An echocardiographic study of diagnostic accuracy, prediction of surgical approach, and outcome for fetuses diagnosed with discordant ventriculo-arterial connections

2007 ◽  
Vol 17 (5) ◽  
pp. 528-534 ◽  
Author(s):  
Cécile J. Pascal ◽  
Ian Huggon ◽  
Gurleen K. Sharland ◽  
John M. Simpson
Keyword(s):  
Ventricular Septal Defect ◽  
Positive Predictive Value ◽  
Diagnostic Accuracy ◽  
Surgical Approach ◽  
Predictive Value ◽  
Aortic Coarctation ◽  
Septal Defect ◽  
Pulmonary Stenosis ◽  
Ventricular Septum ◽  
Intact Ventricular Septum

AbstractOur aims were to examine the diagnostic accuracy of prenatal diagnosis of concordant atrioventricular and discordant ventriculo-arterial connections, the accuracy of predictions made concerning the postnatal surgical approach, and a description of the different subtypes related to outcome. All fetuses were evaluated at a tertiary centre for fetal cardiology between January, 1994, and December, 2003. In this period, we identified congenitally malformed hearts in 1,835 fetuses, of whom 56 (3%) met the criterions of inclusion. Of the total, 30 (54%) had an intact ventricular septum, 9 (16%) had an associated ventricular septal defect, 7 (13%) had a ventricular septal defect and pulmonary stenosis, 1 (2%) had pulmonary stenosis and an intact ventricular septum, 8 (14%) had a ventricular septal defect and aortic coarctation, and 1 (2%) had coarctation of the aorta with an intact ventricular septum. All the discordant ventriculo-arterial connections were correctly identified. For associated ventricular septal defects, the diagnostic sensitivity was 96%, with specificity of 88%, positive predictive value of 85%, and negative predictive value of 97%. For aortic coarctation, the sensitivity was 100%, specificity 96%, positive predictive value 82%, and negative predictive value 100%. The prediction of the surgical approach was accurate in 41 of 48 cases (85%). For those fetuses with pulmonary stenosis and ventricular septal defect, the ratio of the diameters of the pulmonary trunk was shown to be helpful in predicting the possibility of an arterial switch as opposed to the Rastelli type of repair. Of the 49 liveborn infants, 46 were alive at 30 days (94%, with 95% confidence intervals from 83 to 99%), and 43 at one year (88%, 95% confidence intervals from 75 to 95%). Deaths were mainly related to the anatomy of the coronary arteries, and associated cardiac lesions.

Is the function of all cardiac valves after the arterial switch operation influenced by an associated ventricular septal defect?

2011 ◽  
Vol 21 (4) ◽  
pp. 383-391
Author(s):  
J. Gabriel ◽  
H.-H. Scheld ◽  
T.D.T. Tjan ◽  
N. Osada ◽  
Thomas Krasemann
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Arterial Switch Operation ◽  
Pulmonary Stenosis ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Cardiac Valves ◽  
Arterial Switch ◽  
Switch Operation

AbstractA ventricular septal defect in transposition of the great arteries is frequently closely related to the cardiac valves. The valvar function after arterial switch operation of patients with transposition of the great arteries and ventricular septal defect or intact ventricular septum was compared. We analysed the function of all cardiac valves in patients who underwent the arterial switch operations pre- and post-operatively, 1 year after the procedure and on follow-up. The study included 92 patients – 64 with transposition of the great arteries/intact ventricular septum and 28 with transposition of the great arteries/ventricular septal defect. The median age at surgery was 5.5 days in transposition of the great arteries/intact ventricular septum (0–73 days) and 7.0 days in transposition of the great arteries/ventricular septal defect (4–41 days). Follow-up was 51.7 months in transposition of the great arteries/intact ventricular septum (3.3–177.3 months) and 55 months in transposition of the great arteries/ventricular septal defect (14.6–164.7 months). Neo-aortic, neo-pulmonary, and mitral valvar function did not differ. Tricuspid regurgitation was more frequent 1 year post-operatively in transposition of the great arteries/ventricular septal defect (n = 4) than in transposition of the great arteries/intact ventricular septum. The prevalence of neo-aortic regurgitation and pulmonary stenosis increased over time, especially in patients with transposition of the great arteries/intact ventricular septum. The presence of a ventricular septal defect in patients undergoing arterial switch operation for transposition of the great arteries only has a minor bearing for the development of valvar dysfunction on the longer follow-up.

