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

2021 ◽  
Vol 71 (4) ◽  
pp. 1408-12
Author(s):  
Gul Afshan ◽  
Ahmed Usaid Qureshi ◽  
Syed Najam Haider ◽  
Tehmina Kazmi ◽  
Uzma Kazmi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Cardiac Catheterization ◽  
Pulmonary Arteries ◽  
Cross Sectional ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Diagnostic Cardiac Catheterization ◽  
Aortopulmonary Collateral ◽  
Pulmonary Artery Abnormalities

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: , , , , , , .

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

2020 ◽  
Vol 9 (24) ◽  
Author(s):  
Gregory T. Adamson ◽  
Doff B. McElhinney ◽  
Yulin Zhang ◽  
Jeffrey A. Feinstein ◽  
Lynn F. Peng ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Surgical Outcomes ◽  
Pulmonary Circulation ◽  
Cross Sectional Area ◽  
Sectional Area ◽  
Cross Sectional ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Total Cross Sectional Area ◽  
Aortopulmonary Collateral

Background Due 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 Results All 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. Conclusions MAPCA 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.

Deposition of collagen in the alveolar wall of lungs from patients with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries—an ultrastructural study

1994 ◽  
Vol 4 (3) ◽  
pp. 277-284
Author(s):  
Hikaru Matsuda ◽  
Tohru Kuratani ◽  
Yasuhisa Shimazaki ◽  
Keishi Kadoba ◽  
Jyunjiro Kobayashi ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Ultrastructural Study ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Alveolar Wall ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

AbstractAbstract Lung biopsies were taken at surgery from five patients (age 2–13, average 7.6 years) with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. The biopsies were then processed for ultrastructural study, comparing paired samples taken, on the one hand, from a segment connected to central pulmonary arteries and, on the other hand, from a segment supplied directly by collateral arteries. Specimens from patients with isolated ventricular septal defect, and from those without cardiac disease, were used as controls. In the patients with tetralogy of Fallot and pulmonary atresia, all biopsies taken from segments supplied by the major collateral arteries showed marked deposition of collagen in the alveolar wall, with an increase in the thickness of the basement membrane (3.6±1.2 µm, mean±SD) greater than seen in those taken from segments connected to central pulmonary arteries (0.9±0.6, p<0.05). The proportional fibrosis of the alveolar interstitial space was also significantly greater in the biopsies from the segments supplied by collateral arteries (30±9%) compared to those from segments fed by central pulmonary arteries (15±8%, p<0.05). No significant differences were found in these indices between the biopsies from segments connected to the central pulmonary arteries in patients with tetralogy of Fallot and pulmonary atresia and those from patients with isolated ventricular septal defect or normal controls. There was no apparent relation to the pulmonary arterial pressure in these findings. The results suggest that the pulmonary segments fed directly by major aortopulmonary collateral arteries in patients with tetralogy of Fallot and pulmonary atresia seem likely to be afflicted by alveolar wall fibrosis, although the etiology and clinical implications of this finding remain unclear.

The essential echocardiographic features of tetralogy of Fallot

2013 ◽  
Vol 23 (6) ◽  
pp. 871-882 ◽  
Author(s):  
Norman H. Silverman
Keyword(s):  
Tetralogy Of Fallot ◽  
Ventricular Hypertrophy ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
First Trimester ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral ◽  
Subpulmonary Stenosis

AbstractThis presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

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

2020 ◽  
pp. 1-8
Author(s):  
Mohammad Abumehdi ◽  
Deepa Sasikumar ◽  
Milind Chaudhari ◽  
Vinay Bhole ◽  
Phil Botha ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Major Aortopulmonary Collateral Arteries ◽  
Collateral Arteries ◽  
Aortopulmonary Collateral

Abstract Objectives: 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. Background: 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. Methods: 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. Results: 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. Conclusion: 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.

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

2020 ◽  
pp. 1-9
Author(s):  
Dong Zhao ◽  
Keming Yang ◽  
Wei Feng ◽  
Shoujun Li ◽  
Jun Yan ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Survival Curves ◽  
Major Aortopulmonary Collateral Arteries ◽  
Palliative Procedure ◽  
Collateral Arteries ◽  
Kaplan Meier ◽  
Aortopulmonary Collateral

Abstract Objective: 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. Methods: 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. Results: 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. Conclusions: 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.

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