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anomalous left coronary artery from the pulmonary artery
ICD-10 Codes
Description
What is Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA)?
ALCAPA is a rare congenital heart defect in which the left coronary artery arises from the pulmonary artery instead of the aorta. This abnormal connection can lead to serious complications, including:
- Reduced blood flow to the heart muscle
- Increased risk of heart failure and sudden cardiac death
Causes and Symptoms
ALCAPA occurs when there is an abnormal development of the coronary arteries during fetal growth. The exact cause is unknown, but it is believed to be a genetic condition.
Symptoms may appear in infancy or early childhood and can include:
- Chest pain
- Shortness of breath
- Fatigue
- Poor feeding
- Failure to gain weight
Prevalence and Diagnosis
ALCAPA is a rare condition, affecting approximately 1 in 300,000 births [9]. It is often diagnosed through echocardiography or cardiac catheterization.
Treatment and Prognosis
Surgical intervention is necessary to correct the abnormal connection between the left coronary artery and the pulmonary artery. The prognosis for children with ALCAPA who undergo surgery is generally good, but complications can arise if the condition is not treated promptly [3].
References:
[1] - Search result 1: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital heart defect in which the left coronary artery arises from the pulmonary artery instead of the aorta. [2] - Search result 2: The left coronary artery, which carries blood to the heart muscle, is connected to the pulmonary artery instead of the aorta in ALCAPA. [3] - Search result 7: ALCAPA is a rare congenital coronary anomaly that results from abnormal left coronary artery origination from the pulmonary artery. This condition requires surgical intervention for correction. [4] - Search result 8: The left coronary artery, which carries blood to the heart muscle, is connected to the pulmonary artery instead of the aorta in ALCAPA. [9] - Search result 9: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) occurs once per 300,000 births.
Additional Characteristics
- chest pain
- fatigue
- poor feeding
- shortness of breath
- a rare congenital heart defect
- the left coronary artery arises from the pulmonary artery instead of the aorta
- reduced blood flow to the heart muscle
- increased risk of heart failure and sudden cardiac death
- failure to gain weight
- necessary surgical intervention for correction
Signs and Symptoms
Common Signs and Symptoms of Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA)
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that can cause various signs and symptoms in infants and adults. Here are some common symptoms associated with ALCAPA:
- Poor feeding: Infants with ALCAPA may show poor feeding habits, which can be due to discomfort or pain during feeding [1].
- Rapid breathing (tachypnea): Babies with ALCAPA often exhibit rapid breathing, which is a sign of heart failure [3][8].
- Sweating and crying during feeding: Some infants may cry or sweat excessively during feeding, which can be mistaken for colic symptoms [1][6].
- Irritability: Infants with ALCAPA may become irritable due to discomfort or pain [6].
- Pale skin: Pale skin tone is another symptom associated with ALCAPA in infants [6].
- Heart failure: Untreated ALCAPA can lead to heart failure, which manifests as shortness of breath (dyspnea) and rapid breathing (tachypnea) [3][8].
- Murmur: A murmur may be heard during a physical examination, indicating abnormal blood flow through the heart [5].
- Sudden cardiac death: In severe cases, ALCAPA can lead to sudden cardiac death if left untreated [5].
In adults, symptoms of ALCAPA may include palpitations from dysrhythmias, syncope (fainting), dyspnea (shortness of breath), chest pain, and fatigue [7].
Additional Symptoms
- Pale skin
- Sudden cardiac death
- Rapid breathing (tachypnea)
- Sweating and crying during feeding
- Murmur
- Palpitations from dysrhythmias
- poor feeding
- chest pain
- dyspnea
- heart failure
- syncope
- fatigue
- irritability
Diagnostic Tests
Diagnostic Tests for Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA)
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect that requires prompt diagnosis and treatment. The following diagnostic tests are commonly used to diagnose ALCAPA:
- Echocardiogram: An ultrasound test that uses sound waves to create images of the heart structures and blood flow inside the heart. Echocardiography without Doppler may identify abnormal origin of the left coronary artery from the main pulmonary artery [4].
- Electrocardiogram (ECG): A test that measures the electrical activity of the heart, which can indicate abnormal heart rhythms or other cardiac issues.
- Invasive Coronary Angiography (ICA): A standard diagnostic test for ALCAPA diagnosis, which depicts the course of the anomalous coronary artery [3].
- Angiogram: A test that uses X-rays and a contrast agent to visualize the blood vessels in the heart and diagnose any blockages or abnormalities.
- Echocardiogram with Doppler: This test can help identify abnormal blood flow patterns and confirm the diagnosis of ALCAPA.
These diagnostic tests are usually performed under the guidance of a cardiologist, and the results will help determine the best course of treatment for the patient. Early detection and treatment of ALCAPA are crucial to prevent complications and improve outcomes [1].
References: [1] by H Yang · 2018 · Cited by 10 — Electrocardiogram (ECG) was normal, but echocardiography made the diagnosis of ALCAPA. [3] by EA Blickenstaff · 2023 · Cited by 4 — 3. Diagnostic Testing. Invasive coronary angiography (ICA) was the standard for ALCAPA diagnosis as it depicted the course of the anomalous ... [4] Aug 10, 2020 — Echocardiography without Doppler may identify abnormal origin of the left coronary artery from the main pulmonary artery. [5] Exams and Tests · Echocardiogram, which is an ultrasound that views the heart structures and blood flow inside the heart · Electrocardiogram (ECG), which measures ... [6] Tests. If we suspect you have ALCAPA, we will order a variety of imaging and other diagnostic tests to be sure. Some of these may include: Angiogram ...
