Anomalous left coronary artery from the pulmonary artery
January 01, 1970
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]
Anomalous left coronary artery from the pulmonary artery
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.
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 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 dies within the first year if left untreated.[1] 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 for sudden cardiac arrest, heart failure or malignant arrhythmia.[3][4] The mechanism of patient survival to adulthood is not fully understood.[4]
Diagnosisedit
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]
Surgeryedit
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.[5] Surgery on the mitral valve at the same time as ALCAPA correction is controversial.[6] Even if surgery is carried in adulthood, reestablishment of a two-coronary system can make malignant arrhythmia disappear.
Outcome and managementedit
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.[6]
^Cardiology. Michael H. Crawford. Philadelphia. 2010. ISBN978-0-7234-3644-7. OCLC 489073793.{{cite book}}: CS1 maint: location missing publisher (link) CS1 maint: others (link)
^ abPeña, Elena; Nguyen, Elsie T.; Merchant, Naeem; Dennie, Carole (2009). "ALCAPA syndrome: not just a pediatric disease". Radiographics. 29 (2): 553–565. doi:10.1148/rg.292085059. ISSN 1527-1323. PMID 19325065.
^ abKwiatkowski, David M.; Mastropietro, Christopher W.; Cashen, Katherine; Chiwane, Saurabh; Flores, Saul; Iliopoulos, Ilias; Karki, Karan B.; Migally, Karl; Radman, Monique R.; Riley, Christine M.; Sassalos, Peter (2021). "Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study". Seminars in Thoracic and Cardiovascular Surgery. 33 (1): 141–150. doi:10.1053/j.semtcvs.2020.08.014. PMID 32858217. S2CID 221365678.
^Dodge-Khatami, Ali; Mavroudis, Constantine; Backer, Carl L (2002). "Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy". The Annals of Thoracic Surgery. 74 (3): 946–955. doi:10.1016/S0003-4975(02)03633-0. PMID 12238882.
^ abIsmail, Muna; Jijeh, Abdulraouf; Alhuwaymil, Rathath M; Alahmari, Raneem; Alshahrani, Rawan; Almutairi, Reem; Habshan, Fahad; Shaath, Ghassan A (2020-12-01). "Long-Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (ALCAPA) in Children After Cardiac Surgery: A Single-Center Experience". Cureus. 12 (12): e11829. doi:10.7759/cureus.11829. ISSN 2168-8184. PMC7781494. PMID 33409071.
External linksedit
January 01, 1970
anomalous, left, coronary, artery, from, pulmonary, artery, alcapa, bland, white, garland, syndrome, white, garland, syndrome, rare, congenital, anomaly, occurring, approximately, liveborn, children, diagnosis, comprises, between, cases, congenital, heart, dis. 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 Anomalous left coronary artery from the pulmonary arteryOther namesBland White Garland syndromePossible communication between left coronary artery and pulmonary artery in a 45 year old woman with Bland White Garland syndrome SpecialtyCardiology Contents 1 Signs and symptoms 2 Diagnosis 3 Surgery 4 Outcome and management 5 References 6 External linksSigns and symptoms editThe 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 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 dies within the first year if left untreated 1 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 for sudden cardiac arrest heart failure or malignant arrhythmia 3 4 The mechanism of patient survival to adulthood is not fully understood 4 Diagnosis editHistorically 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 editSurgery 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 5 Surgery on the mitral valve at the same time as ALCAPA correction is controversial 6 Even if surgery is carried in adulthood reestablishment of a two coronary system can make malignant arrhythmia disappear Outcome and management editNo 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 6 References edit a b Kubota Hiroshi Endo Hidehito Ishii Hikaru Tsuchiya Hiroshi Inaba Yusuke Terakawa Katsunari Takahashi Yu Noma Mio Takemoto Kazuya Taniai Seiichi Sakata Konomi 2020 Adult ALCAPA from histological picture to clinical features Journal of Cardiothoracic Surgery 15 1 14 doi 10 1186 s13019 020 1048 y ISSN 1749 8090 PMC 6958604 PMID 31931842 Cardiology Michael H Crawford Philadelphia 2010 ISBN 978 0 7234 3644 7 OCLC 489073793 a href Template Cite book html title Template Cite book cite book a CS1 maint location missing publisher link CS1 maint others link a b Pena Elena Nguyen Elsie T Merchant Naeem Dennie Carole 2009 ALCAPA syndrome not just a pediatric disease Radiographics 29 2 553 565 doi 10 1148 rg 292085059 ISSN 1527 1323 PMID 19325065 a b Kwiatkowski David M Mastropietro Christopher W Cashen Katherine Chiwane Saurabh Flores Saul Iliopoulos Ilias Karki Karan B Migally Karl Radman Monique R Riley Christine M Sassalos Peter 2021 Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery A Multicenter Study Seminars in Thoracic and Cardiovascular Surgery 33 1 141 150 doi 10 1053 j semtcvs 2020 08 014 PMID 32858217 S2CID 221365678 Dodge Khatami Ali Mavroudis Constantine Backer Carl L 2002 Anomalous origin of the left coronary artery from the pulmonary artery collective review of surgical therapy The Annals of Thoracic Surgery 74 3 946 955 doi 10 1016 S0003 4975 02 03633 0 PMID 12238882 a b Ismail Muna Jijeh Abdulraouf Alhuwaymil Rathath M Alahmari Raneem Alshahrani Rawan Almutairi Reem Habshan Fahad Shaath Ghassan A 2020 12 01 Long Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery ALCAPA in Children After Cardiac Surgery A Single Center Experience Cureus 12 12 e11829 doi 10 7759 cureus 11829 ISSN 2168 8184 PMC 7781494 PMID 33409071 External links edit Retrieved from https en wikipedia org w index php title Anomalous left coronary artery from the pulmonary artery amp oldid 1194022093, wikipedia, wiki, book, books, library,
