Hemodynamic function after the mustard operation for transposition of the great arteries

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The postoperative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6 % ), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.     

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.     

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.     

Noninvasive estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava

To evaluate a simple noninvasive means of estimating right atrial (RA) pressure, the respiratory motion of the inferior vena cava (IVC) was analyzed by 2-dimensional echocardiography in 83 patients. Expiratory and inspiratory IVC diameters and percent collapse (caval index) were measured in subcostal views within 2 cm of the right atrium. Parameters were correlated with RA pressure by flotation catheter within 24 hours of the echocardiogram (38 were simultaneous). Correlations between RA pressure (range 0 to 28 mm Hg), expiratory and inspiratory diameters and caval index were 0.48, 0.71 and 0.75, respectively. Of 48 patients with caval indexes less than 50%, 41 (89%) had RA pressure greater than or equal to 10 mm Hg (mean +/- standard deviation, 15 +/- 6), while 30 of 35 patients (86%) with caval indexes greater than or equal to 50% had RA pressure less than 10 mm Hg (mean 6 +/- 5). Sensitivity and specificity for discrimination of RA pressure greater than or equal to or less than 10 mm Hg were maximized at the 50% level of collapse. Thus, IVC respiratory collapse on echocardiography is easily imaged and can be used to estimate RA pressure. A caval index greater than or equal to 50% indicates RA pressure less than 10 mm Hg, and caval indexes less than 50% indicate RA pressure greater than or equal to 10 Hg.     

Radionuclide angiocardiography in evaluation of patients after repair of transposition of the great arteries

The ability of first pass radionuclide angiocardiography to detect and quantitate residual intracardiac shunts and systemic venous obstruction after repair of transposition of the great arteries was evaluated in 29 children. Information from radionuclide scans was compared with data obtained during cardiac catheterization. Three children had a residual right to left shunt detected with both methods. There was good agreement between radionuclide and catheterization quantitation of left to right shunt in the nine patients with a residual defect, five of whom had significant shunting. Nineteen patients with signs suggesting superior vena caval obstruction were evaluated with both radionuclide and catheterization methods. In eight, complete obstruction was detected with both techniques; in one additional patient, partial obstruction was found on catheterization only. One of six patients evaluated for possible inferior vena caval obstruction was identified with both techniques. In the group as a whole, information obtained with radionuclide angiography correlated well with cardiac catheterization data in evaluation of residual shunts and obstruction to systemic venous return.     

Two-dimensional echocardiographic assessment of caval and pulmonary venous pathways after the senning operation

This study reports the 2-dimensional echocardiographic appearance of the caval and pulmonary venous pathways after the Senning procedure in 28 patients and establishes normal values for the caval and pulmonary venous pathway dimensions. Eighteen patients had no caval or pulmonary venous obstruction or tricuspid regurgitation at catheterization; 2 had isolated superior vena caval obstruction, 3 had isolated pulmonary venous obstruction, 4 patients had severe tricuspid regurgitation, and 1 had a large residual ventricular septal defect. The caval and pulmonary venous pathways were imaged in modified 4-chamber and transverse views, and the narrowest dimension of each pathway in each view was measured by 2 independent observers. Dimension measurements were then normalized to the cube root of body surface area. Caval and pulmonary venous pathway “dimension products” were obtained by multiplying the normalized dimension in the 4-chamber view by the normalized dimension in the transverse view.All patients with catheterization-proven caval or pulmonary venous obstruction or tricuspid regurgitation had caval or pulmonary venous pathway dimension products outside the normal range, defined by our measurements in the 18 patients with no caval or pulmonary venous obstruction or tricuspid regurgitation. Thus, 2-dimensional echocardiography can provide both quantitative and qualitative information about the caval and pulmonary venous pathways after the Senning procedure.     

