Anomalous left coronary artery from the pulmonary artery | |
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Other names | Bland-White-Garland syndrome |
Possible communication between left coronary artery and pulmonary artery in a 45-year-old woman with Bland-White-Garland syndrome. | |
Specialty | Cardiology |
Anomalous left coronary artery from the pulmonary artery (ALCAPA,Bland-White-Garland syndrome or White-Garland syndrome) is a rare congenital anomaly 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. [1] The anomalous left coronary artery (LCA) usually arises from the pulmonary artery instead of the aortic sinus. 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 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. [2]
The development of symptoms in ALCAPA depends heavily on the amount of collateral development. When 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 and tachypnea, 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 die within the first year if left untreated. [1] Patients having a significant collateral circulation can live to adulthood in rare cases, but often insufficient circulation will cause them to develop chronic ischemia, having a risk for sudden cardiac arrest, heart failure or malignant arrhythmia. [3] [4] The mechanism of patient survival to adulthood is not fully understood. [4]
Historically ALCAPA was diagnosed with conventional angiography. 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. [3]
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 into the aorta, creation of an intra-pulmonary buffer (Takeuchi procedure), and bypass grafting. Establishment of a dual coronary system is the preferred method and if possible reimplantation of the artery is the approach of choice. [5] Surgery on the mitral valve at the same time as ALCAPA correction is controversial. [6] Even if surgery is carried out in adulthood, reestablishment of a two-coronary system can make malignant arrhythmia disappear.
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 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 better understand the natural evolution of a corrected heart. [6]
Cardiology is the study of the heart. Cardiology is a branch of medicine that deals with disorders of the heart and the cardiovascular system. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease, and electrophysiology. Physicians who specialize in this field of medicine are called cardiologists, a sub-specialty of internal medicine. Pediatric cardiologists are pediatricians who specialize in cardiology. Physicians who specialize in cardiac surgery are called cardiothoracic surgeons or cardiac surgeons, a specialty of general surgery.
Coronary circulation is the circulation of blood in the arteries and veins that supply the heart muscle (myocardium). Coronary arteries supply oxygenated blood to the heart muscle. Cardiac veins then drain away the blood after it has been deoxygenated. Because the rest of the body, and most especially the brain, needs a steady supply of oxygenated blood that is free of all but the slightest interruptions, the heart is required to function continuously. Therefore its circulation is of major importance not only to its own tissues but to the entire body and even the level of consciousness of the brain from moment to moment. Interruptions of coronary circulation quickly cause heart attacks, in which the heart muscle is damaged by oxygen starvation. Such interruptions are usually caused by coronary ischemia linked to coronary artery disease, and sometimes to embolism from other causes like obstruction in blood flow through vessels.
Coronary artery bypass surgery, also known as coronary artery bypass graft, is a surgical procedure to treat coronary artery disease (CAD), the buildup of plaques in the arteries of the heart. It can relieve chest pain caused by CAD, slow the progression of CAD, and increase life expectancy. It aims to bypass narrowings in heart arteries by using arteries or veins harvested from other parts of the body, thus restoring adequate blood supply to the previously ischemic heart.
The aortic valve is a valve in the heart of humans and most other animals, located between the left ventricle and the aorta. It is one of the four valves of the heart and one of the two semilunar valves, the other being the pulmonary valve. The aortic valve normally has three cusps or leaflets, although in 1–2% of the population it is found to congenitally have two leaflets. The aortic valve is the last structure in the heart the blood travels through before stopping the flow through the systemic circulation.
Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:
Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO). It is common in patients with a congenital atrial septal aneurysm (ASA).
The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries. The procedure varies for differing congenital heart pathologies. For example in tricuspid atresia, the procedure can be done where the blood does not pass through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. Whereas in hypoplastic left heart syndrome, the heart is more reliant on the more functional right ventricle to provide blood flow to the systemic circulation. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in July 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, presented at the Argentinean National Cardilogy meeting of that year and finally published in 1973.
Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenital heart diseases involving only the primary arteries belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates.
Cor triatriatum is a congenital heart defect where the left atrium or right atrium is subdivided by a thin membrane, resulting in three atrial chambers.
In medicine, collateralization, also vessel collateralization and blood vessel collateralization, is the growth of a blood vessel or several blood vessels that serve the same end organ or vascular bed as another blood vessel that cannot adequately supply that end organ or vascular bed sufficiently.
Coronary artery anomalies are variations of the coronary circulation, affecting <1% of the general population. Symptoms include chest pain, shortness of breath and syncope, although cardiac arrest may be the first clinical presentation. Several varieties are identified, with a different potential to cause sudden cardiac death.
Pulmonaryregurgitation is a condition in which the pulmonary valve is incompetent and allows backflow from the pulmonary artery to the right ventricle of the heart during diastole. While a small amount of backflow may occur ordinarily, it is usually only shown on an echocardiogram and is harmless. More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants further investigation. If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur.
The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.
The bidirectional Glenn (BDG) shunt, or bidirectional cavopulmonary anastomosis, is a surgical technique used in pediatric cardiac surgery procedure used to temporarily improve blood oxygenation for patients with a congenital cardiac defect resulting in a single functional ventricle. Creation of a bidirectional shunt reduces the amount of blood volume that the heart needs to pump at the time of surgical repair with the Fontan procedure.
Anomalous pulmonary venous connection is a congenital defect of the pulmonary veins.
Coronary steal is a phenomenon where an alteration of circulation patterns leads to a reduction in the blood flow directed to the coronary circulation. It is caused when there is narrowing of the coronary arteries and a coronary vasodilator is used – "stealing" blood away from those parts of the heart.
Cardiac magnetic resonance imaging perfusion, also known as stress CMR perfusion, is a clinical magnetic resonance imaging test performed on patients with known or suspected coronary artery disease to determine if there are perfusion defects in the myocardium of the left ventricle that are caused by narrowing of one or more of the coronary arteries.
Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect in which a coronary artery inappropriately arises from the aorta, usually from the incorrect sinus of Valsalva. This anomalous coronary artery often takes an interarterial, intraconal, or intramural course, and is associated with an increased risk of sudden death in children.
The LeCompte maneuver is a technique used in open heart surgery, primarily on infants and children. The maneuver entails cutting the main pulmonary artery and moving it anterior to the aorta before reattaching the pulmonary artery during the following reconstruction of the great vessels. It allows the surgeon to reconstruct the right ventricular outflow tract without needing to connect the proximal and distal sections with a graft. It also enables the surgeon to avoid compressing the coronary arteries and relieves compression of the bronchi in cases where the pulmonary artery is severely dilated or aneurysmal. If both pulmonary arteries are not mobilized adequately, they can become stretched, leading to pulmonic stenosis.
Pulmonary artery agenesis refers to a rare congenital absence of pulmonary artery due to a malformation in the sixth aortic arch. It can occur bilaterally, with both left and right pulmonary arteries being absent, or unilaterally, the absence of either left or right pulmonary artery (UAPA). About 67% of UAPA occurs isolated in the right lung. The absence of pulmonary artery can be an isolated disorder, or accompanied by other related lesions, most commonly Tetralogy of Fallot.
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