Main coronary artery and coronary ostial stenosis in children: Detection by transthoracic color flow Doppler echocardiography

Stenosis (S) of a main coronary artery (CA) or its ostium (CO) are potentially life threatening conditions in children. The echocardiographic diagnosis of CAS and COS has not been described. Pour patients (pt) aged 1 week to 12 years were diagnosed with COS (3 pt) and CAS (1 pt), by echocardiography and confirmed by autopsy (1 pt), surgery (1 pt) and angiography (2 pt). Left COS was associated with an aberrant left CA coursing between the aorta and pulmonary truck (1 pt), and an intramural left CA with d-transposition of the great vessels (1 pt). Right COS was present with normal coursing right CA with aortic valvular stenosis (1 pt). Acquired left main CAS was diagnosed by echocardiography in the fourth pt, 3 years after orthotopic heart transplantation. COS was recognized when a color flow acceleration signal was present proximal to CO which persisted after increasing the Nyquist range beyond the measured maximal spectral velocity in the CA. This finding was not detected in 63 controls with normal CA of which 23 had ventricular hypertrophy. CAS was detected when a coarctated color flow stream was present within the stenosed CA segment with disturbed distal flow. Its percentage narrowing (50%) corresponded well with angiography. All had increased spectral velocity in the CA compared to the normal controls (pt = 45 ± 10 cm/sec, controls = 24 ± 6 cm/sec p < 0.01) and delayed peak diaslolic velocity expressed as a ratio of time to peak velocity to total diastolic velocity duration (pt = .38 ± .04, controls .2 ± .08, p < 0.01). We conclude 1) Prospective color and pulsed Doppler echocardiographic evaluation of the CA in children can detect CAS and COS and help plan the proper intervention. 2) Knowledge of normal echocardiographic CA flow pattern is essential so that abnormal flow patterns as seen with CAS and COS can be detected and a timely diagnosis made.