Anomalous left coronary artery from the pulmonary artery (ALCAPA, Bland-White-Garland syndrome or White-Garland syndrome) is a rare

congenital anomaly A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities ca ...

occurring in approximately 1 in 300,000 liveborn children. The diagnosis comprises between 0.24 and 0.46% of all cases of

congenital heart disease A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular ...

. The anomalous left coronary artery (LCA) usually arises from the

pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...

instead of the

aortic sinus An aortic sinus, also known as a sinus of Valsalva, is one of the anatomic dilations of the ascending aorta, which occurs just above the aortic valve. These widenings are between the wall of the aorta and each of the three cusps of the aortic valv ...

. In fetal life, the high pressure in the pulmonic artery and the fetal shunts enable oxygen-rich blood to flow in the LCA. By the time of birth, the pressure will decrease in the pulmonic artery and the child will have a postnatal circulation. The myocardium which is supplied by the LCA, will therefore be dependent on collateral blood flow from the other coronary arteries, mainly the RCA. Because the pressure in RCA exceeds the pressure in LCA a

collateral circulation Collateral circulation is the alternate circulation around a blocked artery or vein via another path, such as nearby minor vessels. It may occur via preexisting vascular redundancy (analogous to engineered redundancy), as in the circle of Wil ...

will increase. This situation ultimately can lead to blood flowing from the RCA into the LCA retrograde and into the pulmonary artery, thus forming a left-to-right shunt.

Signs and symptoms

The development of symptoms in ALCAPA depends heavily on the amount of collaterals development. When only few collaterals are present, the myocardium will not get enough oxygen and will become ischemic. The symptoms in an infant with ALCAPA include signs of heart failure such as

dyspnea Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...

and

tachypnea Tachypnea, also spelt tachypnoea, is a respiratory rate greater than normal, resulting in abnormally rapid and shallow breathing. In adult humans at rest, any respiratory rate of 1220 per minute is considered clinically normal, with tachypnea b ...

, but sometimes the development is more subtle and the first sign of ischemia can be crying during feeding, sweating, failure to thrive and irritability. Approximately 90% of patients dies within the first year if left untreated. The patients having a significant collateral circulation can live to adulthood in rare cases; their circulation is often not enough and they develop chronic ischemia, having a risk för sudden cardiac arrest, heart failure or malignant arrhythmia. The mechanism of patient survival to adulthood is not fully understood.

Diagnosis

Historically ALCAPA was diagnosed with conventional

angiography Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is perfor ...

. Today echocardiography is easily used. It can provide direct visualisation of the anomalous coronary artery and other associated structural abnormalities, and it can also assess myocardial function. The use of pulse and color-flow doppler can sometimes visualise reversal flow in the pulmonic artery. Other non-invasive methods used are computed tomography (CT) as well as magnetic resonance imaging (MRI) which enable a direct visualisation of the arteries as well as the myocardial viability.

Surgery

Surgery is indicated in all patients with ALCAPA independent of symptoms, since reconnection of the anomalous left coronary artery, to the aortic root, is crucial to the perfusion of the myocardium dependent on that vessel. Several surgical techniques have been described in ALCAPA repair, including reimplantation of the left coronary artery in to the aorta or creation of an intra-pulmonary buffer (Takeuchi procedure), bypass grafting. Establishment of a dual coronary system is the preferred method and if possible reimplantation of the artery is the approach of choice. Surgery on the mitral valve at the same time as ALCAPA correction is controversial. Even if surgery is carried in adulthood, reestablishment of a two-coronary system can make malignant arrhythmia disappear.

Outcome and management

No difference in long-term mortality or left ventricle function has been shown between the different techniques to re-establish a two-coronary system. An exception is the ligation of the anomalous left coronary artery, which today is used in some cases and has a higher mortality. The development of surgical techniques and restoring of two-artery circulation has dramatically increased survival. Close long-term follow-up of these patients is necessary, to diagnose a recurrent left ventricle dysfunction, but also to understand better the natural evolution of a corrected heart.

References

External links

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