The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries

2-D Doppler echocardiography was used to assess the occurrenceof haemodynamic abnormalities in 45 asymptomatic patients, aged4 to 16 years (median 7·4) after a Mustard operationfor transposition of the great arteries. The findings were comparedwith those derived from cardiac catheterization. Thirty-fivecardiac lesions were correctly diagnosed by 2-D Doppler echocardiographyin 23 patients, but on six occasions, minor abnormalities weremissed. 2-D Doppler echocardiography demonstrated systemic venouspathway obstruction of more than 3 mmHg at cardiac catheterizationin nine patients, and in five of the six patients with pulmonaryvenous channel obstruction. A left ventricular outflow tractobstruction (pressure difference > 15 mmHg) was diagnosedcorrectly by Doppler echocardiography in seven patients. Baffleleakage was found in two patients with a left to right shuntof 25% or more of pulmonary bloodflow, but was missed in fiveout of nine patients with small shunts. Tricuspid regurgitationwas well defined in eight patients, The absence of symptomsand a routine examination after a Mustard operation do not ruleout haemodynamic abnormalities. However, these, with the possibleexception of minor baffle leakage, can be detected by 2-D Dopplerechocardiography.     

The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries

2-D Doppler echocardiography was used to assess the occurrenceof haemodynamic abnormalities in 45 asymptomatic patients, aged4 to 16 years (median 7·4) after a Mustard operationfor transposition of the great arteries. The findings were comparedwith those derived from cardiac catheterization. Thirty-fivecardiac lesions were correctly diagnosed by 2-D Doppler echocardiographyin 23 patients, but on six occasions, minor abnormalities weremissed. 2-D Doppler echocardiography demonstrated systemic venouspathway obstruction of more than 3 mmHg at cardiac catheterizationin nine patients, and in five of the six patients with pulmonaryvenous channel obstruction. A left ventricular outflow tractobstruction (pressure difference > 15 mmHg) was diagnosedcorrectly by Doppler echocardiography in seven patients. Baffleleakage was found in two patients with a left to right shuntof 25% or more of pulmonary bloodflow, but was missed in fiveout of nine patients with small shunts. Tricuspid regurgitationwas well defined in eight patients, The absence of symptomsand a routine examination after a Mustard operation do not ruleout haemodynamic abnormalities. However, these, with the possibleexception of minor baffle leakage, can be detected by 2-D Dopplerechocardiography.     

Pulmonary venous and systemic ventricular inflow obstruction in patients with congenital heart disease: detection by combined two-dimensional and Doppler echocardiography

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)     

Comparative values of the precordial and transesophageal approaches in the echocardiographic evaluation of atrial baffle function after an atrial correction procedure

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)     

Haemoptysis as a late complication of a Mustard operation treated by balloon dilation of a superior caval venous obstruction

Haemoptysis was the presenting symptom in a 27-year-old male. He had undergone a Mustard operation for connection of complete transposition at the age of 2 years. For 6 months prior to admission, he had complained of dyspnoea without chestpain, and swelling of the fingers during hard physical work. Chest radiography and computer tomographic scans showed normal features of the pulmonary parenchyma, and no sign of cardiomegaly or vascular stasis. Fiberoptic bronchoscopy demonstrated a blood clot in the upper right bronchus, without any associated abnormalities of the bronchial tree. Doppler echocardiography showed obstruction of the superior caval vein, which was verified by cardiac catheterization. Balloon dilation at the site of obstruction increased the diameter of the vein from 0.5 to 1.7 cm, and the mean pressure in the superior caval vein was reduced significantly from 18 to 10 mmHg. The haemoptysis did not recur, and no complaints of dyspnoea or swelling of fingers during physical activity was reported 2 years later. Transthoracic echocardiography undertaken at this time revealed no obstruction of the superior caval vein. We conclude that hemoptysis is a rare complication of increased venous pressure in the upper body of patients with superior caval venous obstruction, which can be treated by balloon dilation or stenting.     

Accuracy of subcostal two-dimensional echocardiography in prospective diagnosis of total anomalous pulmonary venous connection

Since reparative surgery without cardiac catheterization has been advocated for certain lesions such as total anomalous pulmonary venous connection (TAPVC), it is important to assess the accuracy of two-dimensional (2-D) echocardiography in the prospective diagnosis of this lesion. Abandoning traditional cardiac catheterization and angiography would be justified only if all surgically relevant anatomic features could be consistently displayed noninvasively. Consequently, we reviewed our experience with TAPVC to determine the suitability of subcostal 2-D echocardiography as the sole prospective diagnostic tool. Over a 5-year period, of 2444 infants under 2 years of age who underwent subcostal 2-D echocardiography, 38 were diagnosed as having TAPVC as the only major cardiac defect. There were no false-negative or false-positive cases during the time of the study. Drainage sites were diagnosed correctly in 36 (95%). Of the five cases of mixed-type TAPVC, the second drainage site was missed by 2-D echocardiography in two cases.     

