Magnetic resonance imaging at a high field strength of ventricular septal defects in infants

Magnetic resonance imaging at a high field strength has potential benefits for the study of the heart in infants, which is when most congenital heart disease presents. Seventeen infants with various anatomical types of ventricular septal defect were studied by this technique. Good quality, high resolution, images were obtained in every case. There were no major practical problems. The morphology of the defects in all 17 hearts was displayed in great detail. In some instances, the interpretation of the images resembled that of equivalent images from cross sectional echocardiography. But this new technique allowed imaging in planes that cannot be obtained by echocardiography. One particularly valuable plane gave a face on view of the inlet and trabecular components of the septum. This allowed very precise localisation of defects in these areas. The relation between the defects and the atrioventricular and arterial valves was exceptionally well shown in various different imaging planes. One patient in the series had multiple trabecular defects that were clearly shown. Magnetic resonance imaging gives detailed morphological information about ventricular septal defects.     

Magnetic resonance imaging at a high field strength of ventricular septal defects in infants.

Magnetic resonance imaging at a high field strength has potential benefits for the study of the heart in infants, which is when most congenital heart disease presents. Seventeen infants with various anatomical types of ventricular septal defect were studied by this technique. Good quality, high resolution, images were obtained in every case. There were no major practical problems. The morphology of the defects in all 17 hearts was displayed in great detail. In some instances, the interpretation of the images resembled that of equivalent images from cross sectional echocardiography. But this new technique allowed imaging in planes that cannot be obtained by echocardiography. One particularly valuable plane gave a face on view of the inlet and trabecular components of the septum. This allowed very precise localisation of defects in these areas. The relation between the defects and the atrioventricular and arterial valves was exceptionally well shown in various different imaging planes. One patient in the series had multiple trabecular defects that were clearly shown. Magnetic resonance imaging gives detailed morphological information about ventricular septal defects.     

Double outlet right ventricle. Cross sectional echocardiographic findings, their anatomical explanation, and surgical relevance.

The precise method of surgical repair of double outlet right ventricle depends both on the relation of the interventricular communication to the cardiac outlets and on the course and insertion of the atrioventricular valve tension apparatus. It may be difficult to connect the interventricular communication with one or other outlet or both either because the interventricular communication is too far from the outlets or because atrioventricular tension apparatus interposes between them. This study was carried out in order to establish whether these details could be recognised preoperatively using cross sectional echocardiography. Forty two echocardiograms were reviewed retrospectively from patients with double outlet right ventricle, excluding those with atrioventricular septal defects and atrioventricular discordance. Ten further such patients were studied prospectively. The diagnosis was confirmed at open heart surgery in 19 patients. The relation of the great arteries and their outlet tracts to each other and to the interventricular communication was readily and accurately predicted. Four patients (7.7%) had no infundibular septum. The remaining 48 had such a septum. In 27 (52%) the interventricular communication was overridden by a great artery. In 14 (27%) it was roofed by the ventriculoinfundibular fold, and in 11 (21%) it was confined to the inlet or trabecular septa. The insertion of chordae tendineae limited the possible surgical options in 12 patients (23.1%) who were distributed unpredictably among the above groups. Four patients had straddling atrioventricular valves. In five, tricuspid tension apparatus inserted into the underside of the infundibular septum and, in two, into the roof of the defect. In one patient the mitral valve inserted into the defect floor. Tricuspid tension apparatus inserted into the floor of the defect in a further nine patients, but this does not compromise surgery. Thus in double outlet right ventricle cross sectional echocardiography can provide unique information necessary for planning of rational surgical management.     

