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Journal of the American College of... Mar 1983Two-dimensional echocardiograms of the mitral valve orifice area were obtained in 50 normal pediatric subjects, 15 patients with congenital mitral stenosis and 7...
Two-dimensional echocardiograms of the mitral valve orifice area were obtained in 50 normal pediatric subjects, 15 patients with congenital mitral stenosis and 7 patients with tricuspid atresia. The mitral area was measured near the tips of the mitral valve leaflets from a diastolic cross-sectional image of the left ventricle. The cardiac images were recorded on videotape and later transferred to video disc for high resolution contour tracing. Contour analysis was performed by a special purpose microcomputer system for calculation of the enclosed calibrated area. In normal patients, there was an excellent correlation (r = 0.95) between mitral valve area (MVA) (in cm2) and body surface area (BSA) (in m2) described by MVA = 4.83 X BSA - 0.07. Each patient with mitral stenosis had a mitral valve area that measured less than the third percentile predicted from the normal regression formula. In eight patients in whom the Gorlin formula could be applied, there was excellent correlation (r = 0.95) between echocardiographic and hemodynamic measurements of mitral valve area. Each patient with tricuspid atresia had a very large mitral valve area (greater than 99th percentile of normal). It is concluded that noninvasive measurement of mitral valve orifice area can be accurately achieved by two-dimensional echocardiography in pediatric patients with congenital mitral stenosis, allowing serial measurement of their mitral valve area.
Topics: Child; Child, Preschool; Echocardiography; Humans; Infant; Mitral Valve; Mitral Valve Stenosis; Reference Values; Tricuspid Valve
PubMed: 6826975
DOI: 10.1016/s0735-1097(83)80202-2 -
Cardiology in the Young Feb 2024Atresia of the aortic valve is usually associated with hypoplasia of the mitral valve and the left ventricle. In very rare cases, a ventricular septal defect may be...
Atresia of the aortic valve is usually associated with hypoplasia of the mitral valve and the left ventricle. In very rare cases, a ventricular septal defect may be associated with aortic atresia, when left ventricle and mitral valve are normal-sized, due to the presence of an outflow for the left ventricle through the ventricular septal defect. We present the multi-modality imaging findings of an adolescent girl who presented with breathlessness and was later found to have aortic valvar atresia with a normal-sized left ventricle.
Topics: Female; Humans; Adolescent; Heart Ventricles; Aortic Valve; Heart Septal Defects, Ventricular; Mitral Valve; Dyspnea
PubMed: 38167172
DOI: 10.1017/S1047951123004444 -
British Heart Journal Apr 1977The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular...
The results are reported of a study of 83 necropsied hearts with atresia of the right atrioventricular orifice. It is emphasised that right atrial or atrioventricular orificial atresia is a better term to describe this anomaly than "tricuspid atresia". Use of the latter term can be confusing when the morphologically tricuspid valve is located beneath the left atrium. It is accepted that the definition employed may include cases in which the mitral valve may be atretic, blocking normal exit from the right atrium, but it is argued that such cases would present clinically as "tricuspid atresia" and therefore are correctly designated as right atrial orificial atresia. The results show that the majority of hearts with right atrial orificial atresia have the ventricular morphology of primitive ventricle, most with, but a few without an outlet chamber. However, in a minority of hearts an imperforate membrane interposes between the right atrium and a formed but hypoplastic right ventricle. In two of the hearts, the imperforate membrane showed features of Ebstein's malformation. The hearts could be further subdivided according to the ventriculoarterial connection. Most had normally connected arteries (66 of 83), and all but 2 also had normal relations between the arteries; in these 2 hearts there was "anatomically corrected malposition". Twelve hearts showed transposition, one had double-outlet outlet chamber, and another persistent truncus arteriosus. The remaining 3 hearts, all without outlet chamber, had by definition a double outlet connection. A segmental approach provides the best way of classifying this anomaly, and an embryological explanation is offered for the variations in anatomy observed.
