ALCAPA syndrome: A rare etiology of cardiac arrest in a teenager (original) (raw)
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IP innovative publication pvt. ltd, 2019
ALCAPA is a rare congenital anomaly of the coronary system of the heart in which the left coronary artery, which normally arises from the aorta, takes its origin from the pulmonary artery. It is a cause of myocardial ischemia in infancy and causes up to 90% mortality in the first year of life if left untreated. Diagnosis has become easy with the advent of 2D echocardiography. Surgery is the mainstay of therapy with good short and long term results.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare often fatal congenital coronary artery anomaly that occurs in approximately only 1 of 300,000 live births. Many cardiologists and pathologists may be unaware that cardiac ischemia and sudden death can also occur due to ALCAPA in adults in whom it is even rarer and thus may be missed at autopsy. The adult form may range clinically from asymptomatic to sudden cardiac death. We present four cases of adult ALCAPA that illustrate the variable presentations and survival. Two had died at rest and were previously asymptomatic. The other two died during exertion with history of recent chest pains in one and previous myocardial infarction in childhood in the other. This last case remained asymptomatic until death. These four cases highlight that surgery should be undertaken in all cases, even in those who are asymptomatic as newer imaging makes the diagnosis in life more frequent. Age is not important as cases vary widely in age from 22 to 68 in our small group. Furthermore, the most common site of left coronary artery origin is the left pulmonary sinus but our autopsy study shows that the origin can be variable extending above the pulmonary valve sinus to the main pulmonary artery.
International Journal of Environmental Research and Public Health, 2022
Introduction: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that carries 90% mortality in the first year of life when left untreated. The diagnosis of ALCAPA is rare in adulthood, and it includes a broad spectrum of clinical manifestations, including sudden cardiac death (SCD). Case report: We report a rare case of resuscitated sudden cardiac arrest in a 55-year-old female, who was diagnosed with ALCAPA and underwent successful surgical correction and implantable cardioverter defibrillator (ICD) implantation for secondary prevention. Discussion: ALCAPA diagnosis is not confined to childhood, and it represents a rare cause of life-threatening arrhythmias and SCD in the adult population. Surgical correction is recommended, regardless of age, presence of symptoms or inducible myocardial ischemia. Multimodality imaging is crucial for diagnosis, management planning and follow up. Assessment of the risk of recurrent ventr...
Cardiovascular Journal, 1970
Anomalous origin of the left coronary artery from the pulmonary artery is a serious congenital cardiac defect associated with high mortality rates in infancy. It is undoubtedly a rare defect but its diagnosis during life is possible in most cases, and successful surgery appears within reach. The object of this paper is to report our experience with a fourteen years old girl with this rare anomaly. The presenting symptoms of our patient during infancy were not that are considered classic for this anomaly. Though the child had excessive sweating and symptoms of heart failure during infancy. The girl was suspected to have ALCAPA clinically and echocardiographically finally selective right coronary arteriography established the diagnosis. Keywords: Congenital heart disease; ALCAPA. DOI: http://dx.doi.org/10.3329/cardio.v4i1.9397 Cardiovasc. J. 2011; 4(1): 92-95
A RARE BUT TREATABLE CAUSE OF DILATED CARDIOMYOPATHY: ALCAPA
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease (CHD) which accounts for 0.023% of all congenital heart diseases. It is asymptomatic in many cases and is mostly diagnosed in the first few months of life. If untreated, the mortality rate in the first year of life will be more than 90%. We report a 6-month-old infant admitted to out clinic because of heart failure. Echocardiography revealed a dilated cardiomyopathy and left main coronary artery originates from pulmonary artery. Diagnosis was confirmed by coronary angiography and treated succesfully by surgical procedure.
2016
Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiovascular defect that occurs in approximately 1/300 000 live births or 0.5% of children with congenital heart disease. There are two types of ALCAPA syndrome: the infant type and the adult type. The most infants experience myocardial infarction and congestive heart failure, and approximately 90% die within the first year of life; also, without early surgical intervention they have a dismal prognosis. Materials and Methods We report 3- year experiences from January 2013 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital North East of Iran) that consist of all patients with ALCAPA syndrome. Results The Takeuchi procedure, were successfully performed in five children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). There was no death and significant mitral regurgitation postoperative (n=0) in this short study. All of patient...
2020
Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) syndrome is a potentially lethal and rare congenital cardiac anomaly (for approximately 1 in 300,000 live births). ALCAPA syndrome mostly presents in the first few months of life. Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to congestive heart failure. When pulmonary arterial pressure decreases, it results in reversal of flow so that LCA drains from the right coronary artery through collateral vessels into the pulmonary artery. This phenomenon causes ischemia or eventually infarction of the anterolateral left ventricular wall. Sudden death may occur because of inadequate collateral circulation between LCA and the right coronary artery and/or development of arrhythmia. ALCAPA can be found both in infants and in older individuals, with the absence or presence of strong collateral circulation ...
International Journal of Cardiology, 2007
Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome, is a rare congenital coronary anomaly that results in altered myocardial perfusion and a left to right shunt. It occurs in 1:300000 live births and represents 0.24% to 0.46% of all congenital cardiac diseases. Despite its rarity, it is one of the most common causes of ischemia and infarction in children. Ninety percent of these patients will die within the first year of life if untreated and diagnosing this abnormality in adulthood is extremely rare. Of those patients who survive to adulthood, the average age of sudden cardiac death is 35 years. The initial symptoms of the adult presentation vary widely from progressive dyspnea to sudden cardiac death; therefore, immediate surgical correction is highly recommended upon diagnosis. Understanding the pathophysiology and nature of collateral coronary flow in this congenital anomaly is paramount to the safe anesthetic management of adults with ALCAPA. Here we describe the intraoperative management and echocardiographic findings in a 68-year-old with with recently diagnosed ALCAPA undergoing surgical repair.