Ap window and anomalous origin of right coronary artery from the window

Ap window and anomalous origin of right coronary artery from the window

Ann Thorac Surg 1999;68:557–9 CASE REPORT IZUMOTO ET AL COMPLEX AP WINDOW 557 7. John S, Bashi VV, John CN, Ravikumar E, Kumar HP, Rao S. Use of as...

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Ann Thorac Surg 1999;68:557–9

CASE REPORT IZUMOTO ET AL COMPLEX AP WINDOW

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7. John S, Bashi VV, John CN, Ravikumar E, Kumar HP, Rao S. Use of aspirin as the sole antiplatelet agent following prosthetic valve replacement in Rheumatic heart disease. Ind Heart J 1994;46:341– 4. 8. John S, John C, Saha K, Bashi VV, Ravikumar E. A quarter century experience with mitral valve replacement. Asian Cardiovasc Thorac Ann 1996;4:201–5.

AP Window and Anomalous Origin of Right Coronary Artery From the Window Fig 1. Recent two-dimensional echocardiogram demonstrating the 2SE prosthesis in place.

been low in our patient population. Our patients who are at lower risk of thromboembolic phenomenon have shown promise with low-dose aspirin as the sole antiplatelet agent that appears highly protective against thromboembolic phenomena [7]. It is imperative that patients will have to be in normal sinus rhythm. Over the last year, this patient has been advised to take aspirin, 100 mg daily, as the sole antiplatelet agent to counter thrombogenesis. The antiplatelet effect of aspirin results from irreversible acetylation of cyclo-oxygenase and inhibition of thromboxane A2 production. In a quarter century of experience with 1,129 consecutive mitral valve replacements reported from our center using the SE prosthesis (model 6120) who were monitored with prothrombin time values with coumadin as the anticoagulant, the linearized rates of thromboembolism and bleed were 0.5% and 0.3% per year, respectively [8]. This series was not typical of the western world. The insertion of a biological valve would presumably be favored in patients with Ebstein’s malformation but they lack acceptable durability. To our knowledge, this case report represents one of the longest survivors after SE valve prosthesis in the tricuspid position for Ebstein’s anomaly.

References 1. Kirklin JW, Barratt-Boyes BG. Cardiac Surgery, Vol. 2, 2nd ed. New York: Churchill Livingstone Inc, 1993:1105–30. 2. Danielson GK, Driscoll DJ, Mair DG, Warner CA, Oliver WC. Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg 1992;104:1195–202. 3. Barnard CN, Schrire Y. Surgical correction of Ebstein’s malformation with a prosthetic tricuspid valve. Surgery 1963;54: 302– 8. 4. Hunter SW, Lillehei CW. Ebstein’s malformation of the tricuspid valve. A study of a case, together with suggestions of a new form of surgical therapy. Chest 1958;33:297–304. 5. Hardy KL, May IA, Webster CA, Kimball KG. Ebstein’s anomaly: a functional concept and successful definitive repair. J Thorac Cardiovasc Surg 1964;48:927– 40. 6. Starnes VA, Pitlick PT, Bernstein D, Griffith MI, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg 1991;101: 1082–7. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc

Hiroshi Izumoto, MD, Kazuaki Ishihara, MD, Yutaka Fujii, MD, Kotaro Oyama, MD, and Kohei Kawazoe, MD Departments of Cardiovascular Surgery and Pediatrics, Iwate Medical University Memorial Heart Center, Iwate Medical University, Iwate, Japan

Aortopulmonary window (APW) is a rare malformation. We recently operated on a child with APW, ventricular septal defect, right aortic arch, and anomalous right coronary artery from the APW. This patient also had a chromosomal abnormality. He underwent the repair of this complex lesion in a staged operation. (Ann Thorac Surg 1999;68:557–9) © 1999 by The Society of Thoracic Surgeons

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ortopulmonary window (APW) is a rare malformation and its incidence is reportedly about 0.15% in patients with congenital heart disease [1]. We recently operated on a child with a preoperative diagnosis of ventricular septal defect (VSD), large patent ductus arteriosus (PDA), pulmonary hypertension, and right aortic arch (RAA). During surgery it was found that this child had APW instead of PDA and anomalous origin of the right coronary artery (RCA) from the APW. This patient underwent the repair of both APW and anomalous origin of the RCA, and repair of the VSD in a second operation. In October 1997, a 20-month-old boy was admitted to our institution for evaluation of his congenital heart disease. Immediately after birth, he was diagnosed with laryngomalacia, VSD, atrial septal defect (ASD), and RAA by echocardiography. Since then a pediatrician has followed his condition. During the follow-up period, he experienced failure to thrive and multiple upper respiratory infectious episodes. His height at admission was 77 cm and he weighed 8700 g. A chromosome study was performed and it revealed that the patient’s father had a translocation between chromosomes 3 and 7, and abnormally derived chromosome 3 had been transmitted to the patient [46, XY, der(3) t (3;7) (p25; p 15. 3) pat. ish 22q11.2

Accepted for publication Dec 31, 1998. Address reprint requests to Dr Izumoto, Department of Cardiac Surgery, Iwate Medical University Memorial Heart Center, Iwate Medical University, 1-2-1 Chuodori, Morioka 020-8505, Iwate, Japan.