Anatomic correction of complete transposition with ventricular septal defect in neonates: experience with 42 consecutive cases

1991 ◽  
Vol 1 (1) ◽  
pp. 101-103 ◽  
Author(s):  
Claude Planché ◽  
Alain Serraf ◽  
François Lacour-Gayet ◽  
Jacqueline Bruniaux ◽  
François Bouchart
Keyword(s):  
Left Ventricle ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Arterial Switch Operation ◽  
Systemic Circulation ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Complete Transposition ◽  
The Poor ◽  
Switch Operation

Perhaps paradoxically, it was in the form of complete transposition with a ventricular septal defect rather than an intact ventricular septum that the arterial switch operation was first successfully applied. This was, in part, because of the poor results of the physiologic repair in the presence of a ventricular septal defect, but more because the left ventricle, in the presence of the septal deficiency, is immediately suitable for supporting postoperatively the increased workload imposed on the systemic circulation. Spurred by this success, the procedure was then extended to neonates born with complete transposition and an intact ventricular septum and is currently employed with good results in this group.

scholarly journals Double outlet of both ventricles: morphological, echocardiographic and surgical considerations

2020 ◽  
Author(s):  
Krishna Subramony Iyer ◽  
Ankit Garg ◽  
Sumir Girotra ◽  
Robert H Anderson ◽  
Sushil Azad ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Ventricular Septal Defect ◽  
Surgical Management ◽  
Aortic Coarctation ◽  
Septal Defect ◽  
Surgical Repair ◽  
Ventricular Septum ◽  
Unique Form ◽  
Midterm Results

Abstract OBJECTIVES To describe the morphology, echocardiographic features and surgical management of the entity appropriately described as ‘double outlet of both ventricles’. METHODS Seven patients (5 males, age 0.5–7.5 months) were diagnosed to have a unique form of subarterial ventricular septal defect (VSD) and ventriculo-arterial connection, where a muscular outlet septum straddled the crest of the ventricular septum in a cruciate manner, such that both great arteries were equally committed to both ventricles. Diagnosis was established by echocardiography, with 6 patients submitted to surgical repair by means of intracardiac routing of the left ventricle to the aorta using 2 patches. RESULTS Surgical repair was successful in all 6 patients in whom it was attempted. In addition, 1 patient underwent concomitant repair of aortic coarctation, and 2 had closure of multiple VSDs. We lost 1 patient to follow-up after diagnosis. Follow-up with a range from 3 months to 8 years in the remaining patients revealed all to be clinically well with satisfactory growth of both outflow tracts. CONCLUSIONS We describe a series of patients with the ventriculo-arterial connection best described as ‘double outlet of both ventricles’. Diagnosis is readily established by echocardiography. Good early and midterm results can be expected subsequent to surgical repair using 2 patches for interventricular septation.

Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome

2004 ◽  
Vol 14 (4) ◽  
pp. 402-408 ◽  
Author(s):  
Nili Zucker ◽  
Ilya Rozin ◽  
Aviva Levitas ◽  
Eli Zalzstein
Keyword(s):  
Ventricular Septal Defect ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Clinical Presentation ◽  
Pulmonary Valve ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
History Of ◽  
Absent Pulmonary Valve Syndrome

So-called ‘absent pulmonary valve syndrome’ is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.

scholarly journals Accuracy of MSCT Coronary Angiography with 64 Row CT Scanner—Facing the Facts

2010 ◽  
Vol 4 ◽  
pp. CMC.S3864 ◽  
Author(s):  
M. Wehrschuetz ◽  
E. Wehrschuetz ◽  
H. Schuchlenz ◽  
G. Schaffler
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Coronary Angiography ◽  
Positive Predictive Value ◽  
Diagnostic Accuracy ◽  
Predictive Value ◽  
Diagnostic Potential ◽  
Coronary Artery Stenoses ◽  
Artery Disease ◽  
Msct Angiography

Improvements in multislice computed tomography (MSCT) angiography of the coronary vessels have enabled the minimally invasive detection of coronary artery stenoses, while quantitative coronary angiography (QCA) is the accepted reference standard for evaluation thereof. Sixteen-slice MSCT showed promising diagnostic accuracy in detecting coronary artery stenoses haemodynamically and the subsequent introduction of 64-slice scanners promised excellent and fast results for coronary artery studies. This prompted us to evaluate the diagnostic accuracy, sensitivity, specificity, and the negative und positive predictive value of 64-slice MSCT in the detection of haemodynamically significant coronary artery stenoses. Thirty-seven consecutive subjects with suspected coronary artery disease were evaluated with MSCT angiography and the results compared with QCA. All vessels were considered for the assessment of significant coronary artery stenosis (diameter reduction ≥ 50%). Thirteen patients (35%) were identified as having significant coronary artery stenoses on QCA with 6.3% (35/555) affected segments. None of the coronary segments were excluded from analysis. Overall sensitivity for classifying stenoses of 64-slice MSCT was 69%, specificity was 92%, positive predictive value was 38% and negative predictive value was 98%. The interobserver variability for detection of significant lesions had a κ-value of 0.43. Sixty-four-slice MSCT offers the diagnostic potential to detect coronary artery disease, to quantify haemodynamically significant coronary artery stenoses and to avoid unnecessary invasive coronary artery examinations.

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