Ann Thorac Surg 2004;77:321-322
© 2004 The Society of Thoracic Surgeons
Case report
Coronary artery obstruction due to membranous ridge of the right sinus valsalva associated with Tetralogy of Fallot: syncope mimics anoxic spell
Isao Shiraishi, MDa*,
Masaaki Yamagishi, MDb,
Kentaro Toiyama, MDa,
Yu Osawa, MDc,
Masao Nakagawa, MDc,
Akiyuki Takahashi, MDb,
Keisuke Shuntoh, MDb,
Kenji Hamaoka, MDa
a Department of Pediatrics, Kyoto, Japan
b Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
c Department of Pediatrics, Shiga University School of Medicine, Shiga, Japan
Accepted for publication April 29, 2003.
* Address reprint requests to Dr Shiraishi, Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan 602-8566.
e-mail: isao{at}koto.kpu-m.ac.jp
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Abstract
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We report an infant with Tetralogy of Fallot who had suffered from repetitive attacks of syncope without obvious cyanosis. Careful observation by means of echocardiography and angiography revealed that the attacks resulted from acute coronary artery obstruction due to membranous ridge covering the sinus Valsalva. Surgical resection of the abnormal ridge and repair of Tetralogy of Fallot successfully improved the patient's symptoms. Syncope in children should be extensively investigated to exclude obstruction of the coronary arteries.
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Introduction
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Congenital coronary artery anomalies are a rare but important cause of sudden cardiac death in young population [1]. We report a 1-year-old infant with Tetralogy of Fallot who had repetitive attacks of syncope without obvious cyanosis. The syncope attacks had been diagnosed as anoxic spell of Tetralogy of Fallot. Echocardiography and cardiac angiography revealed an abnormal ridge at the right sinus Valsalva, almost isolating the ostium of the right coronary artery. Surgical repair of the conotruncal anomaly and resection of the membranous ridge successfully improved the patient's symptoms.
A 16-month-old boy with a diagnosis of Tetralogy of Fallot was hospitalized due to repetitive syncope attacks. The attacks suddenly happened without obvious cyanosis and the heart rate fell into marked bradycardia (<40/min). Anoxic spell of Tetralogy of Fallot was initially suspected. However, transthoracic and transesophageal echocardiography visualized an unusual membranelike structure at the right sinus Valsalva (Fig 1A).
At the age of 17 months, cardiac catheterization and angiography were performed. The selective right coronary artery angiography demonstrated hypoplastic right sinus Valsalva and a membranelike ridge isolating the ostium of the right coronary artery (Fig 2A).
These findings suggested that the syncope attacks were induced by the acute obstruction of the right coronary arterial ostium (Fig 1B) rather than the anoxic spell.
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Fig 1. (A) Transesophageal echocardiogram of the patient at the level sinus Valsalva. (B) Schematic diagram of the sinus Valsalva; arrow indicates abnormal membranous ridge. (C and D) Operative findings of the sinus Valsalva. The abnormal ridge (C, arrow) is completely removed from the right sinus Valsalva (D, arrow). (LCA = left coronary artery; LCC = left coronary cusp; NCC = noncoronary cusp; RCA = right coronary artery; RCC = right coronary cusp.)
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Fig 2. (A) Aortogram of the right oblique view before the surgical repair; arrow indicates abnormal membranous ridge. (B) Aortogram of the anterior view of the right sinus Valsalva (arrow) 14 months after the surgery.
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Surgical repair of the conotruncal anomalies and resection of the abnormal ridge was performed. The right coronary cusp was hypoplastic and was almost covered with the membranous ridge originating from the upper portion of the right sinus Valsalva (Fig 1C). The coronary obstruction was successfully treated by complete resection of the abnormal ridge (Fig 1D). Histologic examination revealed fibrous tissue with well-matured fibroblasts. After the surgical repair, the patient has been uneventful for 1.5 years. Follow-up cardiac angiography performed 14 months after the operation demonstrated normal size and movement of the aortic valve leaflets (Fig 2B). Aortic regurgitation was not detected. Chromosomal analysis by fluorescent in situ hybridization did not exhibit deletion in 22q11.2.
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Comment
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We report an infant with acute coronary artery obstruction due to abnormal ridge of the sinus Valsalva associated with Tetralogy of Fallot. The patient presented syncope attacks that had been mixed up with anoxic spell. It appears that the syncope attacks were induced by acute obstruction of the right coronary artery and insufficiency of the sinus node and atrioventricular node artery. The precise diagnosis and the early surgical repair successfully rescued the patient from the life threatening attacks.
Syncope or angina in children, which is produced by coronary ostial obstruction, is a very rare condition and is associated with congenital aortic valve or supravalvular aortic disorders [2–4]. Previous reports described the membranous obstruction at the left main coronary artery. Gibson and coworkers [2] reported two patients with membranous ridge at the left sinus Valsalva and both patients were surgically repaired by removal of the abnormal membranes. Gibbs and colleagues [3] also reported a patient with abnormal membranous ridge and severe aortic regurgitation, which was repaired by replacement of an aortic homograft. Harlan and associates [4] also reported 13 patients with congenital aortic stenosis characterized by hypoplastic left sinus Valsalva associated with a supravalvular ridge. Obstruction of the left main coronary artery is also a characteristic complicating feature of sudden death in infants with supravalvular aortic stenosis in William syndome [5]. Congenital atresia of the left main coronary artery has also been reported [6]. The left main coronary artery is replaced by a fibromuscular cord and the left coronary artery ostium is absent or occluded.
Recent advance in cardiac embryology has revealed that cardiac neural crest cells play an important roles in conotruncal septation, development of proximal coronary arteries, and formation of semilunar valves [7]. Tetralogy of Fallot is the most common cardiac anomaly in deletion in 22q11.2 syndrome that is induced by dysfunction of cardiac neural crest cells [8]. The association of Tetralogy of Fallot and the acute coronary obstruction by the membranous ridge in this patient could be induced by impaired function of neural crest cells.
Unusual cases of coronary arterial obstruction are being reported with increasing frequency. Many of them are suitable for surgical correction. Successful surgery in our patient further emphasizes the importance of awareness of the existence of such abnormalities.
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References
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- Gibbs J.L., Davies J., Qureshi S.A. Coronary artery obstruction associated with congenital aortic valve disease. Br Heart J 1984;52:585-587.[Abstract/Free Full Text]
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Abstract
Introduction
