Outcome and Impact of Associated Left-Sided Cardiac Lesions in Coarctation of the Aorta Diagnosed During Fetal Life

Author(s):  
Stavros Polyviou ◽  
Marietta Charakida ◽  
Owen I. Miller ◽  
Thomas Witter RSCN ◽  
Trisha V. Vigneswaran ◽  
...  
1995 ◽  
Vol 5 (2) ◽  
pp. 132-139 ◽  
Author(s):  
Ana C. Durán ◽  
Luciano Daliento ◽  
Carla Frescura ◽  
Giovanni Stellin ◽  
Valentín Sans-Coma ◽  
...  

AbstractThe morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.


2018 ◽  
Vol 52 ◽  
pp. 184-184
Author(s):  
M. Slodki ◽  
K. Taranenko ◽  
K. Zych-Krekora ◽  
M. Respondek-Liberska

2011 ◽  
Vol 19 (5) ◽  
pp. 487-495 ◽  
Author(s):  
Damien Kenny ◽  
Ziyad M. Hijazi

2006 ◽  
Vol 16 (1) ◽  
pp. 30-33 ◽  
Author(s):  
Colin J. McMahon ◽  
William Reardon

We investigated the prevalence and forms of congenital cardiac malformations in six children with Kabuki syndrome. There were three girls and three boys, diagnosed at a median age of 1.7 years, with a range from 0.7 to 11.1 years. Cardiac lesions were present in five children (83%), specifically complete transposition, tetralogy of Fallot, coarctation of the aorta, ventricular septal defect, and patency of the arterial duct. Characteristic dysmorphic findings were noted in all patients, as well as a strong predisposition to severe problems with feeding in the neonatal period, and developmental delay. Cardiologists should be alert to this syndrome in children who present with the aforementioned constellation of findings, as patients with mild expression of Kabuki syndrome may go unrecognized for a considerable time.


PEDIATRICS ◽  
1951 ◽  
Vol 8 (2) ◽  
pp. 192-203
Author(s):  
ROBERT C. BAHN ◽  
JESSE E. EDWARDS ◽  
JAMES W. DUSHANE

This is a report of three cases in each of which an infant exhibited coarctation of the aorta at the level of a closed or closing ductus arteriosus. In all cases the collateral channels were poorly developed. It appears from the available evidence that death resulted from left ventricular congestive failure secondary to an obstructive hypertension. The development of collaterals to by-pass a coarctation of the aorta seems to depend upon the relation of the aortic coarctation to the aortic mouth of the ductus arteriosus. When the coarctation lies distal to the ductus, collaterals develop. When the coarctation lies proximal to the ductus, adequate collaterals do not develop before birth. When the coarctation lies opposite the aortic mouth of the ductus arteriosus, the development of collaterals during fetal life seems to depend upon the direction that the blood flowing in the ductus arteriosus takes. When the flow is into the aorta proximal to the coarctation, collaterals would be expected to develop; while when the ductal flow is into the descending aorta, adequate collaterals would not be expected to develop in the fetus. The three cases reported fall into the latter category.


2012 ◽  
Vol 23 (4) ◽  
pp. 560-567 ◽  
Author(s):  
Nikolas Christensen ◽  
Helle Andersen ◽  
Ester Garne ◽  
Diana Wellesley ◽  
Marie-Claude Addor ◽  
...  

