Intermediate Type Atrioventricular Septal Defect in the Elderly

Intermediate Type Atrioventricular Septal Defect in the Elderly

1888 CASE REPORT TATEBE ET AL INTERMEDIATE TYPE ATRIOVENTRICULAR SEPTAL DEFECT IN THE ELDERLY FEATURE ARTICLES patient received 40 mEq of potassium...

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1888

CASE REPORT TATEBE ET AL INTERMEDIATE TYPE ATRIOVENTRICULAR SEPTAL DEFECT IN THE ELDERLY

FEATURE ARTICLES

patient received 40 mEq of potassium added to the pump by the perfusionist for hyperkalemic myocardial arrest. Under TCA, the kidney was removed and the IVC was opened just inferior to the hepatic veins to allow removal of tumor or thrombus under direct visualization. In patients 1 and 3, the IVC was chronically occluded and a long segment of IVC was resected en bloc along with the kidney (patient 1) and retro-hepatic IVC. In these two cases, the IVC was not reconstructed because of the presence of well-established collateral venous drainage. In patient 2, the retro-hepatic IVC was repaired primarily after tumor thrombectomy without the need for an interposition graft. After 1 minute of retrograde body perfusion and air removal of the venous and arterial system, the patient was re-warmed on cardiopulmonary bypass. With warming the patient had a spontaneous normal sinus rhythm develop with good hemodynamics and oxygenation. All 3 patients were extubated within the first 24 hours postoperatively and discharged from the intensive care unit to the surgical wards within the first 48 hours. The mean cardiopulmonary bypass time was 127.7 minutes (range, 123 to 134 min), and the mean circulatory arrest time was 23.7 minutes (range, 13 to 44 min). The mean hospital stay was 6.3 days (range, 4 to 8 days). There were no neurologic complications. At last follow-up (February 2005), patients 1 and 3 were alive without any recurrence of disease, whereas patient 2, who had brain metastasis at the time of surgery, expired 12 months after the operation.

Comment Several techniques can be utilized to manage tumors of the IVC that extend to the right atrium [5, 7]. In our institution the standard approach in the past had been to perform TCA through a midline sternotomy in conjunction with a midline abdominal incision. This approach allowed for exploration of the IVC and right atrium and for cannulation to perform TCA. This approach was used in 8 patients at our center between 1988 and 2001. In this small series there was no 30-day mortality, but the mean hospital stay was 25 days (range, 3 to 73). With the introduction of parasternal and “J” incisions for the treatment of IVC tumors, Svensson and colleagues [3] reported hospital stays that averaged 18 days and minimal morbidity. The minimal-access femoral cannulation approach described here provides an excellent option for TCA when it is necessary to open the IVC and extract a clot from the region of the hepatic veins or the right atrium. Femoral cannulation TCA may allow resection of suprahepatic IVC tumors without the need for sternotomy. In each case described here the surgical exposure was excellent and was not compromised by the lack of a sternotomy. Two cases did not require pericardiotomy because the tumor was easily extracted through an opening in the IVC below the diaphragm. In larger tumors or those with significant atrial extension, a subxyphoid pericardiotomy may allow additional exposure. How© 2006 by The Society of Thoracic Surgeons Published by Elsevier Inc

Ann Thorac Surg 2006;81:1888 –90

ever, sternotomies may continue to be required in rare cases in which tumors are densely adherent or invasive at or above the atriocaval junction. Surgeons preparing to resect IVC tumors should consider all these options when planning an operative approach. We propose that many cases of intraatrial IVC tumor extension previously treated with sternotomy can be effectively managed without sternotomy by establishing TCA through femoral cannulation.

References 1. Svensson LG. Minimal-access “J” or “j” sternotomy for valvular, aortic, and coronary operations or reoperations. Ann Thorac Surg 1997;64:1501–3. 2. Fitzgerald JM, Tripathy U, Svensson LG, Libertino JA. Radical nephrectomy with vena caval thrombectomy using a minimal access approach for cardiopulmonary bypass. J Urol 1998;159: 1292–3. 3. Svensson LG, Libertino JA, Sorcini A, Kaushik SD, Marinko E. Minimal-access right atrial exposure for tumor extensions into the inferior vena cava. J Thorac Cardiovasc Surg 2001; 121:589 –90. 4. Okamoto H, Ito T, Morita S, Matsuura A, Yasuura K. Intravenous leiomyomatosis extending into the right ventricle: one-stage radical excision during hypothermic circulatory arrest. Thorac Cardiovasc Surg 1994;42:361–3. 5. Chiappini B, Savini C, Marinelli G. Cavoatrial tumor thrombus: single-stage surgical approach with profound hypothermia and circulatory arrest, including a review of the literature. J Thorac Cardiovasc Surg 2002;124:684 – 8. 6. Ciancio G, Hawke C, Soloway M. The use of liver transplant techniques to aid in the surgical management of urological tumors. J Urol 2000;164:665–72. 7. Vaidya A, Ciancio G, Soloway M. Surgical techniques for treating a renal neoplasm invading the inferior vena cava. J Urol 2003;169:435– 44.

