Cross-sectional echocardiographic diagnosis of systemic venous return.

To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.     

Total anomalous pulmonary venous return in children: importance of echocardiographic diagnosis and early surgery

OBJECTIVE: We studied patients who underwent surgical repair for total anomalous pulmonary venous return at our hospital. We report the importance of diagnosis by echocardiographic imaging before surgical treatment. METHODS: Within the period of 1990-1999, fourteen patients underwent surgical repair of this cardiopathy in our hospital. The type of anomalous drainage was supracardiac in 6 patients, infracardiac in 4, to the coronary sinus in 1, and mixed-type in 3 patients. Eleven cases were diagnosed with an echo-Doppler study, the findings being confirmed intraoperatively. RESULTS: There were 2 early deaths: one occurred in the operating room in a patient with a small left ventricle, and the second one was 35 days postoperatively as a result of a septic complication. Early in the postoperative period our primary goal has steadily been the control and treatment of pulmonary hypertension. After a mean follow-up time of 50 months, only 1 patient needed to be reoperated on and the remainder are symptomless. CONCLUSIONS: That sufficient diagnostic data on total anomalous pulmonary venous return can reliably be obtained by ultrasound scanning so that surgery can be promptly undertaken, and that its surgical risk is currently low and mid-term post-repair outcome is fairly good.     

Anomalous systemic venous return: recognition by two-dimensional echocardiography

The two-dimensional echocardiographic features of anomaliesof systemic and coronary venous return are described as theywere seen in 23 patients with this diagnosis proven at angiography.A left-sided superior vena cava draining to the right atriumvia an enlarged coronary sinus was correctly identified in all17 patients with this condition by observing a characteristicallymoving ovoid structure in the region of the posterior atrioventriculargroove. This structure opacified before the right side of theheart following bolus injections of contrast material from aleft arm vein. Injection from the right arm in 12 patients orfemoral vein in four patients of this group produced contrastechoes within the right heart only. A left-sided superior venacava draining directly to a left atrium in three patients, orleft side of a common atrium in two patients with atrial situsinversus, was identified by the immediate opacification of theleft-sided chamber following injection from the left arm. Two patients with hemiazygos inferior vena caval return to theleft superior vena cava or left atrium were studied using injectionsof contrast from the femoral vein which successfully identifiedthe venous abnormality. Two-dimensional echocardiography, when combined with peripheralvenous injections of contrast will reliably demonstrate anomaliesof systemic venous return, and thus provides important informationbefore more invasive procedures.     

Atrial lipomatous hypertrophy causing reduction in systemic venous return

Lipomatous hypertrophy of the interatrial septum (LHIAS) is a benign condition characterized by an abnormal deposition of adipose tissue in the interatrial septum which appears as a pseudomass with a bilobed shape. We present the case of a 68-year-old obese female patient. LHIAS is an infrequent finding, but with recent improvements in imaging it is increasingly recognized. Cardiac MRI may prove useful in its diagnosis in terms of tissue characterization, as well as for the evaluation of disease extension and haemodynamic compromise.     

Echocardiographic diagnosis of total anomalous pulmonary venous return

Between March 83 and November 84, 20 patients with TAPVC were studied by cross-sectional echocardiography. The diagnosis was confirmed by catheterization, surgery or autopsy in each case. The anomalous drainage of the pulmonary veins was supracardiac in 10 patients, to the coronary sinus in 5, infracardiac in 4, mixed (supracardiac and infracardiac) in 1. The correct diagnosis was prospectively achieved in 17 patients (85); the echocardiographic examination could not show the precise site of the drainage in 2 (10); in only one patient (5) with right isomerism, double inlet indeterminate ventricle and severe pulmonary stenosis, the diagnosis of TAPVC infracardiac was missed. Seven patients (4 with TAPVC supracardiac, 3 to the coronary sinus) were sent to surgery without preoperative catheterization; the diagnosis was confirmed in all. Cross-sectional echocardiography proved to be a reliable diagnostic tool in the assessment of TAPVC. The patients in which the site of anomalous drainage is clearly identified by echo, can be sent to surgery without invasive investigation.     

Cross-sectional echocardiographic diagnosis of a congenital aneurysm of the muscular interventricular septum.

An 18-year-old asymptomatic male patient was detected to have a precordial systolic murmur since early childhood. A cross-sectional and Doppler echocardiographic examination revealed a congenital aneurysm of the trabecular portion of the muscular interventricular septum with a pin-hole communication with the right ventricular cavity.     

Cross-sectional echocardiographic demonstration of biventricular thrombus

Nine months following extensive myocardial infarction, a 60-year-old man presented with intermittent right heart failure. Cross-sectional echocardiography demonstrated biventricular thrombi, the right ventricular thrombus being very close to the tricuspid valve and possibly interfering with its function. Post-mortem examination confirmed the echocardiographic findings.     

