Asian Cardiovasc Thorac Ann 2003;11:80-82
© 2003 Asia Publishing EXchange Ltd
Pulmonary Origin of Circumflex Artery in Aortopulmonary Window
Chalam Mahadevan, FRCS1,
Shiraz Kareem, MBBS1,
Vikram Jitendra, FRCS1,
Natarajan Sivakadaksham, MD1,
MP Naresh Kumar, FRCS1
1 Harvey Heart Hospitals Ltd., Nungambakkam, Chennai, India
For reprint information contact: MP Naresh Kumar FRCS Tel: 91 44 823 4209 Fax: 91 44 822 1355 email: bshivv{at}yahoo.com Harvey Heart Hospitals Ltd. 20, Pycrofts Garden Road, Nungambakkam, Chennai 600 006, India.
 |
ABSTRACT
|
|---|
An aortopulmonary window associated with an anomalous left circumflex coronary artery arising from the adjoining pulmonary sinus in a 4-kg baby boy was successfully repaired by re-routing with a pericardial patch.
|
INTRODUCTION
|
|---|
Aortopulmonary (AP) window is a rare congenital malformation of the conotruncal septum. Severe pulmonary hypertension and failure lead to a poor outcome if not corrected very early in life. It is associated with patent ductus arteriosus in 10%–15% of cases.1 Less common associated lesions are ventricular septal defect, aortic origin of the right pulmonary artery, aortic arch interruption, coarctation of the aorta, right aortic arch, and tetralogy of Fallot.2 Anomalous origin of a coronary artery from the pulmonary artery has been reported in association with this defect, but such cases are very rare.3 Possible association of this anomaly must be kept in mind during closure of the defect as leaving the anomalous coronary artery on the pulmonary artery side of the patch could lead to coronary steal and myocardial ischemia.4
|
CASE REPORT
|
|---|
Echocardiography revealed type 1 AP window with patent ductus arteriosus, atrial septal defect with a left-to-right shunt, and severe pulmonary arterial hypertension in a 5-month-old baby boy weighing 4 kg.5 His right atrial and right ventricular chambers were dilated. Electrocardiographic, hematologic, and biochemical parameters were normal. Surgery was performed under general anesthesia with endotracheal intubation. The electrocardiogram, right internal jugular vein and radial artery pressures, and oral and rectal temperatures were monitored. After a median sternotomy and pericardiotomy, a large type 1 AP window was identified at the level of the aortic sinuses, with normally related great vessels. The ductus arteriosus was dissected and ligated. Under cardiopulmonary bypass and antegrade cold blood cardioplegia, both pulmonary arteries were snared and the aorta was crossclamped. Deep-hypothermic circulatory arrest was employed briefly. Via a vertical aortotomy, an anomalous coronary ostium was seen on the pulmonary sinus adjacent to the AP window, which proved to be the left circumflex coronary artery (Figure 1
). The left anterior descending and right coronary arteries arose from the aortic sinuses. A bovine pericardial patch was used to close the AP window and at the same time, to re-route the anomalous circumflex origin to the aortic side of the defect. This was achieved by suturing the inferior margin of the patch near the root of the pulmonary sinus adjacent to the anomalous circumflex (Figure 2). There was no right ventricular outflow tract gradient or leakage across the patch. A right atriotomy was performed and the ostium secundum atrial septal defect was closed directly. Sinus rhythm resumed after rewarming and reperfusion, and the patient was weaned off cardiopulmonary bypass without problems. Postoperatively, the pulmonary arterial pressure was reduced to less than half of the systemic pressure. The child was extubated after 8 hours, and discharged home on the 6th postoperative day.
