Ann Thorac Surg 2004;78:1451-1453
© 2004 The Society of Thoracic Surgeons
Case report
Bland-White-Garland Syndrome in a 39-Year-Old Mother
Micha Maeder, MDa,b,*,
Paul R. Vogt, MD, FETCSc,
Peter Ammann, MDa,
Hans Rickli, MDa
a Division of Cardiology, Hospital of St. Gallen, St. Gallen, Switzerland,
b Department of Medicine, Hospital of St. Gallen, St. Gallen, Switzerland,
c Division of Surgery, University Hospital of Zürich, Zürich, Switzerland
Accepted for publication July 8, 2003.
* Address reprint requests to Dr Maeder, Division of Cardiology, Department of Medicine, Hospital of St. Gallen, Rorschacherstrasse, CH-9007 St. Gallen, Switzerland
micha.maeder{at}kssg.ch
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Abstract
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We report the case of a 39-year-old mother of 3 children presenting with exertion fatigue. Coronary angiography revealed an anomalous left coronary artery arising from the pulmonary artery. The patient underwent an operation with the direct implantation of the left coronary artery into the ascending aorta. This less symptomatic course stands in contrast to the high risk for sudden death in untreated adults.
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Introduction
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The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is the most common congenital coronary artery anomaly, occurring in 0.26% of patients with congenital heart disease. Ischemia of the left ventricular myocardium, normally supplied by the left coronary artery, occurs as blood flow in the left coronary artery is directed toward the pulmonary trunk after birth. The perfusion of the anterior wall is therefore dependent on the development of collaterals from the right coronary artery. Up to 90% of patients die during the first year of life because of left ventricular failure. The chronic phase of this malformation is characterized by a considerable risk for death due to chronic ischemic congestive heart failure or sudden death. Overall, the life expectancy of patients with unoperated ALCAPA is poor, and of those surviving this condition, 80% present moderately to severely symptomatic heart failure [1, 2].
A 39-year-old previously healthy mother of 3 children was referred to our hospital because of progressive exertion dyspnea and fatigue. Her pregnancies and deliveries had been normal. Systolic and diastolic murmurs were known for years. The patient complained of shortness of breath, palpitations, and chest pain worsening during the past year. Physical examination revealed a prominent holosystolic murmur (grade 3/6) and a mild diastolic decrescendo murmur (grade 2/6) at the lower left sternal border. There were no signs of congestive heart failure. Echocardiography showed sinus rhythm with ST-segment depression and negative T waves in leads I and aVL. An exercise test revealed very limited performance but was otherwise normal. Transthoracic echocardiography demonstrated a huge tortuous right coronary artery originating from the right aortic sinus and a normal-sized left ventricle, with an ejection fraction of 60%. Minor prolapse of the posterior leaflet of the mitral valve with minimal mitral regurgitation was detected. Coronary angiography proved a 9-mm tortuous right coronary artery, marked collateral filling of the left coronary system, and entry of the contrast agent from the left main trunk into the pulmonary artery (Fig 1).
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Fig 1. Coronary angiography showing a dilatated and tortuous right coronary artery (RCA) with a collateral network filling the left anterior descending (LAD) artery. Note the retrograde entry of contrast agent from the LAD artery into the pulmonary trunk.
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The diagnosis of an anomalous left coronary artery originating from the pulmonary artery was made. Right heart catheterization proved a left-to-right shunt, with the ratio of pulmonary blood to systemic blood flow (Qp/Qs) measuring 1.5:1. The patient underwent an operation with the direct implantation of the left coronary artery into the ascending aorta (Fig 2). The defect, which occurred after the excision of the left coronary artery ostium from the pulmonary trunk, was reconstructed, with a vascular prosthesis being anastomosed above the pulmonary valve commissures. Postoperatively, intermittent atrial fibrillation and postcardiotomy syndrome occurred; otherwise, the clinical course was uneventful. Three years after the procedure, the patient's dyspnea had improved by one New York Heart Association class. Echocardiography revealed a normal left ventricular ejection fraction.