Respiratory variations in the right ventricular diameter: an echocardiographic element in the diagnosis of partial isolated pulmonary venous return anomaly

Isolated partial anomalous pulmonary venous drainage (PAPVD), in contrast to atrial septal defect (ASD), does not cancel out the effects of respiration on blood flow in the right ventricle. The aim of this study was to see whether this difference could contribute to the diagnosis of PAPVD without ASD on M mode echocardiography. The diastolic dimensions of the right ventricle on expiration and inspiration were compared in 4 groups of patients aged 2 to 17 years. Group 1 comprised 6 children with PAPVD without ASD; Group 2: 10 children with PAPVD and ASD; Group 3: 11 children with isolated non-restrictive ASD, and Group 4: 10 normal children. Groups 1 and 4 were comparable with a respiratory variation of RV dimension of 10 to 29%. On the other hand, in Groups 2 and 3 the percentage variation was less than 6%. The finding of isolated RV dilatation with normal respiratory variation of its internal dimension should therefore alert the operator to the possible diagnosis of PAPVD without ASD.     

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.     

Ventricular aneurysm: Cross-sectional echocardiographic approach

Current angiographic indexes of ventricular function have proved inadequate for prognostication in patients with ventricular aneurysm. Crosssectional echocardiography can visualize residual myocardium in all four walls of the left ventrlcle. A new echocardiographic technique of calculating residual myocardium is presented. The echocardiographic technique yielded identical information to that of contrast angiography (r = 0.97). An index of residual myocardium was generaled from the cross-sectlonal echocardiogram that correlated with the clinical state of the patients. In patients treated medically it predicted those patients likely to die within 6 months (p < 0.005). Preliminary observations in patients having aneurysmectomy revealed that there were good surgical results in those with an index of residual myocardium of 0.42 or greater, but more patients are necessary to establish the lower limit of a surgically acceptable level of residual myocardium.     

Cross-sectional echocardiographic detection of aortic valve prolapse

To determine the potential for cross-sectional echocardiography to define aortic valve prolapse, 14 controls with a mean age of 51 ± 10 years (SD) with normal cardiac catheterization were studied by cross-sectional echocardiographic left ventricular longitudinal and short axis views to define normal aortic valve morphology and motion characteristics. Similarly, 112 patients with cross-sectional echocardiographic mitral valve morphology and motion characteristics. In all 14 controls the left ventricular longitudinal view revealed the aortic valve as a faint echo reflector with symmetrical cusps whose closure point as well as the cusp tissue itself did not display any downward (or prolapsing) motion toward the left ventricular outflow tract. In the group of 112 patients with mitral valve prolapse, the aortic valve was successfully imaged for detailed analysis in 77. Sixty of these 77 (78%) had a mean age of 38 ± 18 years (SD) and revealed aortic valve morphology motion characteristics similar to controls. All of the remaining 17 patients (mean age 36 ± 21 years, 11 female) revealed a downward displacement (or prolapse) of the aortic valve during diastole. There was an increased echo reflection from the cusp tissue in 16 of 17 and in six of 17, cusp size was asymmetric, producing eccentricity of the coaptation point. All six of these had three aortic cusps seen on the short axis view. The aortic root size was normal in all controls in the 60 patients with mitral valve prolapse without aortic valve prolapse, whereas six of 17 patients with aortic valve prolapse had aortic rood enlargement. When comparing the group with (N = 17) and without (N = 60) aortic valve prolapse, the tricuspid valve was prolapsed in 16 of 17 compared to 31 of 60 (p = NS), and aortic insufficiency was present in four of 17 compared to none of 60 (p ≤ 0.01).In conclusion, cross-sectional echocardiography can identify a subset of patients with mitral valve prolapse who have aortic valve prolapse in which aortic root dilatation and aortic regurgitation may be encountered. Such patients may reflect a more diffuse myxomatous degeneration of the cardiac skeleton.     

Cross-sectional echocardiographic features of ruptured chordae tendineae

Thirty-two patients with mitral regurgitation secondary to rupturedchordae tendineae were studied by cross-sectional echocardiography.Twenty of them subsequently underwent operation. Three signsare described. (1) Non-coaptation of the leaflets (55%). (2)Systolic fluttering echo in the left atrium originating fromthe mitral valve (20%). (3) A previously not described smalldiastolic chaotically moving echo in the short axis sectionof the left ventricle at the level of the papillary muscles(65%). The combined sensitivity of these three signs was 85%.The specificity of the three signs when compared with thoseof 107 patients with mitral regurgitation of different aetiologiesstudied by cross-sectional echocardiography was 99%, 100% and99%, respectively. Six patients were studied after mitral valverepair; restriction of the surgical treated leaflet and reductionof its valve area was seen in all of them, and the disappearanceof the valvar signs of ruptured chordae noted. The persistenceof the small echo sign in the short axis of the left ventricleindicated its chordal origin.     

