Mitral atresia with premature closure of foramen ovale. A rare hemodynamic cause for failure of Blalock-Taussig anastomosis to relieve inadequate pulmonary blood flow.

The clinical, hemodynamic, and surgical findings encountered in the management of a hypoxic male infant with a rare and complex variety of cyanotic congenital heart disease associated with inadequate pulmonary blood flow are described. A poor clinical response to creation of a Blalock-Taussig anastomosis led to the discovery of mitral atresia complicated by premature closure of the foramen ovale and partially relieved by the presence of a levoatriocardinal vein. The subsequent creation of an atrial septal defect enhanced the function of the subclavian artery to pulmonary artery anastomosis and provided palliative relief of hypoxia. Some of the clinical and laboratory findings indicating the presence of additional lesions complicating the picture of a tetralogy of Fallot and requiring additional surgical considerations are discussed. The experience indicates that hemodynamic as well as surgical causes may explain the failure of a systemic artery to pulmonary artery anastomosis to function adequately and should be sought.

Posterior deviation of left ventricular outflow tract septal components without ventricular septal defect.

OBJECTIVE: To describe 11 patients with narrowing of the left ventricular outflow tract caused by angular posterior deviation of both the outlet septum and the upper part of trabecular septum, which was diagnosed by cross sectional echocardiography in all and confirmed by angiocardiography in seven. RESULTS: Four patients had a subaortic systolic pressure gradient ranging from 23 to 70 mm Hg by Doppler echocardiography; cardiac catheterisation showed a significant (60 and 104 mm Hg) systolic pressure gradient in two. In four cases aortic regurgitation and two tricuspid pouches were shown by Doppler echocardiography, angiocardiography, or both. Four cases had a ridge at the angulation point on echocardiographic examination. Three patients were operated on for systolic pressure gradients of the left ventricular outflow tract and one for severe aortic regurgitation. There was proliferation of collagen-rich fibrous tissue in the subendocardial region on histopathological examination of the myectomy material. A ventricular septal defect had been diagnosed previously by contrast echocardiography in one patient; thus ventricular septal defects may close spontaneously over a period of time including fetal life. A subaortic ridge was detected in one patient at follow up. CONCLUSIONS: Deviation of the outlet and trabecular septa should be considered as a cause of ventricular outflow tract obstruction even when no ventricular septal defect is present.

Intracardiac thrombosis diagnosed by echocardiography in childhood: predisposing and etiological factors.

Eleven cases of intracardiac thrombi caused by different factors including protein-C deficiency are presented for discussion of the etiology and predisposing factors of intracardiac thrombi during infancy and childhood, and to stress the importance of protein-C deficiency as an etiological factor. Thrombi were localised in the left heart in five patients and right heart in five patients. One patient had both-sided thrombi. Four of our patients had dilated cardiomyopathy, one had mitral valve hypoplasia, and one had pulmonary valvar stenosis as the predisposing factors for thrombus formation. In three patients whose cardiac anatomies were completely normal, we determined protein-C deficiency as an etiological factor of thrombus formation. One of these had congenital protein-C deficiency and the other two had acquired temporary protein-C deficiency due to sepsis. In conclusion we recommend that protein-C deficiency should be investigated as an etiological factor in all cases of intracardiac thrombi irrespective of whether or not another predisposing factor is identified.

Surgical treatment in tetralogy of Fallot diagnosed by echocardiography.

The purpose of this paper is to present the authors' 3-yr experience of echocardiographic examination of patients with the clinical diagnosis of tetralogy of Fallot, and their evaluation for surgical treatment without prior cardiac catheterization. Among the patients with the clinical diagnosis of tetralogy of Fallot 227 had a definite diagnosis made by M-mode, two-dimensional, Doppler and contrast echocardiography. For the diagnosis of tetralogy of Fallot, ventricular septal defect, pulmonary stenosis, and overriding of the aorta were considered to be fundamental. Ventricular septal defect could be seen easily in the subaortic region by two-dimensional echocardiography. However, in some patients whose ventricular septal defect was not seen clearly, peripheral vein contrast echocardiography was performed. The diameters of pulmonary artery, and main branches at a few millimeters distal to their origin were measured. These parameters were correlated with the aortic diameter for evaluation as to whether they were able to accept the total cardiac output. In patients whose left ventricular end-diastolic dimension was small, shunt operation was preferred. In 115 patients the pediatric cardiologist performing the echocardiography thought that cardiac catheterization was necessary. In these cases the reliability of echocardiography in detecting important cardiac abnormalities was evaluated. Detection of ventricular septal defect, presence of pulmonary valve, detection of stenosis on the pulmonary bifurcation and/or main branches revealed a high sensitivity. Two-hundred-and-one patients diagnosed by echocardiography underwent total correction. In all cases except one the preoperative diagnosis was confirmed by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Double-chambered right ventricle: experience with 52 cases.

