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A magnetic resonance imaging of the heart was obtained in a patient with history of mitral valve replacement, in whom cor triatriatum dexter was suspected. Dense left atrial echo-contrast, visualized during a previous transesophageal echocardiography, was incorrectly interpreted as a large thrombus extending into the pulmonary veins and the patient was referred for cardiac surgical evaluation.
A review of 34 autopsied cases with classical Ebstein's malformation of the tricuspid valves revealed 8 cases with left-sided anomalies. Among these, appearing in 1 case each, were aortic atresia and persistent common atrioventricular canal. The latter 2 conditions were dominant clinically. In the remaining 6 cases the left-sided anomalies were not apparent clinically and probably of no functional significance. These conditions were parachute mitral valve, bicuspid aortic valve, cor triatriatum and pulmonary stenosis, cleft mitral valve, stenosis of individual pulmonary veins, and prolapse of mitral valve.
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.
Due to limitations in transthoracic and occasionally transesophageal 2-D echocardiography with respect to volumetric analysis and morphologic and functional assessment in patients with congenital malformations and valvular heart disease, additional diagnostic tools have been established. In parallel with the rapid evolution in computer technology, 3-D echocardiography has grown into a well-developed technique, such as volume-rendered 3-D reconstruction, capable of displaying dynamic morphology depicting depth of the structures, their attachment, and spatial relation to the surrounding tissue. Nevertheless, the complexity of data acquisition and data processing required for adequate dynamic 3-D echocardiographic imaging and volumetric analysis does not allow to use this approach routinely. The commonly used dynamic 3-D echocardiography means off-line computer-assisted image reconstruction from a series of cross-sectional echocardiographic images using currently available transesophageal and transthoracic transducers. Alternatively, real-time 3-D echocardiography based on novel matrix, phased-array transducer technology has been introduced. Although this technique can be easily combined with any routine examination, its clinical use is limited because of a lower image quality in comparison with dynamic 3-D echocardiography. Up to now, there is no transesophageal approach available using real-time 3-D echocardiography. Recently, dynamic 3-D echocardiographic technique has matured noticeably. Beside the well-known sequential scanning, which is characterized by a fixed probe and patient in space and predetermined motion of the transducer, the freehand scanning using an electromagnetic location system has found its way to clinical environment. The main advantage of this technique is that the transducer can be freely moved by the examiner and, thus, the data set acquired within a routine examination. Also 3-D rendering and display have been developed further. In this respect, especially the "real-time rendering mode" allowing the reconstructed 3-D image to be animated and moved in space and to look at it from different perspectives has gained increasing acceptance. In valvular heart disease, reconstructive surgical treatment is aspired. 3-D echocardiographic imaging is the only technique providing "surgical views" prior to opening the heart. It is capable of distinguishing particular destructive substructures of the valves and the valvular apparatus. Especially in mitral valvular reconstruction, it is of clinical importance to achieve optimal surgical results. With respect to volumetric and mass analysis, 3-D echocardiography is more accurate and reproducible in comparison with conventional 2-D analysis. It provides data independent of geometric assumptions, what may considerably influence the results in the presence of wall motion abnormalities, especially in aneurysmatic ventricles. Volumetric analysis of the aneurysmal portion may also be helpful prior to surgical resection. 3-D echocardiography can also be recommended as a valuable additional approach to atrial septal defect (ASD), corrected transposition of the great arteries, cor triatriatum, and, within limits, to ventricular septal defect (VSD) as well. Especially with respect to ASD and VSD, the potential significance of 3-D echocardiography prior to device closure is emphasized. At present, its additional information in decision-making and the increasing number of clinical cases that can be addressed and answered already justify the clinical use of this technique.