Cross sectional echocardiographic diagnosis of total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection can be diagnosed by cross sectional echocardiography. Information is, however, lacking concerning the diagnostic accuracy of this imaging method and any factors which may influence it. To predict the pulmonary venous connection 463 patients with congenital heart disease who had angiographic confirmation were prospectively examined. Total anomalous pulmonary venous connection was present in 34 (7%) patients and correctly detected in 33 (97% sensitivity). There were two false positive results (99% specificity). All 23 patients with atrial situs solitus with or without associated congenital heart defects were correctly detected. One false negative result occurred in a patient with right atrial isomerism and complex congenital heart disease with decreased pulmonary blood flow. Diagnosis of the type of total anomalous pulmonary venous connection, including the site and other anatomical details, was analysed and was correct in 24 of 34 (71%) patients. Errors included incorrect prediction of the site of total anomalous pulmonary venous connection in five patients with right atrial isomerism, atrioventricular canal defect, and pulmonary atresia, details of confluence interconnection in three of four patients with the mixed type of connection, undiagnosed pulmonary venous obstruction in three of the patients with right atrial isomerism, and failure to predict common pulmonary vein atresia in one patient. Factors which were related to incorrect echocardiographic diagnosis were abnormal atrial situs, mixed total anomalous pulmonary venous connection, and associated congenital cardiac defects, whereas age, weight, sex, clinical condition, and time during the study were not related. It is concluded that cross sectional echocardiography can be used to diagnose accurately total anomalous pulmonary venous connection. This method can be the definitive imaging and diagnostic method in symptomatic infants with total anomalous pulmonary venous connection who have atrial situs solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defect.     

Cross sectional echocardiographic diagnosis of total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection can be diagnosed by cross sectional echocardiography. Information is, however, lacking concerning the diagnostic accuracy of this imaging method and any factors which may influence it. To predict the pulmonary venous connection 463 patients with congenital heart disease who had angiographic confirmation were prospectively examined. Total anomalous pulmonary venous connection was present in 34 (7%) patients and correctly detected in 33 (97% sensitivity). There were two false positive results (99% specificity). All 23 patients with atrial situs solitus with or without associated congenital heart defects were correctly detected. One false negative result occurred in a patient with right atrial isomerism and complex congenital heart disease with decreased pulmonary blood flow. Diagnosis of the type of total anomalous pulmonary venous connection, including the site and other anatomical details, was analysed and was correct in 24 of 34 (71%) patients. Errors included incorrect prediction of the site of total anomalous pulmonary venous connection in five patients with right atrial isomerism, atrioventricular canal defect, and pulmonary atresia, details of confluence interconnection in three of four patients with the mixed type of connection, undiagnosed pulmonary venous obstruction in three of the patients with right atrial isomerism, and failure to predict common pulmonary vein atresia in one patient. Factors which were related to incorrect echocardiographic diagnosis were abnormal atrial situs, mixed total anomalous pulmonary venous connection, and associated congenital cardiac defects, whereas age, weight, sex, clinical condition, and time during the study were not related. It is concluded that cross sectional echocardiography can be used to diagnose accurately total anomalous pulmonary venous connection. This method can be the definitive imaging and diagnostic method in symptomatic infants with total anomalous pulmonary venous connection who have atrial situs solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defect.     

Assessment by cross sectional echocardiography of surgical "mitral valve" disease in children and adolescents

The anatomy of the left atrioventricular valve, a mitral valve unless there is atrioventricular discordance, was determined by cross sectional echocardiography in 15 young patients with congenital lesions and seven with rheumatic lesions. These results were compared with findings at operation. The preoperative diagnosis was accurate in 18 (80%). In the remaining four patients inaccurate echocardiographic diagnosis was due to the mistaken identification of clefts in redundant and multicuspid valves and of absent chordae that were thought to be ruptured chordae. In four patients a subvalvar abnormality was identified by echocardiography. With care, cross sectional echocardiography was a reliable method of defining abnormal anatomy in serious mitral disease and it predicted the need for replacement or the possibility of repair. In the absence of additional lesions invasive investigation was unnecessary.     

Assessment by cross sectional echocardiography of surgical "mitral valve" disease in children and adolescents.

