Tetralogy Of Fallot With Pulmonary Atresia Research Articles

Staged vs Complete Repair in Tetralogy of Fallot With Pulmonary Atresia

BackgroundWe sought to compare outcomes for infants with tetralogy of Fallot with pulmonary atresia (TOF/PA) and confluent pulmonary arteries who underwent staged or primary complete surgical repair. MethodsThis retrospective study included infants undergoing initial surgical intervention between 0 and 60 days of age with TOF/PA without aortopulmonary collaterals from 2009 to 2018 at 20 centers. The primary outcome was days alive and out of the hospital in the first year of life (DAOH365). Secondary outcomes were mortality at 1 year of age and a composite major complication outcome. Multivariable modeling with generalized estimating equations were used to compare outcomes between groups. ResultsOf 221 subjects, 142 underwent staged repair and 79 underwent primary complete repair. There was no significant difference in median DAOH365 between the staged and primary repair groups (317 days [interquartile range, 278-336] vs 338 days [interquartile range, 314-348], respectively; adjusted P = .13). Nine staged repair patients (7%) died in the first year of life vs 5 primary repair patients (6%; adjusted odds ratio, 1.00; 95% CI, 0.25-3.95). At least 1 major complication occurred in 37% of patients who underwent staged repair vs 41% of patients who underwent primary complete repair (P = .75), largely driven by the need for unplanned cardiac reinterventions. ConclusionsFor infants with TOF/PA with confluent pulmonary arteries, a surgical strategy of staged or primary complete repair resulted in statistically similar DAOH365, early mortality, and morbidity.

Neonatal or infantile palliation for Tetralogy of Fallot with or without pulmonary atresia: Modified Blalock–Taussig shunt versus restrictive right ventricle to pulmonary artery connection. A 20-year experience

To compare mortality, morbidity and efficiency of modified Blalock–Taussig shunt (mBTS) versus restrictive right ventricle to pulmonary artery connection (RVPAc) in patients with Tetralogy of Fallot (TOF) and TOF with pulmonary atresia (TOF-PA). We performed a monocentric retrospective review of 198 patients (93 TOF and 105 TOF-PA) who had palliation between 1996 to 2016 in their first year of life. Patients with Major aorto-pulmonary collaterals were excluded. Median follow-up was 11.0 [95% CI: 5.8–16.2] years after palliation. Five patients were lost at the follow-up. Ninety-five patients underwent mBTS, and 103 had an RVPAc. Nakata index before palliation was 129 (80–170) mm 2 /m 2 in mBTS group and 100 (71–146) mm 2 /m 2 in RVPAc group ( P = 0.001). Weight at palliation was similar between mBTS and RVPAc patients ( P = 0.1). Age at the palliation was 12 days (7–24.7) in RVPAc group and 21 days (8–87.5) in mBTS group ( P = 0.0002). One hundred and seventy-five patients underwent repair and 5 are waiting for it. Among them 39 necessitated a repair with a pulmonary valve conduit (23 mBTS and 16 RVPAc P = 0.004). Two patients were unsuitable for repair. Eleven patients died before repair (5.7%). There was no difference in mortality rate regarding the type of palliation ( P = 0.92) or the anatomical group ( P = 0.76). Interstage reintervention before repair was n = 37 (20%). Thirty-three were surgical and four were percutaneous. No difference in reintervention rate between RVPAc or mBTS regarding the age, the weight at palliation or the anatomical group ( P = 0.56). The delay between the palliation and the complete repair was shorter in RVPAc [12.3 (6.3–37.9) months versus 6.5 (4.9–10.3) months for mBTS ( P < 0.01)]. RVPAc provide a better and a faster pulmonary artery growth in our population. It led us to limit the use of RVPAc conduit at the repair. We found no significant difference between mBTS and RVPAc for mortality and interstage reintervention.

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions

BackgroundThe reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions. MethodsThe TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Québec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions. ResultsWe included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations. ConclusionsThe anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.

Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management.

Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus. In more severe cases, however, the ductus arteriosus is absent, and the sole source of pulmonary blood flow is via major aortopulmonary collateral arteries (MAPCAs). The variability in the origin, size, number, and clinical course of these MAPCAs adds to the complexity of these patients. Currently, the goal of management is to establish pulmonary blood flow from the right ventricle (RV) with RV pressures that are ideally less than half of the systemic pressure to allow for closure of the ventricular septal defect. In the long term, patients with ToF-PA are at higher risk for reinterventions to address pulmonary arterial or RV-pulmonary artery conduit stenosis, progressive aortic root dilation and aortic insufficiency, and late mortality than those with less severe forms of ToF.

