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AbstractThe successful diagnosis, surgical planning, and long-term care of children with transposition of the great arteries require high-quality cardiac imaging with echocardiography. Echocardiography must identify the relevant anatomic variants of transposition of the great arteries, such as of ventricular septal defects and aortic arch anomalies. Methodical and detailed imaging of the coronary arteries is particularly important, as translocation of the coronary arteries is a critical component of the arterial switch procedure. Familiarity with the potential coronary artery variants and the ideal imaging planes is essential for an echocardiographer. Knowledge of both the early and late complications following the arterial switch procedure is essential to optimise post-operative echocardiography. These complications can include residual lesions leading to haemodynamic compromise or progressive late phenomena, such as aortic root dilatation and aortic insufficiency. Echocardiography will continue to be the cornerstone to the lifelong management of transposition of the great arteries, and improvements in technology and increased familiarity with modalities such as stress echocardiography will enhance the role of advanced imaging even further.

Left ventricular noncompaction (LVNC) has rarely been described in the fetus.

In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrial septostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.

AbstractIn the past, coronary arterial anomalies have been difficult to diagnose by non-invasive methods. Identification of coronary arterial origins is now a routine part of the standard paediatric echocardiogram. Anomalous origin of a coronary artery from the pulmonary trunk is an extremely important diagnosis to make. Many echocardiographic features are not directly related to the visualisation of the coronary arterial origin. Left ventricular dilation and abnormal ventricular performance are common, along with mitral regurgitation and evidence of collateralisation of the flow from the coronary artery that has an aortic origin. In some cases, the anomalous coronary artery can be seen to arise directly from the pulmonary trunk. Congenital atresia of the main stem of the left coronary artery has a similar echocardiographic presentation, except that its aortic origin is not determined. Anomalous aortic origin of the coronary artery has important implications, as the first presenting symptom can be sudden death. With meticulous attention to the origins of the coronary arteries, echocardiographic diagnosis can also be achieved. In contrast to the anomalous origin of a coronary artery from the pulmonary trunk, ventricular performance is usually normal. Whenever there is doubt as to the definition of the origin of the coronary arteries and, indeed, when there is serious clinical concern that a coronary artery has an anomalous origin, other testing, such as cine-computed tomography, magnetic resonance imaging, or cardiac catheterisation may be indicated for confirmation or to provide greater anatomic detail.

AbstractThere is a collection of rare congenital cardiac defects that can produce significant haemodynamic embarrassment. Owing to their rarity, these lesions may be overlooked or mistaken for other more common congenital cardiac defects. Using careful segmental echocardiographic techniques, such as multiple planes of imaging and inferential Doppler findings, it is possible to identify these lesions and thus plan surgical management. Some of the lesions of importance reviewed include the aortopulmonary window, the aorto-ventricular tunnels, the aorto-atrial tunnels, and aneurysm of the sinuses of Valsalva. Some conditions such as the aortopulmonary window and aneurysm of the sinuses of Valsalva often occur in the setting of other congenital cardiac lesions. At times, the presence of coexisting lesions may mask some of the characteristic features of these defects. With prompt identification, all of these rare conditions are amenable to complete surgical repair. Newer modalities such as three-dimensional echocardiography can further enhance surgical planning.

The purpose of our study in fetal sheep was to assess the measurement agreement between fetal transesophageal echocardiography (FTEE) and conventional maternal transabdominal echocardiography (CMTFE) by the Bland–Altman method. We performed our study in 11 fetal sheep between 95–103 days of gestation (term = 145 days). FTEE was performed by imaging the fetal heart in horizontal planes utilizing a 10-F, 10-MHz intravascular ultrasound catheter. CMTFE was carried out using a 5.0-MHz phased-array transducer replicating the FTEE imaging planes. We found close agreement between FTEE and CMTFE measurements of great vessel and cardiac valvar dimensions. Conversely, the variability between both techniques for measuring ventricular dimensions was inadequate. We conclude that FTEE permits measurement of great vessel and cardiac valve dimensions with high agreement with CMTFE measurements. This finding strengthens the applicability of FTEE as a monitoring tool during experimental open or fetoscopic fetal cardiac interventions.

Summary We report here a patient with interrupted aortic arch and a right descending aorta, in whom the surgical management was complicated by the development of right bronchus compression and unilateral emphysema, due to the presence of the right ductus arteriosus.

We have presented recommendations for the optimum acquisition of quantitative two-dimensional data in the current echocardiographic environment. It is likely that advances in imaging may enhance or supplement these approaches. For example, three-dimensional reconstruction methods may greatly augment the accuracy of volume determination if they become more efficient. The development of three-dimensional methods will depend in turn on vastly improved transthoracic resolution similar to that now obtainable by transesophageal echocardiography. Better resolution will also make the use of more direct methods of measuring myocardial mass practical. For example, if the epicardium were well resolved in the long-axis apical views, the myocardial shell volume could be measured directly by the biplane method of discs rather than extrapolating myocardial thickness from a single short-axis view.