Pulmonary venous obstruction in patients with transposition of the great arteries

We report two cases of complete transposition of the great arteries with pulmonary venous obstruction which was diagnosed by intraoperative transesophageal echocardiography, but was not detected by transthoracic echocardiography and cardiac catheterization. The pulmonary venous obstruction was relieved simultaneously with arterial switch operation. The intraoperative transesophageal echocardiography has great contribution for thorough evaluation of the pulmonary venous return of the patients.     

New Procedure of Treatment for Combined Systemic Venous and Pulmonary Venous Obstruction Following the Mustard Operation for Transposition of the Great Arteries

Three patients aged 4.6, and 9 years were discovered at cardiac catheterization to have combined pulmonary venous and systemic venous obstruction following the Mustard operation for transposition of the great arteries. Relief of systemic venous baffle obstruction may unmask pulmonary venous obstruction. Full evaluation of the pulmonary venous confluence is recommended in any patient with systemic baffle obstruction.     

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.     

Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)     

Coarctation repair without cardiac catheterization in infants

Of 35 infants who underwent an operation for coarctation of the aorta, 15 did not undergo cardiac catheterization before surgery. The diagnoses in all uncatheterized patients were made by clinical examination, chest radiography, ECG, and two-dimensional echocardiography combined with pulsed Doppler echocardiography. Associated anomalies diagnosed by two-dimensional/Doppler echocardiography included a patent ductus arteriosus in all patients, bicuspid aortic valve in six, small ventricular septal defect in four, and complete common atrioventricular canal in one. In no instance was the diagnosis of coarctation in error. In addition, the clinical significance of the ventricular septal defect was judged correctly by two-dimensional echocardiography, and no patient required an early reoperation because of significant left-to-right shunt. Two significant lesions were missed in one patient each: mitral stenosis and aortic stenosis. These diagnoses were missed in patients who were first seen with either profound congestive heart failure or shock. Coarctation of the aorta and associated lesions can be diagnosed accurately by two-dimensional echocardiography. This permits proper patient management without the added risk of cardiac catheterization. Although mitral and aortic lesions may be missed because of low cardiac output, this does not result in management errors.     

Detection of caval obstruction by magnetic resonance imaging after intraatrial repair of transposition of the great arteries

Vena caval obstruction may cause significant morbidity after intraatrial repair of transposition of the great arteries (TGA). Two noninvasive methods of diagnosing vena caval obstruction were compared with cardiac catheterization. Echocardiographically gated magnetic resonance imaging (MRI) and echocardiographic evaluation (2-dimensional saline contrast echocardiography and pulsed Doppler flow measurement) were performed on 15 patients 0.7 to 13.5 years after intraatrial repair of TGA (8 Mustard, 7 Senning). At catheterization, complete superior vena cava or partial caval obstruction (gradient greater than 5 mm Hg from cava to systemic venous atrium) was present in 7 of 15 patients. Superior vena cava obstruction was directly visualized by MRI in both patients with catheterization-proved complete superior vena cava occlusion. A dilated azygous/hemiazygous venous complex (greater than or equal to 5 mm cross-sectional diameter) was seen by MRI in 5 of 7 patients with complex or partial vena caval obstruction and in no patient without vena caval obstruction. MRI showed superior vena caval dilatation (ratio of superior vena caval diameter to aortic diameter greater than 1.45) in 3 of 5 patients with partial vena caval obstruction and in 0 of 8 without vena caval obstruction. Direct visualization of narrowing within the atrium was unreliable for any MRI plane because of the 3-dimensional nature of the intraatrial baffle. Two-dimensional saline contrast echocardiography, successfully performed in 12 of 15 patients, detected complete superior vena caval obstruction only in the 2 patients with catheterization-proved complete superior vena cava occlusion. Contrast echocardiography failed to identify any of the 5 patients with partial vena caval obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)     

Usefulness of color Doppler in the diagnosis of ventricular septal rupture after myocardial infarction

