Asian Cardiovasc Thorac Ann 2008;16:149-151
© 2008 Asia Publishing EXchange Ltd
Late Presentation of Cor Triatriatum with Persistent Levoatrial Cardinal Vein
Cristiana Feltri, MD,
Cinzia Crawley, MD1,
Ian Peart, MD,
Marco Pozzi, MD,
Antonio F Corno, MD
Cardiac Unit
1 Radiology Department, Royal Liverpool Children’s NHS Trust Alder Hey Hospital, Liverpool, United Kingdom
For reprint information contact: Antonio F Corno, MD, Tel: 44 151 252 5713, Fax: 44 151 252 5643, Email: Antonio.Corno{at}rlc.nhs.uk, Cardiac Unit, Royal Liverpool Children’s NHS Trust, Alder Hey, Eaton Road, Liverpool L12 2AP, United Kingdom.
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ABSTRACT
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An asymptomatic 10-year-old boy presented with reduced exercise tolerance and an echocardiographic diagnosis of cor triatriatum. Transthoracic and transesophageal echocardiography failed to reveal the persistent levoatrial cardinal vein discovered at surgery. In patients with late presentation of cor triatriatum with severe mitral inflow obstruction and a small patent foramen ovale, an alternative communication between the posterior collecting chamber and the systemic venous circulation should be sought with alternative imaging techniques.
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INTRODUCTION
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Cor triatriatum is a rare congenital heart defect presenting with various degrees of obstruction between the posterior collecting chamber receiving the pulmonary veins and the true left atrium with an opening towards the mitral valve. The very unusual association with persistent levoatrial cardinal vein can reduce the degree of obstruction and modify the natural history.
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CASE REPORT
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A 10-year-old boy was referred for investigation of a heart murmur and decreased exercise tolerance. Chest radiography showed an enlarged heart and plethoric lungs. Echocardiography (Figure 1
) revealed cor triatriatum with a stenotic membrane across the cavity of the left atrium (velocity, 2 m•s–1). The pulmonary veins were connected to the posterior collecting chamber. A small patent foramen ovale with a minimal left-to-right shunt was present. The right ventricle and pulmonary artery were dilated with elevated pressures, indicated by mild tricuspid regurgitation (3.6 m•s–1). The left ventricle appeared small with impaired systolic function (fractional shortening = 27%). The patient was referred for surgery without further investigation. Intraoperative transesophageal echocardiography confirmed the diagnosis. An enlarged innominate vein was identified, with O2 saturation of 99%. A persistent levoatrial cardinal vein was found and followed back to the posterior aspect of the left atrium where it connected to both left pulmonary veins and then diverged to the right and joined the posterior collecting chamber (Figure 2). The levoatrial cardinal vein was controlled with a tape and snared without any change in systemic O2 saturation or blood pressure. The surgical strategy did not change on finding this dual connection. On normothermic bicaval cardiopulmonary bypass and blood cardioplegia, intracardiac exploration from an oblique right atriotomy confirmed that the right pulmonary veins drained directly into the posterior collecting chamber. The left pulmonary veins drained into the same collecting chamber but via the levoatrial cardinal vein. The connection between the levoatrial cardinal vein and posterior collecting chamber was widely open. The membrane dividing the posterior collecting chamber from the true left atrium, with a very small opening (3–4 mm in diameter) on the posterolateral aspect of the left atrium, was fully resected. Macroscopically, the membrane appeared endothelial on the posterior collecting chamber surface, with layers of muscular tissue on the left atrial side (Figure 2). The levoatrial cardinal vein was doubly legated. The patient was easily weaned from bypass without inotropic support. The pulmonary artery systolic pressure at the end of the procedure was 16 mm Hg. The patient was extubated in the operating room. He had an uneventful postoperative course, and was discharged after 4 days. Follow-up echocardiography at 3 months showed gradual regression of right ventricular dilatation, normal right ventricular pressures and good left ventricular function. The patient remained asymptomatic 10 months after surgery. Postoperatively, a multidetector computed tomography angiogram, using a 16-slide Philips scanner, showed balanced ventricles and confirmed the pulmonary venous anatomy and ligated levoatrial cardinal vein (Figure 3).