Early and long-term results of biventricular repair of tetralogy of Fallot with or without pulmonary atresia

After complete repair, the long-term outcome difference between tetralogy of Fallot without pulmonary atresia (TOF) and tetralogy of Fallot with pulmonary atresia (TOF-PA) without MAPCAs is scarcely described. We performed a retrospective study on patients with a diagnostic of TOF and TOF-PA without MAPCAs who underwent complete repair between 1995 and 2016 in our center. We reviewed 960 patients, divided into two main anatomical groups: TOF ( n = 822, 85.7%), and patients with TOF-PA ( n = 138, 14.3%). Concerning surgical technique repair, three groups were distinguished: TOF without transannular patch ( n = 410, 42.7%), TOF/TOF-PA with transannular patch ( n = 494, 51.5%), and TOF-PA with RVOT tube ( n = 56, 5.8%). After complete repair, overall mortality was 11 (1.1%), five of them occurred before discharge (0.5%). Survival at 10 years after repair was 99.1% for TOF and 96.8% for TOF-PA ( P = 0.036). The median follow-up was 10.0 [95% CI: 6.3–14.1] years after repair. One hundred and sixty-three patients (17%) underwent reintervention (surgery or percutaneous interventions). Freedom from reintervention at 10 years after repair was 92.3%, 82.1%, and 48.7% for TOF without transannular patch, TOF/TOF-PA with transannular patch and TOF-PA with RVOT tube respectively ( P < 0.001 between each groups). In addition, reintervention rate was significantly different between TOF with transannular patch and TOF-PA with pulmonary trunk ( P < 0.001). In multivariate analysis, the absence of pulmonary trunk ( P = 0.013), pulmonary artery branches stenosis ( P = 0.001), prior palliative surgery ( P < 0.001), and reintervention before discharge ( P < 0.001) were reintervention risk factors. The initial anatomy of pulmonary trunk and branches strongly influences the risk of reintervention in TOF with or without PA. Avoid palliative surgery by directly performing a complete repair in the neonatal period would be a strategical option to improve the prognosis of these patients.

Outcome and Associated Findings in Individuals with Pre- and Postnatal Diagnosis of Tetralogy of Fallot (TOF) and Prediction ofEarly Postnatal Intervention.

The aim of our retrospective evaluation was to compare the outcome of patients with prenatal and postnatal diagnosis of Tetralogy of Fallot (TOF) and to analyze prenatal echocardiographic parameters predicting intervention within 30 days postnatal. We evaluated 142 patients in our pediatric heart center and prenatal diagnosis center and prenatal practice Praenatal plus in Cologne between 01/08-06/16. Within the prenatal diagnosis group, 6/74 fetuses (8.1 %) had TOF with pulmonary atresia (TOF-PA), and 6 (8.1 %) had absent pulmonary valve syndrome (TOF-APVS). 14 (18.9 %) had an abnormal karyotype including 9/14 (64.3 %) with microdeletion 22q11.2. 25 (33.8 %) had extracardiac malformation. 4 (5.4 %) had agenesis of ductus arteriosus (DA), 22 (29.7 %) had right aortic arch (RAA) and 9 (12.2 %) had major aortopulmonary collateral arteries (MAPCAs). Within the postnatal diagnosis group, no patient had TOF-PA, 4/68 (5.9 %) had TOF-APVS. 12 (17.6 %) had extracardiac malformations, 9 (13.2 %) had an abnormal karyotype including 2/9 with microdeletion 22q11.2. 10 (14.7 %) had RAA, 9 (13.2 %) had MAPCAs. There were no cases with agenesis of DA. Increasing z-score values of the left/right pulmonary artery (LPA/RPA) prenatally were associated with a lower probability for early postnatal intervention (RPA: p = 0.017; LPA: p = 0.013). Within the prenatal diagnosis group, 12 of 41 (29.3 %) live-born patients with follow-up and intention to treat needed early intervention versus 7 (10.3 %) in the postnatal diagnosis group (p = 0.02). Within the postnatal diagnosis group, there were no deaths, while 2 (4.9 %) post-intervention deaths occurred in the prenatal diagnosis group. There are no significant differences concerning post-intervention survival in the prenatal diagnosis group versus the postnatal diagnosis group. Complex cases may be underrepresented in the postnatal diagnosis group. Smaller RPA/LPA values prenatally seem to be associated with early postnatal intervention.

