Aortopulmonary Collateral Arteries Research Articles

A Novel Perspective on Histopathology Provides Novel Insights into Surgical Effects in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries: A Case-Series Study

Background: Never had literatures characterized the relationship between the property of major aortopulmonary collateral arteries (MAPCAs) and outcomes of selective unifocalization of pulmonary atresia with ventricular septal defects and MAPCAs. Methods: This is a case-series study. Thirteen patients were included. Angiography-based assessment was conducted to determine whether collateral arteries should be unifocalized or treated with intraoperative ligature. Specimens were collected and stained by HE and ET+VG. Results: Twelve patients underwent one-stage unifocalization at a median age of 37 months (range: 6–228 months) and a median weight of 14.0 kg (range: 5.0–49.0 kg), which produced a favorable right ventricle to aortic systolic pressure ratio of no more than 0.5 except in one patient who died. Patients were divided into three groups: Group 1 (n = 6), had no native pulmonary arteries, and collateral arteries supplied all pulmonary blood; Group 2 (n = 6) presented dysplastic native pulmonary arteries on one or both sides, and in some lung lobes or segments, blood was supplied only by collateral arteries; Group 3 (n = 1) had well-developed left and right pulmonary arteries, and collateral arteries, and pulmonary arteries provided blood flow to the same segments. Pathological reports demonstrated two types of collateral arteries: Elastic arteries presented an arborization distribution similar to native pulmonary artery walls, while muscular arteries showed high resistance and distortion. We selectively unifocalized single-supply collateral arteries with morphologic features based on the arborization distribution. Conclusions: We found that there were two kinds of MAPCAs with different histology, and we performed selective UF for MAPCAs that might belong to the elastic artery. Selective unifocalization achieved a low right ventricle to aortic systolic pressure ratio and favorable surgical effects.

Mid-Term Outcomes After Unifocalization Guided by Intraoperative Pulmonary Flow Study.

Repair of tetralogy of Fallot (TOF) with major aortopulmonary collateral arteries (MAPCAs) requires unifocalization of pulmonary circulation, intracardiac repair with the closure of the ventricular septal defect, and placement of a right ventricle (RV) to pulmonary artery (PA) conduit. The decision to perform complete repair is sometimes aided by an intraoperative flow study to estimate the total resistance of the reconstructed pulmonary circulation. We reviewed patients who underwent unifocalization and PA reconstruction for TOF/MAPCAs to evaluate acute and mid-term outcomes after repair with and without flow studies and to characterize the relationship between PA pressure during the flow study and postrepair RV pressure. Among 579 patients who underwent unifocalization and PA reconstruction for TOF/MAPCAs, 99 (17%) had an intraoperative flow study during one (n = 91) or more (n = 8) operations to determine the suitability for a complete repair. There was a reasonably good correlation between mean PA pressure at 3 L/min/m2 during the flow study and postrepair RV pressure and RV:aortic pressure ratio. Acute and mid-term outcomes (median: 3.8 years) after complete repair in the flow study patients (n = 78) did not differ significantly from those in whom the flow study was not performed (n = 444). Furthermore, prior failed flow study was not associated with differences in outcome after subsequent intracardiac repair. The intraoperative flow study remains a useful adjunct for determining the suitability for complete repair in a subset of patients undergoing surgery for TOF/MAPCAs, as it is reasonably accurate for estimating postoperative PA pressure and serves as a reliable guide for the feasibility of single-stage complete repair.

The Clinical Application Value of Selective Unifocalization in the Treatment of Severe Pulmonary Artery Atresia with Ventricular Septal Defect

Background: This study aims to explore the efficacy of selective unifocalization (UF) for major aortopulmonary collateral arteries (MAPCAs) unifocalization in children with pulmonary atresia with ventricular septal defect (PA/VSD). Methods: A retrospective analysis of 13 patients with PA/VSD/MAPCAs who underwent surgery from June 2017 to December 2019. Sex, age, preoperative cardiovascular angiography test results and McGoon ratio were collected. The properties of the collateral arteries were evaluated by angiography, and selective UF for the “dendritic” MAPCAs and ligation of MAPCAs demonstrating distortion and resistance. Results: A total of 13 severe patients underwent one-stage repair, of which 1 case underwent ventricular septal fenestration and died after 2 weeks of ECMO support. The median age was 37 months, and a median weight was 14.0 kg. A right ventricular to aortic systolic pressure ratio (pRV/pAo) of no more than 0.5 was achieved in 12 living patients. Conclusion: Selective unifocalization based on MAPCAs morphology can achieved a good outcome at the early stage. This surgical concept might be provided a novel insight into treatment for some of the subgroups presenting with this complex form of PA/VSD/MAPCAs.

