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Results for “CARDIOVASCULAR DEFECTS, CONGENITAL”

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At least 19 recordsLinked to original sources
PubMed1983

Noninvasive diagnosis of persistent fetal circulation versus congenital cardiovascular defects.

Congenital cardiovascular anomalies associated with right-left atrial or ductal shunts must be excluded before a diagnosis of persistent fetal circulation (PFC) can be made. Despite the advent of 2-dimensional echocardiography (2-D echo), this differentiation can be difficult and may require cardiac catheterization with selective angiography. Fifteen consecutive cases were analyzed in which difficulty was encountered with this differential diagnosis, and experience with the use of cardiac auscultation, the 12-lead electrocardiogram (ECG), arterial blood gas determinations and 2-D echo, both alone and with injection of venous contrast material, is reviewed. Electrocardiographic abnormalities of ventricular axis, hypertrophy or dominance (p = 0.002) and suspicion of cardiovascular disease on 2-D echo (p = 0.011) were the most useful findings in differentiating patients with PFC from those with congenital cardiovascular abnormalities. The ECG was the most sensitive test (100% sensitivity, 90% specificity), while 2-D echo was the most specific (100% specificity, 75% sensitivity). Evidence of right-left shunting at atrial or ductal levels or both did not differentiate between the groups; both groups had evidence of such shunts. A decision tree was developed to facilitate this differential diagnosis, which uses the ECG and 2-D echo. If the ECG reveals no abnormalities of ventricular axis, dominance or hypertrophy, the 2-D echo shows no structural abnormalities, and total anomalous pulmonary venous return and coarctation/interruption of the aorta are specifically excluded, a congenital cardiovascular anomaly is effectively eliminated. We suggest that this approach can optimize the management of the cyanotic newborn with suspected PFC by eliminating the risks of cardiac catheterization and angiography without missing the diagnosis of a major structural cardiovascular anomaly.

Diagnosis, Differential↗
PubMed2005

Association between fetal lymphedema and congenital cardiovascular defects in Turner syndrome.

OBJECTIVES: Turner syndrome (TS) is associated with congenital cardiovascular defects (CCVDs), most commonly bicuspid aortic valve (BAV) and aortic coarctation (COARC), congenital renal anomalies, and fetal lymphedema. It has been theorized that compressive or obstructive effects of fetal lymphedema may actually cause cardiovascular and renal dysmorphogenesis in TS. The objective of this study was to determine whether there is a specific association between a history of fetal lymphedema and CCVDs in monosomy X, or TS, independent of karyotype or general severity of the phenotype. METHODS: This was a prospective study of 134 girls and women who have TS (mean age: 30 years) and were clinically evaluated for evidence of fetal lymphedema, classified as central (signified by the presence of neck webbing) or peripheral (current or perinatal, or dysplastic fingernails). The presence of BAV and/or COARC was detected by magnetic resonance imaging combined with echocardiography, and renal anomalies were determined by ultrasound. RESULTS: There is a strong association between developmental central lymphedema, signified by neck webbing, and the presence of BAV (chi2 = 10) and COARC (chi2 = 8). The association between webbed neck and CCVDs was independent of karyotype. There was, in contrast, no significant association between renal anomalies and webbed neck or CCVDs. CONCLUSIONS: The strong, statistically significant association between neck webbing and the presence of BAV and COARC in TS suggests a pathogenetic connection between fetal lymphatic obstruction and defective aortic development. The presence of neck webbing in TS should alert the clinician to the possibility of congenital cardiovascular defects.

Adult↗
PubMed1990

[Predicting congenital cardiovascular defects on the basis of their immunogenetic characteristics].

The study was undertaken to examine 80 children with congenital heart and vessel malformations, their parents (50 married couples), and 100 families with healthy children. The antigen typing of the HLA system was accomplished by using the Terasaki microlymphocytotoxic test. A statistically significant correlation was found between B35 antigens and septal malformations, vascular diseases, and congenital cardiomyopathies. The unfavorable obstetric history was shown to play a role in the occurrence of developmental malformations. The data of an analysis of HLA antigens in the parents of the children with congenital malformations suggest that a marked HLA antigen compatibility of married couples is one of the cause of pregnancy complications and developmental malformations in their offspring.