The purpose of this study was to determine the sensitivity and specificity of echocardiography for the diagnosis of congenital cardiac abnormalities with multiple lesions. The study was carried out on 80 patients (ages 1 day to 14 years). After clinical evaluation all patients were studied by echocardiography. Cardiac catheterization and angiocardiography were performed, and echocardiographic findings were compared with those obtained by cardiac catheterization. The sensitivity and specificity of echocardiographic diagnosis were determined based on the false-negative and false-positive results. Of the 80 patients, 19 had double-outlet right ventricle, 17 transposition of the great arteries, 10 common atrium, 9 atrioventricular septal defect, 7 single ventricle, 7 corrected transposition, 6 tricuspid atresia, 3 Ebstein's anomaly, and 2 cor triatriatum. All of these entities were visualized correctly by echocardiography (sensitivity 100%, specificity 100%). There were also 12 instances of atrial isomerism with one false-negative diagnosis, 6 pulmonary atresia with two false-negative diagnoses, and 5 total anomalous pulmonary venous connections with one false-negative diagnosis. The total number of individual cardiac lesions was 291. Nineteen false-negative and four false-positive echocardiographic diagnoses were obtained (sensitivity 93%, specificity 99%). It is concluded that double-outlet right ventricle, transposition of the great arteries, atrioventricular septal defect, single ventricle, corrected transposition, and tricuspid atresia can be accurately diagnosed by echocardiography. However, the role of echocardiography is limited for evaluation of right ventricular outflow tract and small patent ductus arteriosus, especially when associated with pulmonary hypertension.
The following is a case report of a 1-month-old patient who developed adverse hemodynamic sequelae during the use of nitric oxide (NO) in the postoperative period for pulmonary hypertension after correction of total anomalous pulmonary venous return. At the time of diagnosis, the patient had evidence of systemic right ventricular pressures estimated by continuous-wave Doppler. He was sedated and paralyzed for hyperventilation in preparation for surgery and underwent pulmonary vein confluence to left atrial anastomosis. Postoperative pulmonary hypertension was managed by hyperventilation, sedation, and paralysis until a sudden onset of systemic-level pulmonary pressure required NO therapy. Satisfactory results were obtained in minutes, but a rebound pulmonary hypertension occurred with concomitant systemic hypertension and no radiographic changes. We suspected left atrial hypertension secondary to a sudden increase in pulmonary blood flow to an noncompliant left ventricle. Discontinuation of NO resulted in stabilization of the hemodynamic profile of the patient and he continued to be managed with paralysis, hyperventilation, and sedation. Based on this experience we suggest that NO should be used with caution in patients with obstructive lesions at the atrial level prior to surgery (mitral valve stenosis and cor triatriatum) or in patients with a poorly compliant left ventricle (cardiomyopathy and left ventricular dysfunction). These entities are unable to tolerate a sudden increase in pulmonary blood return thus creating paradoxical pulmonary hypertension.
Eighty-seven adult patients with congenital cardiopathy were studied by using transesophageal echocardiography (TEE) over a period of 30 months. Transthoracic echocardiography was practiced on all patients, after which TEE with monoplanar and biplanar probes (74 and 15 patients, respectively) was used to confirm principal diagnoses and determine specific information. TEE findings were compared with those of cardiac catheterization in all 87 cases and with those of surgery in 15 cases. Dextrocardia was found in 11 cases and mesocardia in 2. Situs inversus was demonstrated in 6 and levoisomerism in 4. Intracardiac and extracardiac shunts were diagnosed in 20 cases, Ebstein's anomaly in 27, corrected transposition of great vessels in 15, univentricular atrioventricular connection in 7, cor triatriatum in 1, parachute mitral valve in 1, crisscross heart in 1, and double-outlet left atrium in 1. TEE was of particular value in evaluating total anomalous pulmonary venous connection, common atrioventricular canal, Ebstein's anomaly, corrected transposition of great vessels, and univentricular atrioventricular connection and in diagnosis of the rarer congenital cardiopathies.
