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PubMed2001

Heterotaxia as an outcome of maternal diabetes: an epidemiological study.

There are very few publications on the possible relationship between maternal diabetes and infants presenting heterotaxia-asymmetry defects. In mice, there is a relationship between maternal diabetes and heterotaxia, although this is influenced by the fetal genotype. An epidemiological analysis of heterotaxia-asymmetry in children born to diabetic mothers (diabetes mellitus or gestational diabetes) is presented here. The analysis is based in the case-control study of the ECEMC database. However, due to the very small sample size for each type of study of heterotaxia-asymmetry alterations, up to 10 control infants for each case were selected. Although the sample size is small, the results suggest that only maternal diabetes mellitus significantly increases the risk for transposition of great vessels (OR=61.87; CI:7.36-519.82), and transposition of viscera (OR=24.82; CI;1.84-335.44).

Diabetes, Gestational↗
PubMed1990

Intracardiac shunting in children with ventricular septal defect: evaluation with Doppler color flow mapping.

In children with a ventricular septal defect, transseptal blood flow has been demonstrated angiographically to be bidirectional in all but the smallest defects. To investigate this phenomenon noninvasively, two-dimensional Doppler color flow echocardiography was used in 77 patients (aged 1 day to 15.5 years, mean 24 months). During isovolumetric contraction, flow was seen from the left to the right ventricle in 82 (98%) of 84 studies. During isovolumetric relaxation, right to left flow was noted in 72 (96%) of 75 studies. During early diastole in patients with an isolated defect, flow was initially from the right to the left ventricle and subsequently reversed to become left to right in 29 (91%) of 32 studies. In patients with concomitant right ventricular volume overload, flow across the ventricular defect was from the right to the left ventricle throughout diastole in 30 (86%) of 35 studies. In each of four patients with d-transposition of the great vessels and each of two patients with 1-transposition of the great vessels, blood flow was from the morphologic left ventricle to the morphologic right ventricle during isovolumetric contraction and from the morphologic right ventricle to the morphologic left ventricle during isovolumetric relaxation. These results demonstrate 1) the complex nature of intracardiac shunting in children with ventricular septal defect as previously shown by angiography; 2) an intrinsic functional difference in the contractile and relaxation properties of the morphologic left and right ventricles; and 3) differences in diastolic blood flow patterns between children with an isolated defect and those with a ventricular septal defect and right ventricular volume overload (p = 0.0001).

Adolescent↗
PubMed1981

Specificity of systolic anterior motion of anterior mitral leaflet for hypertrophic cardiomyopathy. Prevalence in large population of patients with other cardiac diseases.

The value of systolic anterior motion of the anterior mitral leaflet as a diagnostic marker for hypertrophic cardiomyopathy has been questioned because of its reported occurrence in other heart diseases. To determine the true specificity of systolic anterior motion for hypertrophic cardiomyopathy, 721 echocardiograms from patients with a wide variety of cardiac diseases were reviewed for its presence or absence under basal conditions. Systolic anterior motion of the anterior mitral leaflet was present in only 22 (3.0%) of the 721 patients, giving a specificity of 97 per cent. It was most common in patients with d-transposition of the great vessels (11 of 51, or 21%). With patients having transposition of the great vessels excluded from the analysis, the prevalence of systolic anterior motion of the anterior mitral leaflet was only 1.6 per cent (specificity 98%). Of note, eight of the 11 patients with systolic anterior motion of the anterior mitral leaflet and diseases other than transposition of the great vessels had disproportionate thickening of the ventricular septum, making it exceedingly rare in a patient population with normal septal-free wall thickness ratios (prevalence 0.4%; specificity 99%). Hence, while systolic anterior motion is not pathognomonic of hypertrophic cardiomyopathy, it was an uncommon finding in a large population of patients with a variety of cardiac diseases; when present in such patients systolic anterior motion of the anterior mitral leaflet is usually associated with disproportionate septal thickening.

Adolescent↗
PubMed1993

Transesophageal echocardiography in adults with congenital cardiopathies.

Eighty-seven adult patients with congenital cardiopathy were studied by using transesophageal echocardiography (TEE) over a period of 30 months. Transthoracic echocardiography was practiced on all patients, after which TEE with monoplanar and biplanar probes (74 and 15 patients, respectively) was used to confirm principal diagnoses and determine specific information. TEE findings were compared with those of cardiac catheterization in all 87 cases and with those of surgery in 15 cases. Dextrocardia was found in 11 cases and mesocardia in 2. Situs inversus was demonstrated in 6 and levoisomerism in 4. Intracardiac and extracardiac shunts were diagnosed in 20 cases, Ebstein's anomaly in 27, corrected transposition of great vessels in 15, univentricular atrioventricular connection in 7, cor triatriatum in 1, parachute mitral valve in 1, crisscross heart in 1, and double-outlet left atrium in 1. TEE was of particular value in evaluating total anomalous pulmonary venous connection, common atrioventricular canal, Ebstein's anomaly, corrected transposition of great vessels, and univentricular atrioventricular connection and in diagnosis of the rarer congenital cardiopathies.

