The outcome of mitral regurgitation after the repair of anomalous left coronary artery from the pulmonary artery in infants and older children

Abstract

The ALCAPA repair has excellent survival outcomes in all age groups. MR improves in the majority of infants with revascularization alone. MR worsens or persists in a significant number of older children following the ALCAPA repair, and they are more likely to require reinterventions for the mitral valve on follow-up.

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

“…Similarly, mitral regurgitation is seen in 50% to 75% of patients with ALCAPA, whereas mitral regurgitation was only seen in 9.3% of patients with ACxAPA/ALADAPA. 33,34 These differences can likely be explained by the relatively decreased size of territory supplied by the anomalous coronary artery in ACxAPA/ALA-DAPA, as an aortic-derived vessel from the LCA system is present. Additionally, once collateralization has developed, the anomalous vessel can receive blood from two sources: (1) the opposite LCA and (2) the RCA.…”

Should We Be Screening for Ischaemic Heart Disease Earlier in Childhood?

“…A lot of patients demonstrate a dramatic clinical and echocardiographic improvement, with remodelling seen through reduction in LV size, improved LV function and reduction in mitral regurgitation [ 21 ]. Subsequent additional surgery such as mitral valve repair may be required in some patients and transplantation is rarely required after revascularisation [ 22 , 23 , 24 ].…”

“…Echocardiography is diagnostic demonstrating extensive aortic wall calcification and calcification of the coronary arteries [ 22 ]. Computed tomography may provide further delineation of the extent of coronary, aortic, great vessels and renal artery involvement [ 23 ]. Treatment consists of bisphosphonate therapy with some patients responding well to treatment [ 24 , 25 ].…”