Transthoracic Echocardiographic Assessment of Coronary Flow in the Diagnosis of Right Ventricular-Dependent Coronary Circulation in Pulmonary Atresia with Intact Ventricular Septum.

Right ventricular-dependent coronary circulation (RVDCC) is associated with pulmonary atresia with intact ventricular septum and is defined by two or more epicardial coronary arteries with atresia or severe stenosis resulting in the dependency of coronary supply by retrograde flow. The hypothesis of this study is that coronary Doppler flow patterns on echocardiography can be used to distinguish patients with RVDCC. Between 2007 and 2016, we reviewed 16 patients with pulmonary atresia or critical pulmonary stenosis. Patients were divided into two groups, those with RVDCC (determined by angiography or pathology evaluation) and those without. Echocardiographic evaluation of the coronary arteries included 2-dimensional measurements and pulse wave Doppler flow pattern in 3 epicardial coronary arteries. Velocity-time integral (VTI) and maximal velocity (Vmax) were measured and compared between the two groups. Three coronary flow patterns were demonstrated: (1) all antegrade flow, (2) antegrade to retrograde VTI flow ratio > 1, and (3) antegrade to retrograde VTI flow ratio ≤ 1. Of the 7 patients with RVDCC, 6 (86%) had evidence of flow pattern 3 in ≥ 2 of the 3 coronary arteries in contrast to 0 (0%) of the non-RVDCC patients (p = 0.001). Higher retrograde Vmax was associated with RVDCC (p < 0.001) and coronary artery dilatation with Z-score ≥ + 3 was also associated with RVDCC (p = 0.02). Echocardiographic evaluation of the coronaries can be useful in identifying RVDCC. More retrograde flow in at least two coronary arteries is strongly suggestive of RVDCC. Dilatation of the coronary arteries is also supportive evidence.

Aberrant coronary arteries: a reliable echocardiographic screening method.

BACKGROUND: Aberrant origin (ABO) of a coronary artery (CA) from the contralateral aortic sinus with a subsequent interarterial course is a life-threatening condition. It carries a 28% to 55% risk for a sudden coronary event or death, but there are no reliable screening methods. OBJECTIVE: We sought to determine whether imaging a cross section of a coronary segment in the anterior aortic wall on the long-axis view may be used as an echocardiographic screening sign for ABO CA. METHODS: The echocardiograms of all patients with ABO CA were evaluated for the screening sign and compared with those of age-matched control patients. RESULTS: Between January 1989 and October 2002, we identified 8 patients with ABO CA (median age: 15 years). Of these patients, 4 were symptomatic and 4 were discovered incidentally. The electrocardiogram produced normal findings in 5 of 8 patients, maximal stress test produced normal findings in 5 of 6 patients, and thallium perfusion test produced negative results in 2 of 3 patients. There were 4 patients with ABO in the main left CA, 3 with ABO in the right CA, and 1 with ABO in the circumflex CA branch. The screening sign was readily visible in 7 of the 8 patients (88%), and all 7 of these patients had ABO in a main CA. Only in the ABO in the circumflex CA branch was the screening sign not detected. The 1743 control patients (median age: 14 years) showed normal anterior aortic wall, which was void of any CA segment. In 5 control patients (0.3%, P <.001) the normal right CA was visible on the long-axis view, but was not confused for a false-positive screening sign. CONCLUSION: We concluded that the proposed screening sign for ABO CA is reliable and easily recognizable, and should prompt a comprehensive assessment of the CA.

Congenital Coronary Artery Abnormalities in Children.

After a significant coronary artery abnormality is recognized in a pediatric patient, surgery or appropriate transcatheter intervention should be performed. The risk of fatality from a congenital coronary abnormality far outweighs the small risks of surgical or transcatheter intervention. Angiography, although considered the state-of-the-art method of diagnosis, has significant spatial limitations and is not always diagnostic of aberrant coronary origins from the contralateral aortic sinus. In the hands of an experienced coronary imager, color flow Doppler echocardiography is one of the best diagnostic tools for congenital coronary abnormalities. Symptoms of a coronary abnormality vary from none to a sudden coronary event that may result in death. Awareness of subtle as well as obvious symptoms is essential for a timely intervention. Surgical or transcatheter intervention in an asymptomatic child with a coronary abnormality is controversial, but it is becoming more acceptable due to a better understanding of the risks involved in unrepaired congenital coronary abnormalities. Surgical reimplantation is the treatment of choice for a patients with a pulmonary origin of a coronary artery. Surgical unroofing of the intramural segment is preferable in an aberrant coronary origin from the contralateral aortic sinus. Surgical enlargement of a stenotic ostium is recommended for ostial stenosis. Transcatheter coil embolization is becoming the treatment of choice of large coronary artery fistula.