--- title: Coronary artery anomalies description: What congenital coronary artery anomalies are — origin of the left coronary artery from the pulmonary artery, origin of an artery from the "wrong" sinus of the aorta and coronary fistulae — why they are dangerous for children and athletes, how they are found, what treatment approaches the clinical guidelines describe and when urgent help is needed. type: disease aliases: - Congenital coronary artery anomalies - Anomalous coronary arteries - Bland–White–Garland syndrome - Anomalous left coronary artery from the pulmonary artery - Anomalous aortic origin of a coronary artery - Coronary artery fistula - Coronary-cameral fistula - ALCAPA - AAOCA sources: - tier: guideline org: minzdrav-rf title: Аномалия развития коронарных сосудов year: 2024 url: https://cr.minzdrav.gov.ru/view-cr/829_1 - tier: guideline org: esc title: 2020 ESC Guidelines for the management of adult congenital heart disease year: 2020 doi: 10.1093/eurheartj/ehaa554 url: https://doi.org/10.1093/eurheartj/ehaa554 - tier: guideline org: esc title: 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease year: 2020 doi: 10.1093/eurheartj/ehaa605 url: https://doi.org/10.1093/eurheartj/ehaa605 - tier: reference org: msd-manuals title: Overview of Congenital Cardiovascular Anomalies (MSD Manual Professional Version) year: 2025 url: https://www.msdmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/overview-of-congenital-cardiovascular-anomalies - tier: reference org: msd-manuals title: Sudden Cardiac Death in Athletes (MSD Manual Professional Version) year: 2026 url: https://www.msdmanuals.com/professional/cardiovascular-disorders/sports-and-the-heart/sudden-cardiac-death-in-athletes sources_checked: 2026-10-13 review: null --- Coronary artery anomalies are congenital differences in where the coronary arteries start, how they run or how their end branches are built. The European Society of Cardiology (ESC) names three groups: origin of a coronary artery from the "wrong" place on the aorta, origin of a coronary artery from the pulmonary artery, and coronary fistulae. Most anomalies are found by chance, and often they are not dangerous and need no treatment, but there are exceptions. How the coronary arteries are arranged normally is explained in the article on the [coronary circulation](../../anatomy/coronary-circulation/). The 2024 Russian Ministry of Health guideline is written for children, the ESC guideline on congenital heart disease for adults, and the ESC guideline on sports cardiology for young people and athletes; the text says which source says what. Narrowing of the coronary arteries by atherosclerosis is covered in the article on [coronary heart disease](../coronary-heart-disease/), and acquired changes of the arteries in children in the article on [Kawasaki disease](../kawasaki-disease/).

Normally both coronary arteries start from the aorta: the right one from the right sinus (a bulge at the root of the aorta), the left one from the left sinus. No coronary artery starts from the pulmonary trunk, and the branches of the left coronary artery run around it.

The diagram shows the anomaly the sources describe in most detail: the left coronary artery starts from the pulmonary trunk (ALCAPA, Bland–White–Garland syndrome). The right coronary artery starts from the aorta and is markedly widened and tortuous. Through bypass vessels (collaterals) blood from the right artery reaches the left one and flows along it into the pulmonary trunk: this is "steal". The anterior wall of the left ventricle gets too little blood and oxygen. The number and course of the collaterals in the diagram are schematic.

