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Ultrasound in Obstetrics & Gynecology :... Oct 2019To examine the performance of the routine 11-13-week scan in detecting fetal non-chromosomal abnormalities.
OBJECTIVE
To examine the performance of the routine 11-13-week scan in detecting fetal non-chromosomal abnormalities.
METHODS
This was a retrospective study of prospectively collected data from 100 997 singleton pregnancies attending for a routine ultrasound examination of fetal anatomy, performed according to a standardized protocol, at 11-13 weeks' gestation. All continuing pregnancies had an additional scan at 18-24 weeks and 71 754 had a scan at either 30-34 or 35-37 weeks. The final diagnosis of fetal abnormality was based on the results of postnatal examination in cases of live birth and on the findings of the last ultrasound examination in cases of pregnancy termination, miscarriage or stillbirth. The performance of the 11-13-week scan in the detection of fetal abnormalities was determined.
RESULTS
The study population contained 1720 (1.7%) pregnancies with a fetal abnormality, including 474 (27.6%) detected on the first-trimester scan, 926 (53.8%) detected on the second-trimester scan and 320 (18.6%) detected in the third trimester or postnatally. At 11-13 weeks' gestation, we diagnosed all cases of acrania, alobar holoprosencephaly, encephalocele, tricuspid or pulmonary atresia, pentalogy of Cantrell, ectopia cordis, exomphalos, gastroschisis and body-stalk anomaly and > 50% of cases of open spina bifida, hypoplastic left heart syndrome, atrioventricular septal defect, complex heart defect, left atrial isomerism (interrupted inferior vena cava with normal intracardiac anatomy), lower urinary tract obstruction, absence of extremities, fetal akinesia deformation sequence and lethal skeletal dysplasia. Common abnormalities that were detected in < 10% of cases at 11-13 weeks included ventriculomegaly, agenesis of the corpus callosum, isolated cleft lip, congenital pulmonary airway malformation, ventricular septal defect, abdominal cysts, unilateral renal agenesis or multicystic kidney, hydronephrosis, duplex kidney, hypospadias and talipes.
CONCLUSIONS
A routine 11-13-week scan, carried out according to a standardized protocol, can identify many severe non-chromosomal fetal abnormalities. A summary statistic of the performance of the first-trimester scan is futile because some abnormalities are always detectable, whereas others are either non-detectable or sometimes detectable. To maximize prenatal detection of abnormalities, additional scans in both the second and third trimesters are necessary. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Adult; Congenital Abnormalities; Female; Fetus; Gestational Age; Humans; Nuchal Translucency Measurement; Pregnancy; Pregnancy Trimester, First; Pregnancy Trimester, Second; Pregnancy Trimester, Third; Prenatal Care; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 31408229
DOI: 10.1002/uog.20844 -
European Heart Journal. Case Reports Jan 2022
PubMed: 35106438
DOI: 10.1093/ehjcr/ytab474 -
British Heart Journal Oct 1956
Topics: Humans; Tricuspid Atresia; Tricuspid Valve
PubMed: 13374157
DOI: 10.1136/hrt.18.4.499 -
British Medical Journal Nov 1957
Topics: Humans; Tricuspid Atresia; Tricuspid Valve
PubMed: 13479699
DOI: 10.1136/bmj.2.5056.1283 -
Frontiers in Pediatrics 2014The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to... (Review)
Review
The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered "functional" univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity. In this setting, the surgical repair is univentricular with "Fontan" operation, bypassing the ventricular mass. In other words, functionally univentricular heart is a condition in which, after surgery, only one ventricle sustain systemic circulation. Univentricular hearts (double inlet or absent AV connection) almost invariably show two ventricular chambers, one main and one accessory, which lacks an inlet portion. The latter is located posteriorly when morphologically left and anteriorly when morphologically right. As far as double inlet left ventricle, this is usually associated with discordant ventriculo-arterial (VA) connection (transposition of the great arteries) and all the blood flow to the aorta, which takes origin from the hypoplastic anterior right ventricle, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet left ventricle may be rarely associated with VA concordance (Holmes heart). As far as double inlet right ventricle with posterior hypoplastic left ventricular cavity, ventriculo-arterial connection is usually of double outlet type; thus the term double inlet-outlet right ventricle may be coined. Absent right or left AV connection may develop in the setting of both d- or l-loop, whatever the situs. In this condition, the contra-lateral patent AV valve may be either mitral or tricuspid in terms of morphology and the underlying ventricle (main chamber) either morphologically left or right. Establishing the loop, whatever right or left (also called right or left ventricular topology), is a fundamental step in the segmental-sequential analysis of congenital heart disease.
PubMed: 25072035
DOI: 10.3389/fped.2014.00062 -
Indian Journal of Thoracic and... May 2020Since the intra-extracardiac Fontan (IECF) was popularized by Jonas in 2008, its claimed advantages over the traditional extracardiac or lateral tunnel Fontan are...
OBJECTIVE
Since the intra-extracardiac Fontan (IECF) was popularized by Jonas in 2008, its claimed advantages over the traditional extracardiac or lateral tunnel Fontan are simplicity, suitability for nearly all subsets, flow characteristics, low risk of sinus node artery injury, and possibly, a lower incidence of arrhythmias. In this paper, we present our early experience with this modification of the Fontan operation.
METHODS
Between 2009 and 2018, 10 patients underwent IECF on cardiopulmonary bypass (CPB) and cardioplegic arrest at our institute. Analysis of preoperative, intraoperative, and early follow-up results was performed. A polytetrafluoroethylene (PTFE) graft was sutured proximally to the orifice of the inferior vena cava (IVC) and distally to the ipsilateral bidirectional superior cavopulmonary (BSCP) junction.
RESULTS
Nine patients had undergone a previous BSCP connection, and one patient had a primary IECF. Diagnoses were double outlet right ventricle ( = 2), unbalanced atrioventricular septal defect with associated atrioventricular valve regurgitation ( = 3), single ventricle with anomalies of cardiac situs ( = 2), and tricuspid atresia with borderline pulmonary arteries ( = 1) or tricuspid atresia with borderline pulmonary artery pressures ( = 2). Median aortic cross-clamp and CPB times were 42 min and 82 min respectively. There were no early or late deaths. Median intensive care stay was 3 days (1 to 23 days). There were no arrhythmias. Mean duration of pleural effusions was 9.5 (median 5.5) days. There were no arrhythmias at a median follow-up of 5 years (range 1 month to 9.3 years).
CONCLUSION
The IECF is simple, particularly at reoperations, in borderline patients and those needing concomitant intracardiac procedures. Early results are promising. These patients need constant surveillance.
PubMed: 33061125
DOI: 10.1007/s12055-019-00862-7 -
Progress in Cardiovascular Diseases 2018In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to... (Review)
Review
In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. These patients require lifelong management including a thorough knowledge of their anatomic substrate, hemodynamic status, management of rhythm and ventricular function along with multi organ evaluation. As these patients enter middle age, there is increasing awareness regarding the long-term complications and mortality. This review highlights the long-term outcomes of the Fontan procedure and management of late sequelae.
Topics: Fontan Procedure; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans; Long Term Adverse Effects; Patient Care Management
PubMed: 30236751
DOI: 10.1016/j.pcad.2018.09.005 -
JTCVS Open Dec 2021
PubMed: 36004148
DOI: 10.1016/j.xjon.2021.10.044