Anomalous left coronary artery arising from the pulmonary trunk, the so-called Bland-Garland-White syndrome, is a rare congenital anomaly with a clinical presentation based on myocardial hypoperfusion resulting in ischaemia and infarction.1–3 Therapeutic intervention consists of reimplantation of the left coronary artery in the aorta, resulting over time in improvement of left ventricular function.4
We report an infant with such an anomaly leading to myocardial ischaemia, complicated by an apical aneurysm of the left ventricle. Because the heart failure persisted, we decided to resect the aneurysm.
Case report
A baby girl was referred to the neonatal intensive care unit of our hospital on the 11th day of life because of respiratory failure. She was born after 36 weeks of pregnancy, which was complicated by polyhydramnios and pregnancy-induced diabetes. On physical examination, she was tachypnoic, but with normal heart sounds. A grade III/VI holosystolic high-pitched murmur, and a diastolic rumble, were audible at the apex. Hepatomegaly was present. The electrocardiogram showed sinus rhythm at a rate of 160 beats/min, a QRS-axis of 140°, P-pulmonale, right ventricular hypertrophy, and normal ST-T segments. The chest X-ray revealed cardiomegaly and pulmonary oedema. Transthoracic echocardiography showed biventricular dilation with normal left ventricular function, as well as dilation of both atria. There was grade III mitral regurgitation, and mild tricuspid regurgitation. The coronary arteries were not visualized. Initially the infant was conservatively treated with digoxin, inhibitors of the angiotension converting enzyme, and diuretics. Surgical repair of the mitral valve was not indicated. At the age of two months, she was discharged from hospital.
Over the next 3 months, her condition deteriorated, and she failed to thrive. At the age of 5 months, she was re-admitted to our hospital with signs of respiratory insufficiency and cardiac failure. Physical examination revealed a severely tachy-dyspnoic child without cyanosis. The heart was now hyperdynamic, with a grade IV/VI systolic murmur on auscultation. The electrocardiogram revealed signs of recent infarction of the anterolateral wall of the left ventricle. The chest X-rays showed cardiomegaly and pulmonary oedema. Echocardiographic evaluation demonstrated a dilated heart, with severely impairment of left ventricular function. The right coronary artery appeared to be normal, whereas the left coronary artery was now seen to arise from the pulmonary trunk. The mitral regurgitation was still present, but had decreased in severity.
Considering her clinical deterioration, it was decided surgically to reimplant the left coronary artery into the aorta. After a successful operation, the postoperative period was complicated by persistent heart failure. Echocardiographic evaluation now showed a globally hypokinetic and dilated left ventricle, with a dyskinetic apex. Cardiac catheterization conducted at the age of 7 months, 6 weeks after surgery, confirmed successful reimplantation of the left coronary artery, but showed left ventricular function to be severely depressed, revealing also a large apical aneurysm (Fig. 1).
Figure 1.
Angiogram of the left ventricle, in 30° right anterior oblique projection, showing the apical aneurysm.
Despite maximal inotropic support, it was not possible to wean the child from mechanical ventilation. In this situation, still with a reasonable expectation of future improvement, the apical aneurysm, found to be thin-walled and fibrotic, was removed at the age of 8 months. Within 48 hours, the clinical situation changed remarkably, and she could be weaned from inotropic support and mechanical ventilation. Echocardiography now revealed amelioration of the apical dyskinesia, despite globally depressed left ventricular function.
At the present time, the girl is five years old and doing well, with good exercise tolerance. Medication consists of aspirin and inhibition of angiotension converting enzymes. On echocardiography, the left ventricular end-diastolic dimensions are on the 95th percentile, with a shortening fraction of 27%. There is only mild mitral valvar incompetence.
Discussion
Anomalous origin of the left coronary artery from the pulmonary trunk causes ischaemia of the left ventricle, ultimately resulting in fibrosis of the myocardium.5 Timely reimplantation of the left coronary artery in the aorta generally restores flow of blood to the left ventricular myocardium, with improvement of its function.4 Our patient, however, did not show improvement after reimplantation of the left coronary artery, and needed prolonged supportive therapy. The development of an apical aneurysm contributed substantially to this unstable hemodynamic situation, making resection of the aneurysm inevitable. It is rare for such aneurysm to develop in children with anomalous origin of the left coronary artery from the pulmonary trunk.5–7 Surgical resection of these aneurysms can be undertaken successfully, especially when left ventricular geometry and the valvar structures are preserved.6, 7 In our patient, there was significant improvement of left ventricular function and the clinical condition, albeit without any change in the pre-existing mitral regurgitation.
Biopsies were taken during the surgical intervention, showing fibrosis of the resected aneurysmatic myocardium. In contrast, the biopsies taken from the hypokinetic left ventricular myocardium showed viable cardiomyocytes, suggesting that the myocardium in our patient might recover from the former ischaemia. Follow-up has shown a remarkable improvement of the left ventricular function in keeping with this assumption.
Thus, anomalous origin of the left coronary artery from the pulmonary trunk may, in rare cases, promote formation of left ventricular aneurysms due to myocardial ischaemia and infarction. Should this happen, then based on our experience, we strongly advocate aneurysmectomy.