Adolescent↗
PubMedDate not supplied

[Catheter therapy of congenital cardiovascular defects].

The authors analyze the experience gained in catheter operations performed at their Department over the recent 28 years. A total of 500 balloon (Rashkind) and 35 knife (Park) atrioseptostomies were performed in critical patients with different "blue" congenital diseases over this period. An immediate hemodynamic effect at the operation table was attained in 95% of patients. Transluminal balloon valvuloplasty (TLBVP) was performed in 372 patients with valvular stenosis of the pulmonary artery. The results of this operation depend on the patient's age, anatomy of the defect, and correct choice of the diameter of balloon catheter. This intervention is particularly effective in patients aged under 1. In patients with combined valvular and infundibular stenosis the results of TLBVP depend on the severity of stenosis and age of patient. An attempt at TLBVP of congenital aortal stenosis was undertaken in 67 patients. Valvuloplasty was carried out in 57 patients. The operation was effective in 13 (62%) out of 21 patients aged under 1. The mortality in this group was 5.5%. Balloon valvuloplasty of the pulmonary artery was carried out in 71 patients with cyanotic congenital heart diseases. The intervention helped eliminate the critical state, rise the systolic pressure in the pulmonary artery, improve blood saturation with oxygen, and evade the operation for creation of a systemic-pulmonary anastomosis. Isolated and postoperative stenoses of pulmonary arteries were removed in 65 patients. Six Johnson and Johnson stents were effectively implanted to 3 patients with rigid postoperative stenoses; in 32 patients transluminal balloon angioplasty (TLBAP) for coarctation and recoarctation of the aorta brought about a satisfactory immediate hemodynamic effect. TLBAP of Blalock-Taussig's stenosed anastomosis were performed in 60 patients with various cyanotic congenital heart diseases. Its results were good in 39 (65%) patients, satisfactory in 19 (31.7%), and unsatisfactory in only 2 (3%) cases. Of the novel endocardial interventions, dilatation of the conduit following Rastrelli's operation, creation of a defect of the atrial septum after Fontain's operation, and embolization of the coronary-cardiac fistulas and of patent ductus arteriosus were carried out. This review demonstrates wide use of endocardial surgery methods in the treatment of some congenital heart diseases; in many cases such treatment may be an alternative to surgical interventions.

Adolescent↗
PubMed2003

The implications of common brachiocephalic trunk on associated congenital cardiovascular defects and their management.

A common brachiocephalic trunk is an anatomic variant in which both common carotid arteries and the right subclavian artery arise from the aortic arch via a single trunk. The impact of this condition on associated congenital cardiac malformations is presently unknown. Out of a total of 1480 cardiac catheterizations performed in children over a period of 10 years, we discovered 48 patients (3.2%) to have a common brachiocephalic trunk, of whom 98% had associated congenital cardiac malformations. A spectrum of associated lesions was identified, including left-to-right shunts in 19 patients, right-sided anomalies in 18 patients, left-sided obstructive lesions in 12 patients, and coronary arterial abnormalities in 10 patients, eight of whom had other cardiac defects. Genetic syndromes were present in one-fifth of the cases. When found with left-sided malformations, the common trunk was associated with persistent hypoplasia of the aortic arch, likely related to diminished flow through the arch during development. In each of four patients in whom the brachiocephalic trunk had been used during construction of a palliative shunt, we observed inadequate growth and deformation of the pulmonary arteries. Thus, angiographic identification of a common brachiocephalic trunk may be a marker for the presence of accompanying congenital cardiac defects and coronary arterial abnormalities. Understanding the pathophysiologic effects of the common trunk is important when planning the palliative or corrective procedures, and when assessing the potential benefit of the surgical repair over the long term.

Brachiocephalic Trunk↗