Early and long-term results of biventricular repair of Tetralogy of Fallot with or without pulmonary atresia

After complete repair, the long-term outcome difference between tetralogy of Fallot without pulmonary atresia (TOF) and tetralogy of Fallot with pulmonary atresia (TOF-PA) without MAPCAs is scarcely described. We performed a retrospective study on patients with a diagnostic of TOF and TOF-PA without MAPCAs who underwent complete repair between 1995 and 2016 in our center. We reviewed 960 patients, divided into two main anatomical groups: TOF ( n = 822, 85.7%), and patients with TOF-PA ( n = 138, 14.3%). Concerning surgical technique repair, three groups were distinguished: TOF without transannular patch ( n = 410, 42.7%), TOF/TOF-PA with transannular patch ( n = 494, 51.5%), and TOF-PA with RVOT tube ( n = 56, 5.8%). After complete repair, overall mortality was 11 (1.1%), five of them occurred before discharge (0.5%). Survival at 10 years after repair was 99.1% for TOF and 96.8% for TOF-PA ( P = 0.036). The median follow-up was 10.0 [CI95%: 6.3–14.1] years after repair. One hundred and sixty-three patients underwent reintervention (surgery or percutaneous interventions) (17%). Freedom from reintervention at 10 years after repair was 92.3%, 82.1%, and 48.7% for TOF without transannular patch, TOF/TOF-PA with transannular patch and TOF-PA with RVOT tube respectively ( P < 0.001 between each groups). In addition, reintervention rate was significantly different between TOF with transannular patch and TOF-PA with pulmonary trunk ( P < 0.001). In multivariate analysis, the absence of pulmonary trunk (HR 1.7 IC 95% [1.1–2.6], P = 0.013), pulmonary artery branches stenosis (HR 2.2 IC 95% [1.4–3.5], P = 0.001), prior palliative surgery (HR 2.6 IC 95% [1.8–3.6], P < 0.001), and reintervention before discharge (HR 5.4 IC 95% [3.8–7.5], P < 0.001) were reintervention risk factors. The initial anatomy of pulmonary trunk and branches strongly influences the risk of reintervention in TOF with or without PA. Avoid palliative surgery by directly performing a complete repair in the neonatal period would be a strategical option to improve the prognosis of these patients.

Stenting the complex patent ductus arteriosus in tetralogy of Fallot with pulmonary atresia: challenges and outcomes.

Patent ductus arteriosus (PDA) stenting has gained acceptance for palliation in cyanotic congenital heart disease. The PDA in tetralogy of Fallot with pulmonary atresia (ToF-PA) arises, in the left aortic arch, from underneath the arch and connects to the proximal left pulmonary artery, often resulting in stenosis. The PDA is usually elongated and tortuous, making stent implantation challenging. Shorter duration of palliation, aggravation of branch pulmonary artery stenosis resulting in poor growth and difficulty at surgery makes ductal stenting controversial. Access via the carotid and axillary artery reduces complexity of the procedure and improves success, with recent data demonstrating good pulmonary artery growth. Advances in bioresorbable stents offer future promise and will likely resolve some controversies surrounding PDA stenting in ToF-PA.

The role of cardiac CT angiography in the pre- and postoperative evaluation of tetralogy of Fallot

Tetralogy of Fallot (TOF) is the first described and most common cyanotic congenital heart anomaly that generated the first successful surgical palliation procedure and definitive intracardiac repair. Classical TOF comprises the four typical features of right ventricular outflow tract obstruction (RVOTO), right ventricular hypertrophy (RVH), ventricular septal defect (VSD) and aortic dextroposition. Complex forms of the condition include TOF associated with absent pulmonary valve and TOF with pulmonary atresia (TOF-PA) with or without major aortopulmonary collateral arteries (MAPCAs). The pathophysiological understanding that the tetrad is basically the sequel of a singular abnormality of infundibular malformation, with anterior deviation of the infundibular septum, had major surgical consequences that improved survival. Diagnostic and functional imaging play a key role in the clinical and surgical management of patients with TOF. We revisit the role of traditionally employed imaging modalities (echocardiography and cardiac catheterisation) only briefly as these remain within the domain of cardiology practice. The emphasis of the present review is to outline the role of cardiac computed tomographic angiography (CTA) in the evaluation of TOF, with special reference to the technical considerations and best practice recommendations. Cardiovascular magnetic resonance imaging (CMR) is addressed only in passing, as this service is not currently available at our institution.

