CMR adenosine stress perfusion in pediatrics and congenital heart disease: effects on clinical decision making and outcomes

Background

In contrast to adults with coronary artery disease (CAD), the use of CMR adenosine stress perfusion in pediatrics and in adults with congenital heart disease (CHD) is not well established. The medical literature reveals an absence of experience evaluating the effects of CMR adenosine stress perfusion on clinical decision making and outcomes in these populations.

Specific Aims

Evaluate whether CMR adenosine stress perfusion in pediatrics and adults with CHD affects clinical decision making and outcomes.

Methods

Consecutive patients, who completed CMR adenosine stress perfusion and were < 21yo or > 21yo with CHD, were enrolled. SSFP cine and delayed enhancement CMR (DE-CMR) were performed in a standard manner. Adenosine stress perfusion was performed with administration of adenosine (140 ug/kg/min) for 2-4 minutes and gadolinium (0.1 mmol/kg) using a standard adult protocol. Perfusion defects matching infarct size on DE-CMR and defects corresponding to DE-CMR at the right ventricular insertion site or post-surgical changes were considered negative for ischemia.

Results

51 studies were performed in 46 patients (mean 25.6 years, 32 < 21 years). Diagnoses and symptoms are listed in Tables 1 and 2. 40/51 studies were negative for ischemia and this finding resulted in no further imaging in 37/40 (93%). 3 patients underwent coronary angiography despite the absence of ischemia on stress perfusion, and none had coronary artery stenosis. 11/51 studies revealed ischemia and 8 were consistent with CAD. 3 patients with ischemia were diagnosed with hypertrophic cardiomyopathy (HCM) based on a) ischemia pattern with small punctate perfusion defects in the mid-portion of the ventricular septum, b) other morphologic findings of HCM. A finding of ischemia led to coronary angiography in 7/10 (1 lost to follow-up). The patients who did not undergo coronary angiography had HCM. 5/7 who underwent coronary angiography had coronary artery stenosis in a pattern consistent with stress perfusion findings. Of these 5 patients, 2 have undergone or are scheduled for coronary artery bypass graft (CABG), 1 is listed for cardiac transplant (pulmonary atresia/intact ventricular septum status post Fontan), 1 is undergoing further workup (myocardial bridge) and 1 was lost to follow-up. Survival rate is 100%. The 3 patients with ischemia in the setting of HCM have been restricted from competitive athletics.

Table 1 Diagnoses

Table 2 Presenting Symptom

Conclusions

A negative finding on CMR adenosine stress perfusion often results in no further testing, indicating confidence in the result. A positive result can lead to further work-up and positively affect patient outcomes.

Funding

Institutional.