Additional Diagnostic Tests
- Electrocardiogram (ECG)
- Echocardiogram
- Invasive Coronary Angiography (ICA)
- Angiogram
- Echocardiogram with Doppler
Treatment
Medical Management of ALCAPA
The medical management of Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) involves treating the symptoms associated with congestive heart failure. According to various sources, the treatment includes:
- Diuretics: Medicines that help remove excess fluid from the body, reducing swelling and alleviating shortness of breath [1][3][5][8].
- Afterload reduction medications: Drugs that lower the workload on the heart, making it easier for the heart to pump blood effectively [1][9].
- Inotropic agents: Medicines that make the heart muscle pump harder, increasing the strength and efficiency of the heart's contractions [2][5][8].
These medical treatments are often used in conjunction with corrective surgery, which is considered the standard treatment for ALCAPA. However, medications can be utilized as a temporary measure or in tandem with surgical intervention to manage symptoms and stabilize the patient.
References:
[1] Aug 10, 2020 — Nitrates are peripheral and coronary vasodilators used in the management of angina pectoris, heart failure, and myocardial infarction. ACE ...
[2] by AA Lardhi · 2010 · Cited by 43 — Medical management of ALCAPA consists of standard therapy for congestive heart failure that includes diuretics, afterload reduction drugs, and inotropic agents.
[3] Oct 7, 2022 — Because of the complex pathological mechanisms of myocardial ischemia in patients with ACAOS, a uniform treatment strategy is not applicable.
[5] These symptoms are often treated with medicines such as digoxin, diuretics, blood pressure lowering medicines, and/or blood thinners.
[8] May 27, 2024 — Treatment · Water pills (diuretics) · Medicines that make the heart muscle pump harder (inotropic agents) · Medicines that lower the workload on ...
[9] Aug 10, 2020 — Treatment of congestive heart failure includes carefully using diuretics, afterload reduction medications, and inotropic drugs.
Recommended Medications
- Diuretics
- Afterload reduction medications
- Inotropic agents
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA)
Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital heart defect where the left coronary artery arises abnormally from the pulmonary artery instead of the left coronary sinus. The differential diagnosis for ALCAPA includes:
- Kawasaki disease: This is an acute inflammatory condition that affects the blood vessels and can cause dilatation of the coronary arteries [8].
- Coronary artery–coronary sinus fistula: This is a rare congenital anomaly where there is an abnormal connection between the coronary arteries and the coronary sinus, which can lead to dilatation of the coronary arteries [8].
- Dilated cardiomyopathy (DCM): DCM is a condition where the heart muscle becomes weakened and the chambers become enlarged, leading to poor heart function. ALCAPA should be considered in the differential diagnosis of any young patient presenting with left-sided chamber dilation, LV dysfunction, and angina [5].
- Pediatric dilated cardiomyopathy (DCM): Similar to DCM, pediatric DCM is a condition where the heart muscle becomes weakened and the chambers become enlarged, leading to poor heart function in children. ALCAPA should be considered in the differential diagnosis of any child presenting with left-sided chamber dilation, LV dysfunction, and angina [1].
Diagnostic Tests
The diagnosis of ALCAPA can be challenging but can be made using a combination of non-invasive tests such as:
- Transthoracic echocardiography: This is an ultrasound test that uses sound waves to create images of the heart. It can help diagnose ALCAPA by showing the abnormal origin of the left coronary artery from the pulmonary artery [2].
- Computed Tomography Angiography (CTA): CTA is a non-invasive imaging test that uses X-rays and computer technology to produce detailed images of the blood vessels, including the coronary arteries. It can help diagnose ALCAPA by showing the abnormal origin of the left coronary artery from the pulmonary artery [2].
- Magnetic Resonance Angiography (MRA): MRA is a non-invasive imaging test that uses magnetic fields and radio waves to produce detailed images of the blood vessels, including the coronary arteries. It can help diagnose ALCAPA by showing the abnormal origin of the left coronary artery from the pulmonary artery [2].
References
[1] Aug 10, 2020 — Differential Diagnoses · Coronary Artery Fistula · Dilated Cardiomyopathy (DCM) · Pediatric Dilated Cardiomyopathy (DCM) · Pediatric Mitral ...
[2] by EA Blickenstaff · 2023 · Cited by 4 — Transthoracic echocardiography combined with other noninvasive tests such as CTA or MRA can be used to definitively diagnose the condition.
[5] by L Croll · 2024 — The diagnosis should be on the differential diagnosis of any young patient presenting with left-sided chamber dilation, LV dysfunction, and angina.
[8] by G Mayà-Casalprim · 2020 · Cited by 7 — The differential diagnosis for such dilatation of the coronary arteries includes Kawasaki disease, coronary artery–coronary sinus fistula ...
Note: The references provided are based on the search results and may not be an exhaustive list of all relevant studies.
Additional Differential Diagnoses
- endocardial fibroelastosis
- Coronary arteryâcoronary sinus fistula
- Pediatric dilated cardiomyopathy (DCM)
- dilated cardiomyopathy
- Kawasaki disease
Additional Information
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- MESH:D063748
- IAO_0000115
- A coronary artery anomaly in which the left coronary artery (LCA) branches off the pulmonary artery instead of the aortic sinus.
- oboInOwl#hasExactSynonym
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- rdf-schema#label
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