Ebstein anomaly is a rare heart defect that's present at birth (congenital). In this condition, your tricuspid valve is in the wrong position and the valve's flaps (leaflets) are malformed. As a result, the valve does not work properly. Blood might leak back through the valve, making your heart work less efficiently.
In Ebstein's anomaly of the tricuspid valve, some blood being pushed into the right ventricle “leaks” back into the right atrium because the valve does not close completely. The atrium may become enlarged because of the extra volume of blood; and, in severe cases, heart failure can result.
Ebstein's anomaly is often associated with an atrial septal defect – a small hole in the wall between the two upper chambers of the heart – and heart rhythm abnormalities.
The murmur is a shorter, systolic murmur in older children and adults due to low velocity flow from the hypokinetic, low pressure in the right ventricle. A diastolic murmur may be heard secondary to increased flow through a normal-sized or stenotic tricuspid valve orifice.
38.1). More rare forms of tricuspid atresia have partially delaminated but fused leaflets, and appear as membranes or resemble some features of Ebstein anomaly....Tricuspid Valve Disorders: Atresia, Dysplasia, and Ebstein Anomaly.Classification of Tricuspid AtresiaRelative IncidenceType II: Transposed Great Arteries10–25%VSD and pulmonary atresiaVSD and pulmonary stenosis6 more rows•Jul 27, 2016
This will result in lower oxygen levels in the blood. This is why children with Ebstein's anomaly may be blue or "cyanotic". This is why they have low oxygen saturations. Ebstein anomaly may occur with other heart defects, such as pulmonary valve stenosis or atresia, atrial septal defect or ventricular septal defect.
A box-shaped heart is a radiographic description given to the cardiac silhouette in some cases of Ebstein anomaly. The classic appearance of this finding is caused by the combination of the following features: huge right atrium that may fill the entire right hemithorax.
Newly diagnosed patients with Ebstein anomaly may have been told they have concomitant pulmonary arterial hypertension (PAH), particularly in the presence of cyanosis and right-sided heart enlargement. This is usually a misdiagnosis, because PAH is very rare among Ebstein patients.
Ebstein's anomaly is a rare heart defect that affects the tricuspid valve. Ebstein's anomaly has a wide range of severity — from mild to severe. About one in 10,000 babies is born with this condition.
This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.”7,8 The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly.
Several types of procedures can be used to surgically treat Ebstein anomaly and associated defects.Tricuspid valve repair. ... Tricuspid valve replacement. ... Closure of the atrial septal defect. ... Maze procedure. ... Radiofrequency catheter ablation. ... Heart transplantation.
Symptoms of Ebstein's anomaly depend on the severity of the abnormality and so can range from virtually no symptoms at all to low blood oxygen levels, dyspnea (difficulty breathing), and weakness. In extreme cases, the condition can be fatal.