Ann Thorac Surg 2006;81:1888-1890
© 2006 The Society of Thoracic Surgeons
Case report
Intermediate Type Atrioventricular Septal Defect in the Elderly
Shoh Tatebe, MD, PhD
*
,
Yoshiaki Saji, MD, PhD,
Kenji Aoki, MD,
Setsuo Kuraoka, MD, PhD
Department of Thoracic and Cardiovascular Surgery, Mito Saiseikai General Hospital, Mito City, Japan
Accepted for publication May 24, 2005.
* Address correspondence to Dr Tatebe, Department of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medicine and Dentistry, 757 Asahimachi-Dohri 1, Niigata City, 951-8510 Japan (Email: statebe{at}yahoo.com).
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Abstract
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A 65-year-old woman with intermediate type atrioventricular septal defect had been undiagnosed until initial presentation with congestive heart failure. She underwent surgery when the ventricular septal defect was found to be restrictive. The atrioventricular valve was similar to the common atrioventricular valve, but was connected to the crest of the ventricular septal defect. Surgery included direct closure of the ventricular septal defect, repair of the cleft in the atrioventricular valve, and ostium primum closure. Postoperative echocardiography indicated successful heart repair. We present a review of rare intermediate type atrioventricular septal defect in the elderly, and we discuss the surgical issues in this case.
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Introduction
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Atrioventricular septal defect (AVSD) is a complex cardiac anomaly, the status of which varies according to the type of disease. Patients with complete or intermediate type AVSD rarely survive decades without appropriate treatment. Here we report a 65-year-old patient with intermediate type AVSD, who was undiagnosed until initial presentation. The patient underwent surgery that improved her status. We present a review of intermediate type AVSD in the elderly, and discuss the issues regarding surgical procedures in such cases.
A 65-year-old woman presented at a county hospital with generalized edema. She had suffered from shortness of breath and exertional dyspnea for 2 years. At presentation she was lethargic, and grade III/VI systolic murmur was audible at the left sternal border. Her blood pressure was 130/64 mm Hg, and her pulse was irregular at 88 bpm. Electrocardiography showed atrial fibrillation. Echocardiography revealed an ostium primum defect of the atrial septum, a common atrioventricular valve with severe regurgitation, and interventricular communication. Based on these symptoms, the patient was diagnosed as having AVSD, which was suggested to be of the intermediate type. Treatment with digoxin and diuretics improved her status. Cardiac catheterization showed mild pulmonary hypertension (42/15 mm Hg), marked left to right shunt (ratio of pulmonary blood flow to systemic blood flow, 2.64), and stenosis of the right coronary artery (number 2). Therefore, surgery was performed.
The chest was explored by median sternotomy intraoperatively. Cardiopulmonary bypass was instituted, and the patient was cooled to 32°C. The heart was arrested by crystalloid cardioplegia. The right atrium was opened, and a large ostium primum defect and a restrictive ventricular septal defect (VSD) were observed (Fig 1). The atrioventricular (AV) valve appeared to have two orifices. However, the VSD crest was extended, creating fusion between both bridging leaflets of the common atrioventricular valve. The VSD measured 5 mm in diameter and was closed directly with mattress sutures. Stitches were placed only into the VSD crest or the fibrous tissue surrounding the VSD, or both (Fig 1B). The cleft in the left AV valve was repaired. The orifice of the left AV valve measured 27 mm as determined by using a prosthetic valve sizer. Saline was injected into the left ventricle through a catheter introduced through the left AV valve, which was removed after the left ventricle was filled with saline. This saline injection test indicated that the left AV valve was competent. The ostium primum was closed with autologous pericardium, and the coronary sinus was left in the left side of the heart (Fig 1C). Coronary artery bypass grafting to the right coronary artery was performed concomitantly with autologous saphenous vein. The patient tolerated this procedure well. Postoperative echocardiography revealed no shunts, atrioventricular valve regurgitation, or stenosis of the left ventricular outflow tract. The patient is currently well 3 years after surgery.