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Figure 1. Preoperative echocardiogram. CTM = cor triatriatum membrane, LA = left atrium, LV = left ventricle, PCC = posterior collecting chamber, RA = right atrium, RV = right ventricle.
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Figure 2. (A) Schematic drawing; CTM = cor triatriatum membrane, LA = left atrium, LAA = left atrial appendage, LACV = levoatrial cardinal vein, LPV = left pulmonary veins, PCC = posterior collecting chamber, PFO = patent foramen ovale, RPV = right pulmonary veins; (B) Membrane surfaces facing the collecting chamber; (C) The true left atrium. Arrow indicates the opening (8 x 4 mm) of the posterior collecting chamber to the left atrium.
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Figure 3. Postoperative computed tomography angiogram showing (A) 3D lateral, and (B) 3D posterior multiplanar reconstruction of pulmonary venous anatomy and the ligated levoatrial cardinal vein (black arrows); (C) The left atrial and left pulmonary venous drainage was through a common vein, the ligated levoatrial cardinal vein (white arrow); (D) Four-chamber view showing balanced ventricles. AA = aortic arch, IV = innominate vein, LA = left atrium, LO = levoatrial cardinal vein opening, LPA = left pulmonary artery, LPV = left pulmonary vein, LV = left ventricle, MPA = main pulmonary artery, RA = right atrium, RPA = right pulmonary artery, RPV = right pulmonary vein, RV = right ventricle, SVC = superior vena cava.
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DISCUSSION
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Patients with severe cor triatriatum are usually symptomatic in infancy due to early clinical signs of mitral stenosis and/or a left-to-right shunt. A few patients with a relatively wide or even unrestricted left atrial communication are diagnosed in the 3rd–4th decades or later.1–3 Our patient remained asymptomatic for almost 10 years despite a very small communication between the posterior collecting chamber and the left atrium and a patent foramen ovale to decompress the pulmonary veins. He developed decreased exercise tolerance in the absence of significant respiratory compromise. This clinical picture was probably a consequence of increased pulmonary venous pressure on exercise due to left ventricular inflow obstruction.
The association between cor triatriatum and partial or total anomalous pulmonary venous connection with persistent levoatrial cardinal vein with dual connection is very rare but well described.4,5 In our patient, the connection between the levoatrial cardinal vein and posterior collecting chamber was unrestricted, and simple legation of the former was required. In patients presenting late in life with cor triatriatum with severe obstruction to mitral valve inflow and a small atrial communication, or in those with only one left or right pulmonary vein clearly seen draining into the posterior collecting chamber, the presence of another pulmonary-to-systemic venous communication should be investigated, as a different surgical approach may be required. Considering the difficulties in imaging such lateral extracardiac structures by transthoracic or transesophageal echocardiography in older children or adults, other imaging techniques, such as multidetector computed tomography, are strongly advised.6
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REFERENCES
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- Kaneda T, Onoe M, Matsuda M, Moriwaki S, Mori N. Patent levoatrial cardinal vein without left heart hypoplasia. Ann Thorac Surg 2006;81:740–2.[Abstract/Free Full Text]
- Tsutsumi K, Ookura M. Asymptomatic cor triatriatum incidentally revealed in an elderly patient with mitral stenosis. Jpn J Thorac Cardiovasc Surg 2003;51:452–5.[Medline]
- Caputo S, Santoro G, Morelli C, Sarubbi B, D’Alto M, Russo MG, et al. Subtotal anomalous pulmonary venous connection and left cor triatriatum: a rare diagnosis in adulthood. J Am Soc Echocardiogr 2006;19:836.e1–4.
- Vouhé PR, Baillot-Vernant F, Fermont L, Bical O, Leca F, Neveux JY. Cor triatriatum and total anomalous pulmonary venous connection: a rare, surgically correctable anomaly. J Thorac Cardiovasc Surg 1985:90:443–5.[Abstract]
- Corno AF. Congenital heart defects. Decision making for cardiac surgery. Vol 1 & 2. Darmstadt: Springer-Steinkopff Verlag, 2003 & 2004.
- Chen K, Thng CH. Multislice computed tomography and two-dimensional echocardiographic images of cor triatriatum in a 46-year-old man. Circulation 2001;104:2117.[Free Full Text]
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ABSTRACT
INTRODUCTION