Intermediate Type Atrioventricular Septal Defect in the Elderly Shoh Tatebe, MD, PhD, Yoshiaki Saji, MD, PhD Kenji Aoki, MD, and Setsuo Kuraoka, MD, PhD Department of Thoracic and Cardiovascular Surgery, Mito Saiseikai General Hospital, Mito City, Japan

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 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; e-mail: [email protected].

0003-4975/06/$32.00 doi:10.1016/j.athoracsur.2005.05.086

Ann Thorac Surg 2006;81:1888 –90

CASE REPORT TATEBE ET AL INTERMEDIATE TYPE ATRIOVENTRICULAR SEPTAL DEFECT IN THE ELDERLY

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review of rare intermediate type atrioventricular septal defect in the elderly, and we discuss the surgical issues in this case. (Ann Thorac Surg 2006;81:1888 –90) © 2006 by The Society of Thoracic Surgeons

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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

FEATURE ARTICLES

trioventricular 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.

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.)

saphenous vein. The patient tolerated this procedure well. Postoperative echocardiography revealed no shunts, atrioventricular valve regurgitation, or stenosis of

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CASE REPORT PILLAI ET AL PSEUDO BUDD-CHIARI SYNDROME AFTER CARDIOPULMONARY BYPASS

the left ventricular outflow tract. The patient is currently well 3 years after surgery.

Comment

FEATURE ARTICLES

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. © 2006 by The Society of Thoracic Surgeons Published by Elsevier Inc

Ann Thorac Surg 2006;81:1890 –2

References 1. Bharati S, Lev M, McAllister HA Jr, Kirklin JW. Surgical anatomy of the atrioventricular valve in the intermediate type of common atrioventricular orifice. J Thorac Cardiovasc Surg 1980;79:884 –9. 2. 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 –92. 3. Tandon R, Moller JH, Edwards JE. Unusual longevity in persistent common atrioventricular canal. Circulation 1974; 50:619 –26. 4. Keith JD. Atrial septal defect: ostium secundum, ostium primum, and atrioventricularis communis (common AV canal). In: Keith JD, Rowe RD, Vlad P, eds. Heart diseases in infancy and childhood, 3rd ed. New York: Macmillan, 1978: 380 – 404. 5. Elliott MJ, Jacobs JP. Atrioventricular canal defects. In: Kaiser LR, Kron IL, Spray TL, eds. Mastery of cardiothoracic surgery. Philadelphia: Lippincott-Raven, 1998:742–58. 6. 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;118:642–7.

Heterotaxy Syndrome with Azygous Continuation-Causing Pseudo Budd-Chiari Syndrome After Cardiopulmonary Bypass Jain Bhaskara Pillai, FRCS (C-Th) UK, Jacques Kpodonu, MD, Catherine Yu, FRCPC, and Michael A. Borger, MD, PhD Divisions of Cardiac Surgery and Hepatology, Toronto General Hospital, Toronto, Ontario, Canada

Heterotaxy syndrome with interruption of the inferior vena cava and direct hepatic vein inflow into the right atrium has important implications for cardiac surgery. We report a case causing pseudo Budd-Chiari syndrome after cardiopulmonary bypass. (Ann Thorac Surg 2006;81:1890 –2) © 2006 by The Society of Thoracic Surgeons

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odern cardiac surgery pivots on the conduct of safe cardiopulmonary bypass. Any condition that compromises the extracorporeal circulation has due importance for the surgeon and the patient. We describe how an unrecognized congenital anomaly had significant implication for safe right atrial venous cannulation. A 48-year-old woman underwent routine coronary artery bypass surgery for unstable angina. A standard two-stage venous cannula was inserted into the right atrium with some resistance at the inferior caval orifice. Visual inspection of the inferior-cavoatrial region in the pericardial well and the superior vena cava seemed unremarkable. A

Accepted for publication May 24, 2005. Address correspondence to Dr Pillai, 17 Paxford Close, Vicars Ln, Benton, Newcastle upon Tyne, NE7 7PA United Kingdom; e-mail: jain_freeman@ hotmail.com.

0003-4975/06/$32.00 doi:10.1016/j.athoracsur.2005.05.090