The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.

The accuracy of antenatal fetal echocardiography.

The purpose of this study was to evaluate our experience with a group of patients who were either selected by us or referred by an obstetrician or geneticist with the indication of fetal echocardiography. This prospective study was done on 128 cases between 1996-1998. Maternal age range was between 16 and 41 years (mean: 28.79). Gestational age range was between 15 and 37 weeks (mean: 26). In the postnatal period the newborn babies were reevaluated for cardiovascular system abnormalities by physical examination, ECG, telecardiogram and, if it was necessary, by echocardiography, cardiac catheterization and angiography. By comparing prenatal and postnatal findings, sensitivity and specificity of fetal echocardiographic diagnosis were determined. Among the total cases studied, nine had major congenital heart disease in postnatal evaluation. Two cases had false negative; there were no false positive prenatal diagnoses. Sensitivity of echocardiographic diagnosis was 100 percent and specificity 78 percent. Three patients had paroxysmal atrial tachycardia and two atrioventricular block We concluded that the fetal echocardiography is a very useful technique in the evaluation of the fetal cardiovascular system. However, awkward fetal position, severe maternal obesity, and technologic insufficiency of the echo machine may result in unfavorable scanning conditions. Minuteness of anomaly may also result in a false negative prenatal evaluation.

Echocardiographic assessment of left and right ventricular diastolic functions in children with dilated cardiomyopathy.

We evaluated left and right ventricular diastolic functions by pulsed Doppler echocardiography in 16 children with dilated cardiomyopathy and in 20 healthy age-matched control subjects. The cardiomyopathy group demonstrated an abnormal relaxation pattern of the left ventricle. In the cardiomyopathy group compared to normal subjects, peak early filling velocities (43.3 +/- 11 cm/s versus 60.4 +/- 11 cms/s, p < 0.01), the corresponding velocity-time integrals (3.3 +/- 1.4 cm versus 4.6 +/- 1.2 cm, p < 0.01) and the ratio of peak early filling velocity to late filling velocity (1.22 +/- 0.47 versus 1.49 +/- 0.23, p < 0.05) were significantly lower whereas isovolumic relaxation time was significantly longer (58.9 +/- 19.8 ms versus 49.7 +/- 8.9 ms, p < 0.05). In addition, right ventricular diastolic filling was also impaired in children with dilated cardiomyopathy. Peak early filling velocities (41 +/- 7.9 cm/s versus 47.5 +/- 8.8 cm/s, p < 0.05) and the corresponding velocity time integrals (3.0 +/- 1.0 cm versus 3.87 +/- 1.1 cm, p < 0.05) were significantly decreased, while isovolumic relaxation time was significantly increased (60.6 +/- 16.3 ms versus 52.2 +/- 12.8 ms, p < 0.05) in the cardiomyopathy group. The study suggests that abnormalities of both right and left ventricular diastolic function may occur, and should be searched for, in pediatric patients with dilated cardiomyopathy.

Echocardiographic findings in endomyocardial fibrosis.

An 18-month-old infant diagnosed as having endomyocardial fibrosis by echocardiography is presented. Most patients with endomyocardial fibrosis reported in the literature are either older children or adults. To our knowledge, our patient was the youngest ever to have been reported. Echocardiographic studies showed obliteration of the left ventricular apex and increased echo reflectance at the left ventricular endocardium and subendocardium. The left atrium and right ventricle were significantly enlarged. Doppler echocardiography showed minimal mitral, but significant tricuspid regurgitation. In regard to the contribution of echocardiography in the diagnosis, we recommend this method for suspected cases. Contrary to the other patients reported, there was no thickening of the atrioventricular valves. Mitral valve insufficiency was related to the restriction of the ventricular filling rather than to valve involvement occurring with the disease.

