Ann Thorac Surg 2006;82:731-733
© 2006 The Society of Thoracic Surgeons
Case report
Ebstein's Anomaly Assessed by Real-Time 3-D Echocardiography
Philippe Acar, MD, PhD*,
Sylvia Abadir, MD,
Daniel Roux, MD,
Assaad Taktak, MD,
Yves Dulac, MD,
Yves Glock, MD, PhD,
Gerard Fournial, MD
Medical and Surgical Unit of Pediatric Cardiology, Toulouse, France
Accepted for publication September 6, 2005.
* Address correspondence to Dr Acar, Unité de Cardiologie Pédiatrique, Hôpital des Enfants, 330 Avenue de Grande-Bretagne, B-P 3119, Toulouse Cedex 3, 31026 France (Email: acar.p{at}chu-toulouse.fr).
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Abstract
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The outcome of patients with Ebstein's malformation depends mainly on the severity of the tricuspid valve malformation. Accurate description of the tricuspid anatomy by two-dimensional echocardiography remains difficult. We applied real-time three-dimensional echocardiography to 3 patients with Ebstein's anomaly. Preoperative and postoperative descriptions of the tricuspid valve were obtained from views taken inside the right ventricle. Surface of the leaflets as well as the commissures were obtained by three-dimensional echocardiography. Real time three-dimensional echocardiography is a promising tool, providing new views that will help to evaluate the ability and efficiency of surgical valve repair in patient with Ebstein's malformation.
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Introduction
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Ebstein's malformation is a rare congenital heart disease [1]. Patients with Ebstein's anomaly have a wide spectrum of anatomic abnormalities. The predictors of outcome depend on the severity of the tricuspid valve malformation [2–4]. Precise description of the tricuspid anatomy by conventional two-dimensional echocardiography remains difficult [5].
Three-dimensional (3-D) echocardiography offers a direct view to evaluate the leaflet surface [6]. Very few data were reported on 3-D reconstruction of the tricuspid valve from transoesophageal acquisition [7]. The recent introduction of the transthoracic 3-D matrix array probe allows real-time 3-D acquisition and visualization [8]. Our aim was to obtain preoperative and postoperative description of the tricuspid valve using 3-D echocardiography.
Real-time, 3-D echocardiographic ultrasound was performed with the Sonos 7500 (Philips) using the cardiac matrix probe (2 to 4 MHz). The system scanned a 60° x 60° 3-D pyramid of data. Two orthogonal reference plans were used to localize cardiac structures in the volume. Navigation by cropping inside the volume was performed to obtain 3-D views of the tricuspid valve. Live 3-D images were visualized directly on the ultrasound system.
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Case Reports
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Patient 1
A 6-year-old girl underwent two-dimensional echocardiography because of a systolic murmur. Tricuspid regurgitation was mild. Valve anatomy is described in Figure 1. Because the patient was asymptomatic and the regurgitation was mild, surgery was not indicated and an annual echographic follow-up was scheduled.
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Fig 1. Three-dimensional echocardiography (patient 1). The tricuspid valve was viewed from the right ventricle. The three leaflets with the commissures were visualized from below. Only the septal (S) leaflet had abnormal attachment to the ventricular septum. The anterior (A) and posterior (P) leaflets had normal coaptation.
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Patient 2
A 15-year-old boy was determined to be in functional class II of the New York Heart Association. Cardiac auscultation found a 3/6 holosystolic murmur. Chest roentgenogram showed increased cardiothoracic index to 0.65. Two-dimensional echocardiography found severe tricuspid regurgitation with septal attachment. Valve anatomy is described in Figure 2. The surgeon confirmed that the posterior leaflet was deficient and did not coapte with the septal and anterior leaflets of the tricuspid valve. The tricuspid valve was replaced with a Carpentier-Edwards pericardial bioprosthesis.
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Fig 2. Three-dimensional (3-D) echocardiography (patient 2). The tricuspid valve was viewed from below. Because of a restrictive motion and reduced functional surface, the posterior leaflet did not coapt with the septal (S) leaflet. The posterior (P) commissure displayed a huge hole compared with the continent anterior (A) and septal commissures. The surgeons confirmed the 3-D findings.
