3D Virtual Reality Imaging of Major Aortopulmonary Collateral Arteries: A Novel Diagnostic Modality

Background Major aortopulmonary collateral arteries (MAPCAs), as seen in patients with pulmonary atresia, are arteries that supply blood from the aorta to the lungs and often require surgical intervention. To achieve complete repair in the least number of interventions, optimal imaging of the pulmonary arterial anatomy and MAPCAs is critical. 3D virtual reality (3D-VR) is a promising and upcoming new technology that could potentially ameliorate current imaging shortcomings. Methods A retrospective, proof-of-concept study was performed of all operated patients with pulmonary atresia and MAPCAs at our center between 2010 and 2020 with a preoperative computed tomography (CT) scan. CT images were reviewed by two congenital cardiac surgeons in 3D-VR to determine additional value of VR for MAPCA imaging compared to conventional CT and for preoperative planning of MAPCA repair. Results 3D-VR visualizations were reconstructed from CT scans of seven newborns where the enhanced topographic anatomy resulted in improved visualization of MAPCA. In addition, surgical planning was improved since new observations or different preoperative plans were apparent in 4 out of 7 cases. After the initial setup, VR software and hardware was reported to be easy and intuitive to use. Conclusions This study showed technical feasibility of 3D-VR reconstruction of children with immersive visualization of topographic anatomy in an easy-to-use format leading to an improved surgical planning of MAPCA surgery. Future prospective studies are required to investigate the clinical benefits in larger populations.

The DIAGNOSTIC CARDIAC CATHETRIZATION IN CHILDREN WITH TETRALOGY OF FALLOT: STILL RELEVANT IN DEVELOPING WORLD

Objective: To determine the frequency of various anatomical cardiac anomalies and variations in children with Tetralogy of Fallot diagnosed by cardiac catheterization. Study Design: Cross sectional study. Place and Duration of Study: Children's Hospital and Institute of Child Health Lahore, from Jan 2010 to Dec 2018. Methodology: All children with tetralogy of fallot underwent standard cine-angiograms after obtaining written consent following basic laboratory workup. Results: Out of 425 patients, 398 completed cardiac catheterization. The median age was 6 years (interquartile range 3.5-9 years). Confluent Branch pulmonary arteries were present in 395 (99%) children. Pulmonary artery abnormalities were detected in 72 (18%) patients. Two hundred and eleven (53%) children had 283 major aortopulmonary collateral arteries with 88 having 2 or more major aortopulmonary collateral arteries. Out of all, 195 (92%) had hemodynamically significant Major aortopulmonary collateral arteries (supplying ≥3 lung segments) with 54 (28%) having small (<1.33mm at origin), 105 (54%) moderate (1.33-1.67 mm at origin) and 36 (18%) large (>1.67 mm at origin) caliber. Conclusion: The frequencies of pulmonary artery abnormalities and various anatomic variations missed on echocardiography in the studied population were high. Diagnostic cardiac catheterization is still a relevant invasive diagnostic procedure in children with tetralogy of fallot. Keywords: , , , , , , .

Verifying the Usefulness of Pulmonary Blood Flow Studies in the Correction of Pulmonary Atresia and Ventricular Septal Defect with Major Aortopulmonary Collateral Arteries

Objective. We retrospectively analyzed the surgical results of pulmonary blood flow studies to guide ventricular septal defect (VSD) closure in the correction of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods. A total of 57 children who were diagnosed with PA/VSD/MAPCAs and who underwent intraoperative pulmonary blood flow studies at our hospital between August 2016 and June 2019 were included. Surgery and cardiopulmonary bypass records were collected. The receiver operating characteristic (ROC) curve was used to verify the accuracy of pulmonary blood flow studies to predict VSD closure. Results. Complete VSD closure was achieved in 39 of 57 children (68.42%), with a median age of 2 years and 5 months (range: 7 months to 15 years and 9 months) and a median weight of 11.0 kg (5.7–36.5 kg). Partial VSD repair was recorded for 21 children (36.84%), including 4 children (19.05%) who underwent VSD closure in the later stages and 13 children (61.90%) who were under follow-up and waiting to undergo complete VSD closure. There was only one child (1.75%) with VSD left. After eliminating the data of four unqualified cases, the ROC curve for predicting VSD closure based on 53 pulmonary blood flow studies was obtained at a p value of <0.001, with an area under the curve of 0.922. The maximum Youden’s index was 0.713, which corresponded to an optimal mean pulmonary artery pressure cutoff value of 24.5 mmHg. Conclusion. The functional evaluation provided by pulmonary blood flow studies is highly accurate to predict intraoperative VSD repair. We recommend using pulmonary blood flow studies with a mean pulmonary artery pressure of ≤25 mmHg during blood perfusion at 3.0 L/min/m2 as the standard to repair VSD.