Diagnostic accuracy of CT angiography in infants with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries

BackgroundCT angiography (CTA) is increasingly used for the evaluation of congenital heart disease. ObjectiveThe aim was to determine the diagnostic accuracy of CTA in infants with tetralogy of Fallot with pulmonary atresia (ToF-PA) and major aortopulmonary collateral arteries (MAPCAs). MethodsWe retrospectively evaluated 18 consecutive patients (7 girls; median age, 6 days; range, 1–334 days) with ToF-PA and MAPCAs. Findings on CTA were compared with diagnostic catheterization (n = 16) or intraoperative findings (n = 2) for the number of MAPCAs, their diameter, origin, and supplied lung lobes and for the presence and diameter of central pulmonary arteries. Spearman correlation coefficient was calculated to assess the correlation between diameter measurements on CTA and catheterization. CTA dose-length product and catheterization dose-area product were recorded, and effective radiation doses were calculated with the use of age-specific conversion factors. ResultsAgreement was found between CTA and catheterization or intraoperative findings for the number of MAPCAs, their origin, and supplied lung lobes in all cases. In 11 of 13 patients, CTA accurately demonstrated central pulmonary arteries. A good correlation was found between diameter measurements on CTA and catheterization for MAPCAs (r = 0.83) and central pulmonary arteries (r = 0.82). Median effective doses were 0.9 mSv for CTA and 14.4 mSv for catheterization (P < .001). ConclusionCTA is accurate in the preoperative evaluation of infants with ToF-PA and MAPCAs and is associated with a substantially lower radiation dose than catheterization. Preoperative diagnostic catheterization, therefore, may only be necessary in select patients with small MAPCAs in whom the precise assessment of central pulmonary arteries is required for surgical planning.

The pattern of the coronary arterial orifices in hearts with congenital malformations of the outflow tracts: a marker of rotation of the outflow tract during cardiac development?

Outflow tract defects, including cardiac neural crest defects (so-called conotruncal defects) and transposition of the great arteries, are due to an abnormal rotation of the outflow tract during cardiac development. Coronary orifices are often abnormal in outflow tract defects, particularly in common arterial trunk (CAT). A recent study indicates that abnormal coronary artery pattern in a mouse model with common arterial outlet (Tbx1-/- mouse mutant) could be due to a reduced and malpositioned subpulmonary coronary-refractory myocardial domain. The aim of our study was to demonstrate the relation between coronary orifices pattern in outflow tract defects in human and the abnormal embryonic rotation of the outflow tract. We analyzed 101 heart specimens with outflow tract defects: 46 CAT, 15 tetralogy of Fallot (TOF), 29 TOF with pulmonary atresia (TOF-PA), 11 double-outlet right ventricle with subaortic ventricular septal defect (DORV) and 17 controls. The position of left and right coronary orifices (LCO, RCO) was measured in degrees on the aortic/truncal circumference. The anterior angle between LCO and RCO (α) was calculated. The LCO was more posterior in TOF (31 °), TOF-PA (47 °), DORV (44 °), CAT (63 °), compared with controls (0 °, P < 0.05), and more posterior in CAT than in other outflow tract defects (P < 0.05). The RCO was more anterior in TOF (242 °), TOF-PA (245 °) and DORV (271 °) than in controls (213 °, P < 0.05), but not in CAT (195 °). The α angle was similar in TOF, TOF-PA, DORV and controls (149 °, 162 °, 133 °, 147 °), but significantly larger in CAT (229 °, P < 0.0001). In all outflow tract defects but CAT, the displacement of LCO (anterior) and RCO (posterior), while the α angle remains constant, might be due to incomplete rotation of the myocardium at the base of the outflow tract, leading to an abnormally positioned subpulmonary coronary-refractory myocardial domain. The larger α angle in CAT could reflect its dual identity, aortic and pulmonary.

277: Conotruncal defects: is the ventricular septal defect always the same?

