmonary supravalvar area and a 30 mm Hg gradient across the aortic root, with essentially the entire gradient across the sinotubular junction. Both pulmonary arteries were hypoplastic as well, with a distinct stenosis at the origin of the left pulmonary artery. At operation, the aortic and pulmonary valves were trileaflet, and in both the stenosis was confirmed to be at the level of the sinotubular junction. Pulmonary artery patch angioplasty, in conjunction with a three-sinus Brom-type repair of supravalvar pulmonary stenosis, was performed without incident. The Brom technique was also used to repair the SVAS. The supravalvar ring was resected. An incision into the left sinus was made rightward of the left coronary orifice. The right sinus incision was made to the left of the right coronary orifice. The noncoronary sinus was incised in the midway between the two commissures.Photofixed bovine pericardium was used for patch material. The width of the patches was determined by preoperative echocardiographic assessments of the diameter of the sinotubular junction, sinuses, and aortic annulus. The patches were designed to increase the circumference of the sinotubular junction to approximately the size of the annulus. The sinotubular circumference was determined, as was the annular diameter. The sinotubular circumference was subtracted from the annular circumference, with the difference divided by three to estimate the patch size for each sinus. In this patient, the patches were approximately 8 mm wide. All three patches were sutured to reconstruct each sinus of Valsalva, and the ascending aorta was reanastomosed to the remodeled aortic root. The aortic cross-clamp was removed after deairing. Poor perfusion in the left anterior descending (LAD) coronary artery distribution was noted during rewarming; perfusion in the circumflex artery distribution appeared normal. No obvious technical issues were noted, and no intimal thickening was seen, so the patient was again cooled and the cross-clamp was reapplied. The aorta was reopened, and the left coronary orifice was examined. The ostia appeared to be widely open, and a probe could be easily passed into the LAD. The aorta was reclosed, but again poor perfusion in the LAD distribution was noted after cross-clamp removal. While examining the heart we fortuitously noted that the LAD perfusion improved considerably when the apex of the heart was lifted anteriorly. We then noted a very subtle kink at the origin of the LAD (Fig 1A). The pulmonary artery reconstruction did not appear to be a factor in the kinking of the coronary artery. We again cross-clamped the aorta and removed the left sinus patch. We then created a coronary button and mobilized the coronary extensively. The sinus of Valsalva was recreated with another photofixed bovine pericardial patch. The mobilized coronary button was implanted into the patch by use of the trapdoor technique, and the aorta was reclosed (Fig 1B). The LAD distribution then appeared to have normal perfusion. Left ventricular activity was diminished, but this was thought to be due to repeated cross-clamp periods and a relatively prolonged period of ischemia in the LAD distribution. The patient was then given extracorporeal membrane oxygenation (ECMO) to provide hemodynamic support. The patient was weaned from ECMO 48 hours later, the remainder of the hospital course was uneventful, and she was discharged home on the eighth postoperative day. Echocardiography at the time of discharge revealed normal left ventricular function. The patient remains well 3 years after operation, with normal biventricular function and trivial (<10 mm Hg) gradients across the left and right ventricular outflow tracts.CommentSupravalvar aortic stenosis (SVAS) remains a challenging surgical problem. Some variation of the three-sinus reconstruction technique has been widely adopted, despite the scarcity of data demonstrating improved clinical outcomes in comparison with single- and dual-sinus techniques [1Doty D.B. Polansky D.B. Jenson C.B. Supravalvular aortic stenosis repair by extended aortoplasty.J Thorac Cardiovasc Surg. 1977; 74: 362-371PubMed Google Scholar, 2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 3Kavarana M.N. Riley M. Sood V. et al.Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique.Ann Thorac Surg. 2012; 93: 1274-1279Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. Nevertheless, SVAS is a disease of the entire aortic root, and a symmetric repair offers the potential for greater relief of pressure gradients and may improve coaptation of the aortic valve [4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar].The incision onto the left sinus must be rightward of the left coronary orifice to prevent injury to the left coronary artery. Overenlargement of the aortic sinuses has been described as a potential cause of aortic valvar insufficiency [2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar], but impact on coronary perfusion has not been previously noted. The diameters of the annulus and sinotubular junction are similar, and in this case were assessed by echocardiography. However, echocardiography may not provide an accurate estimation of diameter if the measurement is not made at the greatest diameter. This may have led to oversizing of the patches, leading to kinking of the LAD. Direct measurement of the sinotubular junction and annulus may have provided a more accurate measurement. Another possible explanation is that the sinuses may have also been asymmetrically affected, with