Ventricular Defect Research Articles

Two-Patch Technique for Postinfarction Inferoposterior Ventricular Septal Defect

We describe 4 patients with postinfarction inferoposterior ventricular septal defect treated by the two-patch technique for infarct exclusion operation. The ventricular septal defects were closed using two bovine pericardial patches as follows. The septal patch was sutured to the noninfarcted septum covering the defect, and the free wall patch was sutured to the endocardium adjacent to the posterior papillary muscle. The two patches were sutured together and all infarcted areas were excluded from the left ventricular pressure. This technique seems to be useful in specific circumstances, such as when the ventricular defect is located in the inferoposterior septum.

All Cardiac Right Ventricular Outpouches Are Not Created Equal

Congenital right ventricular diverticula and aneurysms are rare. Clinically, a diverticulum is difficult to distinguish from an aneurysm. Four diverse right ventricular cameral defects or right ventricular outpouches (RVOs) are described together with the management of each. Surgery may be necessary if an RVO has thin walls. However, if an RVO is composed of uniform thick contractile walls, conservative follow-up care likely is appropriate.

H020 Chronic expression of osteopontin in cardiomyocytes led to dilated cardiomyopathy

Osteopontin (OPN) is not expressed in healthy heart while, its expression is dramatically increased in cardiomyocytes and inflammatory cells during cardiomyopathies and heart failure. However its role in the development of heart diseases is not known. To understand whether OPN is involved in cardiomyopathy, we created a transgenic mouse (MHC-OPN) expressing recombinant OPN (rOPN) specifically in cardiomyocytes using aMHC promoter-directed OPN expression and tTA technology. In these mice, rOPN expression could be regulated by doxycyclin oral administration. After birth, MHC-OPN young mice were phenotypically indistinguishable from their littermate controls, but most of them died early between the 8 th and 15 th weeks after birth with a half life of 12 weeks. However, less than 10 % MHC-OPN mice survived and were still alive 30 weeks after birth. Inhibition of recombinant OPN expression by doxycyclin at the beginning of T cell infiltration (5 weeks after birth) or when DCM was initiated (11 weeks after birth) reduced myocarditis and thus avoided the early death. Electrocardiography demonstrated atrio-ventricular and intra ventricular defects. Moreover, echocardiography showed left ventricular dilation without hypertrophy and a systolic dysfunction, as indicated by reduced left ventricular fractional shortening (control mice: 29,8 ± 2,0 %, n = 13 ; and MHC-OPN mice: 13,7 ± 4,0 %, n = 5 ; T test p<0.05). In vitro histology confirmed that mice died because of a dilated cardiomyopathy associated with a strong fibrosis. By immunohistology, we demonstrated that OPN expression in cardiomyocytes induced an important cell infiltration including some macrophages and a large number of fibroblasts and activated CD4+ and CD8+ T cells. All together these experiments suggested that chronic OPN expression is required for DCM development inducing Tcell activation and thus a chronic myocarditis resulting in the dilated cardiomyopathy.

The Evolving Roles of Nuclear Cardiology

The use of cardiac imaging modalities has grown steadily, and cardiac nuclear studies constitute a large part of this number. Nuclear Cardiology is often mistakenly considered a synonym of myocardial perfusion imaging (MPI), but has broader applications, including metabolic imaging, innervation imaging, among other technologies. MPI has been a powerful diagnostic and prognostic tool in the assessment of patients for known or suspected CAD for decades, and is now increasingly used for the evaluation of the anti-ischemic effects of various therapies, according to changes in left ventricular perfusion defect size defined by sequential MPI. Neuronal dysfunction identified with iodine-123-metaiodobenzylguanidine may give information on prognosis in different disease conditions, such as after myocardial infarction, in diabetes and dilated cardiomyopathy. Molecular imaging may identify the predominant cellular population in the atherosclerotic plaque and help predict the likelihood of clinical events. Therefore, although its usefulness is well established, Nuclear Cardiology remains a moving science, whose roles keep in pace with evolving clinical needs and expectations.

