Objectives:
Coronary artery to right ventricular (RV) fistulous connections are often thought to preclude decompression of the small hypertensive RV in pulmonary atresia with intact ventricular septum (PAIVS). With RV decompression, fistulae may cause myocardial steal, hypoxic myocardial perfusion, and wall motion abnormalities (WMA). We report the first series of echo assessment of coronary fistula ligation, a critical step to hypoplastic RV decompression to allow post-natal RV growth and 2 ventricle repair .
Methods:
Coronary fistulae were identified with transthoracic echo plus angiocardiographic confirmation. Intraoperatively, fistulae were localized and ligation assessed with TE and high frequency epicardial surface echo. Follow-up TE and transthoracic echo assessed completeness of fistulae ligation and WMAs.
Results:
Five consecutive patients with severe PAIVS and multiple fistulae were studied. 27 fistulous connections were identified pre-op (mean 5.4/patient). Significance was judged by 1) enlargement of the right or left coronary, 2) size of fistulae, 3) retrograde coronary flow. In 3 of 5, right and left coronaries were enlarged. No interruptions were seen but stenoses were observed on angio. Coronary to aortic root connection was best seen with echo flow. Ligation of 25/27 fistulae produced no WMAs. All had RV decompression. Two small fistulae not ligated were well tolerated. All 5 patients were discharged.
Conclusions:
Pre and intra operative echo provided essential surgical guidance and prompt evaluation of fistula ligation. Significance of the fistulae was determined by echo and angio agreement on coronary size, dilatation, retrograde/bidirectional flow. Coronary fistula analysis is important to RV rehabilitation in PAIVS treatment.