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Fig 1. (A) Intraoperative photograph (surgeon's view). A ventricular septal defect (through which a catheter was passed, see asterisk) beneath the common atrioventricular (AV) valve. (B) Direct closure of the ventricular septal defect. (C) Intraoperative photograph (surgeon's view). Atrial septal defect closure using autologous pericardium. (ASD = atrial septal defect.)
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Comment
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Intermediate type AVSD, also called transitional type AVSD, is defined as "malformation lying between persistent ostium primum with cleft aortic leaflet of the mitral valve and the complete form of common AV orifice" [1]. Intermediate type AVSD is less frequent than complete or partial AVSD, and is rarely encountered in the elderly. There have been a few reports of elderly patients who underwent surgery [2] or were diagnosed at autopsy [3]. Tandon and colleagues [3] reviewed 139 patients with AVSD, and only 5 patients (complete AVSD, 1; intermediate type AVSD, 1; partial AVSD, 3) survived to 46 years of age or older [3]. There have been no reports regarding the natural history of intermediate type AVSD. However, considering the lifespan of other types of AVSD (ie, 2 to 15 years, and 30 years in complete and partial AVSD, respectively) [4], our patient survived for an unusually long time.
The anatomy of intermediate type AVSD varies widely, and the surgical approach is not uniform but must be modified in each case. Therefore previous reports have emphasized recognition and understanding of the anatomy. Bharati and colleagues [1] classified the disease into three types based on AV valve morphology: (1) type I, which is two separate AV valves with cleft; (2) type II, which is AV orifice divided by AV valve tissue or summit of VSD, or both; and (3) type III, which is anterior and posterior bridging leaflets similar to complete AVSD [1]. The present case was similar to type II, with separation of the AV orifice by AV valve tissue and the VSD crest.
Surgery includes VSD closure, repair of the cleft in the AV valve, and ostium primum closure. In closure of the VSD, care must be taken to avoid postoperative heart block. Sutures must not be placed at the posteroinferior rim of the VSD through which penetrating bundle pass [5]. In the present case, fibrous tissue extended continuously to the VSD crest. Therefore sutures were placed only in the fibrous tissue, and no heart block occurred. In addition, the VSD was closed directly in the present case, which may push the AV valve down toward the VSD crest. This may result in left ventricular outflow tract stenosis, which is related to the controversy regarding novel surgery for complete AVSD (ie, "direct repair"). This method applies direct suturing of the common atrioventricular valve to the VSD crest. Nicholson and colleagues [6] reported 47 patients who underwent direct repair, and none of their patients had left ventricular outflow tract stenosis develop. As ventricular septal scoop is usually small in intermediate type AVSD, there is less chance of development of left ventricular outflow tract stenosis when VSD is closed directly. In our case, no left ventricular outflow tract stenosis developed, but careful long-term follow-up is required.
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References
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- Bharati S, Lev M, McAllister Jr HA, Kirklin JW. Surgical anatomy of the atrioventricular valve in the intermediate type of common atrioventricular orifice J Thorac Cardiovasc Surg 1980;79:884-889.[Abstract]
- Ataka K, Ota T, Yoshimura N, Sakata M, Okada M. A successful surgical correction of the intermediate type of common atrioventricular orifice; report of an adult case J Jpn Assn Thorac Surg 1994;42:389-392.
- Tandon R, Moller JH, Edwards JE. Unusual longevity in persistent common atrioventricular canal Circulation 1974;50:619-626.[Abstract/Free Full Text]
- Keith JD. Atrial septal defectostium secundum, ostium primum, and atrioventricularis communis (common AV canal). In: Keith JD, Rowe RD, Vlad P, editors. Heart diseases in infancy and childhood. 3rd ed.. New York: Macmillan; 1978. pp. 380-404.
- Elliott MJ, Jacobs JP. Atrioventricular canal defectsIn: Kaiser LR, Kron IL, Spray TL, editors. Mastery of cardiothoracic surgery. Philadelphia: Lippincott-Raven; 1998. pp. 742-758.
- Nicholson IA, Nunn GR, Sholler GF, Hawker RE, Cooper SG, Lau KC. Simplified single patch technique for the repair of atrioventricular septal defect J Thorac Cardiovasc Surg 1999;118:642-647.[Abstract/Free Full Text]
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Abstract
Introduction