Conotruncal defects (CTD) are a group of cardiac malformations hetero-geneous from an anatomic standpoint but with a common embryologic origin: an abnormal rotation of the outflow tract. The outlet septum is therefore malaligned or absent, resulting in a ventricular septal defect (VSD). To analyze the anatomy of the VSD in hearts with CTD. We reviewed 200 heart specimens with CTD from the anatomic collection of the French Center of Reference for Complex Congenital Heart Defects: 70 Tetralogy of Fallot (TOF), 53 TOF with pulmonary atresia (TOF-PA), 54 common arterial trunk (CAT), and 23 interrupted aortic arch type B (IAA-B). Special attention was paid to the rims of the VSD viewed from the right ventricular side, the relationships between tricuspid and aortic valves, and the anatomy of the outlet septum. The VSD was located between the 2 limbs of the septal band (conoventricular) in all hearts. There was a fibrous continuity between tricuspid and aortic valves in 0% of IAA-B, 66% of TOF, 37% of TOF-PA, 1% of CAT (p<0.005). When present, this continuity always involved the anterior tricuspid leaflet. The outlet septum was demonstrable in 81% of IAA-B, 96% of TOF, 39% of TOF-PA, 0% of CAT (p<0.0001). All CTD share the same VSD, located between the two limbs of the septal band. However, there are some differences regarding the inferior rim of the VSD. The continuity of the aortic valve with the anterior, and not the septal, tricuspid leaflet indicates that this continuity may be a consequence of the malposition of the ventriculo-infundibular fold, along with its outlet septal component, rather than a perimembranous extension of the VSD. Finally, these differences suggest an anatomic continuum from IAA-B to CAT rather than distinct physiological phenotypes, related to various degrees of abnormal rotation of the outflow tract, excessive in IAA-B, incomplete in TOF, TOF-PA and CAT.

Tetralogy of Fallot and Aortic Root Dilation: A Long-Term Outlook

Dilation of the sinus of Valsalva (SoV) has been increasingly observed after repaired tetralogy of Fallot (TOF). We estimate the prevalence of SoV dilation in adults with repaired TOF and analyze possible factors related to aortic disease. Adults with TOF [n = 109, median age 33.2 years (range 18.1 to 69.5)] evaluated at Johns Hopkins Hospital from 2001 to 2009 were reviewed in an observational retrospective cohort study. Median follow-up was 27.3 (range 0.1-48.8) years. SoV dilation was defined as >95 % confidence interval adjusted for age and body surface area (z-score > 2). The prevalence of SoV dilation was 51 % compared with that of a normal population with a mean z-score of 2.03. Maximal aortic diameters were ≥ 4 cm in 39 % (42 of 109), ≥ 4.5 cm in 21 % (23 of 109), ≥ 5 cm in 8 % (9 of 109), and ≥ 5.5 cm in 2 % (2 of 109). There was no aortic dissection or death due contributable to aortic disease. Aortic valve replacement was performed in 1.8 % and aortic root or ascending aorta (AA) replacement surgery in 2.8 % of patients. By multivariate logistic regression analysis, aortic regurgitation (AR) [odds ratio (OR) = 3.09, p = 0.005], residual ventricular septal defect (VSD) (OR = 4.14, p < 0.02), and TOF with pulmonary atresia (TOF/PA) (OR = 6.75, p = 0.03) were associated with increased odds of dilated aortic root. SoV dilation after TOF repair is common and persists with aging. AR, residual VSD, and TOF/PA are associated with increased odds of dilation. AA evaluation beyond the SoV is important. Indexed values are imperative to avoid bias on the basis of age and body surface area.

Tetralogy of Fallot with pulmonary atresia and total anomalous pulmonary venous connection: Surgical management of a rare combination of congenital heart defects

Introduction: The combination of Tetralogy of Fallot with pulmonary atresia (ToF/PA) and total anomalous pulmonary venous connection (TAPVC) is an extremely rare congenital anomaly. We report on a patient with ToF/PA with multifocal lung perfusion through a PDA and major aortopulmonary collaterals (MAPCAs) and obstructed supracardiac TAPVC who underwent surgical treatment.

Stenting the ductus arteriosus: Case selection, technique and possible complications

Ductal stenting is an attractive alternative to conventional shunt surgery in duct dependent congenital heart disease as it avoids thoracotomy and its related problems. With today's generation of coronary stents which have better profile, flexibility and trackability, ductal stenting may be achieved safely and with considerably less difficulty than previously described.As in Blalock-Taussig (BT) shunt, ductal stenting is indicated mainly in duct-dependent cyanotic lesions chiefly in the neonatal period. Unlike the Patent ductus arteriosus (PDA) as an isolated lesion, the ductus in cyanotic heart disease has a remarkable morphologic variability. The ductus tends to arise more proximally under the aortic arch, giving rise to a vertical ductus or occasionally it may arise from the subclavian artery. It also tends to be long and sometimes very tortuous, rendering stent implantation technically impossible. The ductus in these patients may also insert onto one of the branch pulmonary arteries with some stenosis at the site of insertion. The ductus in Tetralogy of Fallot with pulmonary atresia (TOF-PA) tend to exhibit these morphologic features and to a lesser degree in transposition of great arteries with ventricular septal defect and pulmonary atresia (TGA-VSD-PA) and the more complex forms of univentricular hearts. In the preliminary angiographic evaluation, it is important to delineate these morphologic features as the basis for case selection.Ductal stenting may be done by the retrograde femoral artery route or the antegrade transvenous route depending on the ductus morphology and the underlying cardiac lesion. The detailed techniques and essential hardware are described. Finally, major potential complications of the procedure are described. Acute stent thrombosis is the most serious and potentially catastrophic. Emergent treatment with thrombolytic therapy and mechanical disruption of thrombus are required. With proper case selection, appropriate technique and the right hardware ductal stenting provides reasonable short-medium term palliation in duct-dependent cyanotic heart disease.

