An asymptomatic 16-year-old girl with anomalous left coronary artery from pulmonary artery (Bland-Withe-Garland syndrome)

Introduction

Anomalous origin of the left coronary artery from the pulmonary artery (anomalous left coronary artery from pulmonary artery [ALCAPA] or Bland-White-Garland syndrome) is a rare congenital disease. It is present in 1 out of 300 000 live-born children and constitutes 0.24% to 0.46% of congenital heart diseases [1-3]. Untreated disease leads to death in over 90% of children before the first year of age. Adult patients with this disease are very rarely found and asymptomatic patients are completely unique [3]. For the last 10 years diagnoses of ALCAPA in adult patients have been made with the use of multi-slice computed tomography (MSCT) [3, 4].

Case report

We present a case of a 16-year-old girl referred to our hospital from the Paediatric Cardiology Outpatient Clinic for computed tomography of the heart. The reason for referral was the unclear picture of coronary arteries found on echocardiography (large proximal segment of the right coronary artery with a turbulent flow) and multiform echoes – turbulences in the interventricular septum. Echocardiographic examination was performed routinely during the patient’s control visit at the Cardiology Outpatient Clinic. The patient was hospitalized between the 6th and 9th month of age because of severe heart failure with cardiogenic shock and cardiac arrest. The hospitalization lasted 3 months and ended with a diagnosis of myocarditis. The patient’s growth has been normal since then, she has not required any further hospitalizations and has been free from pharmacological treatment since 18th months of age. Currently the patient is free from symptoms with good exercise tolerance. Physical examination shows the presence of a systolic murmur along the left sternal margin. Echocardiographic examination demonstrated (apart from the features described above) normal dimensions of the heart chambers, normal left ventricular contractility and mild mitral valve regurgitation. Multi-slice computed tomography of the coronary arteries was performed. The right coronary artery (RCA) had a typical origin from the right coronary aortic sinus, but with an atypical morphology – it was very wide (9 mm of diameter in the proximal and middle segment) and tortuous (Figure 1). The left coronary artery (LCA) does not originate from the aorta, but from the pulmonary artery (PA). There was a highly developed collateral circulation between the RCA and LCA through the interventricular septum. There were also signs of retrograde filling of the LCA from the RCA with the LCA emptying into the pulmonary trunk (Figure 2). Because of the findings described above the patient was qualified for an invasive examination including coronary angiography, aortography and right heart catheterization. The anomalous origin of the LCA from the PA (Bland-White-Garland syndrome, ALCAPA) was confirmed. The very wide and tortuous RCA provides highly developed collateral circulation to the LCA, which is also wide, but less so than the RCA (Figures 3, 4). The LCA is filled in a completely retrograde manner from the RCA and empties into the PA (retrograde flow). The patient was consulted surgically, but did not agree (she and her parents) to surgical treatment. Exercise test did not show signs of myocardial ischemia with a load of 12 METs.

Discussion

In 85% of cases the symptoms of Bland-White-Gar­land syndrome occur in the first two months of life [3, 5]. Similar pressure in the aorta and pulmonary trunk existing during fetal life enables a similar blood flow in the RCA arising from the aorta and the LCA originating from the PA. After birth there is a decrease in blood pressure and blood oxygenation in the PA and the LCA, which causes myocardial ischemia of the segments supplied by the LCA. The situation is worsened by the closure of the ductus arteriosus, which occurs up to 3 months of age, and at that time the infants usually present with symptoms of severe heart failure caused by ischemia, and if they are left untreated they usually die.

Survival is possible only after development of potent collateral circulation between the RCA and the LCA with reversal of blood flow in the LCA (in the direction of the PA) [3-5]. Such a situation is present in adult patients with Bland-White-Garland syndrome described in the literature: a large, widened and tortuous RCA providing many collaterals to the LCA with reverse flow of blood in the LCA into the PA. This picture was present in the described case in MSCT and coronary angiography. Typical echocardiographic features of this disease consist of widened RCA, reverse flow in the LCA to the PA and highly developed flow in the collaterals present in the interventricular septum [6]. This picture was partially present in our patient.

Prognosis in adult patients with ALCAPA is uncertain or even unfavourable [3-5]. The asymptomatic period is often followed by the onset of heart failure or sudden cardiac death [5, 6]. The authors of a systematic review of data published in 2011 analysed 152 cases of adult patients with ALCAPA and found only 14% of patients who were completely asymptomatic [3]. Other patients presented symptoms of heart failure or angina, heart palpitations and syncope. In most of the patients physical examination demonstrated the presence of a systolic murmur along the left sternal margin (hyperkinetic flow of blood in the functional fistula formed by the connection between the systemic and pulmonary circulation through the collaterals between the RCA and LCA), as observed in our patient.

A particularly important issue is the risk of sudden cardiac death. This type of incident occurred in 7% of patients in the presented review. However, the frequency was higher in younger patients (< 50 years of age) (9%) when compared to patients > 50 years of age (2%). Mean age of patients who died suddenly was 31 years and most of them were completely asymptomatic before that event [3].

These observations lead to the conclusion that all adults with ALCAPA should be qualified for surgical treatment [3-5]. The diagnosis is made at various age of the patients by means of echocardiography (infants), coronary angiography or, more frequently, MSCT. Surgical treatment also depends on the age at which the disease is diagnosed. In infants the LCA is most frequently transplanted from the PA to the aorta, while adults are more frequently treated with bypass to the LCA with ligation at its origin from the PA or with Takeuchi surgery involving the formation of a tunnel through the pulmonary trunk linking the LCA and the aorta [5, 7, 8].

In conclusion, it should be noted for cardiological practice that Bland-White-Garland syndrome might also be present in adults. MSCT examination is an excellent diagnostic tool for this heart defect.

References

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