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Ann Thorac Surg 1999;68:557–9

Postoperatively, the patient’s course has been uneventful except for an acute hepatic injury and a low-grade fever of unknown origin. He was discharged from the hospital a month after operation and 62 days later he underwent successful repair of the malaligned VSD. He is now in New York Heart Association class I, 12 months after initial surgery.

Comment

Fig 1. Intraoperative findings. The arrow indicates the ostium of right coronary artery (RCA) in the aortopulmonary window itself. Lt PA ⫽ left pulmonary artery, Rt PA ⫽ right pulmonary artery.

(Tuplet 1x2)]. The patient’s blood pressure was 88/49 mm Hg, with a pulse rate of 120. There was a grade IV/VI systolic heart murmur at the left sternal border. Echocardiography demonstrated that he had PDA, VSD, persistent left superior vena cava (PLSVC), and RAA. Cardiac catheterization revealed that the Qp/Qs ratio was 2.5, Rp/Rs ratio 1.17, and Pp/Ps ratio 1. Under oxygen administration (70%), the Qp/Qs ratio reached 3.6 and Rp/Rs ratio 0.93. Pulmonary resistance decreased from 8.1 to 5.2 units. Aortography showed that he had a large PDA. With the diagnosis of large PDA in addition to VSD, PLSVC, RAA, and pulmonary hypertension, isolated PDA division through a median sternotomy was electively planned for November 1997. During surgery, a median sternotomy was chosen as the surgical approach for anatomical ease. A wide communication between the main pulmonary artery and the left side of the ascending aorta was found. The RCA appeared to arise from the pulmonary artery. The patient was cannulated with an ascending aortic and biatrial cannulas and placed on cardiopulmonary bypass. The patient was cooled to 18°C rectally and ascending aorta was cross-clamped distally to APW. The crystalloid cardioplegic solution was infused while the pulmonary artery was clamped. A longitudinal opening was made in the pulmonary artery and it was found that the pulmonary arteriotomy was not in the pulmonary artery but in the large APW and the RCA, originating from the APW (Fig 1). The size of the APW was 8 mm on both the aortic and pulmonary sides. A small transverse aortotomy was made and it was determined that the left coronary artery arose from the left coronary sinus. Then, the pulmonary arterial side opening was patched to direct arterial blood to RCA, the patient was rewarmed, and the operation was completed with postbypass modified ultrafiltration. At the end of the procedure, the ascending aortic pressure was 75/33 (mean 50) mm Hg, whereas the right pulmonary arterial pressure was 46/20 (mean 33) mm Hg.

Anomalous origin of the RCA may often complicate operative procedures in congenital cardiac surgery. In the present case, the preoperative diagnosis was VSD, RAA, and large PDA. During surgery, however, it was found that the patient had a large APW of Richardson type I [2] and also that the RCA arose from the APW itself. The diagnosis of PDA was incorrect. With uncertainty concerning the origin of the left coronary artery, we decided to identify the left coronary ostium. There have been a couple of case reports documenting anomalous origin of the left coronary artery associated with APW [3, 4]. There are three technical points that need clarification in surgical management of patients with APW and anomalous origin of RCA from the APW: (1) In administering cardioplegic solution, the cross-clamp should be placed distally to APW and the pulmonary artery should be occluded to prevent the steel of cardioplegic solution. In this specific case, because the origin of left coronary artery was not clearly identified, we employed profound hypothermia in case the left coronary artery transfer is indicated. As an alternative, retrograde cardioplegia could have been employed. (2) Regarding the choice of repair in patients with APW and anomalous origin of RCA from APW, the anatomical repair without [5, 6], or with a patch as in this case, seems a viable repair. (3) The operative treatment in this case could have been completed in a single operation. However, we staged the procedure because of the prolonged cross-clamp time (40 minutes) and preoperative severe pulmonary hypertension. Preoperative angiographic diagnosis of anomalous origin of the RCA is very difficult because both great arteries fill simultaneously in patients with APW [5]. Therefore surgeons should be prepared to cope with coronary artery anomaly in the operative management of APW, although the incidence of such a combination seems very rare. Conotruncal cardiac anomalies such as occurred in the present case are often associated with chromosome 22q11 deletion. In this patient, chromosome 22q11 was not deleted but he had received an abnormally derived chromosome 3. In summary, we treated a child with complex APW with anomalous origin of the RCA from the APW and the patient underwent a successful two-stage operation. We believe this is the first report of such a combination in association with a chromosomal abnormality in the literature.