Three-dimensional transesophageal echocardiography is a new and evolving cardiac imaging technique. We reported our experiences of its clinical applications in 59 patients. A series of special temporal longitudinal views were selected by the frame grabber. Then the computer connected each digitized endocardial surface of the longitudinal views according to their spatial position and reconstructed the three-dimensional, cardiac shaded picture with gray scale. The three-dimensional transesophageal echocardiographic images were divided into three areas. The right area was right anterior to the esophagus and included such structures as the superior vena cava, right atrium, interatrial septum, and left atrium; the size, shape, and location of an atrial septal defect could be clearly shown. In the middle area the origin and the course of the two great arteries could be visualized, thus facilitating the diagnosis of transposition of the great arteries; in patients with obstruction of the right ventricular outflow tract, the circular ridgelike narrowing in the right ventricle was clearly visualized. In the left area the contour and size of the left ventricle and left atrium and the shape and point of coaptation of the mitral valve could be demonstrated; in patients with mitral valve prolapse, part of either leaflet protruded into the left atrium and appeared as a spoonlike depression in the mitral valve. Other entities subjected to three-dimensional transesophageal echocardiographic reconstruction included cor triatriatum, left atrial myxoma, aneurysm of sinus of Valsalva, dissecting aortic aneurysm, mitral stenosis, mitral regurgitation, and mitral valve prolapse.(ABSTRACT TRUNCATED AT 250 WORDS)
During precatheterization M mode echocardiographic examination, 33 infants were identified as having findings including right ventricular enlargement and presence of a line within the left atrial portion of the M mode tracing suggesting the possibility of cor triatiatum or total anomalous pulmonary venous return. Pulsed Doppler echocardiography was used to sample blood characteristics on either side of the left atrial line to determine which line was artifactual and which was indicative of an important structure. Five patients whose blood flow characteristics were different on either side of the left atrial line were subsequently proved to have total anomalous pulmonary venous return at cardiac catheterization. In the remaining infants blood flow characteristics were identical on either side of the line, and catheterization excluded total anomalous pulmonary venous return and cor triatriatum. In all patients who had total anomalous pulmonary venous return, drainage involved a persistent left superior vena cava, and this vascular structure was identified by pulsed Doppler examination from the suprasternal notch. Pulsed Doppler echocardiography appears to be a useful technique for resolution of left atrial lines found on M mode echocardiography.
Congenital anomalies of the atrium, pulmonary venous return, and systemic venous return are often regarded as anatomical contraindications to orthotopic cardiac transplantation. Among our pediatric transplant patients, 10 children aged 3 to 15 years, weighing 9 to 45 kg, and all previously operated on for a total of 18 interventions had 32 anomalies needing correction at the time of transplantation. Besides the 18 instances of great vessel abnormalities, 14 anomalies of the atrium and of the venous return were encountered either alone or in combination: single atrium or previous septectomy (4), hypoplastic left atrium (2), previous Mustard procedure (1), cor triatriatum (1), anomalous pulmonary venous return (3), and anomalous systemic venous return (3). The preparation of the donor heart was modified in four ways: right atrial paraseptal incision, left atrial flap technique, full-length mobilization of the pulmonary arteries, and aortic arch incision. Correction of the atrial and venous return anomalies was carried out at the time of orthotopic transplantation with the following techniques: atrial septation, atrial enlargement, superior systemic venous return reroofing, inferior systemic venous return reroofing, double venous rerouting (pulmonary and systemic), and septal realignment. One child died of pulmonary hypertension in the early postoperative period. After a follow-up ranging from 1 month to 52 months, all survivors are asymptomatic. Based on echocardiography, heart catheterization, and angiography, there are no stenoses and no shunts, and the atrial dimensions are good. Based on the results achieved with these surgical techniques, we conclude that most atrial lesions, anomalous pulmonary venous returns, and anomalous systemic venous returns are correctable at the time of orthotopic transplantation and do not preclude a successful outcome in children.