## Why it happens **Origin of the left coronary artery from the pulmonary artery (ALCAPA).** This is a rare congenital heart defect that is dangerous from the newborn period and infancy. Before birth it causes no problems: blood with roughly the same oxygen content flows into both coronary arteries. After birth the pressure in the pulmonary artery falls, and at about 6–8 weeks blood flow in the left coronary artery drops; the heart muscle of the left ventricle gets too little blood and oxygen. The children who survive are those who have developed a network of bypass vessels from the right coronary artery to the left one. But even then some of the blood from the right artery escapes through the left one into the pulmonary artery: it is "stolen". Without treatment the outlook is poor. **Origin of a coronary artery from the "wrong" sinus of the aorta.** The artery starts from the aorta, but in the wrong place: for example, the left one from the right sinus, or the right one from the left sinus. It is most dangerous when such an artery runs between the aorta and the pulmonary artery, leaves at an acute angle, or its first segment runs within the wall of the aorta: the artery may then be compressed or the blood flow through it may be insufficient, and the heart muscle gets too little blood. In the ESC's view, repeated episodes of this shortage of blood (ischaemia) are likely to lead to scarring of the muscle and to dangerous ventricular arrhythmias during exercise, such as [ventricular tachycardia](../ventricular-tachycardia/). **A coronary fistula** is an abnormal connection between a coronary artery and a chamber of the heart or a vessel, bypassing the small vessels. A lot of blood escapes through a large fistula, and part of the heart muscle does not get enough of it. According to the Russian Ministry of Health paediatric guideline, isolated anomalies are found in 0.6–1.8% of all coronary angiograms. According to the ESC, anomalous aortic origin of a coronary artery (AOCA) is present in 0.44% of adolescents in the general population. In ALCAPA the right coronary artery arises from the aorta and is markedly dilated and tortuous, while the left one arises from the posterior surface of the pulmonary trunk and almost immediately divides into the anterior interventricular (left anterior descending) and circumflex branches. In anomalous aortic origin, the ESC associates the following with myocardial ischaemia: a high orifice, ostial stenosis, a slit-like orifice, acute-angle take-off, an intramural course and its length, an interarterial course and hypoplasia of the proximal artery. Origin of the left coronary artery from the right sinus is less common but more dangerous than origin of the right one from the left sinus. The 3D model shows a normal heart: both coronary arteries start from the aorta, whereas in ALCAPA the left coronary artery would start from the pulmonary trunk. ## Symptoms **In babies with ALCAPA** the first signs are listlessness, pallor, sweating, vomiting and spitting up, shortness of breath and a fast heartbeat. There are attacks of sudden distress during or after feeds; they are called "feeding angina". The disease may start straight away with a severe condition caused by a [heart attack](../myocardial-infarction/). Over time [heart failure](../heart-failure/) and [mitral regurgitation](../mitral-regurgitation/) develop. **In teenagers and adults** with the "adult" type of ALCAPA there may be no symptoms for a long time; teenagers may feel unwell and have chest discomfort during sport, and in adults the first signs are often heart rhythm problems. When an artery starts from the "wrong" sinus of the aorta, the first sign may be chest pain, fainting during exercise or sudden cardiac arrest, but more than two thirds of people have no symptoms. **A coronary fistula** may cause no symptoms for a long time. The most common symptom is shortness of breath out of proportion to effort; adults may have pain in the heart area during exercise and an irregular heartbeat. According to the ESC, ALCAPA can present as a silent or symptomatic myocardial infarction, left ventricular dysfunction, ventricular tachycardia or even sudden cardiac death. According to the Russian Ministry of Health paediatric guideline, in infants the picture may resemble [myocarditis](../myocarditis/), [dilated cardiomyopathy](../dilated-cardiomyopathy/) or congenital mitral regurgitation; mitral regurgitation results from papillary muscle ischaemia and dilatation of the mitral annulus. According to the ESC, small fistulae have a good prognosis without treatment, while medium or large ones are associated with long-term complications: angina, myocardial infarction, arrhythmias, heart failure and [endocarditis](../infective-endocarditis/). ## How it is diagnosed According to the Russian Ministry of Health paediatric guideline, when a coronary artery anomaly is suspected, a child has an [ECG](../../diagnostic/ecg/), a chest X-ray and a [heart ultrasound (echocardiography)](../../diagnostic/echocardiography/) with colour Doppler. An enlarged heart is especially typical of ALCAPA in newborns and infants. CT coronary angiography shows the anatomy of the coronary arteries best. For adults the ESC recommends checking with imaging whether ischaemia appears during physical exercise: the treatment decision depends on it. According to the ESC, an ordinary exercise test rarely reveals ischaemia in anomalous aortic origin, so the diagnosis is made mainly by CT or MRI. Among the groups to be examined, the Russian Ministry of Health paediatric guideline lists newborns with unexplained cardiomegaly and attacks of "feeding angina", and adolescents with new-onset anginal pain or arrhythmias. The ESC recommends (I C) non-pharmacological functional imaging — a nuclear study, echocardiography or cardiac MRI with physical stress — to confirm or exclude myocardial ischaemia. ## How it is treated **ALCAPA.