Primary repair of tetralogy of fallot and major aorto-pulmonary collateral arteries with suspected Noonan syndrome: A rare case

Tetralogy of Fallot (TOF) is a congenital heart disease that includes ventricular septal defect (VSD), pulmonary stenosis (PS), right ventricular hypertrophy (RVH), and an overriding aorta. A cyanotic heart defect can develop aorto-pulmonary collateral (APCA) to supply underperfused pulmonary beds. Genetic syndromes can occur in 20% of patients with TOF. In developing countries, diagnosed genetic syndromes associated with heart defects have not been covered by health insurance, even though outcomes of primary repair remain unknown. This study reported a case of an 11-year-old boy with cyanosis since birth, dyspnoea on effort, growth delays, learning problems, scoliosis, micropenis, hypertelorism, exophthalmos, facial dysmorphism, cryptorchidism, and major aorto- pulmonary collaterals. The patient underwent an echocardiographic study. It showed VSD malalignment with overriding aorta of 48%, critical pulmonary stenosis, and size of pulmonary arteries that were favourable for primary repair. Cardiac catheterization showed classic Fallot with normal coronary arteries and major aortopulmonary collateral arteries (MAPCA) arising. The patient underwent percutaneous coil embolization just before surgery. There was a problem during induction, the anaesthetist found that it was hard to place the ETT because there was membrane-like covering the trachea. Next, a median sternotomy was conducted, and aortic cross-clamping was achieved. Right atriotomy was performed, and it provided exposure to the malalignment VSD. A longitudinal pulmonary arteriotomy was made to expose infundibular stenosis. Infundibulectomy was performed, and it reconstructed the MPA with a pericardial patch. The VSD was closed by using a PTFE patch. The patient was transferred to an ICU afterward. Post-operative echocardiography showed a good outcome, and 15 days after surgery the patient was discharged. It could be concluded that primary repair of TOF with MAPCAs and suspected Noonan syndrome was conducted successfully.

Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot.

BackgroundDue in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs.Methods and ResultsAll infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross‐sectional area were less likely to undergo complete repair.ConclusionsMAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross‐sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.

Stenting of the right ventricular outflow tract as an initial intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries. The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting. Retrospective review of all patients with Tetralogy of Fallot with pulmonary stenosis who underwent right ventricular outflow tract stenting as primary intervention over an 8-year period. Ten patients underwent right ventricular outflow tract stent insertion at a median age of 61 days (interquartile range (IQR) 8.3-155 days). Median weight at stent deployment was 4.2 kg (IQR 3.2-5.7 kg). Oxygen saturations improved from a median of 79% (IQR 76-80%) to 92% (IQR 90-95%), p = 0.012. The median right and left pulmonary artery z score increased from -3.51 (IQR -4.59 to -2.80) and -2.07 (IQR -3.72 to 0.15) to a median of -1.17 (IQR -2.26 to 0.16) p < 0.05, and 0.24 (IQR -1.09 to 1.84) p < 0.05, respectively, at subsequent angiogram. Nine patients underwent further catheterisation. Four patients underwent complete anatomical repair. Only one patient required unifocalisation, as most patients had a native supply to all-important lung segments. Right ventricular outflow tract stenting is a useful procedure in the subset of patients with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries, where native pulmonary arterial growth is required to facilitate repair.

Prenatal ultrasound diagnosis of pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (MARCA)

The case of prenatal diagnosis of pulmonary artery atresia with a ventricular septal defect and major aortopulmonary collateral arteries at 34 weeks is presented. Atresia of the pulmonary artery with a ventricular septal defect and major aortopulmonary collateral arteries (type B) was diagnosed. At 39 weeks birth occurred. After the examination, the prenatal diagnosis was confirmed. The condition of the newborn was rated as satisfactory. On the 7th day the child was discharged under the supervision of a cardiologist. Surgical treatment is planned. The article discusses the issues of differential prenatal diagnosis, modern possibilities of operative treatment and prognosis.

Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism.

