Adult presentation of ALCAPA: Echo and CT diagnosis

Abstract

A healthy 20-year-old male presented with aborted sudden death. Both thoracic echocardiography and cardiac computed tomographic angiography demonstrated the characteristic findings of ALCAPA noting a dilated and tortuous right coronary artery, dilated coronary collateral arteries within the interventricular septum or along the surface of the heart, and visualization of the left coronary artery origin from the posterior aspect of the main pulmonary artery. Both imaging modalities demonstrated all three characteristic findings of this rare coronary anomaly and served as complementary imaging studies for surgical correction.

Anomalous Origin of the Circumflex or Left Anterior Descending Artery From the Pulmonary Artery

“…Although echocardiographic diagnoses of ALADAPA were made, overall it appeared less common than an echocardiographic diagnosis of ALCAPA. 35,36 Reasons for this include the presence of an aortic-derived vessel from the left coronary system and a lesser degree of collateralization compared to ALCAPA. 37 A preoperative diagnosis of ACxAPA/ALADAPA is important, as four operative deaths were associated with misidentification or failure to identify the anomaly.…”

A Multimodality Imaging and Multidisciplinary Approach to Manage Anomalous Right Coronary Artery from the Pulmonary Artery in Pregnancy

“… 7 These connections are not as pronounced as in cases of ALCAPA where color-flow Doppler echocardiography of the myocardium can reveal extensive collaterization, conforming the so-called heart on fire sign. 8 In our patient, the use of CCT allowed for identification of the anterior RV and septal collaterals. Cardiac computed tomography is fundamental in evaluating coronary anatomy and high-risk features of anomalous coronaries including intramural course, slit-like orifice, or acute-angle takeoff, all of which were not present in this patient.…”

Rare Etiology of Reversible Hypokinetic Dilatated Heart Disease in Infants: Bland-White-Garland Syndrome (ALCAPA)

“…The bundle of signs strongly suggesting ALCAPA on Doppler echocardiography are: right coronary artery dilation [33] [39] [40], a right coronary artery diameter to aortic annulus ratio greater than or equal to 0.14 [41], dilated coronary collateral arteries in the interventricular septum [33] [40], increased echogenicity of the anterolateral papillary muscle of the mitral valve [40] [41], predominance of systolic coronary flow at the ostium right coronary on pulsed Doppler [39], a visualization of the left coronary which does not connect to the aorta and with especially a blue flow in its lumen on color Doppler which testifies to the retrograde blood flow of the left coronary artery towards the pulmonary artery [1] [40]. Supra-sigmoid aortography (Figure 10) confirms that only the right coronary, which is large in caliber, originates from the aorta and that it perfuses the left coronary retrogradely [42] [43].…”