Ann Thorac Surg 2005;79:2151-2153
© 2005 The Society of Thoracic Surgeons
Case report
Beating Heart Off-Pump Myocardial Revascularization in an Infant
Thomas Walther, MD, PhDa,*,
Ingo Dähnert, MDb,
Herbert Kiefer, MDc,
Volkmar Falk, MD, PhDa,
Martin Kostelka, MD, PhDa,
Friedrich W. Mohr, MD, PhDa
a Klinik für Herzchirurgie, Herzzentrum, Universität Leipzig, Leipzig, Germany
b Klinik für Kinderkardiologie, Herzzentrum, Universität Leipzig, Leipzig, Germany
c Klinik für Anästhesie, Herzzentrum, Universität Leipzig, Leipzig, Germany
Accepted for publication December 2, 2003.
* Address reprint requests to Dr Walther, Klinik für Herzchirurgie, Herzzentrum, Universität Leipzig, Strümpellstr 39, 04289 Leipzig, Germany (E-mail: walt{at}medizin.uni-leipzig.de).
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Abstract
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A 6-week-old patient weighing 4 kg was admitted for surgical correction of anomalous left coronary artery from the pulmonary artery. Reimplantation of the coronary artery with cardiopulmonary bypass was impossible because the patient also had hereditary spherocytosis, which put him at risk for hemolytic anemia. Thus, closure of the left main stem and left internal mammary artery-left anterior descending coronary artery bypass grafting were performed successfully with the heart beating.
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Introduction
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Anomalous left coronary artery from the pulmonary artery is a serious diagnosis associated with coronary steal and ischemia as well as progressive deterioration in left ventricular function. Standard therapy consists of coronary artery reimplantation or creation of a transpulmonary artery baffle with cardiopulmonary bypass (CPB). Eventually, postoperative circulatory assistance is required. However, in patients who also have hereditary spherocytosis, CPB is contraindicated because of the risk of severe hemolysis leading to multiple-organ failure. We present an alternative approach.
A 6-week-old patient weighing 4 kg, and measuring 55 cm long with hereditary spherocytosis was admitted because of a cardiac malformation. The electrocardiogram revealed myocardial ischemia with a deep Q wave in leads I, II, and aVl and ST segment elevation in V5 and V6. Echocardiography showed a severe decrease in left ventricular function (ejection fraction, 0.21) and moderate to severe (2–3) mitral incompetence. At cardiac catheterization, the diagnosis of anomalous coronary artery from the pulmonary artery with the left main stem originating posteriorly from the main pulmonary artery was confirmed.
Operation was performed through a median sternotomy. Myocardial function was severely depressed, and the heart was enlarged. After careful dissection, the left main stem was closed using two titanium clips. The left internal mammary artery with an internal diameter of less than 1 mm was dissected as a pedicle. After heparinization (100 IU), the proximal left anterior descending coronary artery was exposed using a stay suture and with an Octopus 4 stabilizer applying direct pressure and suction (Fig 1). The anastomosis was performed using interrupted sutures of 8–0 Prolene. Postoperative flow measurement was 18 mL/min, and myocardial function was slightly improved. Primary chest closure was feasible.
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Fig 1. Exposure of proximal left anterior descending coronary artery (LAD) using an Octopus stabilizer. The view is from the head of the patient caudally, and the left side of the patient is to the left. The two tips of the stabilizer are beside the most proximal part of the LAD; the white base of the stabilizer is just above the apex of the heart.
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With moderate inotropic support, the patient was transferred to the pediatric intensive care unit. Extubation was performed after 11 hours. There was a regression of preexisting ST-segment elevation. Inotropic agents were withdrawn gradually, and therapy with an angiotensin-converting enzyme inhibitor was initiated. Echocardiography after 10 days revealed improvement in global left ventricular function (ejection fraction, 0.35), no more akinsis of the left ventricular wall, and only moderate residual (1°) mitral incompetence. Control angiography revealed an open graft with good perfusion of the left anterior descending coronary artery as well as marked improvement in myocardial function (Fig 2). The infant had an uneventful further recovery and was discharged on postoperative day 15.
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Fig 2. Postoperative angiogram, left anterior oblique 90-degree view showing good left internal mammary artery-left anterior descending coronary artery perfusion with satisfactory filling of the septal branches.
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Comment
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Anomalous left coronary artery from the pulmonary artery in combination with hereditary spherocytosis is an extremely rare condition. Standard corrective surgical therapy cannot be performed, as the use of CPB is contraindicated. Only 11 patients (aged 1.3 to 67 years; median age, 16 years) with hereditary spherocytosis and undergoing an open heart operation with CPD have been described in the literature [1–4]. Seven of them had undergone a previous splenectomy when they were older than 5 years.
Preoperatively we discussed whether corrective surgical intervention was feasible in our patient, but we judged the potential risk of hemolytic crisis to be too high and decided on the alternative approach presented. Left internal mammary artery-left anterior descending coronary artery bypass grafting has been performed successfully in 3 children with anomalous left coronary artery from the pulmonary artery using CPB [5]. Thus, although not the current standard, left internal mammary artery-left anterior descending coronary artery bypass is a legitimate approach to treat patients with anomalous left coronary artery from the pulmonary artery. Furthermore, we did not have any other option, as the use of CPB was clearly contraindicated in our patient. Left anterior descending coronary artery ligation alone seems to be acceptable only as an emergency procedure. We are not aware of other reports of successful beating-heart off-pump myocardial revascularization in an infant with a body weight of 4 kg. This operation can be performed successfully even in very young patients.
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References
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- Aoyagi S, Kawano H, Tomoeda H, Hiratsuka R, Kawara T. Open heart operation in a patient with hereditary spherocytosisa case report. Ann Thorac Cardiovasc Surg 2001;7:375-377.[Medline]
- De Leval MR, Taswell HF, Bowie EJW, Danielson GK. Open heart surgery in patients with inherited hemoglobinopathies, red cell dyscrasias, and coagulopathies Arch Surg 1974;109:618-622.[Medline]
- Gayyed NL, Bouboulis N, Holden MP. Open heart operation in patients suffering from hereditary spherocytosis Ann Thorac Surg 1993;55:1497-1500.[Abstract/Free Full Text]
- Dal A, Kumar RS. Open heart surgery in presence of hereditary spherocytosis J Cardiovasc Surg (Torino) 1995;36:447-448.[Medline]
- Brackenbury E, Gardiner H, Chan K, Hickey M. Internal mammary artery to coronary artery bypass in paediatric cardiac surgery Eur J Cardio-thorac Surg 1998;14:639-642.[Abstract/Free Full Text]
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Abstract
Introduction
