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Pulmonary Atresia (pulmonary + atresia)

Distribution by Scientific Domains

Medical Sciences	87%

Selected Abstracts

Collaborative Approach in the Management of Pulmonary Atresia with Intact Ventricular Septum

JOURNAL OF INTERVENTIONAL CARDIOLOGY, Issue 3 2001
F.R.C.P.Article first published online: 8 JUN 200, SHAKEEL A. QURESHI M.B.Ch.B.
[source]

The Benefits of High-flow Management in Children With Pulmonary Atresia

ARTIFICIAL ORGANS, Issue 11 2009
Yasuhiro Fujii
Abstract The high-flow management of cardiopulmonary bypass (CPB; ,2.4 L/min/m2) is a standard strategy used at this institute for children with pulmonary atresia (PA) due to a fear that the blood flow may be diverted by the major/minor aortopulmonary-collateral-arteries and hypervascularization due to long-term hypoxia. The purpose of this study was to describe the validity of high-flow management in children with PA. The CPB records of 23 children with PA who underwent a definitive biventricular repair between Feb 2006 and Nov 2008 were retrospectively reviewed. The mean age at the operation was 33 ± 22 months. The blood-pressure during bypass was controlled with the same protocol. The mean cooling-temperature was 28.4 ± 3.7°C. The mean minimum hematocrit was 25.0 ± 3.4%. The mean maximum bypass flow index at the initiation, the mean maximum flow index during aortic cross-clamping, the mean minimum flow index during aortic cross-clamping, and the mean maximum flow index after rewarming were 3.1 ± 0.5, 3.1 ± 0.5, 2.6 ± 0.4, and 3.2 ± 0.4 L/min/m2, respectively. The higher bypass flow indexes significantly correlated with the lower serum lactate levels. The lowest oxygen delivery during CPB had significant influences on the urine output during bypass (R = 0.547, P = 0.007), the serum lactate levels at the end of CPB (R = ,0.442, P = 0.035), and the postoperative thoracic effusion (R = ,0.459, P = 0.028). A bypass flow index of 2.4 L/min/m2 may not be sufficient and the maximum requirement of bypass flow index may be 3.2 L/min/m2 or more in this patient population. [source]

Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

CLINICAL CARDIOLOGY, Issue 2 2010
Xue-Cun Liang MD
Background The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle. Hypothesis Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS. Methods A total of 27 patients, age 16.5 ± 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity. Results Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%,94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05). Conclusion Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS. Copyright © 2010 Wiley Periodicals, Inc. [source]

The Benefits of High-flow Management in Children With Pulmonary Atresia

Fetal cardiac effects of maternal hyperglycemia during pregnancy

BIRTH DEFECTS RESEARCH, Issue 6 2009
Niamh Corrigan
Maternal diabetes mellitus is associated with increased teratogenesis, which can occur in pregestational type 1 and type 2 diabetes. Cardiac defects and with neural tube defects are the most common malformations observed in fetuses of pregestational diabetic mothers. The exact mechanism by which diabetes exerts its teratogenic effects and induces embryonic malformations is unclear. Whereas the sequelae of maternal pregestational diabetes, such as modulating insulin levels, altered fat levels, and increased reactive oxygen species, may play a role in fetal damage during diabetic pregnancy, hyperglycemia is thought to be the primary teratogen, causing particularly adverse effects on cardiovascular development. Fetal cardiac defects are associated with raised maternal glycosylated hemoglobin levels and are up to five times more likely in infants of mothers with pregestational diabetes compared with those without diabetes. The resulting anomalies are varied and include transposition of the great arteries, mitral and pulmonary atresia, double outlet of the right ventricle, tetralogy of Fallot, and fetal cardiomyopathy. A wide variety of rodent models have been used to study diabetic teratogenesis. Both genetic and chemically induced models of type 1 and 2 diabetes have been used to examine the effects of hyperglycemia on fetal development. Factors such as genetic background as well as confounding variables such as obesity appear to influence the severity of fetal abnormalities in mice. In this review, we will summarize recent data on fetal cardiac effects from human pregestational diabetic mothers, as well as the most relevant findings in rodent models of diabetic cardiac teratogenesis. Birth Defects Research (Part A), 2009. © 2009 Wiley-Liss, Inc. [source]

Disturbed morphogenesis of cardiac outflow tract and increased rate of aortic arch anomalies in the offspring of diabetic rats

