Aberrant origin of the left coronary artery from the right aortic sinus: surgical intervention based on echocardiographic diagnosis.

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.

Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography.

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 echocardiography of normally originating coronary arteries in children.

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/m2 (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 < 0.001, 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.

Congenital coronary artery abnormalities.

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.

Role of fetal echocardiography in the management of isolated fetal heart block with ventricular rate <55 bpm.

Persistent bradycardia is an uncommon cardiac problem in fetuses but carries a high mortality in those with a ventricular rate <55 bpm. Fetal heart block is one of the most common causes of persistent fetal bradycardia (PFB). An optimal method for assessing and monitoring cardiovascular compensation in the setting of PFB due to heart block has not been fully established. We report the application of two-dimensional and Doppler echocardiography in close monitoring of cardiac function and hemodynamics in a third-trimester fetus with a ventricular rate <55 bpm due to heart block, which assisted in successful management of the pregnancy to term. Hemodynamic and cardiac adaptive changes in compromised fetuses, particularly due to heart block, are discussed.