Adult↗
PubMed1976

An unusual form of the transposition complex. Uncorrected levo-transposition with horizontal ventricular septum: Report of two cases.

A distinctive angiographic appearance is described in two patients who had uncorrected levo-transposition of the great vessels. Although levo-transposition with inversion of the ventricles usually results in physiologically corrected transposition, in these patients the anatomy was arranged in such a way as to result in an uncorrected transposition. The following elements were responsible for this physiological condition: normal atrial situs, inverted ventricles with "criss-cross" atrioventricular flow, levo-transposed great arteries. In addition, the morphological right ventricle was hypoplastic, left sided and superior to the left ventricle and the ventricular septum was horizontal in position. The two ventricles were connected via a large ventricular septal defect. The importance of accurate, detailed, preoperative angiographic demonstration of the anatomic situation is stressed.

Adolescent↗
PubMed1999

Pregnancy among women with congenitally corrected transposition of great arteries.

OBJECTIVES: The outcome of pregnancy in congenitally corrected transposition of the great vessels was studied in 22 women. BACKGROUND: Women with congenitally corrected transposition of the great vessels often reach childbearing age. Although reports on the outcome of pregnancy in these women are available, the number of patients is small. METHODS: The medical and surgical databases at the Mayo Clinic were reviewed, and 36 women >16 years old with congenitally corrected transposition of the great vessels were identified. All of them were contacted, and 22 who had pregnancies were identified and the outcome of pregnancy was evaluated. RESULTS: Twenty-two women had 60 pregnancies resulting in 50 live births (83%). Forty-four deliveries (88%) were vaginal and 6 (12%) were by cesarean section. One delivery was premature at 29 weeks. There was one successful twin pregnancy. There were 11 unsuccessful pregnancies. One patient developed congestive heart failure late in pregnancy because of systemic atrioventricular valve regurgitation and required valve replacement in the early postpartum period. One patient had a total of 12 pregnancies, including 1 twin pregnancy and 2 unsuccessful pregnancies. She had multiple pregnancy-related complications, including toxemia, congestive heart failure, endocarditis and myocardial infarction (single coronary artery). No other serious pregnancy-related maternal complications and no pregnancy-related deaths occurred. The mean birth weight of the infants (n = 32) was 3.2 +/- 0.4 kg. None of the 50 live offspring have been diagnosed with congenital heart disease. CONCLUSIONS: Successful pregnancy can be achieved in most women with congenitally corrected transposition of the great arteries. The rate of fetal loss and maternal cardiovascular morbidity is increased. Because of the small number of births, the risk of congenital heart disease in offspring of women with congenitally corrected transposition of the great arteries is uncertain.

Adult↗
PubMedDate not supplied

Transcatheter stent treatment for congenital peripheral pulmonary arterial stenosis.

A total of 5 Johnson and Johnson stents were implanted in two patients with significant residual peripheral pulmonary arterial stenosis. These were a 15-year-old boy with post-open heart surgery for tetralogy of Fallot and a 3 8/12 year-old boy with D-transposition of great vessels. Immediately after balloon dilatation and implantation of the stents, the diameter of the narrowing pulmonary arteries increased significantly from 6.0 +/- 0.8 mm to 13.5 +/- 1.7 mm (P < 0.001) and the systolic pressure gradients across the stenosis of peripheral pulmonary artery dropped significantly from 33.0 +/- 16.0 mmHg to 10.2 +/- 4.4 mmHg (P < 0.01). One year later, repeated cardiac catheterization was performed on both patients. In the patient with tetralogy of Fallot, a 20 mmHg pressure gradient was found between the main and left pulmonary artery. This patient then received another stent implantation to release the residual stenosis. The boy with D-transposition of great vessels had only 9 mmHg gradient between main and right pulmonary artery. Transcatheter placement of the stent is a feasible and effective method to treat certain patients with significant pulmonary arterial stenosis if surgical correction can not be performed.

Adolescent↗
PubMed1975

Ventricular inversion associated with normally related great vessels.

A patient with ventricular inversion with normally related great vessels and double inlet left ventricle is presented. the patient also showed features of Down's syndrome. Association of ventricular inversion with normally related great vessels is rare and leads to a basic functional state like that of complete transposition of great vessels derived from the connections between the ventricles and great vessels. The association of ventricular inversion with Down's syndrome observed in the case reported represents a rarity.

Aorta↗
PubMed1993

[Role of heart transplantation in the treatment of complex congenital malformations in adolescents and adults].