Ten consecutive patients with ventricular septal rupture complicating acute myocardial infarction were studied by means of Doppler echocardiography (including two-dimensional, conventional and color Doppler techniques) and bedside right heart catheterization using a Swan-Ganz catheter. One patient died before an operation could be performed. Seven critically ill patients underwent emergency cardiac surgery without preoperative cardiac catheterization, while in two patients it was also possible to undertake coronary angiography before surgery. Two-dimensional echocardiography diagnosed post-infarction ventricular septal rupture in 6 out of 10 patients. Color Doppler revealed the presence and the location of septal rupture in all 10 patients. The color Doppler diagnosis was confirmed either by surgery or necropsy. The estimates of pulmonary artery pressure, obtained by color Doppler-guided continuous wave Doppler beam, were very close to those measured by simultaneous right heart catheterization. In 3 patients, patch leakage occurred 3 days, 15 days and 1 year after the operation. Two-dimensional echocardiography revealed the patch leakage in only one of 3 patients while its location was visualized by color Doppler in all 3 patients. In one patient the color Doppler diagnosis was confirmed at necropsy. In the remaining 2 patients, a small left-to-right shunt was demonstrated by radionuclide studies. Color Doppler echocardiography is a highly sensitive and rapid technique in the diagnosis of postinfarction ventricular septal rupture. In critically ill patients it offers relevant information and may obviate the need for any invasive preoperate investigation.     

Pulsed Doppler echocardiographic assessment of the pulmonary venous pathway after the Mustard or Senning procedure for transposition of the great arteries

Pulsed Doppler evaluation of pulmonary venous flow was performed in three groups of patients who had undergone either a Mustard or Senning procedure. Group I consisted of 43 patients in sinus rhythm who had undergone the Mustard procedure 9 months or more before the Doppler examination and 16 who were evaluated immediately after surgery. Group II consisted of 12 patients in sinus rhythm who had undergone the Senning procedure 9 months or more before the Doppler evaluation. Group III consisted of eight patients with mid baffle obstruction of the pulmonary venous atrium and seven with isolated stenosis of the pulmonary vein of which all but one had previously had associated mid baffle obstruction. In those without obstruction, the pulmonary venous flow pattern mirrored the left atrial pressure trace, with peak forward flow occurring during the x and y descent. Obstruction produced a specific high-velocity turbulent pattern, whether at the mid baffle or pulmonary venous level. This technique provides reliable noninvasive information about pulmonary venous flow after the Mustard or Senning procedure.     

Diagnosing heart failure in aortic valve stenosis

OBJECTIVE: In aortic valve stenosis (AS), heart failure (HF) omens a high risk of death and is an indication for prompt valve replacement. We studied whether its detection can be facilitated by measuring plasma N-terminal B-type natriuretic peptide (Nt-BNP) or by estimating pulmonary capillary wedge pressure (PCWP) using echocardiography. DESIGN AND SETTING: A cross-sectional cohort study in a university hospital. SUBJECTS AND METHODS: We studied 137 consecutive adult patients referred to our unit for invasive evaluation of isolated AS. All patients underwent cardiac catheterization, measurement of plasma Nt-BNP and estimation of PCWP by Doppler echocardiography of transmitral and pulmonary venous flow velocities. The final diagnosis of HF was based on the combined criteria of dyspnoea on ordinary effort and PCWP >14 mmHg at cardiac catheterization. The performance of Nt-BNP and the PCWP estimate in the detection of HF were studied using receiver operating characteristic (ROC) analysis. RESULTS: Totally 42 patients had HF. A cardiologist's clinical diagnosis of HF had high specificity (94%) but poor sensitivity (66%). With an optimized cut-off point, plasma Nt-BNP had moderate sensitivity (77%) and specificity (79%) for HF; the ROC area was 0.83. The echocardiographic PCWP estimate classified 90% of patients correctly as having normal or truly elevated (>14 mmHg) PCWP. Its sensitivity and specificity for the diagnosis of HF were 80 and 95% respectively; the ROC area was 0.88. With a cut-off point of 12 mmHg, the sensitivity of the PCWP estimate was 85% and specificity, 88%. CONCLUSION: The recognition of HF in patients with AS can be improved by estimating PCWP using Doppler echocardiography of transmitral and pulmonary venous flow velocities.     

Value and limitations of pulsed Doppler echocardiography in the determination of pressure gradients in children

