• Title/Summary/Keyword: ALCAPA syndrome

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A 69-year-old Woman with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery: Surgical Repair Using a Trap-door Flap

  • Lim, Ju-Yong;Chung, Cheol-Hyun;Ma, Dae-Sung;Lee, Seung-Hyun
    • Journal of Chest Surgery
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    • v.44 no.5
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    • pp.358-360
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    • 2011
  • We present a patient with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), which was diagnosed and corrected in her 60 s. The patient is the oldest documented survivor of ALCAPA who underwent a surgical repair. ALCAPA should be corrected surgically to restore the dual coronary system at any age and this case shows that the surgical procedure may be performed safely even in an elderly patient.

An Alternative Surgical Technique for Repair of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery

  • Kim, Young-Su;Lee, Mina;Cho, Yang Hyun;Yang, Ji-Hyuk;Jun, Tae-Gook
    • Journal of Chest Surgery
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    • v.47 no.3
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    • pp.220-224
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    • 2014
  • Background: For the surgical management of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), there have been various techniques that reduce the tension and kinking of the coronary artery during reimplantation to the aorta. The aim of this study is to describe the results of our modified technique of coronary reimplantation for the treatment of ALCAPA. Methods: Between October 2003 and February 2011, seven patients underwent coronary reimplantation with the modified technique (tubing formation with the sinus wall of the pulmonary artery and trapdoor formation at the site of implantation in the aorta). The median follow-up duration was 52 months (range, 4 to 72 months). Clinical outcomes and serial echocardiographic data were reviewed. Results: There was no mortality. One patient had a small amount of cerebral hemorrhage postoperatively and improved without any sequelae. Another patient had left diaphragm palsy and underwent diaphragm plication. Follow-up echocardiogram showed that all patients had normal ventricular function without chamber enlargement. Conclusion: Our modified technique (tubing formation with the sinus wall of the pulmonary artery and trapdoor formation at the site of implantation in the aorta) demonstrated successful clinical outcomes. We conclude that this surgical technique can be a potential alternative for the treatment of ALCAPA.

Early Result of Surgical Management of the Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (관상동맥-폐동맥 이상 기시증에 대한 수술의 조기 결과)

  • Yoon Yoo Sang;Park Jeong Jun;Yun Tae Jin;Kim Young Hwue;Ko Jae Kon;Park In Sook;Seo Dong Man
    • Journal of Chest Surgery
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    • v.39 no.1 s.258
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    • pp.18-27
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    • 2006
  • Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. Material and Method: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. Result: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3$\∼$33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7$\∼$11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114$\pm$37 minutes, and the average aortic cross clamping time was 55$\pm$22 minutes. The average stay of intensive care unit was 5$\pm$3 days, the mean mechanical ventilator time was 38$\pm$45 hours and the hospital stay after operation was 12$\pm$5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2$\pm$ 10.3$\%$ to 60.5$\pm$ 15.8$\%$ within 1 month and to 59.8$\pm$13.9$\%$ within 1 year after operation, the SF (Shortening Fraction) increased from 23.6$\pm$4.7$\%$ to 38.6$\pm$8.4$\%$ within 1 month and to 37.4$\pm$7.9$\%$ within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8$\pm$25.6 mm/$m^{2}$ to 90.3$\pm$ 19.2 mm/$m^{2}$ within f month and to 79.3$\pm$ 15.8 mm/$m^{2}$ within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy Conclusion: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important. But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.

Surgical Treatment of Anomalous Origin of Coronary Artery from the Pulmonary Artery: Postoperative Changes of Ventricular Dimensions and Mitral Regurgitation (관상동맥-폐동맥 이상기시증(Anomalous Origin of Coronary Artery from Pulmonary Artery)의 수술적 치료: 중기 성적과 좌심실 및 승모판 기능의 변화 양상에 대한 연구)

  • Kang, Chang-Hyun;Kim, Woong-Han;Seo, Hong-Joo;Kim, Jae-Hyun;Lee, Cheul;Chang, Yoon-Hee;Hwang, Seong-Wook;Back, Man-Jong;Oh, Sam-Se;Na, Chan-Young;Han, Jae-Jin;Lee, Young-Tak;Kim, Chong-Whan
    • Journal of Chest Surgery
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    • v.37 no.1
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    • pp.19-26
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    • 2004
  • Background: The aims of this study are to verify the result of the surgical treatment of ALCAPA and to identify the postoperative changes of left ventricular dimensions and mitral regurgitation (MR), Material and Method: Fifteen patients operated on since 1985 were included in the study. The patients operated on before 1998 (n=9) showed heterogeneous properties with various surgical strategies and cardiopulmonary bypass techniques. However, six patients were operated on with the established surgical strategy since 1998; 1) Dual perfusion and dual cardioplegic solution delivery through ascending aorta and main pulmonary artery, 2) Coronary transfer by rolled-conduit made of pulmonary artery wall flap, and 3) Additional mitral valvular procedure was not peformed. Result: Median age of the study group was 6 months (1 month to 34 years). The operative methods were left subclavian artery to left coronary artery anastomosis in 1, simple ligation in 2, Takeuchi operation in 2, and coronary reimplantation in 10 patients. The mean follow up period was 5.5<5.8 years (2 months 14 years), There were one early death (6.7%) and one late death. Overall 5-year survival rate was 85.6$\pm$9.6%. The Z-value of left ventricular end-diastolic and end-systolic dimensions were 6.4$\pm$3.0 and 5.1 $\pm$3.6 preoperatively, and decreased to 1.7$\pm$ 1.9 and 0.8$\pm$ 1.6 in 3 months (p<0.05). Significant preoperative MR was identified in 6 patients (40%) and all the patients showed immediate improvement of MR within f month postoperatively. There were 3 cases of reoperation due to coronary anastomosis site stenosis and recurrence of MR. However, there was no mortality nor late reoperation in the patients operated on after 1998. Conclusion: The surgical treatment of ALCAPA showed favorable survival and early recovery of ventricular dimensions and mitral valvular function. Although long-term reintervention was required in some cases of earlier period, all the cases after 1998 showed excellent surgical outcome without long-term problem.