Right atrial isomerism is associated with complex cardiac malformations, particularly single-ventricle lesions; right atrial isomerism is rarely associated with aorto-pulmonary collateral arteries. We report a foetal diagnosis of right atrial isomerism, with an unbalanced atrioventricular septal defect, pulmonary stenosis, total anomalous venous drainage, and significant aorto-pulmonary collaterals diagnosed at 22 weeks' gestation.

Outcomes of management of major aortopulmonary collaterals for pulmonary atresia and ventricular septal defect.

This study aimed to investigate the association between long-term survival and different management of major aortopulmonary collateral arteries in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. From November, 2009 to October, 2018, a total of 98 consecutive patients with pulmonary atresia, ventricular septal defect, major aortopulmonary collateral arteries, and hypoplastic pulmonary arteries treated with modified Blalock-Taussig shunt or right ventricle-pulmonary artery connection were included. Fifty-five patients who received occlusion or ligation of major aortopulmonary collateral arteries during or after palliative procedure were occlusion group, and the other 43 patients were no occlusion group. The early and late outcomes were compared. The mean duration of follow-up was 30.9 months in no occlusion group and 49.8 months in the occlusion group (p < 0.001). Multivariate analysis showed that only no occlusion of major aortopulmonary collateral arteries was predictive of total mortality (Hazard Ratio: 4.42, 95% CI: 1.27 to 15.42, p = 0.02). The Kaplan-Meier survival curves confirmed that patients without occlusion of major aortopulmonary collateral arteries demonstrated worse survival as compared with the occlusion group (p = 0.013). The Kaplan-Meier survival curves of patients who underwent different palliative procedures showed no differences. For patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries when a primary repair is not feasible, those without occlusion of major aortopulmonary collateral arteries have a higher risk of death following an initial palliative procedure compared with patients who underwent occlusion of major aortopulmonary collateral arteries. The occlusion of major aortopulmonary collateral arteries is not associated with a higher rate of complete repair or better improvement of pulmonary artery growth.

Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease

To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease. Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs. Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation. Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.

Association of Preoperative Mixed Venous Oxygen Saturation with Postoperative Segmental Pulmonary Hypertension in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals.

In patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), segmental pulmonary hypertension is common; however, its pathophysiology remains to be elucidated. This study aimed to identify preoperative hemodynamic factors associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs. This study included 12 patients with PA/VSD/MAPCAs who underwent unifocalization and intracardiac repair from 2009 at our institution. Hemodynamic measurements of preoperative and postoperative cardiac catheterization were collected. Patients were considered to have pulmonary hypertension if the mean pulmonary pressure measured postoperatively at any peripheral pulmonary artery was ≥ 20mmHg. The data were compared between patients with and without postoperative segmental pulmonary hypertension; p value < 0.05 was considered statistically significant. Intracardiac repair was performed at the age of 2.1years (range 0.5-28.2years). Unifocalization was performed separately prior to intracardiac repair in two patients and concomitantly in 10 patients. Postoperative cardiac catheterization, performed 1.2years (range 0.2-8.7years) after intracardiac repair, revealed that five patients had segmental pulmonary hypertension. Patients with postoperative pulmonary hypertension had significantly lower preoperative mixed venous oxygen saturation than those without pulmonary hypertension (57% [55-63%] vs. 65% [53-75%], p < 0.05). No significant differences were found between the groups in terms of age at intracardiac repair, preoperative arterial oxygen saturation, and preoperative peripheral pulmonary arterial mean pressure. Lower preoperative mixed venous oxygen saturation was associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs.

Case Report: A forty year-survivor of Tetralogy of Fallot with pulmonary atresia and chronic pediatric shunt thrombosis; findings from cardiac CT scan

Here we illustrate a rare case of Tetralogy of Fallot (TOF) with pulmonary atresia in a 40-year-old survivor patient, despite a chronic pediatric shunt thrombosis. The patient became symptomatic at 38-years-old with progressive dyspnea on exertion and short cyanosis spells. The clinical findings were unspecific except for sinus tachycardia. To reassess cardiac abnormalities and associated intrathoracic malformations, a cardiac CT-scan was performed. Here we explain the mechanism for prolonged survival as an expansion of several and huge major aortopulmonary collateral arteries. This case predicts an exceptional late outcome of untreated TOF.

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.

