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• W3199698891 abstract "Central MessageRVD should be an option for treatment in patients with pulmonary atresia and intact ventricular septum unless the coronary circulation is RV dependent.See Commentary on page 2216.Feature Editor's Introduction—Surgical management of children with pulmonary atresia and intact ventricular septum remains challenging. These patients have a large spectrum of pathological anatomy, including various degrees of coronary to right ventricular (RV) fistulous connections, culminating in the true RV coronary artery dependence. A surgical group from Okayama led by Dr Yasuhiro Kotani presents their experience with these challenging patients and discusses the indications, role, timing, and impact of RV decompression.Igor E. Konstantinov, MD, PhD, FRACS RVD should be an option for treatment in patients with pulmonary atresia and intact ventricular septum unless the coronary circulation is RV dependent. See Commentary on page 2216. Coronary artery anomalies, namely, sinusoidal communications (SC), are commonly seen in patients with pulmonary atresia and intact ventricular septum (PA-IVS), especially in those with a diminutive right ventricle (RV).1Daubeney P.E.F. Delany D.J. Anderson R.H. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study.ACC Curr J Rev. 2002; 11: 90Crossref Google Scholar Therefore, assessment of the coronary arteries is essential before any interventions that could potentially reduce the RV pressure in patients with a relatively smaller RV, especially when the outlet portion is absent because SCs tend to be large.1Daubeney P.E.F. Delany D.J. Anderson R.H. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study.ACC Curr J Rev. 2002; 11: 90Crossref Google Scholar Several reports from the 1990s demonstrated that a large SC with a stenosis in the proximal portion of the left coronary artery (LCA) carried a high risk of death.2Giglia T.M. Mandell V.S. Connor A.R. Mayer J.E. Lock J.E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.Circulation. 1992; 86: 1516-1528Crossref PubMed Scopus (158) Google Scholar, 3Coles J.G. Freedom R.M. Lightfoot N.E. Dasmahapatra H.K. Williams W.G. Trusler G.A. et al.Long-term results in neonates with pulmonary atresia and intact ventricular septum.Ann Thorac Surg. 1989; 47: 213-217Abstract Full Text PDF PubMed Scopus (95) Google Scholar, 4Williams W.G. Burrows P. Freedom R.M. Trusler G.A. Coles J.G. Moes C.A. et al.Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1991; 101: 222-229Abstract Full Text PDF PubMed Google Scholar Since then, it has been considered that RV cannot safely be decompressed in this circumstance. Furthermore, the continued presence of coronary perfusion with unsaturated RV blood could lead to myocardial fibrosis, resulting in arrhythmia and myocardial ischemia. Decompression of the RV (RVD) through pulmonary valvotomy was considered as an optimal treatment for those with a reasonably sized RV in the 1950s.5Cole R.B. Muster A.J. Lev M. Paul M.H. Pulmonary atresia with intact ventricular septum.Am J Cardiol. 1968; 21: 23-31Abstract Full Text PDF PubMed Scopus (25) Google Scholar, 6Bowman F.O. Malm J.R. Hayes C.J. Gersony W.M. Ellis K. Pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg. 1971; 61: 85-95Abstract Full Text PDF PubMed Google Scholar, 7Moulton A.L. Bowman F.O. Edie R.N. Hayes C.J. Ellis K. Gersony W.M. et al.Pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg. 1979; 78: 527-536Abstract Full Text PDF PubMed Google Scholar For those with a tight outlet portion, the combination of repair of the right ventricular outflow tract (RVOT) and construction of a systemic-to-pulmonary arterial shunt has been the mainstay of surgical interventions since its first report in 1971 until today.6Bowman F.O. Malm J.R. Hayes C.J. Gersony W.M. Ellis K. Pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg. 1971; 61: 85-95Abstract Full Text PDF PubMed Google Scholar,8Pawade A. Capuani A. Penny D.J. Karl T.R. Mee R.B.B. Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum.J Card Surg. 1993; 8: 371-383Crossref PubMed Scopus (62) Google Scholar,9de Leval M. Bull C. Stark J. Anderson R.H. Taylor J.F. Macartney F.J. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.Circulation. 1982; 66: 272-280Crossref PubMed Scopus (135) Google Scholar The indications have been considerably refined as our knowledge of the anatomic complexities, together with their correlation to physiology, has grown. However, various controversies remain regarding the indications for RVD. It is still unresolved whether a procedure on the RVOT should be performed in all cases, even when the RV is diminutive, except in cases of right ventricular dependent coronary circulation (RVDCC). Previous reports demonstrated several advantages of RVD in patients with PA-IVS, and one of the important effects is RV growth and subsequent biventricular repair.8Pawade A. Capuani A. Penny D.J. Karl T.R. Mee R.B.B. Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum.J Card Surg. 1993; 8: 371-383Crossref PubMed Scopus (62) Google Scholar,10Ovaert C. Qureshi S.A. Rosenthal E. Baker E.J. Tynan M. Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1998; 115: 1055-1062Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar, 11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar, 12Shaddy R.E. Sturtevant J.E. Judd V.E. McGough E.C. Right ventricular growth after transventricular pulmonary valvotomy and central aortopulmonary shunt for pulmonary atresia and intact ventricular septum.Circulation. 1990; 82: IV157-IV163PubMed Google Scholar An early study looking at the impact of RVD in 22 patients who underwent transventricular pulmonary valvotomy demonstrated that the RV end-diastolic volume increased from 59.1% ± 39.3% of the predicted normal before the initial surgery to 114.6% ± 63.2% at late follow-up catheterization, resulting in the achievement of biventricular repair in more than 90%.12Shaddy R.E. Sturtevant J.E. Judd V.E. McGough E.C. Right ventricular growth after transventricular pulmonary valvotomy and central aortopulmonary shunt for pulmonary atresia and intact ventricular septum.Circulation. 1990; 82: IV157-IV163PubMed Google Scholar Several studies have emphasized the importance of RV myectomy13Bryant R. Nowicki E.R. Mee R.B. Rajeswaran J. Duncan B.W. Rosenthal G.L. et al.Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg. 2008; 136: 735-742.e2Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar or RV overhaul8Pawade A. Capuani A. Penny D.J. Karl T.R. Mee R.B.B. Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum.J Card Surg. 1993; 8: 371-383Crossref PubMed Scopus (62) Google Scholar,11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar,14Sano S. Ishino K. Kawada M. Fujisawa E. Kamada M. Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum.Ann Thorac Surg. 2000; 70: 1501-1506Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar in addition to RVD, which recruited approximately 60% to 87% of patients who achieved a biventricular repair. This result can be altered depending on the severity of hypoplasia of the RV or the tricuspid valve (TV) at birth. Large SC and RVDCC occur most frequently in patients with the smallest RVs, although there are cases when RVDCC is found in patients with a normal RV size.15Mair D.D. Julsrud P.R. Puga F.J. Danielson G.K. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results.J Am Coll Cardiol. 1997; 29: 1359-1364Crossref PubMed Scopus (49) Google Scholar It is universally agreed that a treatment strategy can be constructed on the basis of the understanding of coronary artery circulation.2Giglia T.M. Mandell V.S. Connor A.R. Mayer J.E. Lock J.E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.Circulation. 1992; 86: 1516-1528Crossref PubMed Scopus (158) Google Scholar,16Gentles T.L. Colan S.D. Giglia T.M. Mandell V.S. Mayer J.E.J. Sanders S.P. Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies.Circulation. 1993; 88: II183-II188PubMed Google Scholar Fontan operation is usually planned for patients whose coronary artery circulation is entirely or largely dependent on the RV, and we do not attempt to decompress by surgical/catheter-based intervention or thromboexclusion of the RV.4Williams W.G. Burrows P. Freedom R.M. Trusler G.A. Coles J.G. Moes C.A. et al.Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1991; 101: 222-229Abstract Full Text PDF PubMed Google Scholar,17Waldman J.D. Lamberti J.J. Mathewson J.W. George L. Surgical closure of the tricuspid valve for pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications.Pediatr Cardiol. 1984; 5: 221-223Crossref PubMed Scopus (27) Google Scholar At the time of the Fontan procedure, the RV should be incorporated into the repair to receive oxygenated blood from the left atrium. However, it is important to note that persistent RV hypertension with the presence of SCs potentially leads to the progression of coronary abnormalities such as stenosis, ectasia, and interruptions that can lead to sudden death. A multicenter study from Australia and New Zealand showed that RVDCC was associated with myocardial ischemia and sudden death after Fontan operation.18Elias P. Poh C.L. du Plessis K. Zannino D. Rice K. Radford D.J. et al.Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.Eur J Cardiothorac Surg. 2018; 53: 1230-1236Crossref PubMed Scopus (22) Google Scholar Another key finding of this study is that the occurrence of angina (16%) is not negligible, which makes us think we should somehow intervene to decompress the RV even in patients having Fontan operation (decompression Fontan), so that coronary-related complications could be avoided. For patients in whom a relatively small and distal portion of the coronary artery circulation is RV dependent, one can consider proceeding to a biventricular repair, depending on the size of the RV and TV. The impact of RVD on SC has been discussed since a few decades ago when decompression was considered as an optimal palliation for those with a reasonable RV size.2Giglia T.M. Mandell V.S. Connor A.R. Mayer J.E. Lock J.E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.Circulation. 1992; 86: 1516-1528Crossref PubMed Scopus (158) Google Scholar,19Patel R.G. Freedom R.M. Moes C.A. Bloom K.R. Olley P.M. Williams W.G. et al.Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum. Analysis of factors influencing right ventricular growth.Circulation. 1980; 61: 428-440Crossref PubMed Scopus (71) Google Scholar,20Waldman J.D. Karp R.B. Lamberti J.J. Sand M.E. Ruschhaupt D.G. Agarwala B. Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections.Ann Thorac Surg. 1995; 59: 933-941Abstract Full Text PDF PubMed Scopus (25) Google Scholar In contrast, the natural history of SC is not well elucidated, whether or not this may progress, persist, or regress.20Waldman J.D. Karp R.B. Lamberti J.J. Sand M.E. Ruschhaupt D.G. Agarwala B. Tricuspid valve closure in pulmonary atresia and important RV-to-coronary artery connections.Ann Thorac Surg. 1995; 59: 933-941Abstract Full Text PDF PubMed Scopus (25) Google Scholar In 1974, Freedom and Harrington21Freedom R.M. Harrington D.P. Contributions of intramyocardial sinusoids in pulmonary atresia and intact ventricular septum to a right-sided circular shunt.Heart. 1974; 36: 1061-1065Crossref Scopus (60) Google Scholar described that SC regressed when the RV pressure declined by means of pulmonary valvotomy and infundibulectomy. However, RVD has been considered as “taboo” after further recognition of the anatomic and physiologic characteristics of SC, especially in those with large SCs at the proximal or mid-portion of the fistula compared with distal and apical.4Williams W.G. Burrows P. Freedom R.M. Trusler G.A. Coles J.G. Moes C.A. et al.Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1991; 101: 222-229Abstract Full Text PDF PubMed Google Scholar,22Najm H.K. Williams W.G. Coles J.G. Rebeyka I.M. Freedom R.M. Pulmonary atresia with intact ventricular septum: results of the Fontan procedure.Ann Thorac Surg. 1997; 63: 669-675Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar, 23O'Connor W.N. Cottrill C.M. Johnson G.L. Noonan J.A. Todd E.P. Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance.Circulation. 1982; 65: 805-809Crossref PubMed Scopus (59) Google Scholar, 24Gittenberger-de Groot A.C. Sauer U. Bindl L. Babic R. Essed C.E. Bühlmeyer K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum.Int J Cardiol. 1988; 18: 243-258Abstract Full Text PDF PubMed Scopus (89) Google Scholar In children with RVDCC, RVD leads to a decrease in the intracavitary RV pressure, which subsequently leads to a decrease in the blood supply to the dependent myocardium. Critical myocardial ischemia is caused by the venous blood from the RV that supplies the myocardium. Therefore, RVD is contraindicated in children with RVDCC. Giglia and colleagues2Giglia T.M. Mandell V.S. Connor A.R. Mayer J.E. Lock J.E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.Circulation. 1992; 86: 1516-1528Crossref PubMed Scopus (158) Google Scholar investigated 26 patients with various degrees of SC and concluded that RVD can be safely performed unless stenosis or occlusion is present in both the left ventricle (LV) and RV, namely, RVDCC. Previous authors have advocated RVD, either performed retrogradely by tricuspid valve excision25Hawkins J.A. Kent Thorne J. Boucek M.M. Orsmond G.S. Ruttenberg H.D. Veasy L.G. et al.Early and late results in pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1990; 100: 492-497Abstract Full Text PDF PubMed Google Scholar or by the placement of an RVOT patch.26Squitieri C. di Carlo D. Giannico S. Marino B. Giamberti A. Marcelletti C. Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum.J Thorac Cardiovasc Surg. 1989; 97: 779-784Abstract Full Text PDF PubMed Google Scholar However, these approaches might also cause or increase myocardial ischemia, because the decompressed RV would then offer increased blood flow during diastole, which would probably cause further distal ischemia, as described by Giglia and colleagues.2Giglia T.M. Mandell V.S. Connor A.R. Mayer J.E. Lock J.E. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.Circulation. 1992; 86: 1516-1528Crossref PubMed Scopus (158) Google Scholar For this reason, RVD is recommended in the absence of large SCs because this may promote growth of the RV.19Patel R.G. Freedom R.M. Moes C.A. Bloom K.R. Olley P.M. Williams W.G. et al.Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum. Analysis of factors influencing right ventricular growth.Circulation. 1980; 61: 428-440Crossref PubMed Scopus (71) Google Scholar,25Hawkins J.A. Kent Thorne J. Boucek M.M. Orsmond G.S. Ruttenberg H.D. Veasy L.G. et al.Early and late results in pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1990; 100: 492-497Abstract Full Text PDF PubMed Google Scholar After transpulmonary valvotomy was introduced,9de Leval M. Bull C. Stark J. Anderson R.H. Taylor J.F. Macartney F.J. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.Circulation. 1982; 66: 272-280Crossref PubMed Scopus (135) Google Scholar RVD by means of surgical pulmonary valvotomy was widely used in the 1990s.8Pawade A. Capuani A. Penny D.J. Karl T.R. Mee R.B.B. Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum.J Card Surg. 1993; 8: 371-383Crossref PubMed Scopus (62) Google Scholar Surgical valvotomy under cardioplegic arrest is the most reliable technique responsible for creating the optimal size of the communication between the RV and pulmonary artery. However, it is important to note that a cardioplegic arrest or even temporary pressure unloading of the RV by cardiopulmonary bypass can pose a risk for myocardial ischemia in patients with a large SC. We experienced a case with a large SC to the LCA and right coronary artery who developed severe LV dysfunction after surgical valvotomy under cardioplegic arrest. The patient recovered 3 days later and subsequently underwent Fontan operation, but inadequate myocardial protection or decrease in RV pressure during cardiopulmonary bypass was considered as a cause of LV dysfunction, and no further RVD was attempted during staged palliation. Our early experience showed a favorable outcome by means of off-pump pulmonary valvotomy with BTS through left thoracotomy.11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar,14Sano S. Ishino K. Kawada M. Fujisawa E. Kamada M. Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum.Ann Thorac Surg. 2000; 70: 1501-1506Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar This is technically a demanding surgery, but it makes sense to patients with SC because it can avoid the use of cardiopulmonary bypass. Moreover, it is technically difficult to create a larger communication; thus, there is a lower chance of myocardial ischemia intraoperatively. How big is communication big enough to decompress RV? Our experience indicates that 2 to 4 mm should be sufficient in neonates.11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar,14Sano S. Ishino K. Kawada M. Fujisawa E. Kamada M. Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum.Ann Thorac Surg. 2000; 70: 1501-1506Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar Successful transcatheter pulmonary valvotomy of the RVOT was introduced by the Toronto team in the early 1990s, and this approach has become the treatment of choice in many centers for patients with a good-sized RV with a membranous atresia of the pulmonary valve.27Justo R.N. Nykanen D.G. Williams W.G. Freedom R.M. Benson L.N. Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn.Cathet Cardiovasc Diagn. 1997; 40: 408-413Crossref PubMed Scopus (92) Google Scholar Indications for catheter valvotomy include mild hypoplasia of the RV, that is, potential biventricular candidate, patent outlet portion, valvular atresia, confluent pulmonary arteries, and no RVDCC.10Ovaert C. Qureshi S.A. Rosenthal E. Baker E.J. Tynan M. Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 1998; 115: 1055-1062Abstract Full Text Full Text PDF PubMed Scopus (100) Google Scholar,28Alwi M. Management algorithm in pulmonary atresia with intact ventricular septum.Catheter Cardiovasc Interv. 2006; 67: 679-686Crossref PubMed Scopus (64) Google Scholar However, the influence of catheter valvotomy in a single ventricle, namely, severe hypoplasia of the RV, is not well described. The advantage of transcatheter valvotomy is that it can avoid coronary ischemia related to cardioplegic arrest during open surgery because RV/LV pressure can be monitored during the procedure. In contrast, there is a disadvantage of perforation of the RV outflow, resulting in cardiac tamponade. Successful perforation rates range from 70% to 100%, and periprocedural mortality in the largest published series to date is consistently 5% to 15%.29Alwi M. Choo K.