Abstract 6131: Regadenoson Induces Perfusion Defects of Comparable Extent and Severity as Adenosine: A Quantitative Analysis from the ADVANCE 2 Trial

The quantified total and ischemic left ventricular (LV) perfusion defect size (PDS) is an important determinant of subsequent patient outcome. Therefore it is important to determine whether a new stressor agent induces similar perfusion abnormalities as a standard stressor agent such as adenosine. The ADVANCE 2 was a double-blind randomized trial comparing image results in patients undergoing standard adenosine (Ad) SPECT who were then randomized to either a second Ad SPECT (N=260) or SPECT following pharmacologic stress with Regadenoson (Reg) (N=493). All raw data of gated SPECT images (2 sets/patient) were reconstructed and reoriented in a standard fashion. Quantitative SPECT was performed using a previously validated automated program which determined the extent and severity of left ventricular (LV) perfusion defect size (PDS) and the extent of scintigraphic ischemia. PDS severity was defined as mild, moderate or severe based on the relation to normal pixel count activity (&gt;50%, 26–50%, and 0–25%, respectively). Quantification was performed blinded to randomization and image sequence. Baseline perfusion results were similar (p&gt;.05) between patients randomized to Ad vs Reg with respect to total (10.2±14.8 vs 11.5±15.8), ischemic (4.2±7.8 vs 4.6±8.9) and scar (6.0±10.7 vs 6.9±11.3) LV PDS. Results are summarized in the table below. Linear regression analysis showed a close correlation between Ad vs Reg for total (r=0.97, p&lt;.0001) and ischemic (0.94, p&lt;.0001) LV PDS. In this large database, quantitative SPECT analysis demonstrates that Regadenoson induces a comparable extent and severity of perfusion abnormality as induced with standard adenosine stress. This implies that similar prognostic information should be obtainable with Regadenoson stress as observed with adenosine.

Enhanced recovery of mechanical function in the canine heart by seeding an extracellular matrix patch with mesenchymal stem cells committed to a cardiac lineage

The need to regenerate tissue is paramount, especially for the heart that lacks the ability to regenerate after injury. The urinary bladder extracellular matrix (ECM), when used to repair a right ventricular defect, successfully regenerated some mechanical function. The objective of the current study was to determine whether the regenerative effect of ECM could be improved by seeding the patch with human mesenchymal stem cells (hMSCs) enhanced to differentiate down a cardiac linage. hMSCs were used to form three-dimensional spheroids. The expression of cardiac proteins was determined in cells exposed to the spheroid formation and compared with nonmanipulated hMSCs. To determine whether functional calcium channels were present, the cells were patch clamped. To evaluate the ability of these cells to regenerate mechanical function, the spheroids were seeded on ECM and then implanted into the canine heart to repair a full-thickness right ventricular defect. As a result, many of the cells spreading from the spheroids expressed cardiac-specific proteins, including sarcomeric alpha-actinin, cardiotin, and atrial natriuretic peptide, as well as the cell cycle markers cyclin D1 and proliferating cell nuclear antigen. A calcium current similar in amplitude to that of ventricular myocytes was present in 16% of the cells. The cardiogenic cell-seeded scaffolds increased the regional mechanical function in the canine heart compared with the unmanipulated hMSC-seeded scaffolds. In addition, the cells prelabeled with fluorescent markers demonstrated myocyte-specific actinin staining with sarcomere spacing similar to that of normal myocytes. In conclusion, the spheroid-derived cells express cardiac-specific proteins and demonstrate a calcium current similar to adult ventricular myocytes. When these cells are implanted into the canine heart, some of these cells appear striated and mechanical function is improved compared with the unmanipulated hMSCs. Further investigation will be required to determine whether the increased mechanical function is due to a differentiation of the cardiogenic cells to myocytes or to other effects.

Atrial and ventricular lead insulation defects with resulting inappropriate shocks and end-of-life of the ICD pulse generator in a young bodybuilder with congenital long-QT-syndrome

We report on a 19-year-old male with a congenital Long-QT syndrome who was admitted to our hospital because of insulation defects of both atrial and ventricular ICD leads resulting in inappropriate delivered shocks and a non-responding pulse generator during telemetrical evaluation. The insulation defects led to multiple arc marks within the ICD pocket and there was a short circuit between denuded leads and the electrically active pulse generator. The high current flow generated sufficient heat to damage several circuits of the generator. A new ICD system was implanted successfully and the postoperative course has been remained uneventful for almost 6 years.