The number of pediatric heart transplants for complex congenital heart disease has increased in recent years, but little experience has been reported in the adolescent and adult population. Between 1987 and 1992, 6 patients (mean age 24 years, range 14 to 42) underwent transplant in our institution because of structural congenital heart disease with or without prior palliative operation or definitive repair. The diagnose covered: congenitally corrected transposition of the great vessels, late systemic ventricular failure after surgically corrected transposition of the great arteries, left superior vena cava, and tricuspid atresia with right ventricular hypoplasia. The palliative repairs included modified Blalock-Taussig shunt, cavo-pulmonary Glenn shunt, two aorto-pulmonary Waterstone shunts, two Blalock-Hanlon atrioseptectomies and one pulmonary valvotomy by the method of Brock. One patient had undergone pulmonary artery banding, in two patients atrial repair by Senning's technique had been performed for definitive repair of transposition of the great arteries. Donor cardiectomy was modified to remove complete inflow and outflow tissue and transplantation was performed without prosthetic material in all patients, in no case was deep hypothermic arrest necessary. There was no early or late mortality after a mean follow-up of 28 months. Postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results. There was no early or late mortality after a mean follow-up of 28 months. Postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results. Adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1998

Direct reconstruction of the pulmonary artery during the arterial switch operation: an interesting surgical option with excellent hemodynamic results.

BACKGROUND: In transposition of the great arteries, reconstruction of the neo-pulmonary artery is a challenging surgical detail during the arterial switch procedure. We present early and midterm clinical and hemodynamic results of a direct reconstruction of the pulmonary artery avoiding prosthetic and autologous material. METHODS: Between 1990 and June 1996, a total of 189 patients underwent the arterial switch procedure because of D-transposition of the great vessels. Of them, 47 underwent direct pulmonary artery reconstruction. Mean age at operation was 5.2+/-4.1 days and mean weight was 3.75+/-0.85 kg. Simple transposition of the great arteries was present in 13, transposition of the great arteries plus ventricular septal defect in 27, and more complex forms of transposition of the great arteries in 7 patients. The great vessels were side-by-side in 4 patients and in the anteroposterior position in 43 patients. The technique of direct pulmonary reconstruction includes extensive mobilization of both pulmonary artery branches into the hilum, posterior incision of the mean pulmonary artery into the bifurcation, and resuspension of the posterior commissure of the neo-pulmonary valve. A large anastomosis without any tension is then performed, using the anterior remnant aortic sinus of Valsalva to fit out the expected size of the neo-pulmonary artery. RESULTS: Early mortality was 8.5% (4/47) in this particular group of patients. Postoperative echocardiography was performed before hospital discharge, 3 to 6 months postoperatively, and after a mean follow-up of 24 months. Of the 43 survivors, 37 patients had a pressure gradient across the pulmonary valve of less than 15 mm Hg. Mild pulmonary stenosis (pressure gradient of 15 to 30 mm Hg) was present in 4 and more severe supravalvar stenosis (pressure gradient > 30 mm Hg) in 2 patients. After a mean follow-up of 36 months, there was one redo operation to enlarge the right ventricular outflow tract. CONCLUSIONS: Direct reconstruction of the neo-pulmonary artery-avoiding autologous pericardium and prosthetic material-may represent an interesting option during the arterial switch operation when the great vessels lie in the anteroposterior position. This technique is simple, and the hemodynamic midterm results are very favorable. The incidence of postoperative supravalvar pulmonary stenosis is low, and there may be considerable potential for unlimited tissue growth.

Anastomosis, Surgical↗
PubMed1983

[Diagnostic value of CT in congenital disorders of the great vessels].

Computed tomographic (CT) studies of the cardiovascular system were performed on 850 cases at our institute during the last five years. The aorta was clearly demonstrated by CT from the aortic root to the bifurcation of the iliac artery, and in most cases the main aortic branches including the coronary, brachiocephalic, common carotid, subclavian, celiac, superior mesenteric, renal and iliac arteries were satisfactorily evaluated (Fig. 1-3). Therefore CT renders us encouraging to detect the abnormality of these arteries. This paper described the CT findings of congenital anomalies of the great vessels in 14 patients with 16 anomalies including two cases of l-corrected transposition of the great vessels, two of double aortic arch, one of aneurysm of the sinus of Valsalva, six of patent ductus arteriosus (PDA) and five of right-sided descending aorta, two of which had double aortic arch aforementioned and the remaining three had dextrocardia. The diagnosis of these abnormalities except for PDA were made only by CT. For instance, l-corrected transposition of the great vessels was diagnosed easily by observing the side-to-side relationship of the great vessels, the aorta is situated to the left and anterior to the pulmonary artery. In the case of double aortic arch, not only the left and right aortic arch, but also the degree of narrowing as well as compression of the trachea and esophagus were well evaluated. The diagnosis of aneurysm of the sinus of Valsalva was made by the characteristically marked dilatation of the aorta at the level of sinus of Valsalva on CT. Only in one case of PDA, the ductus connecting the descending aorta to the left pulmonary artery was demonstrated by CT. Plain CT was well tolerable, but enhanced CT was much more informative to detect cardiovascular abnormalities, and moreover, dynamic CT was rewarding in the detailed evaluation of blood flow in the cardiovascular system.

Adult↗