double outlet right ventricle/edema

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Trisomy 16 in the mouse fetus associated with generalized edema and cardiovascular and urinary tract anomalies.

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Murine trisomy (Ts) 16 occurs in the fetal and neonatal progeny of males doubly heterozygous for the Robertsonian metacentric chromosomes Rb(16.17)7Bnr/Rb(9.16)9Rma and "all acrocentric" females. The developmental aspects of this trisomy were studied between day 12 of gestation and birth. So far,

[A rare adult case of double-outlet right ventricle without pulmonary stenosis: an autopsy case].

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Double outlet right ventricle (DORV) is a rare cardiac malformation especially in adulthood. We report a woman with DORV who survived to the age of 33 years. She had not been seriously limited, before she visited our hospital for cardiac evaluation at the age of 25 years. She was cyanotic, and had

[Fetal hydrops caused by cardiac failure in utero: description of 2 cases and pathogenetic considerations].

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Two cases of congestive heart failure in utero with fetal hydrops are reported. The diagnosis was made during pregnancy by means of echography. Cardiac failure, derived from supraventricular tachycardia not associated with structural anomalies of the heart, was observed in one of the patients. By

Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle.

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BACKGROUND We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy. METHODS Patients with a pre or postnatal diagnosis of DORV from 2000 to 2007 were identified and classified into 3

Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis.

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The fate of patients with pulmonary artery sling depends on associated tracheal lesions. Distal tracheal stenosis involving the carina frequently results in lethal obstruction due to secretion or inflammatory edema. Further-more, associated complex cardiac anomalies with excessive pulmonary flow

The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases.

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OBJECTIVE The purpose of this study was to evaluate the accuracy of the prenatal diagnosis of left isomerism and to assess possible diagnostic and prognostic markers. METHODS We conducted a retrospective review of all previously unpublished cases of left isomerism diagnosed in the prenatal and

First-trimester fetal heart block: a marker for cardiac anomaly.

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BACKGROUND Fetal heart block in the second and third trimesters may be caused by transplacental passage of auto-antibodies or cardiac defects. Little is known about the etiology of first-trimester fetal heart block. METHODS Fetal heart block was diagnosed in four patients (negative antibody

[Characteristics and prognosis of interrupted inferior vena cava with azygous continuation].

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Objective: To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Methods: Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent

Microdeletion 22q11 in complex cardiovascular malformations.

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Besides DiGeorge, velocardiofacial and conotruncal anomaly face syndromes, some of the isolated congenital heart diseases have also been associated with a chromosomal deletion in 22q11. These disease entities, which had originally been considered to have a different genetic background, are now

Prenatal diagnosis of left atrial isomerism.

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OBJECTIVE To describe the cardiac anomalies and outcome in the fetus with left atrial isomerism. METHODS All fetuses with a diagnosis of left atrial isomerism between 1998 and 2008 were identified. Gestational age at diagnosis, the nuchal translucency, the karyotype, the cardiac findings and outcome

[Total cavopulmonary connection in complex heart defects with a single functional ventricle].

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Nineteen children aged between 1.5 and 9.8 years (10 patients younger than 4 years) underwent total cavopulmonary connection (TCPC) for complex congenital heart defects other than tricuspid atresia. Diagnoses included double inlet left ventricle (n = 11), transposition of the great arteries with a

Absent aortic valve: a complex anomaly.

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Seven patients (four previously cited and three new cases) with absent aortic valve cusps (leaflets), a rare and underrecognized complex congenital heart defect, are discussed. All patients were male, six full-term and one premature with nonimmunologic hydrops. None underwent operation; all died

Treatment of children with protein - losing enteropathy after fontan and other complex congenital heart disease procedures in condition with limited human and technical resources.

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BACKGROUND Protein-losing enteropathy (PLE) is a disorder characterized by abnormal and often profound enteric protein loss. It's relatively uncommon complication of Fontan and other complex congenital heart disease (CCHD) procedures. Because of the complexity and rarity of this disease process, the

Management of single-ventricle patients with Berlin Heart EXCOR Ventricular Assist Device: single-center experience.

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There are minimal data regarding chronic management of single-ventricle ventricular assist device (VAD) patients. This study aims to describe our center's multidisciplinary team management of single-ventricle patients supported long term with the Berlin Heart EXCOR Pediatric VAD. Patient #1 was a

Deletion of the aryl hydrocarbon receptor-associated protein 9 leads to cardiac malformation and embryonic lethality.

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The aryl hydrocarbon receptor-associated protein 9, ARA9 (also known as XAP2 or AIP1), is a chaperone that is found in complexes with certain xenobiotic receptors, such as the aryl hydrocarbon receptor (AHR) and the peroxisome proliferator-activated receptor alpha (PPARalpha). In an effort to better

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