Clinical analysis of surgical procedures and outcomes for corrected transposition of great arteries with heart anomaly

To determine the outcome of anatomically corrective repair and traditional repair of corrected transposition of great arteries (c-TGA) with heart anomaly. From April 2002 to December 2006, nineteen patients including fourteen male and five female with c-TGA, underwent operations, age ranged from 2 to 22 years old and weight ranged from 10 to 48 kg. Fifteen of them received anatomically corrective repair and the other four received traditional repair. Eighteen patients were referred to SLL (segmental anatomy) in situs solitus while fifteen of them with levocardia and three with dextrocardia. One patient was referred to IDD (segmental anatomy) in situs inversus with levocardia. Associated cardiac lesions included ventricular defect in eighteen patients, double outlet of right ventricle in one patient, pulmonary stenosis in seventeen patients and pulmonary hypertension in two patients. The operative procedures to anatomically correct atrioventricular discordance included an atrial switch plus a ventricle-arterial switch. The atrial switch was performed using the modified Senning procedure (n=13), Senning procedure (n=1) and Mustard procedure (n=1). The ventricle-arterial switch was performed using a Rastelli procedure (n=13) or an arterial switch (n=2). The patients underwent Mustard and Rastelli procedure had received bi-direct Gleen shunt due to postoperative high pressure of superior vena cava. Three patients underwent traditional cardiac repair because of small ventricular septal defect and one patient was reoperated to undergo traditional cardiac repair because of left ventricular failure after received anatomically corrective repair. In the patients received anatomically corrective repair, there was one early operative death received a modified Senning atrial switch and an arterial switch. The cause of death was acute myocardial failure due to imperfect coronary transfer. The postoperative complications included severe low cardiac output syndrome (n=1), temporary atrioventricular block (n=1) and thorax cavity fluidify (n=1). The survivors were followed up for 6 months to 4 years. All were sinus cardiac rhythm and in NYHA class I or II. There was no death in the patients received traditional repair. Four patients were followed up for 1 year. Three patients were in NYHA I or II class and one patient in class II. Anatomically corrective repair of c-TGA can be performed with good operative survival and intermediate-term outcome. The patients with good right ventricular function and well developed tricuspid valve who were difficult to undergo anatomically corrective repair might be fit to receive traditional repair.

Syncope Due to Paroxysmal Atrioventricular Block in a Patient With Systemic Sclerosis: A Case Report

A 79-year-old woman with systemic sclerosis was admitted to our hospital because of syncope. On admission, electrocardiogram showed progression of intraventricular conduction defect. Chest radiograph showed marked cardiomegaly. Echocardiogram revealed deterioration of left ventricular systolic function. We suspected progressive myocardial disease with Stokes-Adams attack. When we were preparing a temporary pacemaker, paroxysmal atrioventricular block with asystole for 15 seconds and convulsion occurred. Electrophysiological study showed His-ventricular block and sinus node dysfunction. A permanent pacemaker was implanted. In systemic sclerosis, progression of ventricular conduction defect may warrant prompt electrophysiological study and prophylactic pacemaker implantation.

Fetal Bifid Left Ventricular Diverticulum Presenting With Ventricular Ectopy

We report the diagnosis and outcome of a fetus with an unusual left ventricular wall defect. A 34-week fetus presented for echocardiographic evaluation because of an irregular cardiac rhythm. Fetal echocardiography revealed frequent ventricular ectopy and an “accessory” ventricular chamber diagnosed as a left ventricular diverticulum (LVD). The ectopy resolved in utero but recurred after delivery. Angiography revealed a “bifid” LVD. Inderal therapy suppressed the ventricular ectopy. The association between ventricular ectopy and LVD is unusual, but the prognosis of LVD even with arrhythmia is excellent.

Myocardial Pitx2 Differentially Regulates the Left Atrial Identity and Ventricular Asymmetric Remodeling Programs

The Pitx2 gene regulates left-right (L/R) asymmetrical cardiac morphogenesis. Constitutive Pitx2 knock out (ko) mice die before birth and display, among other defects, right atrial isomerism, atrial and ventricular septal defects, and double outlet right ventricle. The myocardial role of the gene has not been dissected. In particular, how Pitx2 regulates the differential L/R cardiac identity program is not clear. Additionally, the relation between Pitx2 ko ventricular defects and the gene expression pattern is not understood. In this article we analyze Pitx2 myocardial function during mouse heart development. By in situ hybridization analysis we show that myocardial Pitx2 expression delineates the remodeling of the left atrioventricular canal, the inner curvature, the ventral part of the interventricular ring, and the ventral portion of the right and left ventricle. By genetic analysis using an allelic series of Pitx2 mutants, among which a myocardial specific ko (ko(myo)) we show it has a crucial role in this process. Pitx2 ko(myo) mutants survive to adulthood, when they present strong cardiac morphological and functional defects. Confocal analysis of embryonic Pitx2 ko(myo) hearts reveals delayed cardiomyocyte development in the ventricular but not in the atrial Pitx2 null areas. Conversely, selective left atrial BMP10 mRNA downregulation which normally occurs at fetal stages is not found in the Pitx2 ko(myo) mice. This is the first evidence for distinct Pitx2 action in mediating L/R atrial identity and asymmetrical ventricular remodeling.