The clinical features, diagnostic studies and surgical treatment of a subvalvular mitral aneurysm of the left ventricle are described. The infant presented at 9 weeks of age with large apical ventricular septal defects and pulmonary hypertension. The subvalvular aneurysm was an incidental finding. Both lesions were treated surgically.

Diagnostic quality images of the fetal heart in utero can be obtained as early as 18 to 20 weeks of gestation. The cardiac structures can be imaged primarily by cross-sectional echocardiography and augmented by a combination of simultaneous M-mode echocardiography and range-gated pulsed Doppler ultrasonography. Cross-sectional images from planes through the fetal heart equivalent to planes that can be obtained after birth can be generated in utero. In a study of 168 pregnancies, 10 structural cardiac abnormalities have been defined. These abnormalities and others that have been reported indicate the potential for in utero cardiac diagnosis. The recognition of structural congenital heart disease in utero has been helpful in genetic counseling, planning the method of labor and delivery and making decisions regarding termination of pregnancy and planning postnatal care. Fetal echocardiography offers the potential to change the pattern of health care delivery to those with suspected congenital heart disease.

To determine which morphologic features are associated with early death, the complete echocardiograms and medical records of 16 consecutive patients with Ebstein's anomaly and concordant atrioventricular connections who presented in the fetal (n = 5) or neonatal (n = 11) period were reviewed. The cohort was classified into two groups on the basis of survival at 3 months. Group 1 consisted of seven patients who died at ≤3 months of age, and Group 2 consisted of the nine surviving patients.

The purpose of this study was to determine whether measurement of aortic blood flow velocity and acceleration by pulsed Doppler ultrasound can be used to assess the global performance of the systemic right ventricle noninvasively in young patients who have undergone intraatrial repair of aortopulmonary transposition. The effect of age at surgery on right ventricular performance in these patients was also studied. Pulsed Doppler velocity profiles of aortic blood flow were analyzed at a mean age of 5.5 years in 24 patients who had undergone intraatrial repair of aortopulmonary transposition either in early infancy (mean age 3.4 ± 1.3 weeks) or later (mean age 8.5 ± 6.5 months). Velocity and acceleration variables in these patients were compared with the same variables in 24 age-matched normal subjects and with the performance of their own right ventricle as assessed by two-dimensional echocardiographic measurement of ejection fraction.

Ten consecutive patients with a coronary artery fistula, aged 1 day to 4 years, were studied by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. All patients underwent cardiac catheterization, and seven patients had surgical closure of the fistula. The origin, course and site of drainage of the coronary artery fistula were correctly identified prospectively by echocardiographic examination in all patients. Color flow imaging was particularly helpful in visualizing the site of drainage of the fistula.

Doubly committed subarterial (supracristal, subpulmonary) ventricular septal defects are often complicated by aortic regurgitation resulting from aortic valve herniation into the defect. The clinical, echocardiographic and catheterization findings in 48 patients aged 0.3 to 46.4 years (median 9.5) with a doubly committed subarterial ventricular septal defect were reviewed. Aortic valve herniation was present in 38 (79%) and 55% of these had aortic regurgitation. The prevalence of both findings increased gradually with advancing age.

A coronary-cameral fistula was inspected clinically by two-dimensional and pulsed Doppler ultrasound. At cardiac catheterization a fistulous connection between the left coronary artery and the right ventricle was observed. Contrast echocardiography using agitated saline solution injected into the aortic catheter clearly showed the passage of microcavitations into the left and the right ventricles confirming the connection of the fistula to both chambers. Contrast echocardiography is a valuable technique that can help define the site of drainage of coronary artery fistulas.

Fetal echocardiography has allowed prenatal diagnosis of heart disease to become a reality over the last decade. Recently, pulsed Doppler flow studies have been applied to the examination of the fetal cardiovascular system. In fetuses with dysrhythmias, complex structural heart malformations, nonimmune hydrops fetalis, or mothers receiving nonsteroidal prostaglandin synthetase inhibitors, pulsed Doppler echocardiographic studies are clinically indicated. These studies involve noninvestigational tests that provide information of critical clinical import. These studies may occasionally, require energy intensities that exceed the 510(k) limits. The physician should be held responsible for educating patients to the potential risks versus benefits of any imaging study they undergo, but appending potentially alarming labels to transducers, machines, or cathode-ray tube displays can only serve to undermine the fabric of the physician-patient relationship without achieving the desired goal of protection of the patient.

Thirty-one children with congenital heart disease were examined for tricuspid regurgitation by four methods: (1) auscultation, (2) pulsed Doppler echocardiography, (3) saline contrast echocardiography, and (4) right ventricular angiography. Tricuspid regurgitation was detected in three children by auscultation, in 20 by pulsed Doppler echocardiography, in 21 by saline contrast echocardiography, and in 20 by right ventricular angiography. To determine the prevalence of tricuspid regurgitation in children suspected of having congenital heart disease, we reviewed 5417 Doppler echocardiograms performed between 1983 and 1985. Tricuspid regurgitation was detected in 399 of 4670 children (8.5%) with congenital heart disease and in 26 of 106 newborns (25%) with respiratory distress. By comparison, tricuspid regurgitation was detected in only 19 of 641 (3%) normal children. Tricuspid regurgitation is uncommon in normal children, but its incidence in children with congenital heart disease and/or respiratory distress is high.