Prevalence and optimal management strategy for aortic regurgitation in tetralogy of Fallot

Aortic regurgitation (AR) in the tetralogy of Fallot (TOF) is not frequent, but when present it impacts significantly on surgical management. Furthermore, the incidence of late AR development has been increasing, along with surgical interest in current practices. Pre- and post-operative studies on 427 patients (TOF, 374; TOF/PA (TOF with pulmonary atresia), 53) who survived corrective operation were reviewed. AR (> or =mild) was detected in 28. Nine had AR preoperatively, while 25 (including six with preoperative AR) exhibited AR post-operatively. In the 19 who developed AR post-operatively, the aortic root diameter (AoRoD) and indexed AoRoD (%AoRoD) were 42+/-11 mm and 166+/-36%, increased from the preoperative values of 30+/-10mm and 149+/-24%. AR-free rate at 20 years was 95.1% of all cases studied, 84.3 vs 96.5% in TOF/PA vs classic TOF (P<0.0001), and 82.2 vs 97.0% in bulboventricular VSD vs infracristal VSD (P<0.0001). Older age at repair, and bulboventricular VSD were identified as risk factors for the progression of AR. Aortic valvuloplasty (AVP; n=5) or replacement (AVR; n=4) was performed nine times in eight patients before (n=1), during (n=4), or late after TOF repair (n=4); all showed improvement of NYHA class. Survival- and reoperation-free survival curves showed no significant difference between patients with or without AR. After repair of TOF, careful observation for a late progression of AR is needed for the optimal timing of surgical intervention, especially in patients who repaired at higher age with a dilated aortic root or in patients with bulboventricular VSD.

Successful Primary Correction of Tetralogy of Fallot with Pulmonary Atresia and Aortopulmonary Window in a 2,220-g Neonate with a Valved Bovine Jugular Vein Conduit

Prenatal diagnosis of tetralogy of Fallot with pulmonary atresia (TOF/PA) was confirmed in a newborn with a birth weight of 2,095 g. Additionally, an aortopulmonary window (APW) type I was diagnosed on echocardiography. The operation was performed at the age of 4 weeks due to congestive heart failure. The APW was closed with a pericardial patch, the ventricular septal defect (VSD) with a Dacron patch, and the right ventricular outflow tract (RVOT) was reconstructed with a 12-mm bovine jugular vein valved conduit (Contegra, Medtronic Inc., Minneapolis, MN, USA). At 15-month follow-up, the patient is in excellent clinical condition without medication. On echocardiography, the conduit showed a mean gradient of 11 mmHg with first-degree insufficiency.

Tetralogy of fallot, pulmonary atresia, and diminutive pulmonary arteries

The surgical management of classical tetralogy of Fallot (TOF), with right ventricular-to-pulmonary artery continuity and pulmonic stenosis, has been well established and standardized, and both early and late results are good, even in critically ill infants. In other patients with pulmonary atresia with ventricular septal defect, however, significant additional therapeutic challenges are seen, primarily because of the wide range of origin, size, and distribution of pulmonary blood supply found in this entity. The most fortunate subset of patients with tetralogy of Fallot with pulmonary atresia (TOFPA) are those with valvar pulmonary atresia, a ductus-dependent pulmonary arterial circulation and essentially normal pulmonary arteries. At the other end of anatomic complexity are those patients with TOF-PA without a patent ductus arteriosus (PDA) and with diminutive or absent central pulmonary arteries. In these patients pulmonary blood flow is supplied exclusively by aortopulmonary collateral arteries (APCAs). Because of the significant variations in pulmonary arterial structure and blood flow, no single effective treatment plan is applicable to this diverse group of patients. Generally,