scholarly journals Dilatation of the Great Arteries in an Infant with Marfan Syndrome and Ventricular Septal Defect

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
L. Rozendaal ◽  
N. A. Blom ◽  
Y. Hilhorst-Hofstee ◽  
A. D. J. Ten Harkel
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Marfan Syndrome ◽  
Aortic Root ◽  
Dna Analysis ◽  
Septal Defect ◽  
Normal Limits ◽  
Congenital Contractural Arachnodactyly ◽  
Pulmonary Artery Dilatation

We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits. Eventually the diagnosis of MFS was confirmed by DNA analysis.

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.

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect

2000 ◽  
Vol 70 (1) ◽  
pp. 119-123 ◽  
Author(s):  
Zahid Amin ◽  
Doff B McElhinney ◽  
V.Mohan Reddy ◽  
Phillip Moore ◽  
Frank L Hanley ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Atresia

Double aortic arch: an elusive diagnosis in a child with CHD

2021 ◽  
pp. 1-3
Author(s):  
Mariana Lemos ◽  
Miguel Fogaça da Mata ◽  
Ana Coutinho Santos
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Aortic Arch ◽  
Ct Angiography ◽  
Septal Defect ◽  
Vascular Ring ◽  
Pulmonary Atresia ◽  
Double Aortic Arch ◽  
Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

scholarly journals Echocardiographic evaluation of ventricular septal defect haemodynamics

2007 ◽  
Vol 135 (9-10) ◽  
pp. 541-546
Author(s):  
Vesna Miranovic
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Body Surface ◽  
Septal Defect ◽  
Left Ventricular ◽  
The Body ◽  
Control Group ◽  
Healthy Children ◽  
And Control ◽  
The Relationship

Introduction Ventricular septal defect (VSD) is an opening in the interventricular septum. 30-50% of patients with congenital heart disease have VSD. Objective The aim of the study was to determine the dependence of the left ventricular diastolic dimension (LVD), left ventricular systolic dimension (LVS), shortening fraction (SF), left atrium (LA), pulmonary artery truncus (TPA) on the body surface and compare their values among experimental, control and a group of healthy children. Values of maximal systolic gradient pressure (Pvsd) of VSD were compared with children from one experimental and control group. Method Children were divided into three groups: experimental (32 children with VSD that were to go to surgery), control (20 children with VSD who did not require surgery) and 40 healthy children. Measurements of LVD, LVS, SF, LA, TPA were performed in accordance to recommendations of the American Echocardiographic Association. The value of Pvsd was calculated from the maximal flow velocity (V) in VSD using the following formula: Pvsd=4xV? (mm Hg). Results For children from the experimental group, the relationship between the body surface and the variability of the LVD was explained with 56.85%, LVS with 66.15%, SF with 4.9%, TPA with 58.92%. For children from the control group, the relationship between the body surface and the variability of LVD was explained with 88.8%, LVS with 72.5%, SF with 0.42%, PA with 58.92%. For healthy children, the relationship between the body surface and the variabilitiy of the LVD was explained with 88.8%, LVS with 88.78%, SF with 5.25% and PA with 84.75%. There was a significant statistical difference between average values of Pvsd in the experimental and control group (p<0.02). Conclusion The presence of the large VSD has an influence on the enlargement of LVD, LVS, SF, TPA. The enlargement of the size of the pulmonary artery depends on the presence of VSD and there is a direct variation in the magnitude of the shunt. There is a relationship and significant dependence of the LVS and LVD on the body surface. There is no statistically significant dependence between SF and body surface.

Coarctation of the Left pulmonary artery associated with congenitally corrected transposition

1999 ◽  
Vol 9 (2) ◽  
pp. 207-209 ◽  
Author(s):  
Martial M. Massin ◽  
Götz von Bernuth
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Left Pulmonary Artery ◽  
First Report ◽  
Ductal Tissue ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

AbstractWe describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.

Prenatal diagnosis of complete transposition of the great arteries and dextrocardia in the fetus with left juxtaposition of the atrial appendages and subpulmonary ventricular septal defect

2020 ◽  
Author(s):  
I.N. Daminov
Keyword(s):  
Prenatal Diagnosis ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Arterial Switch Operation ◽  
Ductus Arteriosus ◽  
Health Improvement ◽  
Complete Transposition ◽  
Switch Operation ◽  
Third Degree Atrioventricular Block

The case of prenatal ultrasound diagnosis of complete transposition of the great arteries and dextrocardia in the fetus with left juxtaposition of the atrial appendages and subpulmonary ventricular septal defect in the third trimester is presented. The postnatal echocardiography confirmed the prenatal diagnosis. At 2 months of life, first surgical intervention under extracorporeal circulation was performed: the arterial switch operation and narrowing of the dilated pulmonary artery root, closure of patent foramen ovale, ligation of patent ductus arteriosus. At the age of 8 months, the child underwent a second operation: closure of ventricular septal defect with the transventricular approach and plastic surgery of the pulmonary artery. After an operation third-degree atrioventricular block (bradyarrhythmias) has occurred and 2 weeks later patient's health improvement was achieved, and he underwent implantation of a single-chamber pacemaker. Currently, the child is 2 years old, physical and mental development corresponds to age and he remains under the supervision of specialists.

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