Left ventricular size following shunt operation in tetralogy of Fallot.

This study was performed in 24 patients with tetralogy of Fallot in whom shunt operation was performed instead of total correction because of small left ventricular end-diastolic dimension. Left ventricular end-diastolic dimension was measured using M-mode and two-dimensional echocardiography pre- and at least one year postoperatively. There was no change in the postoperative left ventricular size in two patients. However, in the other 22 patients, the left ventricular dimension was increased to 70 to 103 percent of normal left ventricular size. According to the findings of this study, we can conclude that the patients in whom shunt operation was performed would most likely have an increased left ventricular size over time.

Congenital sick sinus syndrome with breath holding and severe syncope episodes during infancy. A case report.

Sick sinus syndrome is a rare cause of bradycardia in children without structural heart disease. A case of profound sinus bradycardia, sinus arrest with junctional escape, and pauses in a two-year-old infant with breath-holding and syncope episodes is presented. As a result of these clinical symptoms and electrocardiographic findings, the patient with sick sinus syndrome underwent implantation of transvenous ventricular pacemaker. He has been well and asymptomatic since the insertion of the pacemaker. In the differential diagnosis of an infant with breath-holding and syncope episodes, when these symptoms in particular cannot be explained by other common reasons, sick sinus syndrome should be kept in mind. This case also illustrates the importance of electrocardiographic studies for the diagnosis.

Ventricular diastolic filling indices in pulmonary stenosis.

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

Detection of intracardiac left-to-right shunts in children by contrast echocardiography and the radionuclide technique.

This study was carried out in the Departments of Pediatrics and Nuclear Medicine at Hacettepe University Faculty of Medicine between April 1986 and March 1988 with the aim of detecting left-to-right shunts in 70 patients with isolated ventricular septal (VSD) defect, isolated atrial septal defect (ASD) and patent ductus arteriosus, by using the radionuclide technique together with contrast echocardiography. In some patients, the results were confirmed by both cardiac catheterization and angiocardiography. Most of the data were confirmed during surgery. A good correlation was found between the results of contrast echocardiography and radionuclide angiography. The difference between the QP/QS value and the grade of positive contrast effect was tested statistically and "p" was found to be less than 0.001. Therefore, we conclude that these methods could be applied together to determine the indication for surgery in cases with ASD or VSD.

Two-dimensional and Doppler echocardiographic findings of congenital coronary artery fistula.

Three patients with coronary artery fistula confirmed by coronary angiography and surgery were studied using two-dimensional and Doppler echocardiography. Two of these patients had left coronary artery-right ventricle fistula. The proximal dilatation of the coronary arteries was visualized in all three patients by two-dimensional echocardiography. The course of the dilated right coronary artery and left circumflex artery was demonstrated by two-dimensional echocardiography in two patients. We were able to detect the disturbed flow at the drainage site in one patient by using the conventional Doppler echocardiography. In conclusion, it should be emphasized that two-dimensional and Doppler echocardiography can be useful methods in diagnosing coronary artery fistula.

Two-dimensional and Doppler echocardiography in the diagnosis of absent pulmonary valve syndrome (surgery without catheterization).

The majority of severely affected infants with absent pulmonary valve syndrome develop varying degrees of respiratory distress. Cardiac catheterization and angiocardiography are still performed in the diagnosis of these infants prior to surgery. However, considering the high risks of these invasive investigations for severely symptomatic infants, diagnosis using only noninvasive methods becomes important. In this regard, we present three cases of absent pulmonary valve syndrome, diagnosed pre-operatively by both two-dimensional and Doppler echocardiography. The diagnosis was confirmed by cardiac catheterization and angiocardiography in two cases and by surgery in a symptomatic infant. Therefore, we are of the opinion that the two-dimensional and Doppler echocardiographic methods are most reliable in diagnosing absent pulmonary valve syndrome, and that severely symptomatic infants can be referred for surgery without catheterization.

The angiocardiographic analysis of 73 patients with double-outlet right ventricle.