Outcomes After Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: A Tailored Approach in a Developing Setting

Objectives: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is complex and diverse that has led to a variety of treatment strategies. Experience has been largely obtained in the advanced countries. The clinical diversity is greater in China. We evaluated our surgical approaches and outcomes of these patients.Methods: We reviewed 127 patients undergoing varied surgeries in our center in 2010–2019.Results: Thirty patients underwent single-stage complete repair by unifocalizing MAPCAs and VSD closure (aged 3.9–131.4 months, median 22) with 3 (10%) early deaths. Ninety-seven underwent the first-stage rehabilitation strategy including systemic-to-pulmonary shunt in 29 (aged 0.5–144 month, median 8), and palliative RV-PA conduit in 68 (aged 2.2–209.6 months, median 14) with 5 (5.2%) early deaths. Eight-one patients (63.8%) eventually achieved complete repair with a median right/left ventricular (RV/LV) pressure ratio of 0.7 (ranged 0.4–1.0). Fourteen patients (11.0%) accepted palliation as final destination. Survival for the entire cohort was 89.5, 85.2, and 76.1% at 1, 5, and 10 years, respectively. Survival for those undergoing complete repair was 88.2 and 76.6% at 1 and 5 year, respectively. RV/LV pressure ratio ≥0.8 was risk factor for mortality (HR10.3, p = 0.003).Conclusions: Our cohort, the largest from China, had distinctive clinical features with substantially wider age range and higher RV/LV pressure ratio. Using the combined approaches tailored to individual patients, complete repair was achieved in 64% of patients. The early and intermediate outcomes are acceptable compared to many of the previous reports.

Successful surgical management of cyanotic congenital heart disease complicated by pulmonary aspergillosis

Pulmonary aspergillosis associated with cyanotic congenital heart disease is a rare condition, which is known to have a poor prognosis. We report a case of a 21-year-old woman with truncus arteriosus and major aortopulmonary collateral arteries who underwent primary Rastelli procedure after thoracoscopic lobectomy for the management of progressive pulmonary aspergillosis.

Fate of the Arterial Origin of Major Aortopulmonary Collateral Arteries After Unifocalization

Background: During unifocalization procedures for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, collateral arteries are either ligated or detached. Not much is known of the fate of the remaining arterial origins in the long term. Available computed tomography (CT) or magnetic resonance (MR) imaging of the intrathoracic arteries was examined to investigate possible abnormalities at the former position of the collateral arteries as well as ascending aortic diameters. Methods: From 1989 to 2018, we performed 66 unifocalization procedures in 39 patients. One hundred and twenty-nine collateral arteries were ligated or detached. In 52% (15) of the surviving patients (with a total of 55 ligated or detached collaterals), sufficient imaging of the thoracic aorta from CT (11) and/or MR (9) was available for evaluation. Results: The median interval between unifocalization procedure and imaging was 15 years (interquartile range [IQR]: 9-19 years). In 93% (14) of the scanned patients, 18 blunt ends were detected at the location of a former collateral artery. No aneurysm formation of the descending aorta was observed. The median diameter of the ascending aorta was 35 mm (IQR: 31-40 mm). During follow-up, no aortic dissection or rupture occurred. Conclusions: Aortic imaging late after unifocalization showed abnormalities in 93% of the scanned patients. Abnormalities consisted mostly of blunt ends of the former collateral artery. We recommend to include routine imaging of the aorta during late follow-up to detect eventual future abnormalities and monitor aortic diameters. Ascending aortic diameters showed slight dilatation with no clinical implications so far.