急性心筋梗塞後の左室心筋欠損に対しパッチ接着術を行った 1 症例

急性心筋梗塞後の左室心筋欠損に対しパッチ接着術を行った 1 症例

A complex clinical case linking genetic disorders and infectious disease

Dr. Leopoldo Di Iorio: On the basis of this clinical presentation, four main hypotheses were considered and simultaneously investigated. First, a cardiogenic shock was considered (subjects with Down’s syndrome can often have multiple cardiac abnormalities, such as atrium-ventricular channel, ventricular or atrial defects, Fallot’s tetralogy), but echocardiography excluded significant alterations of the parietal kinesis or structural abnormalities. Second, the presence of genital mycosis, marked hypocalcaemia, several symptoms indicative of acute adrenocortical deficiency (ions disorders, hypoglycaemia, hypotension, bradycardia) were all suggestive of type I polyglandular autoimmune syndrome (PAS, a syndrome characterised by muco-cutaneous candidiasis, hypoparathyroidism and Addison’s disease) [1]. However, results from specific laboratory tests (presence of hypercortisolaemia associated with normal levels of ACTH and PTH) definitely ruled out this clinical hypothesis. Third, we excluded a neurogenic shock by performing a brain CT, which did not document signs of ischaemic or haemorrhagic lesions, but found an enlargement of the IV ventricle associated with congenital olivary cerebello-pontine atrophy.

First-Trimester Use of Selective Serotonin-Reuptake Inhibitors and the Risk of Birth Defects

About 10% of pregnant women experience symptoms of clinical depression. Many of them receive antidepressants, particularly selective serotonin-reuptake inhibitors (SSRIs). While SSRIs are not major teratogens, recent reports cite an increased risk of birth defects in general and of congenital heart defects, craniosynostosis, and omphalocele in connection with SSRI treatment. The investigators examined associations between maternal SSRI treatment in the first trimester of pregnancy and the risk of birth defects in mothers of 9849 infants with, and 5860 without birth defects who participated in the Slone Epidemiology Center Birth Defects Study. Participants completed a standardized interview in person or by phone within 6 months after delivery. This analysis was limited to women who conceived in the years 1993 to 2004. Infants with chromosomal defects or metabolic disorders were excluded. In contrast to other reported studies, taking SSRIs was not associated with a significantly increased risk of craniosynostosis, omphalocele, or heart defects. The odds ratio (OR) for cardiac defects overall was 1.2, with a 95% confidence interval (CI) of 0.9 to 1.6. Maternal use of sertraline was significantly associated with omphalocele (OR, 5.7; 95% CI, 1.6-20.7) and septal cardiac defects (OR, 2.0; 95% CI, 1.2-4.0). Taking paroxetine carried a significantly increased risk of right ventricular outflow tract obstruction defects (OR, 3.3; 95% CI, 1.3-8.8). No increase in risk was noted when pregnant women took other SSRIs or other types of antidepressants. Maternal use of SSRIs in early gestation was not associated with an overall increase in risk of cardiac defects in this study. While individual drugs of this class may confer an increased risk of specific defects, these abnormalities are rare and the absolute risk level is small.

Replacing damaged myocardium

Heart failure survival after diagnosis has barely changed for more than half a century. Recently, investigation has focused on differentiation of stem cells in vitro and their delivery for use in vivo as replacement cardiac contractile elements. Here we report preliminary results using mesenchymal stem cells partially differentiated to a cardiac lineage in vitro. When delivered to the canine heart on an extracellular matrix patch to replace a full-thickness ventricular defect in vivo, they improve regional mechanical function. The delivered cells were also tracked, and some became myocytes with mature sarcomeres.

