Anomalous left coronary artery from the pulmonary artery

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Anomalous left coronary artery from the pulmonary artery
Other namesBland-White-Garland syndrome
Usg ALCAPA.jpg
Possible communication between left coronary artery and pulmonary artery in a 45-year-old woman with Bland-White-Garland syndrome.
SpecialtyCardiology Edit this on Wikidata

Anomalous left coronary artery from the pulmonary artery (ALCAPA, Bland-White-Garland syndrome or White-Garland syndrome) is a very rare congenital anomaly and is occurring in approximately 1 in 300.000 liveborn children. The diagnos comprise between 0.24 and 0.46% of all cases of congenital heart disease.[1] The anomalous left coronary artery (LCA) usually arise 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 blod to fled 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 therefor 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, 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]

Signs and symtoms

The development of symtoms in ALPACA highly depends on the amount of collaterals development. When only few collaterals are present, the myocardium will not get enough oxygen and will become ischemic. The symtoms in an infant with ALPACA include signs of heart failure such as dyspnea, tachypnea, but sometimes is the development is more subtile and the first sign of ischemia can be crying during feeding, sweating, failure to thrive and irritability. Approximately 90% dies within the first year if left untreated.[1] The patiens having a significant collateral circulation can live to adulthood in rare cases, hence the circulation is often not enough and they will develop chronic ischemia, having a risk för sudden cardiac arrest, heart failure or maligant arrhythmia.[3][4] The mechanism to its very extent, which patient survival to adulthood, is not fully understood.[4]


Historically ALPACA was diagnosed with conventional angiography. Today echocardiography is easily used, and has been well described as a method of diagnosing ALPACA. Echocardiography can provide direct visualisation of the anomalous coronary artery and other associating structural abnormalities, and it can also assess myocardial function.The use of pulse and color-flow doppler can sometimes visualise reversal flow in pulmonic artery. Other non invasive methods used is computed tomographic (CT) as well as magnetic resonance imaging (MRI) which enables a direct visualisation of the arteries as well as the myocardial viability.[3]


Surgery is indicated in all patients with ALPACA independent of symtoms, 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 ALPACA repair, including reimplantation of the left coronary artery in to the aorta or creation of an intra pulmonary buffel (Takeuchi procedure), bypass grafting. Establishment of a dual coronary system is the preferred method and if possible reimplantion of the artery is the matter of choice.[5] Whether surgery on the mitral-valve at the same time as ALPACA correction is controversial.[6] Even if surgery is done in adulthood, reestablishment of a two coronary system has made malignant arrhythmia disappeared.

Outcome and Management

No difference in longterm mortality or left ventricle function has been show between the different techniques to reestablish a two coronary system. The exception of this is the ligation av 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 this patients are necessary, in terms of diagnose a recurrent left ventricle dysfunction but also to better understand the natural evolution of a corrected heart.[6]


  1. ^ 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.
  2. ^ Cardiology. Michael H. Crawford. Philadelphia. 2010. ISBN 978-0-7234-3644-7. OCLC 489073793.{{cite book}}: CS1 maint: others (link)
  3. ^ a b Peña, 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.
  4. ^ 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.
  5. ^ 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.
  6. ^ 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.

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