Repair of Narrowed Coronary Sinus without Connecting Left Superior Vena Cava
Takashi Miyamoto, MD, PhD,
Yoshio Doi, PhD, MD,
Kentaro Ikeda, MD1,
Tohru Kobayashi, MD, PhD1,
Tomio Kobayashi, MD, PhD1
Department of Cardiovascular Surgery
1 Pediatric Cardiology Gunma Children’s Medical Center Gunma, Japan
Takashi Miyamoto, MD, Tel: +81 279 52 3511, Fax: +81 279 52 2045, Email: yonomiyataka{at}msn.com, 779 Shimohakoda, Hokkitsu, Shibukawa, Gunma 377-8577, Japan.
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ABSTRACT
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We successfully repaired a markedly narrowed coronary sinus without connecting a left superior vena cava in a 3-year-old boy with an atrial septal defect.
Key Words: Coronary Sinus • Coronary Vessel Anomalies • Vena Cava
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INTRODUCTION
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A left superior vena cava (LSVC) sometimes drains directly to the left atrium in an anomaly of the coronary sinus ostium. This is usually seen in atrial isomerism. Markedly narrowed coronary sinus is a very rare cardiac anomaly. Obstruction of coronary sinus drainage may lead to myocardial ischemic problems with consequent coronary venous hypertension.
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CASE REPORT
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A 3-year-old boy weighing 12 kg was referred to our institution for treatment of an atrial septal defect (ASD). He was on diuretics. Two-dimensional echocardiography demonstrated a large ASD measuring 20 mm, with left-to-right shunting. Preoperative catheterization showed a pulmonary-to-systemic blood flow ratio of 6.8, mean pulmonary arterial pressure of 26 mm Hg, mean right atrial pressure of 8 mm Hg, and mean left atrial pressure of 9 mm Hg. Pulmonary resistance was 0.7 U. Angiocardiography revealed an LSVC draining directly to the left atrium; the left ventricular end-diastolic volume was 157% of normal. On echocardiography, a large ASD was detected, and an unroofed coronary sinus to the left atrium was suspected. At operation, an LSVC was found in addition to a right superior vena cava, with no bridging vein connecting them. After establishing cardiopulmonary bypass with bicaval cannulae, the LSVC was snared with silk suture. After cardiac arrest, a right atriotomy was performed. There was marked narrowing of the ostium of the coronary sinus within the right atrium (Figure 1
). The LSVC returned directly to the upper corner of the left atrium (Figure 2). The stenotic coronary sinus orifice was enlarged with a 5-mm incision under guidance using a bougie. The ASD was closed with a pericardial patch, leaving the coronary sinus to the right atrium. The LSVC was divided on the cardiac side, and the cardiac end was closed. After declamping, the LSVC was relocated to the tip of the right atrial appendage. The patient was easily weaned from cardiopulmonary bypass, with regular sinus rhythm. Angiography at 1 month postoperatively revealed no obstruction of the coronary sinus or systemic vein.
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Figure 1. Preoperative left superior vena cavography revealing the left superior vena cava returning directly to the upper corner of the left atrium. There was no bridging vein between the left and right superior venae cavae.
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Figure 2. There was markedly narrowed ostium (*) of the coronary sinus within the right atrium. The sucker tip was inserted into the left atrium through the atrial septal defect.
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DISCUSSION
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Anomalies involving the coronary sinus are often associated with other venous anomalies of either the systemic or pulmonary circulation. Coronary sinus atresia is usually associated with an unroofed coronary sinus, constituting a small right-to-left shunt. There are some reports of coronary sinus orifice atresia with persistent LSVC.1–4 This is seen when some of the cardiac veins fail to join the coronary sinus. Blood spouts out from the narrow orifice that is formed in response to partial obstruction during early development, without connecting the LSVC. A diagnosis of coronary sinus orifice atresia is usually made at cardiac catheterization when retrograde flow is detected though the LSVC.3,4 Transthoracic echocardiography may demonstrate a retrograde LSVC. In our case, transesophageal echocardiography detected the coronary sinus orifice without a connecting LSVC. However, the narrowed coronary sinus orifice was not recognized. Selective angiography of the coronary artery defined the coronary sinus orifice atresia.
The crux of the surgical treatment of this anomaly was enlargement of the narrow coronary sinus orifice and patch closure of the large ASD, leaving the coronary sinus orifice to the right atrium because of the hazardous absence of a landmark for the atrioventricular node. In this patient, it was necessary to rule out coronary sinus orifice atresia by selective angiography of the innominate vein and coronary artery. It is important to avoid lethal coronary venous hypertension, myocardial hypoperfusion and damage to the atrioventricular node.
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REFERENCES
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- Salminen JT, Hakala T, Pihkala J, Mattila I, Puntila J, Sairanen H. Coronary sinus orifice atresia with left superior vena cava in patients with univentricular heart. Ann Thorac Surg 2006; 81:e16–7.[Abstract/Free Full Text]
- Oshima Y, Doi Y, Misaki T, Ichida F. Surgical repair of coronary sinus orifice atresia. Eur J Cardiothorac Surg 2005;27:351–2.[Abstract/Free Full Text]
- Takabayashi S, Shimpo H, Yokoyama K. Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia. Gen Thorac Cardiovasc Surg 2007;55:197–9.[Medline]
- Fulton JO, Mas C, Brizard CP, Karl TR. The surgical importance of coronary sinus orifice atresia. Ann Thorac Surg 1998; 66:2112–4.[Abstract/Free Full Text]
Asian Cardiovasc Thorac Ann 2009;
17:186-187
© 2009 by SAGE Publications
DOI: 10.1177/0218492309103311
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ABSTRACT
INTRODUCTION
