Presentation
CMR is a powerful tool in the armamentarium of pediatric cardiology and health care workers caring for patients with congenital heart disease (CHD), but a successful study still presents major technical and clinical challenges. [books.google.com]
Infants are usually cyanotic and may present chest pain and dyspnea on exertion. [orpha.net]
The clinical presentation of DORV varies. On one end of the spectrum, the infant may present with profound cyanosis, while on the other end may have fulminant congestive heart failure. [news-medical.net]
Our patient presented with dyspnea on exertion, but was otherwise asymptomatic. [e-sciencecentral.org]
Presentation in adulthood is very rare. [dergipark.ulakbim.gov.tr]
Entire Body System
- Sudden Infant Death Syndrome
[…] to 0.5% of all congenital heart disease. [14] However, true incidence may be greater than previously recognized, and it is possible that cases are being misdiagnosed or presenting as sudden infant death syndrome. [jfcmonline.com]
Neurologic
- Irritability
Presenting complaints are typically nonspecific, and include diaphoresis, irritability, wheezing and respiratory distress. Precordial auscultation may reveal a murmur. [radiopaedia.org]
Echocardiography, 26, 1232-1235. doi:10.1111/j.1540-8175.2009.00977.x [ 4 ] Mahle, W.T. (1998) A dangerous case of colic: Anomalous left coronary artery presenting with paroxysms of irritability. [scirp.org]
If CHF is present, the infant appears distressed and exhibits tachypnea, tachycardia, diaphoresis, and irritability. [chd-uk.co.uk]
< 2 years of age) Most common type (>75%) Poor collateral circulation Symptoms begin around 2 months of age, coinciding with reduction in pulmonary vascular resistance -> coronary steal -> ischemia Present with signs of CHF or Dilated Cardiomyopathy Irritability [pedemmorsels.com]
Symptoms Symptoms of ALCAPA in an infant include: Crying or sweating during feeding Irritability Pale skin Poor feeding Rapid breathing Symptoms of pain or distress in the baby (often mistaken for colic) Symptoms can appear within the first 2 months of [ufhealth.org]
Workup
Systemic examination and baseline laboratory workup were in normal range. X-ray chest showed bilateral perihilar infiltrates. Cardiac shadow appears to be magnified in supine position. [apspjcaserep.com]
Treatment
Management and treatment Early diagnosis and surgical treatment are mandatory in order to prevent cardiac failure and pulmonary hypertension. [orpha.net]
With early treatment such as surgery, most babies do well and can expect a normal life. Routine follow-ups with a heart specialist (cardiologist) will be needed. [mountsinai.org]
In cases of ALCAPA several surgical treatment options have been proposed9. [romanianjournalcardiology.ro]
Prognosis
Treatment and prognosis Prognosis depends significantly on the extent of collateral formation, however, most infants die within the 1 st year of birth 4. [radiopaedia.org]
Prognosis Patients treated early in life have good short and long-term outcome. If left untreated, the disease may lead to congestive cardiac failure and pulmonary hypertension, with 30% of infants dying within 3 months. [orpha.net]
Etiology
Etiology Either of two etiologies can be responsible for ALCAPA: the bulbus cordis undergoes abnormal septation into the aorta and pulmonary trunk persistence of the pulmonary buds with concomitant involution of the aortic buds that are precursors of [radiopaedia.org]
Etiology The pathogenesis still remains unknown, although an association has been suggested with the 22q11.2 deletion syndrome (see this term). [orpha.net]
(Etiology) Currently, the causative factor of Anomalous Left Coronary Artery from the Pulmonary Artery is unknown. [dovemed.com]
Inadequate myocardial perfusion likely causes significant chest pain and these symptoms of myocardial ischemia may be misinterpreted as routine infantile colic. [3] Etiology Inheritance is not a factor for anomalous origin of the left coronary artery [emedicine.medscape.com]
Etiology Theories have been described to explain the origin of a coronary artery from the pulmonary trunk specifically related to the embryologic division of the truncus arteriosus. [omicsonline.org]
Epidemiology
Summary Epidemiology The incidence is estimated at 0.1% of all congenital cardiac anomalies and around 200 cases have been described in the world literature. [orpha.net]
Chugh SS, Reinier K, Teodorescu C, Evanado A, Kehr E, et al. (2008) Epidemiology of sudden cardiac death: clinical and research implications. ProgCardiovasc Dis 51: 213-228. [omicsonline.org]
We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition. [revespcardiol.org]
No specific association with any noncardiac anomalies is noted. [4] Epidemiology United States data Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare, congenital cardiac anomaly accounting for approximately 0.25 [emedicine.medscape.com]
Pathophysiology
Provides comprehensive coverage of physiology, pathophysiology, pharmacology, and nursing management related to care of the critically ill child. Includes detailed Nursing Care Plans for select disorders. [books.google.com]
Wesselhoeft H, Fawcett JS, Johnson AL (1968) Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. [link.springer.com]
[…] etiologies can be responsible for ALCAPA: the bulbus cordis undergoes abnormal septation into the aorta and pulmonary trunk persistence of the pulmonary buds with concomitant involution of the aortic buds that are precursors of the coronary arteries Pathophysiology [radiopaedia.org]
Prevention
Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. [nemours.org]
Management and treatment Early diagnosis and surgical treatment are mandatory in order to prevent cardiac failure and pulmonary hypertension. [orpha.net]
Early repair is carried out to avoid pulmonary vascular disease of the lung.[4] The repair should be contemplated within the first six months of life to prevent severe pulmonary vasculature obstructive disease. [apspjcaserep.com]
When coronary artery anomalies like ALCAPA are diagnosed, urgent surgery is often indicated in order to prevent myocardial ischemia, malignant ventricular arrhythmias and sudden cardiac death 8. [romanianjournalcardiology.ro]