1/27. Successful total correction of complete atrioventricular canal, total anomalous pulmonary venous drainage and unroofed coronary sinus in an infant.An infant with complete atrioventricular canal, total anomalous pulmonary venous drainage into the left superior vena cava and an unroofed coronary sinus successfully underwent total correction. A homograft vein was used to connect the proximal left superior vena cava to the right atrium and can be recommended when other methods of correction of unroofed coronary sinus cannot be used.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/27. Oral-facial-digital syndrome with Y-shaped fourth metacarpals and endocardial cushion defect.We report on a boy with pseudo-cleft of the upper lip, cleft palate, bifid uvula, lobulated tongue, hypoplasia of the epiglottis, both preaxial and central polydactyly of the hands (Y-shaped fourth metacarpals), bilateral preaxial polydactyly of the feet, postaxial polydactyly of the left foot, hearing impairment, and congenital heart disease with endocardial cushion defect. These clinical manifestations resembled oral-facial-digital syndrome type II (OFDS II, Mohr syndrome) or type VI (Varadi syndrome), associated with an atrioventricular canal. Clinical variability of OFDS II has been observed repeatedly. To the best of our knowledge, this is the first reported case of OFDS II with Y-shaped fourth metacarpals. In addition to Y-shaped fourth metacarpals, Mohr syndrome plus atrioventricular canal and hypoplasia of the epiglottis may represent an additional subgroup of OFDS.- - - - - - - - - - ranking = 0.4keywords = canal (Clic here for more details about this article) |
3/27. Atrioventricular canal defect and hypoplastic left heart syndrome as discordant congenital heart defects in twins.We report on a twin pair presenting with atrioventricular canal defect (AVCD) with right ventricular dominance in one twin, and classic hypoplastic left heart syndrome (HLHS) in the other. According to the developmental-mechanistic approach, AVCDs belong to the group of extracellular matrix abnormalities, whereas classic HLHS is included among flow lesions. Twin pairs with congenital heart defect (CHD) generally have concordant defects by mechanistic group. The occurrence of AVCD and classic HLHS in twins or siblings has never been reported. Interestingly, hypoplasia of the left ventricle is the anatomic characteristic which unifies the discordant CHDs observed in our twins. The occurrence of CHD in both members of the twin pair implies a strong influence of genetic factors. At present, the genetic basis determining the different cardiac phenotypes observed in our twins is unknown. The report of these peculiar associations may be useful to stimulate further studies and shed light on the etiology of CHDs.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
4/27. Unusual association of hypertrophic cardiomyopathy with complete atrioventricular canal defect and down syndrome.Hypertrophic cardiomyopathy typically presents as an isolated cardiac lesion. Transient hypertrophic cardiomyopathy in infancy has been described as a result of exposure to maternal metabolic disorders or to corticosteroids. In addition, hypertrophic cardiomyopathy has been described in association with genetic syndromes and, in rare cases, as a primary lesion associated with other congenital heart defects. We describe the unusual association of hypertrophic cardiomyopathy and complete atrioventricular canal defect in an infant with trisomy 21.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
5/27. Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cor triatriatum using ventricular septal defect translocation.A 17-month-old girl underwent successful single-stage anatomic repair using a technique of ventricular septal defect translocation for complete atrioventricular canal, double-outlet right ventricle, pulmonary stenosis, cor triatriatum, and left superior vena cava to an unroofed coronary sinus. We describe the repair and present clinical and angiographic data from 9 years follow-up.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
6/27. Macroreentrant atrial flutter around a common atrioventricular canal in an infant with complicated congenital heart anomaly.This report describes a 2-year-old male infant after repair of complicated congenital heart anomalies including common atrioventricular canal (CAVC), in which macroreentrant atrial flutter around the CAVC was demonstrated by electroanatomic mapping (CARTO) using a single catheter. Radiofrequency catheter ablation at the isthmus between the CAVC and the inferior vena cava eliminated the atrial flutter successfully.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
7/27. Pulmonary venous stenosis as an associated manifestation of left-sided obstructive lesions in an infant with partial atrioventricular canal defect.patients with partial atrioventricular canal defect (PAVC) who present with congestive heart failure within the first year of life are usually found to have associated left-sided obstructive lesions. We present a case of a child with PAVC who subsequently developed progressive pulmonary venous stenosis as a manifestation of her disease.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
8/27. tetralogy of fallot with complete atrioventricular canal: a report of successful surgical treatment.A case of successful surgical repair of tetralogy of fallot with complete atrioventricular canal in 4 year old female child is reported. Double patch technique and combined right atrial and right ventricular (RV) approach were used to repair this defect. The ventricular patch was tailored wide and redundant anteriorly in the form of a sail to prevent subaortic obstruction. Right ventricular outflow tract obstruction was relieved through an infundibular incision with subsequent use of subannular pericardial patch. The relevant literature on the management of this rare anomaly is briefly reviewed.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
9/27. Di George anomaly with atrioventricular canal.We report the first case of Di George anomaly associated with atrioventricular canal. This combination of anomalies may represent a chance occurrence of two situations happening in the same patient or, alternatively, the result of a single unknown embriogenetic mechanism.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
10/27. Single papillary muscle and ostium primum defect.Parachute mitral valve (single papillary muscle) is commonly associated with complete atrioventricular (AV) canal. We report a case of ostium primum defect associated with single papillary muscle.- - - - - - - - - - ranking = 0.2keywords = canal (Clic here for more details about this article) |
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