BIRTH DEFECTS RESEARCH, Issue 12 2004
Daniël G.M. Molin
Abstract BACKGROUND Maternal diabetes (MD) is a risk factor for offspring to develop cardiovascular anomalies; this is of growing clinical concern since the number of women in childbearing age with compromised glucose homeostasis is increasing. Hyperglycemia abrogates cardiovascular development in vitro; however, a link to cardiovascular defects in diabetic offspring remains to be investigated. METHODS We have studied cardiovascular development in offspring of MD rats by examining serial histological sections of GD 12.0,18.0 offspring. Development of pharyngeal arch artery malformations was analyzed and related to intracardiac anomalies. RESULTS Pharyngeal arch artery and intracardiac defects were present in 27 of 37 MD GD 13.0,18.0 offspring. Early sixth arch arteries showed abrogated arteriogenesis, whereas fourth arch artery defects developed as a result of abnormal remodeling. Morphometrical analysis showed increased apoptosis in regressing artery segments and reduced apoptosis in persisting artery segments. Double outlet right ventricle with infundibular stenosis (tetralogy of Fallot) was predominantly found in combination with sixth artery defects and pulmonary atresia. As confirmed by morphometric analysis and three-dimensional (3D)-reconstructions, outflow tract defects coincided with endocardial cushion hypoplasia. Cases with teratology of Fallot additionally showed a shorter outflow tract. No relation with apoptosis or disturbed neural crest cell migration was found. CONCLUSIONS Our data uniquely demonstrate mechanistic differences involved in the development of sixth and fourth artery anomalies. Whereas increased apoptosis induces fourth artery anomalies, pulmonary outflow obstruction abrogates sixth artery differentiation independent of apoptosis. The model presented allows analysis of diabetic conditions on cardiovascular development in vivo, essential for elucidating this teratology. Birth Defects Research (Part A), 2004. © 2004 Wiley-Liss, Inc. [source]

Transcatheter occlusion of a residual muscular ventricular septal defect using an Amplatzer duct occluder in a child with congenitally corrected transposition of the great arteries

CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 2 2006
Gary E. Stapleton MD
Abstract Transcatheter occlusion has become an acceptable alternative to surgery in patients with congenital muscular and residual post-surgical ventricular septal defects (VSD). We present a case of an 11 year old male with congenitally corrected transposition of the great arteries, dextrocardia, pulmonary atresia, VSD, and advanced second degree atrioventricular block who underwent successful transcatheter occlusion of a residual post-surgical VSD with an Amplatzer duct occluder, in preparation for transvenous pacemaker implantation. © 2006 Wiley-Liss, Inc. [source]

Cannulation of patent arterial duct in patients with pulmonary atresia and ventricular septal defect

CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 3 2005
Bhava R.J. Kannan DM
Abstract During cardiac catheterization in patients with pulmonary atresia and ventricular septal defect, cannulation of the arterial duct was attempted in 23 children via antegrade approach. It was successful in all but two children. Injection of contrast through antegradely placed catheter resulted in improved opacification of pulmonary arteries. This technique facilitated transcatheter dilation of spontaneously closed arterial duct in two children. © 2005 Wiley-Liss, Inc. [source]

Percutaneous occlusion of a pulmonary aneurysm causing hemoptysis in a patient with pulmonary atresia and aortopulmonary collaterals

CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 2 2005
Gordon E. Pate
Abstract A 28-year-old male was referred for cardiac catheterization because of recurrent severe hemoptysis necessitating resuscitation and subsequently preventing weaning from ventilation. He had a history of pulmonary atresia, ventricular septal defect, overriding aorta with right-sided aortic arch diagnosed at birth. Eisenmenger's syndrome ensued and he was not felt to be suitable for corrective cardiac surgery. He had multiple major aortopulmonary collateral vessels to both lungs with a large aneurysm in an artery to the right lower lobe, which was suspected to be the source of his bleeding. Occlusion of this aneurysm was achieved percutaneously using an Amplatzer septal occluder device. He had no further bleeding and was successfully weaned from ventilation. Six months later, he has recovered to his functional baseline and has not had any further episodes of hemoptysis. © 2005 Wiley-Liss, Inc. [source]

Congenital heart disease in 111 225 births in Belgium: birth prevalence, treatment and survival in the 21st century