The aim of this study was to evaluate the results of pulsed Doppler echocardiography in assessing pressure gradients in children despite the theoretical limitations of this technique in the measurement of high velocity blood flow (due mainly to the phenomenon of "aliasing"). 20 patients with an average age of 6.7 years (range 3 months to 19 years) were studied by 2D echocardiography and pulsed Doppler within 48 hours of cardiac catheterisation. Valvular stenosis was present in 14 cases (aortic, 7, pulmonary, 7). There were 3 cases of infundibular obstruction and 2 vascular stenosis (coarctation of the aorta and stenosis of a branch of the pulmonary artery). One patient had stenosis at the origin of a prosthetic tube graft. The gradient was estimated from the Doppler flow curves using simplified Bernoulli formula (P = 4 X maximal jet velocity). In 17 patients (gradients of 20 to 90 mmHg) an excellent correlation was observed between the pulsed Doppler and haemodynamic results (r = 0.90). In 3 cases with gradients over 80 mmHg it was not possible to quantify the gradient but pulsed Doppler fixed an inferior limit of 80 mmHg. Therefore, using a 3 or 2.25 MHz probe at the low depths of investigation encountered in childhood, pulsed Doppler gave a reliable indication of pressure gradients less than or equal to 80 mmHg. These results and the non-invasive nature of the method make pulsed Doppler a particularly interesting complementary examination in children or babies with stenotic cardiac lesions.     

Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection

Pulmonary venous flow was evaluated by pulsed Doppler echocardiography in 38 patients with total anomalous pulmonary venous connection. Twenty-nine of these 38 had no associated intracardiac anomaly (Group I), and 9 had complex intracardiac anatomy associated with low pulmonary blood flow (Group II). In Group I the drainage was infracardiac in nine, supracardiac in seven, intracardiac in eight and mixed in five. In both groups, in those with venous obstruction the flow in the individual pulmonary veins and ascending or descending vein was nonphasic, varying only with respiration. Flow in the absence of obstruction was phasic, varying with the cardiac cycle. Distal to a site of obstruction the flow was nonlaminar and of high velocity irrespective of the amount of pulmonary blood flow. The pulsed Doppler technique provides important physiologic information in the patient with total anomalous pulmonary venous connection before surgical intervention.     

Effect of atrial fibrillation and mitral regurgitation on calculated mitral valve area in mitral stenosis

Forty-nine patients with mitral stenosis (MS) were studied by Doppler echocardiography and 2-dimensional (2-D) echocardiography to assess the ability of Doppler ultrasound to accurately measure mitral valve orifice area and to assess whether atrial fibrillation (AF) or mitral regurgitation (MR) affected the calculation. Twenty-four patients underwent cardiac catheterization. Mitral valve area by Doppler was determined by the pressure half-time method. Mean mitral valve area of all 49 patients by Doppler and 2-D echocardiography correlated well (r = 0.90). There was good correlation between Doppler and 2-D echocardiography in patients with pure MS in sinus rhythm (r = 0.88), in patients with MR (r = 0.93) and in patients with AF (r = 0.96). In the 7 patients with pure MS in sinus rhythm, there was good correlation between Doppler, 2-D echocardiography and cardiac catheterization (r = 0.95). In patients with either MR or AF, cardiac catheterization appeared to underestimate mitral valve orifice compared with both Doppler and 2-D echocardiography (p less than 0.05). Doppler echocardiography can estimate valve area in patients with MS regardless of the presence of MR or AF.     

Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection

External obstruction of the pulmonary venous return was detected by two-dimensional (2-D) and pulsed Doppler echocardiography and was confirmed by angiography and at surgery in two infants with isolated supracardiac total anomalous pulmonary venous connection (TAPVC) to the left vertical vein. In both infants, the left vertical vein ascended behind the left pulmonary artery and anterior to the left main stem bronchus. This arrangement produces mechanical obstruction to both vertical vein flow and pulmonary venous drainage. Early detection and surgical correction of obstructed pulmonary venous return are necessary to prevent severe right-sided heart failure and death. Doppler echocardiography is recommended when evaluating infants with TAPVC for possible anatomic or mechanical obstruction to pulmonary venous return.     

Two-dimensional and pulsed Doppler echocardiography in the postoperative evaluation of total anomalous pulmonary venous connection

The role of combined two-dimensional and pulsed Doppler echocardiography in the postoperative assessment of patients with total anomalous pulmonary venous connection was evaluated. Twenty-two cases with a median age of 9.5 weeks at the initial examination were evaluated. Serial ultrasound examinations were performed throughout the study period. The ultrasound results were compared with chest radiographs obtained during the same period. Of the 22 patients, 16 had normal pulmonary venous flow profiles characterized by low-velocity laminar flow. Of this group 12 had persistent radiographic postoperative pulmonary edema that cleared in all by 4 months. Six patients with pulmonary venous obstruction were identified, the diagnosis being confirmed at catheterization or autopsy. The venous flow pattern in this group was uniformly high velocity and turbulent. It was possible to localize the site of obstruction in each case. Although pulmonary edema was present in each patient, the chest radiograph did not provide reliable information as to the exact site of obstruction. Combined two-dimensional and Doppler echocardiography is a useful adjunct in the postoperative evaluation of patients with total anomalous pulmonary venous connection.