AbstractObjectiveTo describe the epidemiology of chromosomal and non-chromosomal cases of atrioventricular septal defects in Europe.MethodsData were obtained from EUROCAT, a European network of population-based registries collecting data on congenital anomalies. Data from 13 registries for the period 2000–2008 were included.ResultsThere was a total of 993 cases of atrioventricular septal defects, with a total prevalence of 5.3 per 10,000 births (95% confidence interval 4.1 to 6.5). Of the total cases, 250 were isolated cardiac lesions, 583 were chromosomal cases, 79 had multiple anomalies, 58 had heterotaxia sequence, and 23 had a monogenic syndrome. The total prevalence of chromosomal cases was 3.1 per 10,000 (95% confidence interval 1.9 to 4.3), with a large variation between registers. Of the 993 cases, 639 cases were live births, 45 were stillbirths, and 309 were terminations of pregnancy owing to foetal anomaly. Among the groups, additional associated cardiac anomalies were most frequent in heterotaxia cases (38%) and least frequent in chromosomal cases (8%). Coarctation of the aorta was the most common associated cardiac defect. The 1-week survival rate for live births was 94%.ConclusionOf all cases, three-quarters were associated with other anomalies, both chromosomal and non-chromosomal. For infants with atrioventricular septal defects and no chromosomal anomalies, cardiac defects were often more complex compared with infants with atrioventricular septal defects and a chromosomal anomaly. Clinical outcomes for atrioventricular septal defects varied between regions. The proportion of termination of pregnancy for foetal anomaly was higher for cases with multiple anomalies, chromosomal anomalies, and heterotaxia sequence.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Meysonnier Marechal ◽  
S Di Filippo ◽  
N Pangaud ◽  
C Galoin Bertail ◽  
H Joly ◽  
...  

Abstract The objective of this study was to define echocardiographic (ECHO) parameters during fetal life, to predict postnatal aortic coarctation (COA). Material and methods This prospective single-center study from 2010 to 2018 included all fetus diagnosed with isolated ventricular and/ or great vessels asymetry (right heart dominance). Complex CHD were excluded. The cohort was divided in patients with coarctation after birth (COA) and those free from coarctation (noCOA). Left heart, aortic and ductus measurements were collected serially at second trimester (T2), third trimester (T3) ad Z-scores were assessed. COA and noCOA were compared, sensitivity (Se) and specificity (Spec) and ROC curves (cut-off values) were assessed for each parameter. Results 67 cases were included: 34 (50.7%) ranged in group COA and 33 (49.3%) in noCOA. Coarctation occurred more frequently if suspected at T2 (70.6% in COA vs 30.3% in noCOA, p=0.001). Mitral annulus (T3) was 8.6mm in COA vs 10.3mm in noCOA (p=0.002) with cut-off <7.3mm (Se= 50%, Spec= 93.6%, OR= 14.5). Aortic annulus diameter (T3) was 4.8mm in COA vs 6mm in noCOA (p=0.005), with cut-off <5.4mm (Se= 65%, Spec= 77%, OR= 6.3). Aortic isthmus diameter (T3) was 1.4mm in COA vs 2.8mm in noCOA (p=0.003), with cut-off <3mm (Se= 91%, Spec= 65%, OR= 18.9). Ductus arteriosus/ aortic isthmus ratio (T3) was 2.5 in COA vs 1.8 in noCOA (p=0.01) with cut-off >1.57mm (Se= 90.5%, Spec= 62%, OR= 15.4). Conclusion Mitral annulus <7.3mm, aortic annulus <5.4mm, aortic isthmus <3mm and ductus/isthmus ratio >1.57 assessed during 3rd trimester of fetal life may help to predict postnatal coractation of the aorta. These results have to be confirmed by larger prospective studies.


2003 ◽  
Vol 13 (6) ◽  
pp. 565-567 ◽  
Author(s):  
Christian Schreiber ◽  
Sabine Bleiziffer ◽  
Rüdiger Lange

Our long-term follow-up has revealed that symmetrical development of the breasts is significantly impaired in pre-pubescent female patients subsequent to an anterolateral thoracotomy. Although standard posterolateral and anterolateral right-sided thoracotomies are used for correction of “simple” cardiac lesions such as patency of the arterial duct, coarctation of the aorta, or atrial septal defect, the required partial transection of large muscle groups, and injury to the developing tissues of the breast, may contribute to an unfavourable cosmetic outcome. Over the years, many surgeons, mostly specialising in thoracic procedures, have advocated mini- or axillary thoracotomies. In an attempt to improve surgical and cosmetic outcome, we have now adopted such a muscle-sparing approach, using a small horizontal midaxillary incision. We have now successfully employed the technique to close atrial septal defects in the oval fossa in 17 pre-pubescent females.


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