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Comment
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Bland, White, and Garland first reported the association of ALCAPA with a hypertrophied left ventricle and attacks of dyspnea, pallor, and profuse sweating in a boy who died at the age of 3.5 months [1, 2].
During fetal life, ALCAPA does not cause hemodynamic problems, because systemic and pulmonary pressures are equal and provide antegrade flow in both the right and the left coronary arteries. However, symptoms occur as pulmonary arterial pressure diminishes after birth and flow in the left coronary artery reverses [1, 3]. Insufficient collateral flow from the right coronary artery and a coronary steal from the left coronary artery into the pulmonary trunk result in malperfusion of the left ventricular myocardium, with the right coronary artery becoming large and tortuous. Previous existence or the rapid development of collateral vessels between the right and the left coronary arteries may prevent ischemia [1, 3]. These children will survive to adolescence or adulthood, whereas about 80% to 90% will die during the first year of life from heart failure or sudden cardiac death if surgical correction is not performed [3]. Survival to adulthood is unusual, and patients older than 50 years are very rare [4].
A systolic murmur of ischemic mitral regurgitation is present in almost 70% of cases [3]. Sometimes, the murmur is continuous as a sign of collateral flow [5]. Echocardiography may reveal previous anterolateral myocardial infarction and signs of left ventricular hypertrophy, with or without a partial left bundle branch block [1, 5]. Echocardiography may reveal the presence of a dilatated and tortuous right coronary artery in the parasternal short axis as well as difficulties in or the impossibility of finding the left coronary artery. The identification of retrograde diastolic blood flow of an abnormal vessel that originates from the wall of the pulmonary trunk by color Doppler flow mapping identifies ALCAPA [5], confirmed by angiography.
Our patient did not have symptoms until the age of 38 years and had 3 uncomplicated pregnancies and deliveries. Nightingale similarly reported 2 patients with uneventful pregnancies [6]. We suppose that these women were asymptomatic because of an optimal balance between sufficient collateral flow from the right to the left coronary arteries and a minimal coronary steal from the pulmonary artery.
Even in adults with only minor symptoms, surgical intervention is recommended because of the high risk for sudden death [4]. Whenever possible, a 2-coronary system will be established by the direct reimplantation of the left coronary artery into the ascending aorta. Alternative options include the ligation of the left coronary artery ostium combined with aortocoronary bypass grafting using venous grafts, the internal mammary artery, or the radial artery [4, 7]. Jin demonstrated complete recovery of left ventricular function after the direct reimplantation of the left coronary artery into the aorta within months in most infants, as well as a reduction of left ventricular hypertrophy and left ventricular dilatation. These changes occurred much faster in infants than in older children [8] and might be even slower or incomplete in adults. Despite improvement or even normalization of left ventricular function after surgical intervention, limitations of myocardial blood flow persist even years afterward [9]. The present case illustrates that ALCAPA can be asymptomatic over a long time, even during pregnancy. Nevertheless, the risk for sudden death warrants surgical intervention, even though patients may be asymptomatic.
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References
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- Perlof JK. Anomalous origin of the left coronary artery from the pulmonary trunk. Perlof JK. The clinical recognition of congenital heart disease. 4th ed. Philadelphia: W.B. Saunders; 1994. p. 546–561
- Moodie DS, Fyfe D, Gill CG, et al. Anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) in adult patients: long-term follow-up after surgery. Am Heart J. 1983;106:381–388[Medline]
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- Nightingale AK, Burrell CJ, Marshall AJ. Anomalous origin of the left coronary artery from the pulmonary artery: natural history and normal pregnancies. Heart. 1998;80:629–631[Abstract/Free Full Text]
- Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg. 1992;103:1049–1058[Abstract]
- Jin Z, Berger F, Uhlemann F, et al. Improvement in left ventricular dysfunction after aortic reimplantation in 11 consecutive pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery. Eur Heart J. 1994;15:1044–1049[Abstract/Free Full Text]
- Seguchi M, Nakanishi T, Nakazawa M, et al. Myocardial perfusion after aortic implantation for anomalous origin of the left coronary artery from the pulmonary artery. Eur Heart J. 1990;11:213–218[Abstract/Free Full Text]
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Abstract
Introduction