Cross-Sectional echocardiographic diagnosis of azygos continuation of the inferior vena cava

Azygos continuation of the inferior vena cava has importance for both the invasive diagnosis of congenital heart disease by catheterization and for surgical treatment. Cross-sectional echocardiography was used to examine 1,000 patients (1 day to 16 years, mean 3.3 years) who also had angiographic or surgical confirmation. Twenty-eight patients (3%) had azygos continuation (left 13, right 14, bilateral 1) and, in 26 patients, the hepatic portion of the inferior vena cava was absent. Azygos continuation was prospectively detected in all and was directly visualized in subcostal scans as a venous structure posterior to the aorta coursing behind the heart and not entering the inferior aspect of either atrium in 26/28 (93%). Azygos connection to the ipsilateral superior vena cava or atrium was correctly predicted in all. The inferior vena cava was visualized in all patients without azygos continuation, except one neonate with omphalocele. We conclude that cross-sectional echocardiography can accurately detect azygos continuation of the inferior vena cava and predict its side and connection.     

Aggressive mediastinal fibrosis, a rare cause of superior vena cava obstruction--case report and review of the literature

An aggressive mediastinal fibrosis was found to be a cause of superior vena caval obstruction in 1 to 2% of patients. Symptoms can be discrete for a long time, but the progressing disease can cause many symptoms. A 56-year-old man who, for half a year, suffered from thoracic pain during deep inspiration, and then rapidly developed superior vena caval obstruction caused by aggressive mediastinal fibrosis is discussed. The operative treatment consisted in complete replacement of the superior vena cava.     

Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant

A 24-yr-old woman with hemolytic anemia developed multiple thrombosis of the hepatic vein and inferior vena cava. She was found to have circulating lupus anticoagulant that could have been causally related to the thrombosis and hence the Budd-Chiari syndrome. On her first admission to the hospital vena cava and hepatic vein catheterizations revealed partial thrombotic occlusion of the cava at the level of the diaphragm, which was subsequently transformed into complete membranous obstruction. The right hepatic vein, which was patent on the first admission, was also completely occluded. These observations support the theory that membranous obstruction of the inferior vena cava is a sequela to inferior vena caval thrombosis rather than a congenital anomaly.     

Comparison of central venous and inferior vena caval pressures

Inferior vena caval pressures were measured in 60 patients undergoing cardiac catheterization and compared with central venous pressure from within the right atrium. Mean pressures within the abdominal inferior vena cava were essentially the same as mean right atrial pressure, suggesting that the inferior vena cava provides a useful safe alternative for measuring central venous pressure.     

Unusual collateral vessel from right subclavian vein to left atrium,a rare complication of superior vena cava obstruction

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49‐year‐old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi‐lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.     

Tamponade caused by localized compression of the superior vena cava and right atrium after aortic valve replacement under extracorporeal circulation

The authors report a case of localised compression of the right atrium due to a loculated intrapericardial haematoma after open heart surgery. The patient suddenly developed signs of superior vena caval obstruction during the third postoperative week. The diagnosis was made by 2D echocardiography and superior vena cavography. The authors review the literature and discuss the main clinical features of localised cardiac tamponade, underlying the value of 2D echocardiography in the postoperative management of cardiac surgical patients.     

An elusive persistent left superior vena cava draining into left atrium

A case report of a persistent left superior vena cava draining into left atrium with a fibromuscular left ventricular outflow tract obstruction and a small atrial septal defect. The anomalous vessel escaped detection during two right and left heart catheterizations from the right arm and open heart surgery. It was an incidental finding during cardiac catheterization from the left arm and the anatomy was confirmed by contrast echocardiography.     

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.     

Complete transposition of the great arteries: visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography

Subcostal oblique 2-dimensional echocardiography was performed in 64 infants younger than 2 years with complete transposition of the great arteries (TGA) (situs solitus, concordant atrioventricular and discordant ventriculoarterial connections). All patients examined before cardiac catheterization had a correct diagnosis by 2-dimensional echocardiography using the subcostal oblique views. Twelve patients had associated left ventricular (LV) outflow tract obstruction and 7 had right ventricular (RV) outflow obstruction. The standard parasternal views failed to diagnose obstruction in 1 patients with LV outflow obstruction and 5 with RV outflow obstruction; the subcostal left oblique cut and long axis of the left ventricle visualized all left-sided obstructions, and right-sided obstructions were correctly displayed in 5 of 7 cases using a combination of left oblique and right oblique cuts. Two-dimensional echocardiographic subcostal oblique views allow an excellent definition of the morphologic characteristics of RV and LV outflow tracts in patients with TGA and improve the diagnosis of the outflow obstruction in these malformations.     