The anatomy of the left atrioventricular valve, a mitral valve unless there is atrioventricular discordance, was determined by cross sectional echocardiography in 15 young patients with congenital lesions and seven with rheumatic lesions. These results were compared with findings at operation. The preoperative diagnosis was accurate in 18 (80%). In the remaining four patients inaccurate echocardiographic diagnosis was due to the mistaken identification of clefts in redundant and multicuspid valves and of absent chordae that were thought to be ruptured chordae. In four patients a subvalvar abnormality was identified by echocardiography. With care, cross sectional echocardiography was a reliable method of defining abnormal anatomy in serious mitral disease and it predicted the need for replacement or the possibility of repair. In the absence of additional lesions invasive investigation was unnecessary.     

Acquired abnormalities of the tricuspid valve--an ultrasonographic study

In a retrospective ultrasonographic study, 32 cases of acquired disease of the tricuspid valve were detected amongst 7000 consecutive patients. Patients with congenital heart disease (except when the seat of an acquired disease) and with prosthetic heart valves, were excluded. There were twenty-one cases of rheumatic disease, all having additional involvement of the mitral valve. Prolapse (5 patients), bacterial endocarditis (2 patients), rupture of papillary muscle (1 patient), cardiac tumours (2 patients) and carcinoid heart disease (1 patient) were also identified. Acquired disease of the tricuspid valve is infrequently encountered during routine cross-sectional echocardiography but its recognition is clinically important.     

Cross sectional echocardiography for recognition of ventricular topology in atrioventricular septal defect.

The cross sectional echocardiograms of 150 children with atrioventricular septal defects were reviewed to determine the spatial orientation of the ventricles. In 125 cases with usual atrial arrangement (situs solitus), ventricular topology showed the right hand pattern. Of the 25 patients with atrial isomerism, 13 had similar right hand topology but 12 (48%) had the left hand pattern. This finding was more common in hearts in the right side of the chest (dextrocardia), but there was no significant correlation between ventricular topology and number of atrioventricular valves or the ventriculoarterial connection. In part because of inferences for disposition of the conducting tissue, knowledge of ventricular topology is important when operation for atrioventricular septal defects is considered. Ventricular topology can be promptly recognised by cross sectional echocardiography.     

Cross sectional echocardiography for recognition of ventricular topology in atrioventricular septal defect

The cross sectional echocardiograms of 150 children with atrioventricular septal defects were reviewed to determine the spatial orientation of the ventricles. In 125 cases with usual atrial arrangement (situs solitus), ventricular topology showed the right hand pattern. Of the 25 patients with atrial isomerism, 13 had similar right hand topology but 12 (48%) had the left hand pattern. This finding was more common in hearts in the right side of the chest (dextrocardia), but there was no significant correlation between ventricular topology and number of atrioventricular valves or the ventriculoarterial connection. In part because of inferences for disposition of the conducting tissue, knowledge of ventricular topology is important when operation for atrioventricular septal defects is considered. Ventricular topology can be promptly recognised by cross sectional echocardiography.     

