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Doppler pressure gradients (PGs) are routinely used as a surrogate for catheter peak-to-peak gradient (PPG) for referring pediatric patients with aortic stenosis (AS), pulmonary stenosis (PS), and coarctation of the aorta (CoA) for intervention but do not always predict the catheter PPG accurately, which results in misclassification of lesion severity. We hypothesized that recovered pressure (RP) accounts for the discrepancy between Doppler PG and catheter PPG. We aimed to study the occurrence of clinically significant RP in AS, PS, and CoA.Simultaneous Doppler and catheter PGs were prospectively measured in 82 patients (median age, 12.2 months; weight, 7.5 kg) with isolated AS (n = 30), PS (n = 24), and CoA (n = 28), and agreement before and after correcting for RP were analyzed. RP was calculated from a fluid dynamic-based equation. Effects of lesion geometry on the magnitude of RP were analyzed.Recovered pressure-corrected Doppler peak instantaneous gradient (PIG) had significantly closer agreement and correlation with the catheter PPG (P .001) than the uncorrected PIG and mean Doppler gradients. Recovered pressure-corrected Doppler PIG predicted PPG with high specificity and accuracy in all lesions (95% CI, 36%-97% and 85%-100%, respectively, P .05 for both). RP magnitude was weakly related (r = 0.33 to 0.47) to valve area and inversely related (r = -0.22 to -0.34) to downstream vessel area.Significant RP occurs in congenital AS, PS, and CoA, accounting for misclassification of lesion severity by Doppler PIG. The RP magnitude is at a maximum in mild to moderate stenotic outflow lesions, with small-size downstream vessels causing the most misclassification of lesion severity by Doppler PIG.

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

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 enlarge-ment of a stenotic ostium is recommended for ostial stenosis. Transcatheter coil embolization is becoming the treatment of choice of large coronary artery fistula.

An athletic 15-year-old girl with aberrant left coronary artery from the right coronary sinus, presented with syncope during exercise. Trans-thoracic echocardiography was the only imaging technique that clearly demonstrated her anomaly. The results of magnetic resonance and selective coronary angiographic imaging were inconclusive. Surgical intervention was successfully performed on the basis of the echocardiographic diagnosis.

Congenital coronary artery abnormalities are rare and account for approximately 0.1 to 2% of congenital heart defects. They may pose significant risk of mortality or morbidity to the patient. The pediatrician and the pediatric cardiologist should be aware of their subtle but very serious presentations and diagnostic steps to be undertaken to pinpoint the diagnosis. Prevention of serious complications from these abnormalities can be achieved by making the appropriate diagnosis and performing timely surgical intervention. This review will discuss the most common congenital coronary artery abnormalities and their management.

Persistent bradycardia is an uncommon cardiac problem in fetuses but carries a high mortality in those with a ventricular rate

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.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.The echocardiograms of all patients with ABO CA were evaluated for the screening sign and compared with those of age-matched control patients.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.We concluded that the proposed screening sign for ABO CA is reliable and easily recognizable, and should prompt a comprehensive assessment of the CA.

The use of color Doppler interrogation significantly improves accuracy in the evaluation of coronary artery anatomy. This was demonstrated in a patient in whom the left coronary artery originated from the posterior and medial aspect of the pulmonary artery, a particularly difficult coronary anomaly to detect by 2-dimensional imaging. In addition to the coronary artery anomaly, this patient also had an arcade mitral valve. (J Am Soc Echocardiogr 2001;14:641-3.)

Coronary artery stenosis (CAS) and coronary ostial stenosis (COS) are potentially life-threatening conditions. The echocardiographic diagnosis of CAS and COS in children has not been described. We report on the transthoracic echocardiography (TTE) findings of CAS and COS in children. Six patients, aged 1 week to 12 years, with clinically confirmed COS (n = 5) and CAS (n = l) were diagnosed by TTE. Their echocardiographic findings were compared with 26 healthy control subjects of a similar age range. Left COS was associated with an aberrant left coronary artery (CA) from the contralateral aortic sinus (n = 2), an intramural left CA with d-transposition of the great vessels (n = l), and supravalvular aortic stenosis (n = l). Right COS was present in a patient with aortic valvular stenosis. Acquired left main CAS was diagnosed in the sixth patient 3 years after orthotopic heart transplantation. Coronary ostial stenosis was recognized when a color flow acceleration signal was present proximal to and extending into the coronary ostium (CO). Coronary artery stenosis was detected when a coarctated color flow stream was present within the stenosed CA segment with turbulent distal flow. These findings were not detected in the control cohort who demonstrated laminar CA and CO flow signals. All patients had increased spectral velocity in the CA distal to the stenosed segment (patients = 50 +/- 5 cm/s, controls = 24 +/- 6 cm/sec; P.01). Delayed peak diastolic velocity seemed to indicate severe stenosis. We conclude that (1) CO acceleration signals and turbulent coarctated CA flow signals are abnormal findings in TTE coronary Doppler assessment. They indicate COS and CAS, respectively. (2) Knowledge of the normal TTE CA flow velocity patterns is essential so that abnormal velocity signals such as seen with CAS and COS can be recognized and a timely diagnosis made.

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 ± 23 versus 24 ± 6 cm/sec, p = 0.0004), increased diastolic flow (16 ± 15 versus 6 ± 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% ± 14% versus 20% ± 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m 2 ( r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight ( p r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve. (J Am Soc Echocardiogr 1998;11:409-20.)