View larger version (45K):
[in this window]
[in a new window]
|
Figure 1. Aortopulmonary (AP) window with anomalous origin of the left circumflex (Cx) from the pulmonary artery (PA). LAD = left anterior descending artery, RCA = right coronary artery.
|
|
View larger version (45K):
[in this window]
[in a new window]
|
Figure 2. Pericardial patch closure of aortopulmonary window with re-routing of the left circumflex (Cx) artery to the aortic sinus. LAD = left anterior descending artery, PA = pulmonary artery.
|
|
|
DISCUSSION
|
|---|
Anomalous origin of a coronary artery from the pulmonary artery, usually the circumflex and occasionally the right coronary, is rarely associated with AP window.3,6 In the developmental phase, coronary arteries arise as angiogenic buds from the developing truncus, and in the process of conotruncal septation, they remain on the aortic side. When the septum is maldeveloped leading to an AP window, a coronary artery can remain on the pulmonary side. Identification of this anomaly, either preoperatively or intraoperatively, is vital. Surgical septation of AP window leaving the coronary artery arising from the pulmonary side can lead to a left-to-right shunt, coronary steal, and myocardial ischemia. Delayed presentation of ischemia has been reported due to septation of an AP window without recognizing the anomalous origin of a coronary artery on which grafting had been carried out 14 years after the correction.4
Many methods of correction of an anomalous coronary artery have been reported, based on the anatomical position of its origin.7,8 Ligation of the origin and bypassing the anomalous vessel with a venous or arterial graft has been described.8 Re-routing the anomalous artery through the AP window into the aorta is the preferred method if anatomy permits. In our case, a pericardial patch was used as a baffle to re-route the anomalous circumflex artery to the aorta. As the AP window was large and the origin of the anomalous circumflex was adjacent to the defect, this was easily achieved by suturing the inferior margin of the patch along the floor of the pulmonary sinus.
|
REFERENCES
|
|---|
- Friedman W, Silverman N. In: Congenital heart disease in infancy and childhood in heart disease: a textbook of cardiovascular medicine, 6th ed. Braunwald E, Zipes DP, Libby P, editors. 2001:1505–91.
- Morell VO, Feccia M, Cullen S, Elliott MJ. Anomalous coronary artery with tetralogy of Fallot and aortopulmonary window. Ann Thorac Surg
1998;66:1403–5.[Abstract/Free Full Text]
- D’Souza VJ, Chen MY. Anomalous origin of the coronary artery in association with aorticopulmonary window. Pediatr Cardiol
1996;17:316–8.[Medline]
- Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest
1994;106:1920–2.[Abstract/Free Full Text]
- Mori K, Ando M, Takao A, Ishikawa S, Imai Y. Distal type of aortopulmonary window: report of 4 cases. Br Heart J
1978;40:687–9.
- Brouwer MH, Beaufort-Krol GC, Talsma MD. Aortopulmonary window associated with an anomalous origin of the right coronary artery. Int J Cardiol
1990;28:384–6.[Medline]
- Raanani E, Abramov D, Abramov Y, Birk E, Vidne BA. Individual anatomy demands various techniques in correction of an anomalous origin of the left coronary artery in the pulmonary artery. Thorac Cardiovasc Surg
1995;43:99–102.[Medline]
- Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katohgi T, Yozu R, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg
1979;78:7–11.[Abstract]
Antonio F Corno, MD, FECTS, FRCS2
2 Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
|
INVITED COMMENTARY
|
|---|
This Case Study is a timely opportunity to increase the awareness of readers, particularly those less frequently involved in the surgical treatment of infants with congenital heart defects, on this rare but potentially dangerous association: anomalous coronary artery in the presence of aortopulmonary window. Although this particular association is quite rare, more than 10 years ago, we reported 10 cases found in the literature, with an incidence of 5.1% and very high surgical mortality (4/9; 44%).1 Several other cases have been reported since. Therefore, everyone involved in the diagnosis and surgical treatment of congenital heart defects should have an adequate threshold for the identification of this potentially dangerous association.
In particular, the surgeon approaching a patient with aortopulmonary window, without a clear preoperative identification of the coronary artery anatomy with regard to origin and course, must keep in mind the following points: very careful dissection has to be performed in the area surrounding the aortopulmonary defect, to avoid any damage to the anomalous coronary artery; both pulmonary arteries need to be occluded at the start of cardiopulmonary bypass, to perfuse both coronary arteries; the aortic crossclamp needs to be placed well distal to the defect, to administer the cardioplegic solution to both coronary arteries; the aortic (or pulmonary artery) incision must allow precise identification of the origin of the anomalous coronary artery; patch closure of the defect through a pulmonary artery incision must ensure precision, safety, and freedom from distortion of the origin of the anomalous coronary artery.
|
REFERENCES
|
|---|
- 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.[Medline]
TOP
ABSTRACT
INTRODUCTION