** The Russian Ministry of Health paediatric guideline recommends surgery for all children as soon as the diagnosis has been confirmed by tests; the ESC recommends surgery for adults too. The aim is to restore the blood supply of the heart through two coronary arteries from the aorta; in small children the preferred method is to reimplant the left coronary artery directly into the aorta. The surgeon chooses the type of operation, above all according to the anatomy of the defect. In severe heart failure medicines are given, but they do not replace surgery: the Russian Ministry of Health paediatric guideline calls surgery the only recognised effective treatment of heart failure in this defect. **Origin from the "wrong" sinus of the aorta in adults.** The ESC recommends surgery for people with typical angina if they have proven ischaemia in the area supplied by that artery or high-risk anatomy; for people without symptoms whose right artery starts from the left sinus, with no ischaemia and no high-risk anatomy, the ESC does not recommend surgery. **Fistulae.** According to the Russian Ministry of Health paediatric guideline, large fistulae in children are closed whether or not there are symptoms, by surgery or through a catheter, while small fistulae without symptoms are not closed. According to the ESC, the main reasons for closure in adults are symptoms, complications and a significant shunt of blood. **Sport.** In its recommendations for people under 40, the ESC advises against most competitive sports with a moderate or high demand on the heart if the artery leaves at an acute angle or runs between the large vessels. For the most dangerous form — when the artery leaves at an acute angle with a slit-like opening and a narrowed lumen and runs between the aorta and the pulmonary artery — the ESC advises against any sport other than low-intensity skill sports until successful surgery, whether or not there are symptoms. After surgery the ESC allows a return to all sports to be considered no earlier than 3 months later, if there are no symptoms and a maximal exercise test shows no ischaemia and no complex arrhythmias. Whether a person can do sport is decided by the doctor after tests. **Follow-up.** The Russian Ministry of Health paediatric guideline recommends follow-up by a paediatric cardiologist for all children with an anomaly, whether or not they have had surgery. When the anomaly is found in time, the outlook is good in the vast majority of cases, but only if it has been corrected by surgery. ESC (I C): surgery is recommended in ALCAPA; reconstruction of a two-coronary system, including coronary transfer, is preferred; coronary artery bypass grafting with closure of the anomalous artery is reserved for cases in which transfer is not feasible. In asymptomatic individuals whose anomalous artery does not course between the large vessels and has no slit-like orifice or intramural course, the ESC states that competition may be considered after adequate counselling on the risks, provided there is no inducible ischaemia (IIb C). The ESC gives no sport recommendations for people over 40 because of the paucity of studies. According to the Russian Ministry of Health paediatric guideline, after surgery for ALCAPA stenosis of the ostium and trunk of the reimplanted left coronary artery and progression of mitral regurgitation are possible, and after fistula closure, recanalisation of the fistula. ## When to call an ambulance In a baby with ALCAPA the disease may present straight away with a severe condition caused by an extensive heart attack, sometimes with shock. Call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) if a baby has attacks of sudden distress with pallor, sweating and worsening shortness of breath, especially during or after feeds. The Russian Ministry of Health paediatric guideline warns that with any of the signs described, the child must be taken without delay to a cardiac surgery hospital. In a teenager or adult, chest pain or fainting during exercise may be the first sign of a coronary artery anomaly. Then call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK), and all the more so if the person suddenly collapses unconscious: according to the MSD Manual, in cardiac arrest the most important thing is to recognise it at once and start cardiopulmonary resuscitation (CPR) and, if an automated external defibrillator (AED) is nearby, to use it. The Russian Ministry of Health paediatric guideline lists acute coronary insufficiency, worsening heart failure or heart rhythm problems, and infective endocarditis as reasons for emergency admission of a child to hospital. As with any heart condition, call an ambulance for fainting, chest pain or severe shortness of breath: this is the site's general safety rule, repeated at the bottom of every page.