the left sinus requiring less patch augmentation in comparison with the other sinuses. Finally, the angle of the left coronary takeoff may have been a contributing factor, inasmuch as even a mildly acute takeoff angle may predispose to kinking if the sinus is surgically enlarged.This report demonstrates that patch enlargement of the left sinus may effectively lengthen the left main coronary artery and cause kinking of the coronary arteries. Multiple techniques for repair of supravalvar aortic stenosis (SVAS) have been described. The most commonly used techniques include a simple onlay patch, the Doty two-sinus technique, and variations that involve reconstructing all three sinuses, including the Brom and Meyer techniques [1Doty D.B. Polansky D.B. Jenson C.B. Supravalvular aortic stenosis repair by extended aortoplasty.J Thorac Cardiovasc Surg. 1977; 74: 362-371PubMed Google Scholar, 2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 3Kavarana M.N. Riley M. Sood V. et al.Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique.Ann Thorac Surg. 2012; 93: 1274-1279Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar]. Data definitively supporting one technique over another have been elusive, but most groups have adopted some form of three-sinus reconstruction in the belief that this type of repair is more symmetric and may offer some degree of hemodynamic benefit [4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. The present case describes an unusual complication of a three-patch repair of SVAS. A 4-month-old 6-kilogram infant with Williams syndrome, SVAS, and supravalvar pulmonary stenosis was referred for surgical repair. Preoperative catheterization demonstrated a 60 mm Hg gradient across the pulmonary supravalvar area and a 30 mm Hg gradient across the aortic root, with essentially the entire gradient across the sinotubular junction. Both pulmonary arteries were hypoplastic as well, with a distinct stenosis at the origin of the left pulmonary artery. At operation, the aortic and pulmonary valves were trileaflet, and in both the stenosis was confirmed to be at the level of the sinotubular junction. Pulmonary artery patch angioplasty, in conjunction with a three-sinus Brom-type repair of supravalvar pulmonary stenosis, was performed without incident. The Brom technique was also used to repair the SVAS. The supravalvar ring was resected. An incision into the left sinus was made rightward of the left coronary orifice. The right sinus incision was made to the left of the right coronary orifice. The noncoronary sinus was incised in the midway between the two commissures. Photofixed bovine pericardium was used for patch material. The width of the patches was determined by preoperative echocardiographic assessments of the diameter of the sinotubular junction, sinuses, and aortic annulus. The patches were designed to increase the circumference of the sinotubular junction to approximately the size of the annulus. The sinotubular circumference was determined, as was the annular diameter. The sinotubular circumference was subtracted from the annular circumference, with the difference divided by three to estimate the patch size for each sinus. In this patient, the patches were approximately 8 mm wide. All three patches were sutured to reconstruct each sinus of Valsalva, and the ascending aorta was reanastomosed to the remodeled aortic root. The aortic cross-clamp was removed after deairing. Poor perfusion in the left anterior descending (LAD) coronary artery distribution was noted during rewarming; perfusion in the circumflex artery distribution appeared normal. No obvious technical issues were noted, and no intimal thickening was seen, so the patient was again cooled and the cross-clamp was reapplied. The aorta was reopened, and the left coronary orifice was examined. The ostia appeared to be widely open, and a probe could be easily passed into the LAD. The aorta was reclosed, but again poor perfusion in the LAD distribution was noted after cross-clamp removal. While examining the heart we fortuitously noted that the LAD perfusion improved considerably when the apex of the heart was lifted anteriorly. We then noted a very subtle kink at the origin of the LAD (Fig 1A). The pulmonary artery reconstruction did not appear to be a factor in the kinking of the coronary artery. We again cross-clamped the aorta and removed the left sinus patch. We then created a coronary button and mobilized the coronary extensively. The sinus of Valsalva was recreated with another photofixed bovine pericardial patch. The mobilized coronary button was implanted into the patch by use of the trapdoor technique, and the aorta was reclosed (Fig 1B). The LAD distribution then appeared to have normal perfusion. Left ventricular activity was diminished, but this was thought to be due to repeated cross-clamp periods and a relatively prolonged period of ischemia in the LAD distribution. The patient was then given extracorporeal membrane oxygenation (ECMO) to provide hemodynamic support. The patient was weaned from ECMO 48 hours later, the remainder of the hospital course was uneventful, and she was discharged home on the eighth postoperative day. Echocardiography at the time of discharge revealed normal left ventricular function. The patient remains well 3 years after operation, with normal biventricular function and trivial (<10 mm Hg) gradients across the left and right ventricular outflow tracts. CommentSupravalvar aortic stenosis (SVAS) remains a challenging surgical problem. Some variation of the three-sinus reconstruction technique has been widely adopted, despite the scarcity of data demonstrating improved clinical outcomes in comparison with single- and dual-sinus techniques [1Doty D.B. Polansky D.B. Jenson C.B. Supravalvular aortic stenosis repair by extended aortoplasty.J Thorac Cardiovasc Surg. 1977; 74: 362-371PubMed Google Scholar, 2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 3Kavarana M.N. Riley M. Sood V. et al.Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique.Ann Thorac Surg. 2012; 93: 1274-1279Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. Nevertheless, SVAS is a disease of the entire aortic root, and a symmetric repair offers the potential for greater relief of pressure gradients and may improve coaptation of the aortic valve [4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar].The incision onto the left sinus must be rightward of the left coronary orifice to prevent injury to the left coronary artery. Overenlargement of the aortic sinuses has been described as a potential cause of aortic valvar insufficiency [2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar], but impact on coronary perfusion has not been previously noted. The diameters of the annulus and sinotubular junction are similar, and in this case were assessed by echocardiography. However, echocardiography may not provide an accurate estimation of diameter if the measurement is not made at the greatest diameter. This may have led to oversizing of the patches, leading to kinking of the LAD. Direct measurement of the sinotubular junction and annulus may have provided a more accurate measurement. Another possible explanation is that the sinuses may have also been asymmetrically affected, with the left sinus requiring less patch augmentation in comparison with the other sinuses. Finally, the angle of the left coronary takeoff may have been a contributing factor, inasmuch as even a mildly acute takeoff angle may predispose to kinking if the sinus is surgically enlarged.This report demonstrates that patch enlargement of the left sinus may effectively lengthen the left main coronary artery and cause kinking of the coronary arteries. Supravalvar aortic stenosis (SVAS) remains a challenging surgical problem. Some variation of the three-sinus reconstruction technique has been widely adopted, despite the scarcity of data demonstrating improved clinical outcomes in comparison with single- and dual-sinus techniques [1Doty D.B. Polansky D.B. Jenson C.B. Supravalvular aortic stenosis repair by extended aortoplasty.J Thorac Cardiovasc Surg. 1977; 74: 362-371PubMed Google Scholar, 2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 3Kavarana M.N. Riley M. Sood V. et al.Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique.Ann Thorac Surg. 2012; 93: 1274-1279Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. Nevertheless, SVAS is a disease of the entire aortic root, and a symmetric repair offers the potential for greater relief of pressure gradients and may improve coaptation of the aortic valve [4Kaushal S. Backer C.L. Patel S. Gossett J.G. Mavroudis C. Midterm outcomes in supravalvular aortic stenosis demonstrate the superiority of multisinus aortoplasty.Ann Thorac Surg. 2010; 89: 1371-1377Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 5Metton O. Ben Ali W. Calvaruso D. et al.Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results?.Ann Thorac Surg. 2009; 88: 588-593Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar]. The incision onto the left sinus must be rightward of the left coronary orifice to prevent injury to the left coronary artery. Overenlargement of the aortic sinuses has been described as a potential cause of aortic valvar insufficiency [2Hazekamp M. Kappetein A.-P. School P.H. et al.Brom's three-patch technique for repair of supravalvar aortic stenosis.J Thorac Cardiovasc Surg. 1999; 118: 252-258Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar], but impact on coronary perfusion has not been previously noted. The diameters of the annulus and sinotubular junction are similar, and in this case were assessed by echocardiography. However, echocardiography may not provide an accurate estimation of diameter if the measurement is not made at the greatest diameter. This may have led to oversizing of the patches, leading to kinking of the LAD. Direct measurement of the sinotubular junction and annulus may have provided a more accurate measurement. Another possible explanation is that the sinuses may have also been asymmetrically affected, with the left sinus requiring less patch augmentation in comparison with the other sinuses. Finally, the angle of the left coronary takeoff may have been a contributing factor, inasmuch as even a mildly acute takeoff angle may predispose to kinking if the sinus is surgically enlarged. This report demonstrates that patch enlargement of the left sinus may effectively lengthen the left main coronary artery and cause kinking of the coronary arteries. Coronary Artery Complications After Three-Patch Repair of Supravalvar Aortic Stenosis: Recognition and ManagementThe Annals of Thoracic SurgeryVol. 99Issue 6PreviewWe read with interest the report of Delius and colleagues [1] describing coronary ischemia after the three-patch repair for supravalvar aortic stenosis. They encountered kinking of the origin of the left anterior descending coronary artery that was subsequently managed successfully by reimplanting the left coronary artery into the left sinus patch. Full-Text PDF
Referência(s)