Abstract 1791: Ventriculo - Coronary Connections in Hypoplastic Left Heart: A 4 year Prospective Study
Background: Prior reports of ventriculo-coronary connections (VCC) in hypoplastic left heart (HLH) are limited to case reports and pathologic specimens. No prospective comprehensive series of live cases has been reported. We conducted a prospective study to determine the incidence, effect on survival and echocardiographic features of VCC in HLH.
Methods: We analyzed 100 consecutive HLH cases for the presence, effect on survival, anatomy and Doppler flow details of VCC. All had aortic and mitral valve diameter and LV volume <Z −4. Cases were classified as mitral and aortic atresia, mitral and aortic stenosis or mitral stenosis and aortic atresia (MSAA).
Results: VCC were exclusive to and present in 56% of the MSAA subtype. VCC were present in 15% of all HLH. Only 2/15 died. VCC were not statistically associated with higher mortality. In one case with very large and bilateral VCC connections, VCC contributed to early death. The other died >6 months after surgery unrelated to VCC. All VCC had trans-myocardial course with connection to the LV cavity, turbulent color Doppler flow in a dilated and/or tortuous coronary artery and dominant usually retrograde systolic coronary artery flow pattern. The number of VCC per case was 1 VCC in 6, 2 VCC in 5 and 3 VCC in 4. The frequency of various VCC to LV connection sites was apex = 11, posterior wall = 10, near the mitral annulus = 5, septum = 2. The frequency of various coronary artery involvement was posterior descending = 12, anterior descending = 11, circumflex = 3, right coronary = 2. VCC were associated (p < 0.05) with MSAA, EFE, LVH and small ascending aorta size. VCC were not associated (p > 0.1) with sex, bodyweight, age at or type of palliation, atrial septal communication, pulmonary vein obstruction, RV dysfunction, TR grade, arrhythmia, ECMO, bypass, cross clamp or circulatory arrest times.
Conclusions: VCC are common, occurring in 56% of HLH-MSAA. Except when very extensive, VCC do not contribute to mortality. Detailed echo analysis is feasible.