Drainage of a persistent left superior vena cava to the coronary sinus is a common congenital lesion associated with dilatation of the coronary sinus. We report on 4 cases in which marked enlargement of the coronary sinus was present, immediately superior to and partially occluding the mitral valve, with consequent obstruction to left ventricular inflow. In 1 patient, presenting with cardiac failure in early infancy, this abnormality was the major defect, mimicking the features of cor triatriatum, and surgical correction led to significant symptomatic improvement. In 3 other patients this abnormality was associated with other congenital lesions, and it is postulated that in these cases impairment of flow into the left ventricle in the presence of an atrial septal defect elevated the left atrial pressure and increased the shunt across the atrial septum, thereby increasing the symptoms and clinical findings. Surgical reduction of the enlarged coronary sinus was performed in each case through the interatrial septum, and the technique is described.
To investigate the hypothesis that embryologic abnormalities in the venous valves may be associated with abnormal cardiac development, we examined the right atrial morphologic characteristics in 20 hearts with underdevelopment of the right heart and 17 normal hearts. In the study group, 16 (80%) of the patients had significantly enlarged eustachian valves, one (5%) was slightly enlarged, and three (15%) were smaller than expected. Five (25%) had cor triatriatum dexter. In comparison, eustachian valves in the control specimens were prominent in only one (6%), normal in five (29%), and almost absent in eight (47%). The thebesian valve was also more prominent in the study cohort when compared with controls (p < 0.05). No other morphologic features of the right atrium analyzed in this study differed from those found in normal specimens. We speculate that failure of the venous valves to regress appropriately may create abnormalities in fetal circulation that predispose the fetus to maldevelopment of the right heart structures.
Two patients with supravalvular stenosing ring of the left atrium are described. In 1 patient with an associated ventricular septal defect and Wolff-Parkinson-White syndrome, the diagnosis of supravalvular stenosing ring was only suspected. This patient underwent correction but died 34 days after the operation because of pulmonary embolism. In the second patient a preoperative diagnosis was not made, and this contributed to his death following correction of tetralogy of Fallot. The association of these two anomalies is very rare. Differential diagnosis from other congenital anomalies producing obstruction of left atrial flow is discussed. The divergent microscopical features of the membrane in supravalvular stenosing ring of the left atrium and in cor triatriatum are described. The value of cardiac catheterization, angiography, and echocardiography as diagnostic aids is emphasized. The hazards of not recognizing and diagnosing this anomaly when associated with other cardiac malformations are pointed out.
The extant nomenclature for pulmonary venous anomalies is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The basis for classification are the prenatal errors of embryologic development. The major categories include: partially anomalous pulmonary venous connection, totally anomalous pulmonary venous connection, atresia of the common pulmonary vein, cor triatriatum, and stenosis or abnormal number of pulmonary veins. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Potential diagnostic-related risk factors are presented.
Obstruction to pulmonary venous return may be associated with a number of congenital cardiovascular abnormalities occurring both before and after surgery. Hemodynamic assessment by cardiac catheterization is often difficult. A noninvasive method for detection and quantitation of obstruction to systemic ventricular inflow would be clinically useful. Two-dimensionally directed pulsed and continuous wave Doppler echocardiography was performed before cardiac catheterization in 31 patients thought clinically to have possible obstruction to left ventricular inflow or pulmonary venous return. Primary diagnoses included transposition of the great arteries after the Mustard or Senning procedure in nine patients, total anomalous pulmonary venous connection in nine (in two after surgical repair), cor triatriatum in eight (in four after surgical repair), congenital mitral stenosis in four (in one after surgical repair) and mitral atresia in one. Severe obstruction was defined as a mean pressure gradient at catheterization of greater than or equal to 16 mm Hg at any level of the pulmonary venous return or of the systemic ventricular inflow. Severe obstruction was predicted if Doppler examination measured a flow velocity of greater than or equal to 2 m/s across any area of inflow obstruction. At catheterization, 12 patients (39%) had severe obstruction to left ventricular inflow or pulmonary venous return and all obstructions were correctly detected by Doppler echocardiography. The site of pulmonary venous obstruction was localized by two-dimensionally directed pulsed Doppler study. Patients with a lesser degree of obstruction had a lower Doppler velocity, but none had a maximal Doppler velocity of greater than or equal to 2 ms/s.(ABSTRACT TRUNCATED AT 250 WORDS)
A 52-year-old man with a symptomatic condition-a huge left coronary artery fistula simulating a cor triatriatum sinister on transthoracic echocardiography-was referred to our center for surgery. Coronary angiography unequivocally established the diagnosis of a left coronary artery fistula and contrast opacification of the right atrium was observed. However, as a result of the size of the fistula, the entry site could not be defined with certainty. A transesophageal echocardiography (TEE) was then performed, and the opening site was clearly identified in the postero-lateral wall of the right atrium. After the surgical treatment transesophageal echocardiography was repeated, and it was confirmed that the surgical treatment had succeeded.
