P4646Prospective study for antenatal diagnosis of coarctation of the aorta

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.

PEDIATRICS ◽  
1969 ◽  
Vol 44 (6) ◽  
pp. 1024-1028
Author(s):  
P. B. Deverall ◽  
J. F. N. Taylor ◽  
G. S. Sturrock ◽  
Eoin Aberdeen

Hemodynamic signs of coarctation of the aorta were present in a neonate dying in cardiac failure. A cerebral arteriovenous fistula was found at autopsy. No obstructive lesion of the aortic arch was present. Development of the aortic isthmus may be impaired if diminished flow through this segment is present. Reduced flow may be present if most of the systemic output is diverted to a fistula proximal to the isthmus, distal systemic flow being maintained by flow from right-to-left through the ductus arteriosus. Spontaneous duct closure after birth may then be followed by a reduction in distal systemic flow, resulting in signs suggestive of coarctation.


PEDIATRICS ◽  
1960 ◽  
Vol 26 (1) ◽  
pp. 109-121
Author(s):  
Irving H. Glass ◽  
William T. Mustard ◽  
John D. Keith

A review of 108 cases of coarctation of the aorta diagnosed under 1 year of age, and admitted to The Hospital for Sick Children, Toronto, over a 12-year period, is presented. Preductal coarctation accounted for 90% of the fatalities under 1 year. Patency of the ductus arteriosus occurned in 98% of cases in the first 6 months of life. Forty-nine per cent of cases of coarctation of the aorta under 1 year of age have major cardiac anomalies other than patent ductus: ventricular septal defect, 33%; transposition of great vessels, 10%; and atrial septal defect, 6%. Problems in diagnosis occurred when blood pressure readings were equivocal. Operative mortality for coarctation of the aorta in the first year of life was 41%, while after 1 month of age it was only 29%. Mortality in patients not operated on during the first year of life was 50%, while for those less than 1 month of age with symptoms and signs of decompensation and not operated on, the mortality was 87%. In dealing with coarctation of the aorta in the first year of life the following are recommended: 1) When blood pressure readings in the arms and legs are equivocal, the readings should be repeated frequently until the diagnosis is clarified; 2) aortogram should be performed on patients difficult to diagnose; 3) babies presenting with symptoms in the first month of life should be operated on promptly unless they show a dramatic response in a 12-hour trial of treatment with digitalis; 4) babies after 1 month of age who respond to digitalis may be kept on this medication until adequate adjustment in circulatory hemodynamics has taken place and surgery can be performed at the optimum age.


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.


PEDIATRICS ◽  
1975 ◽  
Vol 56 (4) ◽  
pp. 562-569
Author(s):  
Norman S. Talner ◽  
Michael A. Berman

The sequence of events leading to the development of acute obstruction in two infants with coarctation has been defined by clinical, hemodynamic, and angiographic studies. One infant had normally related great arteries and an isolated coarctation, while the other infant had transposition with a hypoplastic right ventricle, a ventricular septal defect, narrowing of the aortic isthmus, and a localized site of coarctation. In each instance, a posterior aortic shelf-the basic pathologic lesion in coarctation-could be observed angiographically. Signs of aortic obstruction were absent, however, as long as the ductus arteriosus was widely patent. Following ductal obliteration, femoral pulsations diminished, a peak systolic pressure difference was recorded between the ascending and descending aorta, and a discrete area of juxtaductal coarctation was seen. The basic malformation (posterior aortic curtain) would appear to exist in utero, possibly as an aortic branch point. This lesion is nonobstructive as long as blood can traverse the aortic isthmus through the aortic end of the ductus into the descending aorta. Postnatally, as the ductus arteriosus undergoes goes constriction at its aortic insertion, signs of acute aortic obstruction may become apparent. Normal femoral arterial pulsations during the newborn examination do not definitively exclude coarctation. Pediatricians should recheck at 2 weeks of age if the infant is asymptomatic or sooner if there are signs of cardiac failure to establish the presence or absence of this defect.


2019 ◽  
Vol 11 (4) ◽  
pp. NP239-NP243
Author(s):  
Neha Bansal ◽  
Preetha L. Balakrishnan ◽  
Sanjeev Aggarwal

We report the case of a premature newborn diagnosed with coarctation of the aorta after spontaneous closure of ductus arteriosus who was successfully managed with prostaglandin E1 infusion until surgical repair could be performed. This case, together with a review of the literature, suggests an important role for prostaglandin in the management of coarctation even in the absence of a patent ductus arteriosus. The putative mechanism for the utility of prostaglandin infusion is that it may relieve the obstruction in neonates with severe coarctation by not only opening of the ductus but, in select cases, relaxing the ductal tissue encircling the aortic isthmus region. We also found a possible dose dependence of the efficacy of the prostaglandin infusion when the ductus is closed.


2018 ◽  
Vol 28 (12) ◽  
pp. 1489-1490
Author(s):  
Katsutoshi Nakano ◽  
Taiyu Hayashi ◽  
Hiroshi Ono

AbstractA male neonate with d-transposition of the great arteries was diagnosed with the concomitant anomaly of left circumflex aortic arch and right-sided ductus arteriosus, which formed a vascular ring. Initial postnatal echocardiography had demonstrated an obscured aortic isthmus mimicking coarctation of the aorta, which could be a diagnostic clue to circumflex aortic arch.


Author(s):  
Katrin Fricke ◽  
Petru Liuba ◽  
Constance G. Weismann

AbstractThe aim of the study is to identify reliable quantitative fetal echocardiographic predictors for postnatal development of coarctation (CoA). In this retrospective study, we included 65 fetuses with a prenatally suspected, isolated CoA, born 2010–2018. Dimensions of the cardiac structures, aortic, and ductal arches expressed as ratios and Z-scores were analyzed in relation to outcome. Fetuses that developed CoA postnatally (34%) exhibited significantly smaller Z-scores of left cardiac structures from the mitral valve to the aortic isthmus. The most sensitive and specific predictors were a carotid-subclavian artery index (CSAI) of < 0.78 (92.3% sensitivity, 96.8% specificity) or a product of isthmus-to-duct ratio in the three-vessel trachea view (3VT) and the mitral-to-tricuspid valve ratio (I/D3VTxMV/TV) of < 0.37 (100% sensitivity, 94.6% specificity). When comparing different Z-score datasets, we observed large and highly significant differences. Postnatal CoA can be predicted with high accuracy during fetal life using CSAI or I/D3VTxMV/TV. The latter may be particularly useful if adequate sagittal aortic arch images cannot be obtained. As significant and clinically unacceptable differences in Z-scores were observed for the same measurements, this calls for a large multi-center collaboration to generate reliable fetal echocardiographic Z-scores.


2004 ◽  
Vol 14 (2) ◽  
pp. 206-209 ◽  
Author(s):  
Gerald Wendelin ◽  
Erwin Kitzmüller ◽  
Ulrike Salzer-Muhar

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.


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.


PEDIATRICS ◽  
1989 ◽  
Vol 83 (2) ◽  
pp. 200-203
Author(s):  
Gerard R. Martin ◽  
Lowell W. Perry ◽  
Charlotte Ferencz

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P&lt;.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P&lt; .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P&lt;.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P&lt;.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."


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