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis: Prevalence and predictors

Objectives. To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development.Background. Systemic venous collaterals have been found after cavopulmonary anastomosis.Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis.Results. After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 ± 5 mm Hg versus 11 ± 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 ± 4 mm Hg vs. 11 ± 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 ± 2 mm Hg vs. 6 ± 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 ± 3 mm Hg vs. 5 ± 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12–1.58, p = 0.001) for their presence.Conclusions. Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.     

Absent right superior vena cava with left superior vena cava draining to an unroofed coronary sinus

We describe the case of a 1-month-old infant with a complete atrioventricular septal defect with right dominance, situs solitus, and drainage from the persistent left superior vena cava to the coronary sinus. Corrective surgery was carried out without previous cardiac catheterization. During the operation, the right superior vena cava was found to be absent. Cyanosis and head-and-neck edema were observed in the immediate postoperative period. Transthoracic echocardiography carried out after injection of a small volume of stirred saline into an epicranial vein demonstrated the presence of microbubbles in the left cardiac cavities. A second operation was performed to prevent drainage from the left superior vena cava to the left atrium (via the unroofed coronary sinus) and to insert a PTFE conduit between the innominate vein and the right atrial appendage. The outcome was excellent. In this report, the embryological, clinical, diagnostic and therapeutic characteristics of this entity are discussed.     

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.     

Retrograde non trans‐septal balloon mitral valvotomy in mitral stenosis with interrupted inferior vena cava,left superior vena cava,and hugely dilated coronary sinus

A 22‐year‐old woman with severe mitral stenosis was referred to us for further evaluation and management. She was found to have severe mitral stenosis, severe tricuspid regurgitation with dilated right atrium and right ventricle with persistent left superior vena cava and hugely dilated coronary sinus. Valve was suitable for balloon mitral valvotomy. Cardiac catheterization showed interrupted inferior vena cava with azygos continuation to right atrium and large left superior vena cava draining to coronary sinus which was very much dilated. Right trans‐jugular approach was tried for balloon mitral valvotomy, but was unsuccessful due to a very large right atrium and coronary sinus. Retrograde non trans‐septal approach was used and balloon valvotomy was done successfully using a 24 mm × 40 mm TYSHAK balloon without any major complication. Reduction in the transmitral pressure gradient on cardiac catheterization data and transthoracic echocardiography confirmed successful procedure. Balloon mitral valvotomy can be done successfully in patients with the above unusual cardiac anatomy with no major procedural complications. © 2015 Wiley Periodicals, Inc.     

静脉畸形、迂曲、狭窄时永久起搏导线置入的方法探讨

经静脉造影或观察导丝走形证实 6例患者存在静脉畸形、迂曲、狭窄 ,其中 5例高龄患者置入永久起搏器时 ,其上腔静脉系统迂曲、狭窄 ,无法使用起搏器穿刺套装内的导丝及鞘管将导线送到起搏部位 ,另 1例为永存左上腔静脉合并有右上腔静脉缺如。试用 175cm 0 .0 35长导丝以及 6 8FINPUT鞘替代普通起搏器穿刺套装。结果 :使用175cm 0 .0 35长导丝以及 6 8FINPUT鞘顺利地将起搏导线送入右心房中下部 ,安全地完成置入手术 ,无并发症。结论 :一旦送入导线或导丝困难 ,应积极地进行血管造影 ,不应盲目的推送 ,使用 175cm长导丝增加支撑力 ,结合IN PUT鞘管通过狭窄或纡曲延长的血管段 ,给起搏导线提供一个光滑的通道 ,可顺利的将起搏导线送入心房及心室。