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Patient 3
A 10-year-old boy underwent surgical repair of Ebstein's malformation. Severe tricuspid regurgitation was corrected by De Vega annuloplasty and enlargement of the anterior leaflet with pericardial patch. Two years after the surgery, the patient was asymptomatic with trivial tricuspid regurgitation. Anatomy and function of the repaired tricuspid valve are described in Figure 3.
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Fig 3. Postoperative three-dimensional (3-D) echocardiography (patient 3). The tricuspid (T) and mitral (M) valves were viewed from below. (A) Early diastole (mitral and tricuspid valves are open). (B) End diastole (mitral valve is closed and tricuspid valve is open). (C) Systole (mitral and tricuspid valves are closed). The surgically enlarged anterior leaflet was the only mobile leaflet. The tricuspid valve repair created a functional monocusp with trivial regurgitation without stenosis.
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Comment
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We report the first description of Ebstein's anomaly using real-time 3-D echocardiography. Ebstein's anomaly of the tricuspid valve consists of various degrees of inferior displacement of the proximal attachments of the septal leaflet [1–4]. Because the apical four-chamber plane provides good visualization of the septal leaflet, two-dimensional echocardiography allows the initial diagnosis of Ebstein's anomaly [5]; however downward displacement could involve the anterior and posterior leaflets of the tricuspid valve. Precise description of the tricuspid anatomy could be difficult from only the two-dimensional planes. The surface of the tricuspid leaflets as well as the commissures could be displayed by 3-D echocardiography. The previous 3-D system was cumbersome due to the transoesophageal approach and the time needed for reconstruction [7]. Introduction of the transthoracic 3-D matrix array probe allowed the use of real-time 3-D echocardiography in routine [8]. Three-dimensional echocardiography allows the cardiologist and the surgeon to evaluate the ability and efficiency of surgical valve repair.
In conclusion, real-time 3-D echocardiography is a feasible method in addition to conventional two-dimensional echocardiography to evaluate tricuspid valve anatomy and function in patients with Ebstein's malformation.
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References
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- Chen JM, Mosca RS, Altmann K, et al. Early and medium-term results for repair of Ebstein's anomaly J Thorac Cardiovasc Surg 2004;127:990-998.[Abstract/Free Full Text]
- Celermajer DS, Bull C, Till JA, et al. Ebstein's anomalypresentation and outcome from fetus to adult. J Am Coll Cardiol 1994;23:170-176.[Abstract]
- Chauvaud S, Berrebi A, d'Attellis N, Mousseaux E, Hernigou A, Carpentier A. Ebstein's' anomalyrepair based on functional analysis. Eur J Cardiothorac Surg 2003;23:525-531.[Abstract/Free Full Text]
- Marianeschi SM, McElhinney DB, Reddy M, Silverman H, Hanley FL. Alternative approach to the repair of Ebstein's malformationintracardiac repair with ventricular unloading. Ann Thorac Surg 1998;66:1546-1550.[Abstract/Free Full Text]
- Gussenhoven EJ, Stewart PA, Becker AE, et al. "Offsetting" of the septal tricuspid leaflet in normal hearts and in hearts with Ebstein's anomalyAnatomic and echographic correlation. Am J Cardiol 1984;54:172-176.[Medline]
- Acar P, Laskari C, Rhodes J, Pandian NG, Warner K, Marx G. Determinants of mitral regurgitation after atrioventricular septal defect surgerya three-dimensional echocardiographic study. Am J Cardiol 1999;83:745-749.[Medline]
- Ahmed S, Nanda NC, Nekkanti R, Pacifico AD. Transesophageal three-dimensional echocardiographic demonstration of Ebstein's anomaly Echocardiography 2003;20:305-307.[Medline]
- Acar P, Dulac Y, Taktak A, Abadir S. Real time three-dimensional fetal echocardiography using cardiac matrix probe Prenatal Diagn 2005;25:370-375.[Medline]
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Abstract
Introduction