With color Doppler flow mapping (CFM), we studied 16 children with total anomalous pulmonary venous connection (TAPVC), which was confirmed at cardiac catheterization, surgery, or autopsy in all but one case. The drainage was supracardiac in nine children, cardiac in four, and infracardiac in three. Obstruction to pulmonary venous return was present in seven children. Increased variance, reflecting disturbed blood flow, as well as increased velocities and aliasing were present in all patients. In patients without obstruction turbulence was present in the right atrium and throughout the common pulmonary venous structures. In patients with obstruction a discrete site of increased turbulence and velocity was identified at the site of obstruction. CFM allows rapid differentiation between normal and abnormal venous and arterial structures in TAPVC. In patients suspected of having TAPVC with obstruction, CFM complemented by pulsed Doppler facilitates the determination of the site of obstruction. CFM allows a more rapid appreciation of the anatomy in TAPVC than can be achieved by two-dimensional imaging alone.

New Doppler ultrasound technology has caused a change in the approach to the diagnosis of congenital heart disease. Pulsed Doppler ultrasound continues to be of great value for measuring cardiac output ventricular performance and for calculating pulmonary to systemic flow ratios. Continuous wave Doppler ultrasound by virtue of its ability to detect high velocity accurately remains the premier technique for determining pressure drop across valves or across shunts. Pulsed Doppler ultrasound remains a valuable test for defining many lesions including patent ductus arteriosus.

Two-dimensional echocardiography of cardiac situs, connections, and malpositions from a variety of planes is discussed.

Saline contrast echocardiography was performed in 889 children from June 1976 through February 1988. One-third of these studies were in postoperative patients. A patent foramen ovale was identified by finding right to left shunting on venous contrast injection in 37% of 127 children studied with a structurally normal heart. The incidence of such shunting was greater at younger ages (55% younger than 1 month versus 22% older than 1 month). In most patients with an atrial or ventricular septal defect, some right to left shunting was demonstrable. The technique was useful in distinguishing different forms of atrial septal defect and identifying muscular ventricular septal defects that were difficult to image directly. The technique was used in the catheterization laboratory to aid in the identification of congenital coronary artery fistulas and was diagnostic in two cases of pulmonary arteriovenous malformation. In patients with situs abnormalities, the technique was useful in identifying the systemic venous connections to the atria.

Changes in the Doppler indexes, acceleration time (AcT), right ventricular ejection time (RVET), AcT/RVET ratio and pulmonary artery peak velocity were measured as were changes in pulmonary artery pressure and pulmonary vascular resistance in 21 children with pulmonary hypertension due to a large interventricular communication. In 11 children pulmonary vascular resistance was >4.6 U/m2(mean 8.6 ± 1.6), whereas in 10 it was <4.5 U/m2(mean 3.4 ± 0.2). Although both groups demonstrated acceleration time and AcT/RVET values above normal, there were no significant differences in these values between the groups with high and low pulmonary vascular resistance. With administration of a pulmonary vasodilator pulmonary vascular resistance decreased in 11 responders by >50% of baseline values (from 5.3 ± 0.7 to 1.6 ± 0.3 U/m2), whereas in the 10 nonresponders mean pulmonary vascular resistance decreased from 7.0 ± 1.9 to 4.9 ± 1.1 U/m2. There was no significant change in the Doppler indexes except for an increase in pulmonary artery peak velocity in the responders from 1.34 ± 0.07 to 1.66 ± 0.06 m/s (p < 0.001).

Background:Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart complex incapable of supporting an entire cardiac output or in patients with complicated anatomy. Methods:From July 1992 to April 1997, 23 patients (median age 5.2 years) underwent partial biventricular repair. In 15 of these cases the entire repair, including bidirectional cavopulmonary anastomosis, intracardiac repair, and right ventricular outflow reconstruction, was performed as a planned procedure at our institution. The other eight patients had previously been placed on a Fontan track and had undergone bidirectional cavopulmonary anastomosis; their circulations were converted to a partial biventricular circulation. Results:There were no early deaths. Complete atrioventricular block developed in two patients with straddling tricuspid valve. At a median follow-up of 17 months, there were no late deaths and three patients had undergone reintervention. Partitioning of the pulmonary arteries to create a classic Glenn anastomosis with antegrade flow to the left lung was performed in one case; another patient underwent an atrial septectomy, and the third patient required revision of tricuspid valve repair. All patients are in New York Heart Association functional class I. Conclusions:Partial biventricular repair is a versatile strategy that can be used to manage a variety of forms of complex congenital heart disease. Cases for which the repair is useful include those in which complete biventricular repair is unlikely to be achieved because of limited size or function of the right side of the heart and those in which a patient with a ventricle capable of supporting inferior vena caval return was previously placed on a Fontan track. (J Thorac Cardiovasc Surg 1998;116:21-7)