Topics: Aortic Valve Stenosis; Coronary Vessel Anomalies; Coronary Vessels; Ebstein Anomaly; Heart Atria; Heart Defects, Congenital; Heart Ventricles; Humans; Pulmonary Valve Stenosis; Tricuspid Valve
PubMed: 869977
DOI: 10.1136/hrt.39.4.414 -
Pediatric Cardiology 1999Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity... (Comparative Study)
Comparative Study
Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity and death. The purpose of this study was to determine the incidence of contralateral atrioventricular valve (AVV) pathologies in mitral valve atresia (MA) and tricuspid valve atresia (TA). We retrospectively reviewed the echocardiographic data of 50 neonates with MV and 20 with TA. Appearance of the papillary muscles, chordae tendinae, and valve leaflets was assessed. AVV regurgitation was semiquantitated by color-flow Doppler and the AVV annulus diameter was measured and indexed to body surface area. MV abnormalities were found in 9 of 20 (45%) of patients with TA. The MV was myxomatous in 9 patients, the leaflets were redundant in 5 patients, and prolapsing occurred in 4 patients. Mild regurgitation was found in 2 patients. In 18 of 20 (90%) patients MV annulus size was larger than 95% of predicted normal values. TV abnormalities were found in 12 of 50 (24%) patients with MA. The TV was myxomatous in 4 patients, prolapsing in 2, and redundant in 3, and moderate TV regurgitation was found in 3 patients. In 29 of 50 (58%) patients TV annulus size was larger than 95% of predicted normal values. Contralateral AVV abnormalities in tricuspid and mitral valve atresia are common and should be assessed carefully before surgical procedures.
Topics: Blood Flow Velocity; Echocardiography, Doppler; Female; Follow-Up Studies; Fontan Procedure; Humans; Infant, Newborn; Male; Mitral Valve; Mitral Valve Insufficiency; Pulmonary Artery; Retrospective Studies; Tricuspid Atresia; Tricuspid Valve Insufficiency
PubMed: 10089244
DOI: 10.1007/s002469900440 -
Annals of Pediatric Cardiology 2020Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of...
Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of the mitral valvar orifice has previously been reported in the setting of mirror-imaged atrial arrangement. We report a neonate with unguarding of the mitral valvar orifice in the setting of usual atrial arrangement, but with discordant atrioventricular connections and pulmonary atresia.
PubMed: 32030042
DOI: 10.4103/apc.APC_4_19 -
International Journal of Cardiology Feb 1986We studied hearts with aortic atresia to determine the relationship between cardiac morphology and fetal cardiac blood flow. We compared measurements of aortic,...
We studied hearts with aortic atresia to determine the relationship between cardiac morphology and fetal cardiac blood flow. We compared measurements of aortic, pulmonary, and tricuspid valve circumference and right ventricular wall thickness among 37 hearts with aortic atresia and mitral hypoplasia, 14 hearts with aortic atresia together with mitral atresia and 24 normal hearts. Right ventricular free wall thickness and tricuspid and pulmonary valve annular circumferences were greater and aortic circumferences were smaller in hearts with aortic atresia than in normal hearts (P less than 0.05). Between the subgroups of aortic atresia, the aortic circumference was 5 +/- 1 mm in those with mitral atresia compared to 7 +/- 2 mm in association with mitral hypoplasia (P less than 0.05). The morphologic differences between the subgroups of aortic atresia are consistent with differences in fetal blood flow. Left heart blood flow was likely to be greater during development in those hearts with aortic atresia and mitral hypoplasia than in those with the combination of aortic and mitral atresia.
Topics: Aorta; Fetal Heart; Fetal Monitoring; Humans; Infant, Newborn; Mitral Valve; Pulmonary Artery; Tricuspid Valve
PubMed: 3943933
DOI: 10.1016/0167-5273(86)90220-2 -
The Journal of Medical Investigation :... Feb 1999The surgical strategy in infants with mitral valve stenosis or atresia without diminutive ascending aorta remains to be established, including the potential for...