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CASE REPORT IMOTO ET AL NORWOOD PROCEDURE

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References 1. Kirklin JW, Barratt-Boyes BG. Aortopulmonary window. In: Kirklin JW, Barratt-Boyes BG, eds. Cardiac surgery 1st Ed. New York: Churchill Livingstone, 1986:934. 2. Richardson JV, Doty DB, Rossi NP, Ehrenhaft JT. The spectrum of anomalies of aortopulmonary septation. J Thorac Cardiovasc Surg 1979;78:21–7. 3. Bourlon F, Kreitmann P, Jourdan J, et al. Anomalous origin of left coronary artery with aortopulmonary window. Thorac Cardiovasc Surg 1981;29:91–2. 4. Corno A, Pierli C, Lisi G, Biagioli B, Grossi A. Anomalous origin of the left coronary artery from an aortopulmonary window. J Thorac Cardiovasc Surg 1988;96:669–71. 5. Luis SV, Ashraf MH, Gula G, Radley-Smith R, Yacoub M. Anomalous origin of the right coronary artery with aortopulmonary window; functional and surgical considerations. Thorax 1980;35:446– 8. 6. Burroughs JT, Schumutzer KJ, Linder F, Neuhans G. Anomalous origin of the right coronary artery with aorticopulmonary window and ventricular septal defect. J Cardiovasc Surg 1962;3:142– 8.

Norwood Procedure Without Circulatory Arrest Yutaka Imoto, MD, Hideaki Kado, MD, Yuichi Shiokawa, MD, Kohji Fukae, MD, and Hisataka Yasui, MD Cardiovascular Surgery, Fukuoka Children’s Hospital and Division of Cardiovascular Surgery, Kyushu University, Fukuoka, Japan

In the Norwood procedure for hypoplastic left heart yndrome, the distal descending thoracic aorta was cannulated just superior to the diaphragm through median sternotomy. In combination with cerebral perfusion through the graft anastomosed to the innominate artery, which was used as a systemic-to-pulmonary shunt later, this technique enabled us to completely avoid circulatory arrest and deep hypothermia throughout the operation. (Ann Thorac Surg 1999;68:559 – 61) © 1999 by The Society of Thoracic Surgeons

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irculatory arrest with deep hypothermia is commonly used during the aortic arch reconstruction in hypoplastic left heart syndrome (HLHS). Although it is a useful technique, potential risks of complications such as neurological damage [1] and renal failure [2] cannot be ignored. We introduced a new cardiopulmonary bypass technique using arterial cannulation of the distal descending thoracic aorta through median sternotomy combined with cerebral perfusion through the innominate artery to avoid circulatory arrest. Details of this technique are described in this report. The patient was a 15-day-old girl with a body weight of 2.550 g. She had aortic atresia and mitral stenosis with Accepted for publication Jan 4, 1999. Address reprint requests to Dr Imoto, Cardiovascular Surgery, Fukuoka Children’s Hospital, 2-5-1 Tojinmachi, Chuo-ku, Fukuoka 810-0063, Japan.

© 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc

Fig 1. Intraoperative photograph of the cannula inserted into the descending aorta. Des Ao cannula ⫽ arterial cannula in the descending aorta; G ⫽ graft sewn to the innominate artery. The heart is slightly elevated.

hypoplastic left ventricle. Outer diameters of the ascending and descending aortas were 3.5 mm and 6.0 mm, respectively. After median sternotomy, expandedpolytetrafluoroethylene (E-PTFE) graft, 3.5 mm in diameter, was sewn to the innominate artery. Cardiopulmonary bypass was commenced with bicaval venous cannulas and with the graft at the innominate artery as an arterial line. Then the heart was gently elevated and a small incision was made in the posterior pericardium, to the left of the inferior vena cava (Fig 1). The descending aorta was identified easily and localized dissection was carried out to expose it clearly. An angled, metal-tipped cannula (2.1 mm in outer diameter, Japan Medical Supply Co, Hiroshima, Japan) was inserted through a purse string suture of 4-0 polyester placed in the aorta. Thereafter, extracorporeal circulation (ECC) with single pump and double arterial lines was established (Fig 2A). Pumpflows were maintained at 180 mL/min/kg, and the rectal temperature was lowered to 30.4°C. After cross-clamping the distal arch, the left subclavian artery and the descending aorta, the ductus arteriosus was ligated and coarctectomy was performed. The aortic arch and the descending aorta were then anastomosed with continuous 7-0 polydioxanone suture in an end-to-end fashion. The atrial septal defect was enlarged through the right atriotomy. The pulmonary trunk was transected and the distal end was closed. After clamping all the arch vessels 0003-4975/99/$20.00 PII S0003-4975(99)00590-1