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(Circulation. 2007;115:e177-e178.)
© 2007 American Heart Association, Inc.

Images in Cardiovascular Medicine

Ebstein’s Malformation With Imperforate Tricuspid Valve

Gunter Kerst, MD; Renate Kaulitz, MD; Ludger Sieverding, MD; Gerhard Ziemer, MD; Michael Hofbeck, MD

From the Department of Cardiology, University Children’s Hospital (G.K., R.K., L.S., M.H.), and the Department of Thoracic, Cardiac, and Vascular Surgery, University Hospital (G.Z.), Tübingen, Germany.

Correspondence to Michael Hofbeck, MD, Department of Pediatric Cardiology, University Children’s Hospital, Hoppe-Seyler-Str 1, 72076 Tübingen, Germany. E-mail michael.hofbeck@med.uni-tuebingen.de

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A neonate was admitted at the age of 8 days with a history of increasing cyanosis and tachydyspnea after a normal pregnancy and delivery at 42 weeks’ gestation. A transthoracic echocardiography appeared to indicate pulmonary atresia with an intact ventricular septum. The child was intubated, intravenous prostaglandin E₁ was started, and the patient was transferred to our hospital.

The newborn presented with central cyanosis while being ventilated with 100% oxygen. No heart murmur was perceptible. Transthoracic echocardiography revealed marked apical displacement of the tricuspid valve leaflets (Figure 1 and Movie I). Linear attachments of the anterior and the mural leaflets resulted in complete separation of the inlet portion from the apical trabecular and outlet portions of the right ventricle (Figure 2 and Movie II). On color Doppler, we were unable to demonstrate either antegrade or regurgitant flow across the valve (Figure 3 and Movie III). There was a restrictive apical muscular ventricular septal defect with shunting from the left to the distal right ventricle, resulting in some antegrade flow in the right ventricular outflow tract (Figures 3, 4, 5, and 6 and Movies III, IV, V, and VI). There was no Doppler gradient across the right ventricular outflow tract, and the pulmonary valve, although small, appeared morphologically normal. Under therapy with prostaglandin E₁, there was left-to-right shunting across the patent ductus arteriosus. There also was a patent foramen ovale with right-to-left shunting at the atrial level. The patient underwent placement of a central aortopulmonary shunt . . . [Full Text of this Article]