Real-time three-dimensional transthoracic echocardiography

BACKGROUND: Complex anatomy of intra-cardiac structures requires spatial orientation of image in three dimensions for better understanding and enhanced image interpretation. We evaluated the feasibility and efficacy of the emerging 'real-time three-dimensional transthoracic echocardiography' technique for comprehensive assessment of cardiac anatomy, physiology, pathomorphology and pathophysiology in patients with structural heart disease. METHODS AND RESULTS: Patients with structural heart disease (n=152) were evaluated by conventional two-dimensional transthoracic echocardiography and real-time three-dimensional transthoracic echocardiography using standard protocol. Fifty-six cases were of rheumatic etiology with multi-valvular involvement (mitral stenosis: 32; mitral regurgitation: 29; tricuspid regurgitation: 8; aortic valve disease: 11) and 21 cases of non-rheumatic valvular heart disease. A total of 38 congenital heart disease patients were examined including 23 patients with atrial septal defect. Left ventricular function (n=20) and right ventricular function (n=10) were also assessed using dedicated software. CONCLUSIONS: Results of real-time three-dimensional transthoracic echocardiography mitral valve area assessment by planimetery are comparable to two-dimensional transthoracic echocardiography with additional information about surface anatomy of leaflets and the subvalvular apparatus in real time with clear demarcation of commissural fusion and scallops of leaflets. Enface view of atrial septal defect with direct visualization of shape, size and number of defects, tricuspid valve area by planimetery, right ventricular shape, objective assessment of ventricular volumes and regurgitation vena contracta area are the fields where three-dimensional transthoracic echocardiography was of additive value to conventional two-dimensional transthoracic echcardiography. This study proves clinical feasibility of real-time three-dimensional transthoracic echocardiography but requires further validation of quantitative observations.     

Cross sectional echocardiographic feature in carcinoid heart disease. A mechanism for tricuspid regurgitation in this syndrome

In a patient with severe tricuspid regurgitation and mild pulmonary stenosis due to carcinoid heart disease cross sectional echocardiography showed nodular thickening and coaptation of the tricuspid leaflets at the beginning of systole. The leaflets were, however, seen to be increasingly pulled apart as right ventricular systole proceeded. This finding, which is probably due to traction on the leaflets by the thickened chordae tendineae, is therefore a mechanism of valvular incompetence, perhaps accounting for the particularly severe tricuspid regurgitation seen in carcinoid heart disease.     

Echokardiographische Diagnostik angeborener Herzfehler im Erwachsenenalter

Echo and Doppler echocardiographic procedures have gained special importance in the diagnostics of congenital diseases in adults. These procedures permit detailed visualization of the pathomorphology of the heart as well as reliable evaluation of the hemodynamic changes. There are differentiated indications for the various procedures, such as transthoracic and transesophageal echocardiography, Doppler and color-Doppler echocardiography, contrast echocardiography and 3-dimensional echocardiography. This article discusses the opposition of the various echo and Doppler echocardiographic procedures with respect to the diagnostics of the most frequent non-operated congenital diseases in adults. The pathomorphology of the various congenital diseases will be summarized and then the important echocardiographic criteria presented which are decisive for the diagnostic procedure. In simple congenital malformation of cardiac valves, such as bicuspid aortic valve (Figure 1: aortic ring abscess), pulmonary valve stenosis (Figure 2), Ebstein's anomaly (Figure 3) or malformations of the mitral valve (Figure 4: cleft in the anterior mitral cusp), the diagnosis can often be made using transthoracic echo and Doppler echocardiography, and the severity of the defect determined. However, the sonographic conditions, especially in adults, are frequently too limited to permit recognition of detailed smaller changes, so that transesophageal examination is required to finally confirm the diagnosis in these patients. In the diagnostics of diseases of the left ventricular outflow tract and the thoracic aorta, such as subvalvular aortic valve stenosis (Figure 5), the sinus of Valsalva aneurysm or the coarctation of the aorta (Figure 6), the left ventricular outflow tract can be evaluated morphologically from a transthoracic procedure and the accelerations of flow can be recorded by continuous wave Doppler. If there is no sclerosis of the fibrous membrane, these can often not be depicted by transthoracic procedures, so that a supplementary transesophageal examination is meaningful. This is required in any case for diseases of the descending thoracic aorta. In the case of congenital lesions, such as atrial septal defects (Figure 7: anomalous pulmonary venous return, Figure 8: 3-dimensional visualization of an atrial septal defect, Figure 9: sinus venosus defect), ventricular septal defect or a patent ductus arteriosus Botalli (Figure 10), color-Doppler and contrast echocardiography have become especially important. Transesophageal examination is also indicated for these congenital diseases for direct depiction of the defect as well as for precise evaluation of the shunt. Moreover, in atrial septal defects, it has been shown that a 3-dimensional echocardiography provides additional advantage with respect to spatial relationship of the defect to the other cardiac structures, as well as presenting dynamic changes during a heart cycle. Extensive knowledge of complex congenital heart disease, such as tetralogy of Fallot (Figure 11), complete transposition of the great arteries, congenitally corrected transposition of the great arteries (Figure 12), the double-outlet right ventricle, truncus arteriosus communis, the cor triatriatum, tricuspid atresia (Figure 13) or the univentricular heart (Figure 14) usually requires performance of a transthoracic echo- and Doppler echocardiographic examination to assess the pathomorphological changes and to examine hemodynamics. In the majority of patients, supplementary transesophageal echocardiography and an echo contrast examination are important. Initial examinations using 3-dimensional echocardiography are very promising in this connection and with respect to the exact spatial presentation of pathoanatomical structures.     