Three-Dimensional Printing, Virtual Reality and Mixed Reality for Pulmonary Atresia: Early Surgical Outcomes Evaluation

Single-stage unifocalisation for pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA) requires a high degree of three-dimensional (3D) anatomical imagination. A previous study has reported the application of a 3D-printed heart model with virtual reality (VR) or mixed reality (MR). However, few studies have evaluated the surgical outcomes of the 3D model with VR or MR in PA/VSD patients. Three-dimensional (3D) heart models of five selected PA/VSD patients were derived from traditional imageology of their hearts. Using VR glasses, the 3D models were also visualised in the operating room. Both the 3D-printed heart models and preoperative evaluation by VR were used in the five selected patients for surgical simulation and better anatomical understanding. Mixed reality holograms were used as perioperative assistive tools. Surgical outcomes were assessed, including in-hospital and early follow-up clinical data. The use of these three new technologies had favourable feedback from the surgeons on intraoperative judgment. There were no in-hospital or early deaths. No reintervention was required until the last follow-up. Three (3) patients developed postoperative complications: one had right bundle branch block and ST-segment change, one had chest drainage >7 days (>40 mL/day) and one had pneumonia. The preoperative application of a 3D-printed heart model with VR or MR helped in aligning the surgical field. These technologies improved the understanding of complicated cardiac anatomy and achieved acceptable surgical outcomes as guiding surgical planning.

Unifocalization and pulmonary artery reconstruction in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery before referral

ObjectiveThe study objective was to characterize and analyze outcomes in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who had undergone surgery elsewhere before referral (prereferral surgery). MethodsPatients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery between 2001 and 2019 at our center were reviewed. Prereferral surgery and unoperated patients were compared, as were subsets of prereferral surgery patients who had undergone different types of prior procedures. Primary outcomes included complete repair with survival to 6 months, death, and perioperative metrics. ResultsOf 576 patients studied, 200 (35%) had undergone a wide range and number of prior operations elsewhere, including 92 who had pulmonary blood supply through a shunt and 108 who had a right ventricle pulmonary artery connection. Patients who underwent prereferral surgery with an existing right ventricle pulmonary artery connection had undergone more prereferral surgery procedures than those with a shunt and were more likely to have a right ventricle outflow tract pseudoaneurysm or pulmonary artery stent (all P < .001) at the time of referral. The cumulative incidences of complete repair and death were similar regardless of prereferral surgery status, but the cumulative incidence of complete repair with 6-month survival was higher (P = .002) and of death lower (P = .18) in patients who had prior right ventricle pulmonary artery connection compared with those who had received a prior shunt only. ConclusionsOur comprehensive management strategy for tetralogy of Fallot and major aortopulmonary collateral arteries can be applied with excellent procedural results in both unoperated patients and those who have undergone multiple and varied procedures elsewhere.

Truncus Arteriosus - modified Van Praagh\u2019s Type 3A and Anesthesia: a case report.

BackgroundOne of the rare complex congenital anomalies is truncus arteriosus—modified Van Praagh’s type 3A. Survival of this type of truncus arteriosus child beyond infancy without surgical treatment is unreported. Anesthesiologists do anesthetize children with complex congenital heart disease during the cardiac catheterization study. The final diagnosis of such children is often made after the anesthesia and cardiac catheterization study. We report a 12-year-old with truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple Major Aorto-Pulmonary Collateral Arteries. (MAPCAs) for the right lung, who is surviving without surgical treatment.Case presentationA 12-year-old girl was brought by her parents to Meenakshi Hospital at Thanjavur (India) with complaints of shortness of breath during respiratory infection. The patient was diagnosed to have congenital heart disease at 6 years of age and not on any treatment. There was no history of cyanotic spell. Her echocardiography revealed tetralogy of Fallot, situs solitus, levocardia, large mal-aligned ventricular septal defect with bidirectional shunt, VSD size 12 mm, pulmonary atresia, moderate tricuspid regurgitation (TR pressure gradient, 103 mmHg), thickened aortic valve, grade II aortic regurgitation, right ventricular hypertrophy, intact interatrial septum, dilated right atrium/right ventricle, dilated coronary sinus, and persistent left superior vena cava, good biventricular function 65%, multiple MAPCAs, no coarctation of aorta, normal veno atrial, atrio-ventricular connections, normal pulmonary venous drainage, and no pericardial effusion. She underwent cardiac catheterization study for further evaluation under anesthesia. Her final diagnosis was truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple MAPCAs for right lung, (truncus arteriosus—modified Van Praagh’s type 3A).ConclusionAn anesthesiologist may be encountering such patients during cardiac catheterization study or emergency non-cardiac surgery, where an understanding of the complex anatomy (the aorta, left pulmonary artery, coronary artery, all arising from the common arterial trunk, the truncus arteriosus) and the physiology of their circulation would help in safe anesthesia. From our report, we conclude intra venous ketamine along with regional analgesia would be safe for sedating such patients coming for cardiac catheterization study.

Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand

Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). Data about the outcomes in developing countries is scarce. We therefore conducted a retrospective study to assess survival rates and mortality risks of 90 children with PA/VSD at Siriraj Hospital, Thailand during 2005–2016. Patients with single ventricle were excluded. Survival and mortality risks were analyzed at the end of 2018. The median age of diagnosis was 0.5 (0–13.8) years. The patients’ PAs were categorized into four groups: 1) PA/VSD with confluent PAs (n = 40), 2) PA/VSD with confluent PAs and MAPCAs (n = 21), 3) PA/VSD with non-confluent PAs and MAPCAs (n = 12), and 4) PA/VSD with small native PAs and MAPCAs (n = 17). Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. Significant mortality risks were the presence of associated anomalies and non-confluent PAs.

Classic Imaging Findings of Tetralogy of Fallot with 64-slice Multidetector Computed Tomography

Background &amp; objective: The present study was intended to demonstrate the role of Multidetector Computed Tomography (MDCT) in defining the extracardiac vascular abnormalities including the pulmonary arterial tree, Major Aortopulmonary Collateral Arteries (MAPCAs), patent ductus arteriosus (PDA), and detection of the common and uncommon findings in Fallot’s Tetralogy cases for proper pre-surgical evaluation.&#x0D; Methods: A retrospective review of all multidetector CT images acquired to evaluate suspected cases of Tetralogy of Fallot, presented at Ibrahim Cardiac Hospital &amp; Research Institute between July 2017 and July 2018, was done. This comprised of a total of 35 cases who were examined by 64-slice MDCT. The patients were divided into four main groups: those with classic Tetralogy of Fallot with no associations, those with common associations, those with uncommon associations and those with a combination of common and uncommon associations.&#x0D; Results: In the present study, majority of the patients had levocardia (93.6%), situs solitus (96.9%) with none of the patients having either atrio-ventricular or arterio-ventricular discordance. Major findings of the patients were VSD (93.8%), right ventricular outflow tract obstruction (65%) with ventricular hypertrophy (75%). All patients had overriding of aorta with over 60% of the patients having 50% and 28% &gt; 50% overriding. Approximately 3.1% of the patients had MPA atresia. MPA stenosis was detected in 93.3% of the cases.&#x0D; Conclusion: A customized approach to MDCT imaging improves the diagnostic accuracy and reduces unnecessary prolongation of the study and sedation times. A careful preoperative perception of the complex cardiovascular anatomy in patients with Tetralogy of Fallot aids in exposing the patients to a directed and prepared surgical approach.&#x0D; Ibrahim Card Med J 2019; 9 (1&amp;2): 16-22

Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

BackgroundPulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization.MethodsWe have an experience with well over 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. There were three pathways employed to accommodate the various forms of this heart defect. The dominant pathway is midline unifocalization, which was utilized in 80% of all patients. Less frequently employed strategies included creation of an aortopulmonary window (15%), and other approaches (5%).ResultsFor the patients who underwent a midline unifocalization, 85% had a single-stage complete repair while 15% had a unifocalization and shunt. The operative mortality was 1.5% for the single-stage complete repair group vs. 8% for the unifocalization/shunt group. Ultimately, 97% of the midline unifocalization patients were repaired. However, the mean right ventricle to aortic pressure ratio was lower in patients who had a single-stage complete repair compared with an initial unifocalization/shunt (0.36±0.09 vs. 0.42±0.07).ConclusionsThe surgical treatment of PA/VSD/MAPCAs is made more complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. The algorithm utilized at Stanford takes into account the anatomic heterogeneity and the results demonstrate that a high percentage of patients achieve full repair.

A road map for collaterals: Use of 3-dimensional techniques in tetralogy of Fallot pulmonary atresia with major aortopulmonary collateral arteries.

Treatment of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA) remains challenging.1,2 Surgical repair involves unifocalization and arterioplasty of collaterals and frequently diminutive pulmonary arteries (PAs).2 These vessels are frequently torturous and complex, limiting the utility of traditional imaging modalities (eg, echocardiography and angiography).3 We present images from a novel visualization strategy to enhance preoperative understanding of the anatomy.