PMC10157036_01
A 12-lead ECG illustrated normal sinus rhythm without ST-segment and T-wave changes (A); chest x-ray showed scoliosis, cardiac enlargement and briefly normal vascular markings on both sides, along with rightward mediastinal shift (B); transthoracic echocardiography revealed normal size of four chambers, left ventricular, and valvular function (C); color Doppler in parasternal short-axis view showed abundant collateral blood signals within the ventricular septum (D); an abnormal continuous blood flow directed into the pulmonary artery was also observed (E). LV, left ventrium; RVOT, right ventricular outflow tract; AV, aortic valve; LCA, left coronary artery.
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A 12-lead ECG illustrated normal sinus rhythm without ST-segment and T-wave changes (Figure 1A). Chest x-ray showed scoliosis, cardiac enlargement, and briefly normal vascular markings on both sides, along with a rightward mediastinal shift (Figure 1B). At parasternal short-axis view, a red shunting flow seemingly from the left coronary artery (LCA) into the pulmonary artery (PA) was seen by CDFI, and a continuous shunt following the path of this vessel, which appeared as low velocity with pronounced diastolic phase on pulsed Doppler was also detected (Figures 1C-E and Supplementary Videos S1A,S1B).
Transradial coronary angiography was performed; however, multiple attempts failed to engage the left main coronary artery (LM) (A); right coronary angiography revealed an extremely large and tortuous RCA arising from the RCC with significant collateral flow from RCA to LCA (B). The blood flow terminated in the pulmonary artery through the LCA and delayed enhanced-imaging demonstrated the LCA filling via extensive intercoronary collateral circulation, which supported the diagnosis of ALCAPA (C,D). LA, left main coronary artery; LAD, left anterior descending; PA, pulmonary artery; RCA, right coronary artery.
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MDCTA revealed that the LCA originated from the right side of main pulmonary trunk and then bifurcated into the left anterior descending artery and the left circumflex artery (A-C); MDCTA showed the compensatory dilated and tortuous RCA (D); an UAPA was incidentally revealed by MDCTA (E); and major aortopulmonary collateral artery was shown (F), which was missed with TTE and CAG. ALCAPA, anomalous origin of the left coronary artery from the pulmonary artery; LCA, left coronary artery; LM, left main coronary artery; LPA, left pulmonary artery; MAPCAs, major aortopulmonary collateral arteries; MPA, main pulmonary artery; UAPA, unilateral absence of pulmonary artery.
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The RCA was diffusely enlarged and tortuous, arising from the right coronary sinus as normal, which further confirmed the diagnosis of ALCAPA (Figures 3A-C). TTE and CAG (Figures 3D-F). Major aortopulmonary collateral artery was shown (Figure 3F). Through a more comprehensive analysis of the MDCTA images, we have confirmed that the circulation supplying the right lung originates from the right subclavian artery, with a potential contribution from a collateral bronchial artery and that there was no evidence of collateral circulation within the left lung tissue.