Cross sectional echocardiographic feature in carcinoid heart disease. A mechanism for tricuspid regurgitation in this syndrome.

In a patient with severe tricuspid regurgitation and mild pulmonary stenosis due to carcinoid heart disease cross sectional echocardiography showed nodular thickening and coaptation of the tricuspid leaflets at the beginning of systole. The leaflets were, however, seen to be increasingly pulled apart as right ventricular systole proceeded. This finding, which is probably due to traction on the leaflets by the thickened chordae tendineae, is therefore a mechanism of valvular incompetence, perhaps accounting for the particularly severe tricuspid regurgitation seen in carcinoid heart disease.     

Profile of right-sided endocarditis: an Indian experience

The clinical profile of right-sided infective endocarditis in India was studied from a review of records of patients with infective endocarditis admitted to this hospital. From November 1982 to November 1989, 109 patients with infective endocarditis showed vegetations on cross-sectional echocardiography confirming the diagnosis of infective endocarditis. In 19 (17.4%) patients, only the right side of the heart was involved: specifically the tricuspid valve alone in 10; tricuspid and pulmonary valves in 4; tricuspid valve and right ventricular outflow tract in 1; tricuspid valve and right ventricular free wall in 1; pulmonary valve alone in 2; and bifurcation of pulmonary trunk in 1. Eleven patients (57.9%) had underlying congenital heart disease whereas the remaining 8 patients (42.1%) did not have any underlying heart disease. The latter group, therefore, had isolated right-sided infective endocarditis. Previous illnesses leading to isolated right-sided infective endocarditis were: puerperal sepsis in 4; septic abortion in 1; staphylococcal pneumonia in 2; and epididymoorchitis in one. Eight out of 11 patients with congenital heart disease did not report any previous illness. In the remaining 3, right-sided endocarditis followed cardiac surgery in one; dental extraction without prophylaxis in one; and pulmonary balloon valvoplasty in one. All patients with isolated right-sided infective endocarditis had features of septicaemia, but a murmur of tricuspid regurgitation was audible in only 4 (50%) of them. We conclude that, unlike western reports, the pattern of right-sided infective endocarditis in India is different. No drug addict with right-sided infective endocarditis was seen; puerperal sepsis and septic abortion were the commonest causes of isolated right-sided infective endocarditis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Live 3D echocardiography with the pediatric matrix probe

Three-dimensional echocardiography (3DE) enables new views of heart valves and the septa to be imaged. While the previous 3DE system was cumbersome, the recent introduction of live 3DE allowed for routine use of the technique in adult patients. Here, we report our initial experiences in adapting live 3DE and the adult matrix probe to the pediatric population. Thirty-four 3DE examinations were performed on children, aged 1 day to 12 years (n = 23; median 4 years) and fetuses 20-33 weeks in gestation (n = 11; median 25 weeks), many of whom had various congenital heart diseases. The pediatric matrix probe (2-7 MHz) was used for 2D, Doppler, and 3DE. New modalities of the Vision 2007 (Philips) were applied: live, full volume, thick slice, 3D color Doppler, the QLAB system for navigation, and cropping. The pediatric matrix probe allows for complete 2D and 3D echocardiography, and new acoustic windows are now available to perform live 3DE. The higher frequency of the probe increases the 3D image resolution obtained in neonates and fetuses. This advancement allows new views of the aorta, pulmonary valve, septa and intra cardiac anatomy to be captured. Real time 3DE is a feasible method in addition to conventional 2D echocardiography for evaluating congenital heart disease.     