Magnetische Navigation in der elektrophysiologischen Diagnostik und Therapie

Electrophysiological stimulation and ablation is currently performed with manually deflectable catheters of different lengths and curves. Disadvantages of conventional therapy are catheter stiffness, limited local stability, risk of dislocation or perforation, and reduced tissue contact in regions with difficult access. Fluoroscopy to control catheter movement and position may require substantial radiation times. Magnetic navigation was first applied for right heart catherization in congenital heart disease in 1991; the first electrophysiological application took place in 2003. Today, an ablation electrode with small magnets is aligned in the patient's heart by two external magnets positioned at both sides of the thorax. Antegrade and retrograde movement of the distal catheter tip are performed via an external device on the patient's thigh. Three-dimensional MRI scans acquired before intervention can be merged with electroanatomical reconstruction, leading to further reductions of radiation burden. During treatment of supraventricular tachyarrhythmias high local precision of magnetically guided catheters, good local stability, and a substantially reduced radiation time have been reported. First applications in ventricular tachyarrhythmias and complex congenital cardiac defects indicate a comparable effect. Limitations of this therapy are the application in left atrial procedures (open irrigated ablation catheters not yet available), difficult transaortic retrograde approach (high lead flexibility), and the considerable costs. Magnet-assisted navigation is feasible during percutaneous coronary interventions of tortuous coronary arteries and in positioning guidewires in coronary sinus side branches for resynchronisation therapy. Future applications will be complex left atrial procedures, magnetically guided cardiac stem cell therapy, local drug application, and extracardiac vessel therapy.

Endoscopic access closure for direct implantation of valved stents.

The off-pump trans left ventricular approach provides an alternative option for insertion of stented aortic valves of any size for endovascular replacement. One of the key steps in this procedure is the repair of the ventricle after catheter withdrawal. This study evaluates the reliability of a new device for sutureless and quick repair of the left ventricle access. The Amplatz-nitinol occluder consists of two square heads that squeeze the ventricle wall between them thus sealing the ventricular defect. In four adult pigs weighing 55 kg, left thoracoscopy was performed to open the pericardium and visualise the cardiac apex. Following a heparin injection (100 U/kg) under ICUS and fluoroscopic control, we inserted a 30 F sheath into the epigastric area through the cardiac apex up into the left ventricle thus simulating the approach for an off-pump aortic valve replacement. The sheath was then removed and the ventricle closed with the occluder. Animals were followed-up for three hours; the haemodynamics and pericardial bleeding were recorded. The animals were then sacrificed and the gross anatomy of the heart was examined. The device was successfully deployed in four animals in less than one minute. ACT was above 200 seconds in all cases. All animals survived the procedure with a mean arterial pressure of 50 +/- 15 mm Hg. Bleeding during deployment was 80 +/- 20 ml and over a 3 hour period was 800 +/- 20 ml. Examination of the gross anatomy examination demonstrated the correct positioning of the device. The occluder is easy to use and the procedure is feasible and reproducible. However, the occluder design requires technical improvements in order to reduce bleeding before it can be used clinically.

Endoscopic access closure for direct implantation of valved stents.

The off-pump trans left ventricular approach provides an alternative option for insertion of stented aortic valves of any size for endovascular replacement. One of the key steps in this procedure is the repair of the ventricle after catheter withdrawal. This study evaluates the reliability of a new device for sutureless and quick repair of the left ventricle access. The Amplatz-nitinol occluder consists of two square heads that squeeze the ventricle wall between them thus sealing the ventricular defect. In four adult pigs weighing 55 kg, left thoracoscopy was performed to open the pericardium and visualise the cardiac apex. Following a heparin injection (100 U/kg) under ICUS and fluoroscopic control, we inserted a 30 F sheath into the epigastric area through the cardiac apex up into the left ventricle thus simulating the approach for an off-pump aortic valve replacement. The sheath was then removed and the ventricle closed with the occluder. Animals were followed-up for three hours; the haemodynamics and pericardial bleeding were recorded. The animals were then sacrificed and the gross anatomy of the heart was examined. The device was successfully deployed in four animals in less than one minute. ACT was above 200 seconds in all cases. All animals survived the procedure with a mean arterial pressure of 50 +/- 15 mm Hg. Bleeding during deployment was 80 +/- 20 ml and over a 3 hour period was 800 +/- 20 ml. Examination of the gross anatomy examination demonstrated the correct positioning of the device. The occluder is easy to use and the procedure is feasible and reproducible. However, the occluder design requires technical improvements in order to reduce bleeding before it can be used clinically.