Most publications on atrial isomerism are autopsy or case reports. The authors review 41 consecutive children operated on from 1980 through to 1996 with emphasis on associated cardiac anomalies, surgical procedures and outcome. Left atrial isomerism was present in 23 patients. Interruption of the inferior vena cava (56%), atrio-ventricular septal defect (47%), common atrium (38%) and cor triatriatum sinistrum (30%) were the most common diagnoses. Biventricular repair was achieved in 17 children and total cavo-pulmonary connection in two. Three underwent staged palliation: modified Blalock-Taussig shunt for two and bidirectional Glenn anastomosis for one. The remainder received a cardiac pacemaker. One patient died early after repair. Two underwent reoperation to correct a regurgitant left atrio-ventricular valve: one of these, in another hospital, had peroperative death. Three died later. Actuarial survival rate after repair and total cavo-pulmonary connection that was stabilized after 2 years was 84%. In the 18 children with right atrial isomerism, pulmonary atresia or stenosis predominated (89%) with discordant ventriculo-arterial connection (72%), atrio-ventricular septal defect (72%), 'single' ventricle (55%) and extracardiac total anomalous pulmonary venous drainage (50%). Biventricular repair was achieved in two patients and complete Fontan circulation in eight. The other eight underwent various staged palliative procedures and correction of extracardiac total anomalous pulmonary venous drainage. Five patients died postoperatively: two in our unit after modified Blalock-Taussig shunt and total cavo-pulmonary connection, three in other hospitals after repair (n = 1) and Fontan (n = 2). Five died later. One was lost for review. Survival after repair and Fontan stabilized after 6 months at 49%. In conclusion, the cardiovascular malformations associated with left atrial isomerism can often be successfully corrected. Those accompanying right atrial isomerism usually preclude a biventricular repair, require staged palliation and carry a poor prognosis.
OBJECTIVE: Patients with mitral valve disease and suffering of atrial fibrillation of more than 1 year's duration have a low probability of remaining in sinus rhythm after valve surgery alone. Intraoperative radiofrequency ablation was used as an alternative to simplify the surgical maze procedure. METHODS: Seventy-two patients with mitral valve disease, aged 63+/-11 years ranging from 31 to 80 years, underwent valve surgery and radiofrequency energy applied endocardially, based on the maze III procedure to eliminate the arrhythmia. The right-sided maze was performed on the beating heart and the left-sided maze during aorta cross-clamping. RESULTS: Surgical procedures included mitral valve repair (n=38) or replacement (n=34) and in addition tricuspid valve repair (n=42), closure of an atrial septal defect (n=2) and correction of cor triatriatum (n=1). The left-sided maze needed 14+/-3 min extra ischemic time. There were two in-hospital deaths (2.7%) and three patients (4.2%) died during follow-up of 20+/-15 months. Among 67 surviving patients, 51 patients (76%) were in sinus rhythm, two patients (3%) had an atrial rhythm and eight patients (12%) had persistent atrial fibrillation or atrial flutter. Four patients had a pacemaker implanted, in one patient because of sinus node dysfunction. Doppler echocardiography in 64 patients demonstrated right atrial contractility in 89% and left atrial transport in 91% of patients. CONCLUSIONS: Intraoperative radiofrequency ablation of atrial fibrillation is an effective and less invasive alternative for the original maze procedure to eliminate atrial fibrillation.