Real time two dimensional echocardiography in constrictive pericarditis

Real time two dimensional echocardiography was performed in five patients with constrictive pericarditis. The echocardiogram showed several typical features: (1) The heart was normal in size or decreased; the ventricles were small and the atria enlarged. (2) The pericardium was seen as a single or double dense, rigid shell surrounding both ventricles and the apex; its immobility was striking. (3) The inferior vena cava and hepatic veins were greatly dilated in keeping with the severe clinical venous congestion. (4) Both interventricular and interatrial septa bulged into the left side of the heart on inspiration. (5) The atrioventricular valves were hypermobile. (6) A prominent ventricular diastolic filling halt was seen. The two dimensional echocardiogram excluded other causes of severe cardiac failure such as congestive cardiomyopathy and valvular heart disease, while the dense immobile pericardial echo or echoes and the “normal” texture of the myocardial echoes made the diagnosis of restrictive cardiomyopathy unlikely.     

Cross sectional subcostal echocardiography: atrioventricular septal defects and the short axis cut

A short axis echocardiographic cut of the heart from the subcostal approach was used to study the atrioventricular junction in 47 infants and children with congenital heart disease and 20 with normal hearts. Examination of the diastolic openings of both atrioventricular valves was able to establish normal developments of the valves and annuli even when this was found in cases of complex congenital heart disease. In 30 patients with atrioventricular septal defects the technique distinguished between a partial defect (when the two atrioventricular valves were linked transseptally) and a complete defect (when there was only one atrioventricular valve). A range of atrioventricular attachments was seen in these patients. Short axis echocardiography from the subcostal approach reliably identifies different forms of atrioventricular septal defects by defining the anatomy of the atrioventricular valves during maximal diastolic expansion.     

Cross sectional subcostal echocardiography: atrioventricular septal defects and the short axis cut.

A short axis echocardiographic cut of the heart from the subcostal approach was used to study the atrioventricular junction in 47 infants and children with congenital heart disease and 20 with normal hearts. Examination of the diastolic openings of both atrioventricular valves was able to establish normal developments of the valves and annuli even when this was found in cases of complex congenital heart disease. In 30 patients with atrioventricular septal defects the technique distinguished between a partial defect (when the two atrioventricular valves were linked transseptally) and a complete defect (when there was only one atrioventricular valve). A range of atrioventricular attachments was seen in these patients. Short axis echocardiography from the subcostal approach reliably identifies different forms of atrioventricular septal defects by defining the anatomy of the atrioventricular valves during maximal diastolic expansion.     

An echocardiographic study of perimembranous ventricular septal defect with left ventricular to right atrial shunting

Twenty nine patients with isolated perimembranous ventricular septal defects were investigated by M mode, cross sectional, and pulsed Doppler echocardiography. Tricuspid valve anomalies were present in all six patients with a left ventricular-right atrial shunt but in only six (26%) of 23 patients who had interventricular shunts only. Systolic flutter of the tricuspid valve was shown in five (83%) of the six patients with a ventriculoatrial shunt but not in the other patients. Systolic turbulence in both the right ventricle and right atrium was detected by Doppler echocardiography only in patients with ventriculoatrial shunting. A perimembranous ventricular septal defect with left ventricular to right atrial shunt can be diagnosed by its combined M mode, cross sectional, and pulsed Doppler echocardiographic features.