A Late Complication in a Surgically Corrected ALCAPA Patient.

Late complications in surgically corrected ALCAPA patients are rare. We describe an interesting case of a patient with a thrombosed giant right coronary artery aneurysm which was discovered on a chest X-ray. (Level of Difficulty: Intermediate.).

Our patient is a 54-year-old woman with ALCAPA (anomalous origin of the left coronary artery [LCA] from the pulmonary artery) who had a successful reimplantation of the LCA on the aorta, combined with a mitral valve repair at the age of 44 years.

Her physician had ordered a chest X-ray image because of upper airway complaints: coughing, persistent fever despite antibiotic treatment; she had no cardiac complaints. The chest X-ray (Figures 1A and 1E) demonstrated an increased right-side heart shadow, after which she was referred to our outpatient clinic. We measured a blood pressure of 95/65 mm Hg and pulse of 56 beats/min, and physical examination showed no signs of heart failure. The shortness of breath was no longer present.

Figure 1

Postoperative X-Ray and CT Images After Surgical Correction and Late Complication

(A to D) Postoperative images from 2013. (A) X-ray showing a slightly increased heart shadow due to cardiomegaly. (B, C) Axial computed tomographic scan; arrows indicate the serpentine course of the right coronary artery; a dilated left anterior descending coronary artery is also seen (arrowhead). (D) Three-dimensional reconstruction; arrow indicates the right coronary artery. (E to H) Images from 2020.

The echocardiogram showed new wall motion abnormalities of the inferior wall and a decreased right ventricular function compared with the echocardiogram of 2016, compatible with an ambulant myocardial infarction, with the right coronary artery (RCA) as the most likely culprit.

Subsequent CT imaging showed extensive dilation of the RCA to 80 mm with extensive intraluminal thrombus (Figures 1B to 1D and 1F to 1H). She was referred to a tertiary center for coronary angiography (Video 1) and surgical resection of the RCA aneurysm and a CABG (aorta-RCA saphenous vein graft).

The Bland-White-Garland syndrome, or ALCAPA, is a very rare congenital heart disease, with an incidence of 1 in 300,000. Flows in both the RCA and LCA are equal before closure of the ductus arteriosus. A decrease in pulmonary vascular resistance compared with systemic vascular resistance is at the base of changes in preferential flows in the coronary bed after birth. Closing of the ductus arteriosus leads to ischemia, severe heart failure, and early death when no collaterals are formed (infant type). When extensive collaterals develop (adult type), the blood flow from the RCA is enough to survive. Continuous increased flow in the RCA leads to dilation and outward remodeling of the coronary artery. Although the mechanism of RCA dilation in postsurgical ALCAPA patients has not been described, we hypothesize that RCA dilation results from a pathophysiologic mechanism similar to aneurysm formation in general or arteriogenesis, in which increased flow and shear stress trigger vascular inflammation and increased expression of metalloproteinases and growth factors, eventually leading to increased vessel diameter. Giant RCA aneurysm with a thrombus, most likely due to the low flow state in the aneurysm, in a corrected ALCAPA patient as in our case is extremely rare.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.