-K. Radzi N.A.M. Samion H. Pau K.-K. Hew C.-C. Concomitant stenting of the patent ductus arteriosus and radiofrequency valvotomy in pulmonary atresia with intact ventricular septum and intermediate right ventricle: early in-hospital and medium-term outcomes.J Thorac Cardiovasc Surg. 2011; 141: 1355-1361Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar,30Lamers L. Garn B. Ellsworth E. Graziano J.N. Decreased incidence of right-ventricular outflow tract complications using a retrograde snare technique for radiofrequency pulmonary valve perforation.Pediatr Cardiol. 2012; 33: 1275-1280Crossref PubMed Scopus (5) Google Scholar Patients with a tight outlet portion may require surgical intervention, such as Blalock–Taussig (BT) shunt, due to inadequate antegrade pulmonary blood flow or recurrent RVOT obstruction. Figure 1 shows our strategy for RVD. Our strategy includes RVD whenever possible from the first palliation.11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar Transarterial surgical pulmonary valvotomy is performed with a modified BTS at the first palliation. The size of communication between the RV and pulmonary artery is 3 to 4 mm in neonates as for the target of RV/LV pressure less than 1.0, when no SC was present. In patients with SC, the communication is adjusted to approximately 2 mm to achieve RV/LV pressure 1.0 or greater. For patients with a large SC to LCA, the RV/LV pressure is kept greater than 1.0. RVD is postponed until second or third palliation when the patients have a tight/no outlet portion not suitable for pulmonary valvotomy in the neonatal period (Figure 2). Patients are routinely assessed for RV pressure, RV size, and coronary anatomy/flow direction every 3 to 6 months during the first 2 years of life, as we believe that this is a crucial period for patients if the RV pressure, coronary fistula, and RV size change. Multistaged RVD by catheter and surgical intervention is performed, which causes the RV/LV pressure to gradually decrease over time, resulting in SC not demonstrated by RV angiography at the definitive repair (Figure 3). Our experience showed that in 23 patients who had SC at birth, SC regressed after multistaged decompression in 12 patients (52%), including 8 patients whose fistulas completely disappeared.11Kotani Y. Kasahara S. Fujii Y. Eitoku T. Baba K. Otsuki S. et al.A staged decompression of right ventricle allows growth of right ventricle and subsequent biventricular repair in patients with pulmonary atresia and intact ventricular septum.Eur J Cardiothorac Surg. 2016; 50: 298-303Crossref PubMed Scopus (23) Google Scholar Absolute contraindications for RVD include RVDCC (Figure 4). For patients with RVDCC with total coronary atresia with progressive myocardial ischemia, that is, ST change with or without hemodynamic instability and arrhythmia, aortic to RV shunt has been previously described.31Sakurai H. Sakurai T. Ohashi N. Nishikawa H. Aortic to right ventricular shunt for pulmonary atresia with intact ventricular septum and bilateral coronary ostial atresia.J Thorac Cardiovasc Surg. 2018; 156: e17-e20Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar,32Said S.M. Marey G. Greene R. Griselli M. Hiremath G. Aggarwal V. et al.The double shunt technique as a bridge to heart transplantation in a patient with pulmonary atresia with intact septum and right ventricular-dependent coronary circulation.JTCVS Tech. 2021; 7: 216-221Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar This is the most difficult group of patients with PA-IVS, and patients may not survive without intervention. Aortic to RV shunt with either direct anastomosis to RV31Sakurai H. Sakurai T. Ohashi N. Nishikawa H. Aortic to right ventricular shunt for pulmonary atresia with intact ventricular septum and bilateral coronary ostial atresia.J Thorac Cardiovasc Surg. 2018; 156: e17-e20Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar or anastomosis to tricuspid valve32Said S.M. Marey G. Greene R. Griselli M. Hiremath G. Aggarwal V. et al.The double shunt technique as a bridge to heart transplantation in a patient with pulmonary atresia with intact septum and right ventricular-dependent coronary circulation.JTCVS Tech. 2021; 7: 216-221Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar prevents coronary ischemia and works as a bridge to heart transplantation or the Fontan pathway. For those without any evidence of myocardial ischemia, even RVDCC with total coronary atresia, the indication for aortic to RV shunt is not well discussed. We had a patient with total coronary atresia (Figure 4, C and D), who did not have any signs of myocardial