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Ebstein anomaly and Trisomy 21: A rare association.

Siehr SL, Punn R, Priest JR, Lowenthal A - Ann Pediatr Cardiol (2014)

Bottom Line: This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Lucile Packard Children's Hospital, Palo Alto, California, USA.

ABSTRACT
This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

No MeSH data available.


Related in: MedlinePlus

Apical view demonstrating a broad, severe aliasing tricuspid valve regurgitation jet from the right ventricle (RV). LV = Left ventricle
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F3a: Apical view demonstrating a broad, severe aliasing tricuspid valve regurgitation jet from the right ventricle (RV). LV = Left ventricle

Mentions: Following initial discharge, the patient was readmitted multiple times to the hospital for failure to thrive despite maximal caloric intake. Therefore, the patient underwent tricuspid valvuloplasty and VSD closure, with maintenance of an atrial level communication on day of life #108. A postoperative echocardiogram revealed moderate-to-severe tricuspid regurgitation, estimating a gradient of 39 mmHg from right ventricle to right atrium and right-to-left shunting across the atrial communication. Initially, weight gain was steady and oxygen saturations remained above 80%; however, over several months the patient again failed to gain weight despite adequate caloric intake. An echocardiogram 6.5 months postoperatively [Figure 3a–c] demonstrated severe tricuspid regurgitation, right-to-left shunting across the atrial communication, and new mild left upper pulmonary vein stenosis, with a mean gradient of 3-4 mmHg. Cardiac catheterization revealed diffuse pulmonary vein hypoplasia and left-sided pulmonary vein stenosis, with mean gradients of 4-11 mmHg. Repeat surgical intervention of the tricuspid valve was considered, however, given the low likelihood of improvement on account of pulmonary vascular disease and high-expected morbidity, surgery was not recommended.


Ebstein anomaly and Trisomy 21: A rare association.

Siehr SL, Punn R, Priest JR, Lowenthal A - Ann Pediatr Cardiol (2014)

Apical view demonstrating a broad, severe aliasing tricuspid valve regurgitation jet from the right ventricle (RV). LV = Left ventricle
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3959069&req=5

F3a: Apical view demonstrating a broad, severe aliasing tricuspid valve regurgitation jet from the right ventricle (RV). LV = Left ventricle
Mentions: Following initial discharge, the patient was readmitted multiple times to the hospital for failure to thrive despite maximal caloric intake. Therefore, the patient underwent tricuspid valvuloplasty and VSD closure, with maintenance of an atrial level communication on day of life #108. A postoperative echocardiogram revealed moderate-to-severe tricuspid regurgitation, estimating a gradient of 39 mmHg from right ventricle to right atrium and right-to-left shunting across the atrial communication. Initially, weight gain was steady and oxygen saturations remained above 80%; however, over several months the patient again failed to gain weight despite adequate caloric intake. An echocardiogram 6.5 months postoperatively [Figure 3a–c] demonstrated severe tricuspid regurgitation, right-to-left shunting across the atrial communication, and new mild left upper pulmonary vein stenosis, with a mean gradient of 3-4 mmHg. Cardiac catheterization revealed diffuse pulmonary vein hypoplasia and left-sided pulmonary vein stenosis, with mean gradients of 4-11 mmHg. Repeat surgical intervention of the tricuspid valve was considered, however, given the low likelihood of improvement on account of pulmonary vascular disease and high-expected morbidity, surgery was not recommended.

Bottom Line: This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Cardiology, Stanford University, Lucile Packard Children's Hospital, Palo Alto, California, USA.

ABSTRACT
This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

No MeSH data available.


Related in: MedlinePlus