Holoprosencephaly at 9 Weeks 6 Days in a Triploid Fetus

Received October 29, 2006, from the Fetal Medicine Center (W.S., I.L.), Department of Obstetrics and Gynecology, and Cytogenetics Laboratory (C.B.), Clinica Las Condes, Santiago, Chile. Manuscript accepted for publication November 2, 2006. We thank Filiberto Guerra, MD, for performing the postmortem examination and Medison Co, Ltd (Seoul, Korea) for an equipment loan. This work was supported by Sociedad Profesional de Medicina Fetal “Fetalmed” Limitada, Chile. Address correspondence to Waldo Sepulveda, MD, Fetal Medicine Center, Clinica Las Condes, Casilla 208, Santiago 20, Chile. E-mail: fetalmed@yahoo.com Abbreviations 3D, 3-dimensional oloprosencephaly is a rare intracranial abnormality arising from failure of the prosencephalon to cleave during early embryonic life, which results in different degrees of lateral ventricular fusion and facial defects.1 This condition is classified according to brain structures into lobar, semilobar, and alobar, the latter being the most severe form.1 In this report, we describe one of the earliest prenatally diagnosed cases of holoprosencephaly, in which 3-dimensional (3D) imaging was used to assist in the diagnosis. A 27-year-old woman, gravida 4, para 2, spontaneous aborta 1, was referred at the menstrual age of 9 weeks 6 days for sonographic evaluation because of a suspected fetal brain anomaly detected at the dating scan. Her medical and obstetric histories were unremarkable, and the current pregnancy was otherwise uncomplicated. Transvaginal sonographic evaluation at referral revealed a live singleton fetus with a crown-rump length of 34 mm and a large anechoic area behind the forehead (Figure 1). With the assistance of 3D eXtended Imaging technology (Accuvix XQ; Medison Co, Ltd, Seoul, Korea), including Multislice View and Volume CT, multiple sections of the fetal brain were obtained, which helped in selecting the best plane to show the single ventricular cavity and fused thalami (Figure 2). A follow-up scan 1 week later revealed a 45-mm live fetus with alobar holoprosencephaly, midfacial hypoplasia, small orbits, a suspected small omphalocele, and an increased nuchal translucency thickness of 5.6 mm (Figure 3). The placenta appearance was normal. Trophoblast culture obtained from chorionic villus sampling revealed a 69,XXY karyotype. The patient miscarried at 13 weeks. Pathologic examination of the fetus and placenta confirmed the prenatal diagnosis (Figure 4).

Arterial switch operation in patients with Taussig–Bing anomaly — influence of staged repair and coronary anatomy on outcome☆

This study evaluated the results of arterial switch operation and closure of ventricular defects (ASO+VSDc) for double outlet right ventricle with sub-pulmonary ventricular septal defect (Taussig-Bing anomaly). Between 1988 and 2003, 33 patients (25 male, 76%) with Taussig-Bing anomaly underwent ASO+VSDc (median age 39 days, 1 day-2.1 years). The relationship of the great arteries was antero-posterior (Group I, n=19) or side-by-side (Group II, n=14). Coronary anatomy (Yacoub's classification) was exclusively type A or D in Group I and predominantly type D or E in Group II (64%). Incidence of sub-aortic obstruction and aortic arch obstruction was similar in Group I and II (37% vs 57%, p=0.25 and 84% vs 79%, p=0.98, respectively). Twenty-five patients (76%) had one-stage total correction. Risk factors were analysed using multivariable analysis. Follow-up was complete (median interval of 6.2 years; range, 0.6-15.2 years). There were three early (9%) and one late death. Actuarial survival was 88+/-6% at 1 and 10 years. There were two early and four late re-operations. Freedom from re-operation was 90+/-5% and 75+/-9% at 1 and 10 years. Four patients required cardiological re-interventions. Freedom from re-intervention at 5 and 10 years was 79+/-9%. On multivariable analysis, complex coronary anatomy (type B and C) was a risk for early mortality (p<0.001) but all other anatomical variables and staged strategy did not influence early or actuarial survival. The ASO+VSDc approach can be applied to Taussig-Bing anomaly with acceptable mortality and morbidity and it is the procedure of choice at our institution. Anatomical variables did not influence outcomes with this strategy. A staged strategy is still appropriate in complex cases.