The surgical strategy in infants with mitral valve stenosis or atresia without diminutive ascending aorta remains to be established, including the potential for biventricular repair as a definitive operation. Our surgical experience of six infants with mitral valve stenosis (4 patients) or atresia (2 patients) without diminutive ascending aorta was evaluated based on three important factors: left ventricular volume; the nature of the systemic outflow obstruction; and the type of mitral valve anomaly. Two patients with systemic outflow tract diameter less than 65% of normal underwent systemic outflow tract reconstruction, and the other patients with outflow tract diameter more than 68% of normal were able to maintain systemic circulation without repair. Only one patient with mitral valve stenosis without left ventricular outflow tract obstruction underwent a successful open mitral valvotomy as a biventricular repair after first-stage palliation. The left ventricle of the other patients did not grow after first-stage palliation. Due to progressive subaortic narrowing, pulmonary artery banding should be avoided in patients with mitral atresia due to absent atrioventricular connection who are future Fontan candidates. Most patients with this lesion can be expected to become candidates for safe Fontan-type repair.
Topics: Aorta; Humans; Infant; Infant, Newborn; Mitral Valve; Mitral Valve Stenosis; Surgical Procedures, Operative; Ventricular Function, Left
PubMed: 10408159
DOI: No ID Found -
Catheterization and Cardiovascular... 1984Six infants, four with transposition of the great arteries and one each with mitral atresia and tricuspid atresia, who did not improve following balloon atrial...
Six infants, four with transposition of the great arteries and one each with mitral atresia and tricuspid atresia, who did not improve following balloon atrial septostomy are presented. Each patient underwent transcatheter blade atrial septostomy with resultant improvement of interatrial mixing (immediate improvement in three transposition patients and delayed improvement in one) or interatrial obstruction (in the mitral and tricuspid atresia patients). It is suggested that blade atrial septostomy is a safe and effective technique for nonsurgical palliation of congenital heart defects to enlarge a restrictive interatrial communication, especially if balloon atrial septostomy has not been successful.
Topics: Cardiac Catheterization; Follow-Up Studies; Heart Atria; Heart Defects, Congenital; Heart Septum; Heart Ventricles; Humans; Infant; Infant, Newborn; Mitral Valve; Palliative Care; Pulmonary Valve; Pulmonary Veins; Transposition of Great Vessels; Tricuspid Valve
PubMed: 6207930
DOI: 10.1002/ccd.1810100405 -
Seminars in Thoracic and Cardiovascular...To assess the phenotypic variations found among hearts diagnosed at autopsy with hypoplastic left heart syndrome, with attention to implications related to this syndrome...
To assess the phenotypic variations found among hearts diagnosed at autopsy with hypoplastic left heart syndrome, with attention to implications related to this syndrome as an acquired disease of fetal life, rather than being the consequence of abnormal embryogenesis. We assessed 119 specimens, from 2 archives, diagnosed initially as representing hypoplastic left heart syndrome. Among the 119 specimens, the majority of which had been entered into the archives prior to the availability of surgical treatment for the syndrome, 36 (30%) had the combination of mitral and aortic atresia, 26 (22%) had mitral and aortic stenosis, and 57 (48%) had mitral stenosis combined with aortic atresia. Of the hearts with combined atresia, 92% (33 specimens) had slit-like left ventricles, compared to 12% (3 specimens) of hearts with stenosis of both aortic and mitral valves, and 2 hearts (4%) with mitral stenosis and aortic atresia (P < 0.001). Hypoplasia of the left atrial appendage was present in half (18 specimens, 51%) of those with combined atresia, as opposed to just 18% (10 specimens) of mitral stenosis combined with aortic atresia (P = 0.001). Small left ventricles with valves deemed proportional in size were found in 11 (42%) of those with combined mitral and aortic stenosis. Fibroelastosis was significantly more common in the hearts with mitral stenosis compared to those with mitral atresia (76% vs 11%, P < 0.001). The ascending aorta was significantly smaller in the hearts with aortic atresia. The variability in the morphologic findings support the notion that the lesions seen represent acquired disease occurring subsequent to closure of the embryonic interventricular communication, rather than representing abnormal embryogenesis.
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Mitral Valve; Mitral Valve Stenosis
PubMed: 32092382
DOI: 10.1053/j.semtcvs.2020.02.019 -
American Heart Journal Jun 1987
Topics: Aorta; Echocardiography; Heart Septal Defects; Humans; Infant, Newborn
PubMed: 3591623
DOI: 10.1016/0002-8703(87)90675-2