Cross-sectional echocardiographic detection of rheumatic mitral regurgitation

Short-axis, cross-sectional echocardiograms of the mitral valve were performed in 20 consecutive patients with rheumatic mitral valve disease to determine if mitral regurgitation could be detected. In four patients, cross-sectional echocardiograms were technically inadequate. Of the 16 remaining patients, 8 had no significant mitral regurgitation on cineangiography, and 8 had significant regurgitation. Two independent observers reviewed videotapes of the cross-sectional echocardiograms without knowledge of the cineangtograms or clinical findings. In each case, the presence of significant mitral regurgitation was correctly predicted by viewing the cross-sectional echocardiograms. The thickened leaflets of the mitral orifice could easily be identified during both diastole and systole. Closure of the two leaflets was best observed early in systole before caudal movement of the anulus caused the orifice to move out of range of the ultrasonic beam. Complete closure of the leaflets was seen in patients without mitral regurgitation. Failure of closure of small areas of either the medial or lateral aspect of the valve was associated with insignificant mitral regurgitation. Failure of both sides to close or failure to close in the center of the valve indicated significant mitral regurgitation. Thus, it appears that significant mitral regurgitation in patients with rheumatic mitral valve disease can be detected by qualitative analysis of short-axis, cross-sectional echocardiograms.     

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 unruptured right sinus of Valsalva aneurysm dissecting into the interventricular septum

We present a case of an unruptured right coronary sinus of Valsalva aneurysm with dissection into the interventricular septum diagnosed pre-operatively by cross-sectional echocardiography. The unique echocardiographic features of this rare, although potentially fatal congenital lesion, are described.     

Instantaneous pressure-flow velocity relations of systemic venous return in patients with univentricular circulation

OBJECTIVE—To assess the pressure and flow velocity relations and respiratory variability of the systemic venous and hepatic venous return in patients with univentricular circulation.
PATIENTS—15 selected patients who had undergone cavopulmonary anastomosis (10) or atriopulmonary anastomosis (5). Mean age at operation was 55.1 months (range 9 to 145). Studies were done at 75.5 (32.6) months (mean (SD)) after the operation.
SETTING—Tertiary referral centre.
METHODS—Patients were studied using simultaneous recordings of ECG, pressure trace, respirometer trace, and pulsed Doppler echocardiography. Mean systemic venous pressure and pulmonary vascular resistance did not differ significantly between the two patient groups.
RESULTS—After total cavopulmonary anastomosis, systemic venous pressure tracings showed a flattened pressure curve without any dependence on cardiac or respiratory cycle. After atriopulmonary anastomosis, right atrial pressure tracings showed a significantly higher "a" wave corresponding to atrial contraction, without any respiratory variability. Pulsed Doppler examination of the superior and inferior caval vein and hepatic vein after total cavopulmonary anastomosis did not show a reverse flow after atrial contraction. The inspiratory to expiratory velocity ratio of antegrade flow revealed a significant dependence of flow on changes in intrathoracic pressure in the intra-atrial tunnel, caval veins, and hepatic vein. During expiration, decrease or cessation of antegrade hepatic venous flow was documented. After an atriopulmonary anastomosis, there was a biphasic antegrade venous flow pattern without significant respiratory variation.
CONCLUSIONS—After total cavopulmonary anastomosis, there was marked respiratory dependence of systemic and hepatic venous return, whereas after an atriopulmonary anastomosis venous flow pattern varied according to cardiac cycle and pressure trace. The effects of total cavopulmonary anastomosis on venous return might counteract its other haemodynamic advantages.


Keywords: Fontan operation; Doppler echocardiography; systemic venous flow pattern     

Differential diagnosis of anomalous pulmonary venous return. A clinical-roentgenological study

The paper presents an analysis of the results of clinical and roentgenological examination of 88 patients with anomalous pulmonary venous return (42 females, 46 males), aged 2 weeks to 33 years. This defect has highly diverse anatomical variants. Its most frequent form is partial anomalous drainage (70.5%), while total drainage is much less frequent (29.5%). As regards the return level of the pulmonary veins, the site occurring most frequently is the supracardial one (63%), followed by the cardial (20%), mixed (11%) and infracardial site (6%). The authors compare the findings of X-ray examination with the results of catheterization of the right and left heart, selective angiocardiography (62) and the findings made intraoperatively (82), and assess the value of routine X-ray examination for differential diagnosis of partial and total anomalous pulmonary venous drainage at different return levels.