The purpose of this study was to determine the cardiac anatomy of patients with double-outlet right ventricle by angiocardiography. A total of 73 patients between the ages of one day and 11 years were examined. The aorta was on the right side of the pulmonary artery in 23 cases (32%), right anterior in 20 (27%) and right posterior in 17 cases (23%). Pulmonary stenosis was found in 53 patients (73%) and subaortic stenosis in six cases. Ventricular septal defect (VSD) was subaortic in 39 cases (52%), remote type in 17 (23%), doubly committed in 10 (13%), and subpulmonic in 9 (12%). Double VSD was noted in two patients. Pulmonary hypertension was more frequent in subpulmonic ventricular septal defect (78%). The most common associated anomalies were atrial septal defect (34%), anomalous coronary arteries (12%) and endocardial cushion defect (10%). Aortic root angiography was not satisfactory in half of the cases with coronary arterial anomaly. In conclusion, double-outlet right ventricle is a complex anomaly, all of whose cardiac features can be successfully demonstrated in detail by echocardiography and angiocardiography. However, in order to determine the anatomy of the coronary arteries, selective coronary angiography may be necessary in some patients.

"Acquired" subvalvular aortic stenosis after repair of several congenital cardiac defects.

Discrete subvalvular aortic stenosis is a progressive lesion. In this report we presented nine patients who had no significant left ventricular-aortic obstruction at initial cardiac catheterization or echocardiographic examination, but later developed significant subvalvular aortic stenosis. Associated lesions included ventricular septal defect in three, patent ductus arteriosus in two, aorticopulmonary window in one, tetralogy of Fallot in one, supramitral membrane in one, and ventricular septal defect and patent ductus arteriosus in one case. Nine patients were diagnosed with subvalvular aortic stenosis 18 months to eight years after surgical correction. Eight of the patients required surgery for subvalvular obstruction. In conclusion, discrete subaortic stenosis is a rare, late complication of the surgical repair of several congenital heart defects. It is a progressive lesion after surgery; therefore these patients require careful follow-up.

Peripheral vein contrast echocardiography in atrial septal defect.

This study was carried out on 233 children suspected clinically of having atrial septal defect with the aim of investigating the diagnostic capability of peripheral venous contrast echocardiography. The transfer of contrast material from the right atrium into the left atrium was evaluated as "positive contrast", while noncontrast blood, passing from the left atrium into the right atrium was termed "negative contrast". Positive contrasts were quantitated in four grades. A significant negative contrast effect was graded 3- or 4-. Three positive, 4+ and/or 3-, 4- contrast effects were considered definite evidence of an atrial septal defect. Among the cases with the above findings 92 underwent surgical closure of atrial septal defect. The procedure was successful in all patients operated; the size of the defect was large. This result demonstrates that the method applied is a safe and reliable one. However, in a group of cases without the above echocardiographic findings the presence of an atrial septal defect was detected by cardiac catheterization and angiocardiography. Therefore, we can conclude that the method applied is not a sensitive, but a specific one, for definite detection of atrial septal defect.

Computerized diagnosis of respiratory disorders. SVM based classification of VAR model parameters of respiratory sounds.

INTRODUCTION: This article is part of the Focus Theme of Methods of Information in Medicine on "Biosignal Interpretation: Advanced METHODS for Studying Cardiovascular and Respiratory Systems". OBJECTIVES: This work proposes an algorithm for diagnostic classification of multi-channel respiratory sounds. METHODS: 14-channel respiratory sounds are modeled assuming a 250-point second order vector autoregressive (VAR) process, and the estimated model parameters are used to feed a support vector machine (SVM) classifier. Both a three-class classifier (healthy, bronchiectasis and interstitial pulmonary disease) and a binary classifier (healthy versus pathological) are considered. RESULTS: In the binary scheme, the sensitivity and specificity for both classes are 85% ± 8.2%. In the three-class classification scheme, the healthy recall (95% ± 5%) and the interstitial pulmonary disease recall and precision (100% ± 0% both) are rather high. However, bronchiectasis recall is very low (30% ± 15.3%), resulting in poor healthy and bronchiectasis precision rates (76% ± 8.7% and 75% ± 25%, respectively). The main reason behind these poor rates is that the bronchiectasis is confused with the healthy case. CONCLUSIONS: The proposed method is promising, nevertheless, it should be improved such that other mathematical models, additional features, and/or other classifiers are to be experimented in future studies.