ischemia in the first weeks of life. The decision was made to perform only a modified BT shunt. The patient tolerated the procedure well and went home, but died of respiratory infection 3 months later. However, we do not have a clear answer that this patient deteriorated because of myocardial ischemia due to an untreated RVDCC. Patients without an outlet portion of the RV were considered to have difficulty decompressing the RV even without RVDCC. We included 6 patients without an outlet portion of the RV and 5 patients who underwent Fontan completion, except 1 with 1+1/2 ventricular repair. Patients with a huge SC to LCA are at a high risk of myocardial ischemia, and RVD is considered only if the shunt flow can be restricted by surgical ligation of the fistula. Ligation of the fistula has been described by Foker and colleagues.33Foker J.E. Setty S.P. Berry J. Jain P. Catton K. Gittenberger-de-Groot A.C. et al.Treatment of right ventricle to coronary artery connections in infants with pulmonary atresia and intact ventricular septum.J Thorac Cardiovasc Surg. 2008; 136: 749-756Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar We ligate the fistula when the patients present with a huge SC without aortocoronary atresia. Coronary/RV angiography with a balloon occlusion test of SC can be considered in cases of coronary ischemia brought about by the ligation of the fistula due to atresia or stenosis at the proximal portion of the coronary artery where SC drains into. However, this does not always exclude the possibility of coronary ischemia upon ligation of the SC as a balloon occlusion test is often technically difficult and unreliable. For the technical aspect of fistula ligation, the communication between the coronary artery and RV is usually well identified from the epicardial side as its caliber changes. An epicardial echocardiogram also helps to show the communication site. A pair or 2 interrupted Prolene sutures with a pledget are used to close the communication. Ligation is usually performed at the Glenn shunt, as we think patients post-BTS are hemodynamically unstable due to parallel circulation, and to avoid a sudden change in coronary flow by the ligation, which may be influenced by the physiology of the BT shunt. For patients undergoing Fontan operation, we attempt to ligate the SC or RVOT reconstruction/stenting RVOT whenever technically feasible to achieve “decompression Fontan.” The indication of decompression Fontan includes no RVDCC, similar to the indication for RVD. However, careful attention should be given to patients with a large SC to LCA who do not assess the details of coronary/SC anatomy with a balloon occlusion test, as it cannot exclude the possibility of coronary ischemia when RV is decompressed. There is no universal agreement as to whether RV should be decompressed in patients with diminutive RV who are undergoing Fontan palliation, because concerns still remain about myocardial ischemia when there is a sudden change in the coronary flow upon the RVD, especially in those with a large SC and stenosis at the proximal coronary arteries. The long-term effects of RVD in Fontan circulation are still unknown, and careful follow-up is necessary.Figure 2Right ventriculography in patient with a tight/no outlet portion. Right ventriculography shows (A) rudimentary tight outlet portion (arrow) and (B) no outlet portion, that is, bipartite RV (arrow).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Staged decompression of the RV and change in SC. A, Day 9—SC seen and RV/LV pressure was 1.69. B, Three months of age—SC still seen, RV/LV pressure decreased to 1.06. C, Two years after 1+1/2 ventricular repair—no SC demonstrated, RV/LV pressure was 0.45.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 4RV-dependent coronary circulation in 2 patients. Patient 1 had a monopartite RV (A) with atresia of proximal segment of the LCA (B). Patient 2 had had a monopartite RV (C) with atresia of proximal segments of both coronary arteries (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT) There is no doubt that RVD facilitates RV growth in patients with a decent RV size without a large SC. Our experience demonstrated that it would be safe to decompress the RV if the RV pressure is kept at least equal to the LV pressure. Further decompression can be achieved by multistaged intervention, because this gentle, stepwise decompression does not seem to compromise the coronary perfusion. Although the exact mechanisms are unknown, we believe that the regression of SC will reduce the risk of coronary-related complications. Therefore, a long-term follow-up is necessary." @default.
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