Asian Cardiovasc Thorac Ann 2007;15:332-334
© 2007 Asia Publishing EXchange Ltd
Multiperforated Atrial Septal Aneurysm and a Single Coronary Artery
Julian E Losanoff, MD,
Bruce W Richman, MA1,
James W Jones, MD2
Department of Surgery, University of Chicago, Chicago
1 Department of Surgery, University of Missouri-Columbia, Columbia
2 The Center for Health Policy and Medical Ethics, Baylor College of Medicine, Houston, USA
For reprint information contact: Julian E Losanoff, MD, Tel: 1 312 330 5659, Fax: 1 313 576 1002, Email: jelosanoff{at}yahoo.com, Department of Surgery (11S), John D. Dingell VA Medical Center, 4646 John R, Detroit, MI 48201, USA.
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ABSTRACT
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Multiperforated atrial septal aneurysm (ASA) with left-to-right shunt and elevated pulmonary arterial pressure is rare. We present an adult patient with this unusual anomaly combined with a single coronary ostium emerging from the right sinus of Valsalva. The ASA was excised and the defect repaired successfully.
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INTRODUCTION
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Atrial septal aneurysms (ASAs) are generally rare but their incidence varies depending on the patient population: literature reports cite 64% in fetuses with atrial arrhythmias, 26% in congenital anomalies, 1–2% in pediatric cardiac patients, 1–8% in normal subjects, and 1% in multiple consecutive autopsies.1–3 Perforated ASAs are peculiar because of the associated hemodynamic disturbances, including small shunts presenting with thromboembolic events, and significant left to right shunts paralleling the features of atrial septal defects.1–3 Perforated ASAs have been classified into four types: A (with persistent foramen ovale); B (with atrial septal defect); C (with two or few perforations in clusters), and D (with multiple, irregularly spread perforations).3 Recent experience with transcatheter treatment of ASAs suggests that the method is potentially successful for the first three ASA types (A–C), with open surgery indicated for type D.3
The congenital occurrence of a single coronary artery to supply the entire heart is rare, with an incidence of 0.019%.4 The anomaly has been described as a cause of cardiac ischemia, congestive heart failure, and sudden death.5 Its importance has not been fully determined because only a few have been described in living patients. To our knowledge, no report describes a combination of ASA and a single coronary artery.
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CASE REPORT
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A 41-year-old female patient was admitted to the Emergency Department complaining of dyspnea and retrosternal discomfort. She gave a history of orthopnea, cough, and hemoptysis for the prior six months. Physical examination revealed a 3/6 diastolic flow murmur along the left sternal border. Transesophageal echocardiography revealed right ventricular hypertrophy, prominent pulmonary arteries, and a multiperforated ASA. Cardiac catheterization indicated pulmonary hypertension, left-to-right shunt (47% in the atrium level), impaired cardiac output, and anomalous coronary blood supply represented by an absent left coronary artery ostium, a single right coronary artery, and a rudimentary circumflex artery arising from the right coronary ostium and coiling around the left ventricle posteriorly (Figure 1
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Figure 1. Coronary angiogram showing normal right and circumflex arteries arising from the right coronary ostium (arrow).
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Hyperkalemic cardioplegia at 4°C was infused. The heart stopped immediately; myocardial temperatures were monitored and cardioplegia was ceased when the temperature fell below 4°C. The surgical findings included marked pulmonary artery dilatation, right ventricle hypertrophy, and a hypoplastic left ventricle. The myocardium was supplied by a well-developed right coronary artery; no left main coronary artery was found. There were no macroscopic signs of coronary atherosclerosis. The interatrial septum was thin-walled and contained a fenestrated 2 cm aneurysm. The aneurysm was excised and the defect repaired with a Dacron patch. Only one dose of cardioplegia was necessary. The cardiopulmonary bypass was discontinued efficiently without sympathomimetic drips. The patient’s postoperative recovery was uneventful.
The septal specimen appeared as a trabeculated, slightly nodular and focally fenestrated tan-yellow fibrous sheath measuring 2.7 x 2.1 x 0.1 cm (Figure 2). Histologically, serial sections of the ASA were compared to septa from normal individuals of the same age. No scar tissue or notable differences in cellular morphology, extracellular matrix, vascularity, endothelial lining, or collagen contents were found.
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DISCUSSION
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The constellation of multiple abnormalities in the heart’s gross structure suggests a genetic etiology of the reported condition. Anomalies of the coronary arteries’ origin are commonly associated with other major congenital cardiovascular anomalies.4 Based on tissue analysis, we hypothesize that the etiology of the ASA’s multiple perforations was related to an embryonic dysmorphogenesis rather than acquired tears in the ASA. The aneurysm’s perforations were regular in shape and with smooth margins. Scar tissue, which would have been evident in case of a septal tear, was absent. Physiologic vascular supply throughout the specimen suggests that the fenestrations of the septum were not produced by ischemia or tissue necrosis. No necrotic tissue was found. Collagen was distributed evenly, as seen in multiple trichrome preparations, and its volume was equal to that found in healthy hearts. This first detailed histologic ASA study contradicts earlier data linking the ASA pathogenesis to mixoid degeneration and increased collagen content.1
Significant shunts through fenestrated ASAs cause right ventricular volume overload and heart failure, hence the necessity for prompt defect closure. The treatment methods depend on the aneurysm’s morphology. Successful transcatheter closure of types A–C ASAs has been reported using the Amplatzer septal (ASD) and persistent foramen ovale (PFA) occluders (AGA Medical Corporation, Golden Valley, MN, USA), and the Cardio-Seal device (Nitinol Medical Technologies, Boston, MA, USA).3 Open surgical repairs are currently reserved for type D ASAs.3 Depending on the aneurysm’s size and extent of fenestrations, defect closure using endogenous tissue or prosthetic material may be used.6 The literature lacks randomized trials with long-term follow-up data, which potentially makes the treatment approaches intuitive or based on individual preferences.
This unusual case illustrates a pathological constellation of cardiac anomalies not previously described. Our patient’s ASA exemplifies the life-threatening consequences such lesions can cause in the later stages. Her single right coronary artery reminds us that the condition’s serious long-term prognosis requires close follow-up.4,5 Awareness of the anomaly’s clinical significance can spare diagnostic efforts and avoid misdiagnosis of occlusive coronary disease.
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REFERENCES
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- Angelini P, Wilansky S, Gaos C, Montazavi A, Boncompagni E, Cooley DA. Prolapsing large aneurysm of the atrial septum simulating a right atrial mass. Cathet Cardiovasc Diagn 1992;26:122–6.[Medline]
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- Antonellis J, Rabaouni A, Kostopoulos K, Margaris N, Kranidis A, Salahas A, et al. Single coronary artery from the right sinus of Valsalva, associated with absence of left anterior descending and an ostium-secundum-type atrial septal defect: a rare combination. A case report. Angiology 1996;47:621–5.[Medline]
- Passman RS, Ferrari VA, Holland GA, Herling IM, Kolansky DM. Single coronary artery: an angiographic and MRI case report. Cathet Cardiovasc Diagn 1997;40:177–8.[Medline]
- Kondo T, Iwasaki K, Hiramatsu K, Katakura M, Shirota T, Yamada T, et al. Partial resection of atrial septal aneurysm with multiple fenestrations–a case report. Angiology 1988;39:838–42.[Medline]
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ABSTRACT
INTRODUCTION