ACTA PAEDIATRICA, Issue 3 2009
Philip Moons
Abstract Aim: To investigate the birth prevalence, treatment modalities and short-term survival of children with congenital heart disease who were born in 2002. Methods: We undertook a retrospective review of medical records of all patients who were born in 2002, and were diagnosed, treated and/or followed-up in one of the seven-paediatric cardiology programmes in Belgium. Results: In 111 225 births, 921 children with congenital heart disease were detected, yielding a birth prevalence of 8.3 per 1000. The most frequently occurring conditions were ventricular septal defects (VSDs) (33%), ostium secundum atrial septal defects (18%) and pulmonary valve abnormalities (10%). Thirty-nine percent of the children either had a cardiosurgical operation or catheter intervention. In this study, 4% of the children died. The actuarial survival at 6 months and 1 year of age was 97% and 96%, respectively and remained stable after then. Compared to other heart defects, mortality was higher in univentricular physiology, pulmonary atresia with VSD, left ventricle outflow obstruction and tetralogy of Fallot. Conclusion: Survival of congenital heart disease is excellent and continued to improve in the early 21st century. New therapeutic options are increasingly used. This study provides baseline data for the longitudinal follow-up of this cohort. [source]

Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

Coronary arteries in fetal life: physiology, malformations and the "heart-sparing effect"

ACTA PAEDIATRICA, Issue 2004
R ChaouiArticle first published online: 2 JAN 200
The present knowledge of coronary arteries in prenatal diagnosis is reviewed with a focus on three aspects: the physiology and visualization of coronary flow, malformations involving the coronary arteries, and the "heart-sparing effect". Visualization of coronary arteries in a healthy human fetus is possible in real-time and colour Doppler during the last 10wk of gestation when ultrasound conditions are excellent. Visualization at an earlier gestational age (up to 13 wk) is feasible mainly in association with malformations and impending hypoxia. The main coronary malformations that can be visualized in utero are the ventriculo-coronary communications in fetuses with pulmonary atresia. In the last few years, interest has been focused on the "heart-sparing effect", defined as the increased perfusion of the coronary arteries in fetuses with severe growth restriction and abnormal Doppler velocimetry in the peripheral vessels. Increased perfusion detectable with colour and pulsed Doppler is a late sign of fetal compromise in hypoxaemia. It confirms animal experiments that have demonstrated dilatory reserves of the fetal coronary arteries under chronic hypoxaemia. The outcome of 21 fetuses showing the "heart-sparing effect" before 32 wk gestation was poor: nine fetuses died in utero and two after birth, the median weight at birth was 630 g. In summary, our knowledge of the coronary arteries in the fetus is based on the diagnostic means used in prenatal diagnosis. New information in this field may also contribute to a better understanding of coronary heart disease later in life. [source]

Development of the coronary vasculature and its implications for coronary abnormalities in general and specifically in pulmonary atresia without ventricular septal defect

ACTA PAEDIATRICA, Issue 2004
AC Gittenberger-de Groot
Aim: Coronary vascular anomalies are an important factor in congenital heart disease in the neonate. However, our knowledge of the pathomorphogenesis is still defective. Material and methods: 1) Study of coronary anomaly variations in congenital heart disease using specimens and 2) study of the role of epicardium-derived cells (EPDC) and neural crest cells in coronary vascular formation using quail-chicken chimeras. Results: The clinical and pathological data revealed the existence of ventriculo-coronary arterial communications during fetal life before pulmonary atresia was established. This supported a primary coronary developmental anomaly as the origin of some cases of pulmonary atresia as opposed to other cases in which the pulmonary orifice atresia was the primary anomaly. Our experimental work showed the high relevance of the development of the epicardium and epicardium-derived cells for the formation of the coronary vasculature, and showed the coronary vascular ingrowth into the myocardium and subsequently into the aorta and the right atrium. The absence of epicardium-derived cells leads to embryonic death, while delayed outgrowth could result in the absence of the main coronary arteries to pinpoint orifice formation. In these cases, the circulation was maintained through ventriculocoronary arterial communications. Neural crest cells were important for the patterning of the coronary vasculature. We have extended this knowledge to a number of other heart malformations. Conclusions: Coronary vascular anomalies are highly linked to the development of extracardiac contributors like the epicardium and the neural crest. A proper interaction between these cell types and the myocardium and aortic arterial wall are important for normal vascular development. [source]