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Comparative evaluation of the pulmonary arteries in patients with right ventricular outflow tract obstructive lesions.

The purpose of this prospective study was to assess and compare the roles of CT, sonography, and cineangiography in the evaluation of the central pulmonary arteries. Twenty patients with severe cyanotic congenital heart disease were evaluated. In six patients, cineangiography failed to identify pulmonary arteries (four right, two left) that were seen by CT. Narrowing within the right pulmonary artery was seen more clearly by CT and sonography than by cineangiography in one-third of patients. By contrast, narrowing of the origin of the left pulmonary artery shown by cineangiography was not detected by either CT or sonography in four of four patients. CT has a complementary role and should be performed when the pulmonary arteries are not well seen by cineangiography or sonography, confirmation of the patency and adequacy of the pulmonary arteries is necessary before complete repair, or documentation of interval growth of the pulmonary arteries is desired after palliative surgery.

Adolescent↗

[Fixed subvalvular left ventricular outflow tract obstruction. Comparison of the diagnostic usefulness of mono- and bi-dimensional echocardiography].

Because Cross-Sectional Echocardiography offers the advantage of correct spatial orientation, it is said to permit a more exact localization of the level of the outflow tract obstruction and a determination of the nature of the lesion. Our experience confirms that B-Mode Echo is more reliable in diagnosing an extensive sub-valvular narrowing, while the existence of a thin sub-valvular membrane can also be detected by M-Mode examination. The latter has moreover proven very useful in that it records indirect signs of obstruction, such as a mid-systolic closure of the aortic cusps. We therefore suggest that both methods should be employed in studying these patients.

Aortic Stenosis, Subvalvular↗

Left ventricular rhabdomyoma. A case report.

BACKGROUND: Cardiac tumours in infancy are rare, with a predominant incidence of rhabdomyoma. Diagnosis can best be obtained by pre-cordial and transoesophageal echocardiography. Because of the tendency for spontaneous regression conservative therapy is generally recommended. A rare indication for surgical treatment is described. METHODS EXPERIMENTAL DESIGN: case report. SETTING: Pediatric cardiology and cardiac surgery in a university hospital. PATIENT: a case of a rhabdomyoma of the left ventricle is described which was attached to the left ventricular outflow tract and the left coronary cusp of the aortic valve, causing outflow obstruction of the left ventricle. INTERVENTION: the tumour was removed surgically using cardiopulmonary bypass. MEASURE: achievement of relief of left ventricular outflow obstruction. RESULTS: Immediate and complete relief of the left ventricular outflow tract was achieved with preservation of the aortic valve. The patient recovered well. CONCLUSIONS: Cardiac rhabdomyomas can usually be treated conservatively. Surgical therapy can be successfully applied when rhabdomyoma results in hemodynamic problems or arrhythmias.

Echocardiography, Transesophageal↗

[Left ventricular remodeling in patients with hypertrophic obstructive cardiomyopathy treated with percutaneous alcohol septal ablation: an echocardiographic study].

INTRODUCTION: In patients with hypertrophic obstructive cardiomyopathy, obstruction in the left ventricular outflow tract may generate more hypertrophy. Our aim was to evaluate the impact of reducing ventricular outflow tract obstruction on left ventricular hypertrophy and remodeling after alcohol septal ablation. PATIENTS AND METHOD: 20 patients with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation were included. Doppler echocardiography was performed in all patients at baseline, immediately after alcohol septal ablation, and at 3 and 12 months' follow-up. Left ventricular diameters and wall thickness and pressure gradients in the ventricular outflow tract were determined. RESULTS: Immediately after alcohol septal ablation, ventricular outflow tract pressure gradient decreased from 63.0 27.7 to 28.2 24.7 mmHg (p < 0.001), without significant changes in left ventricular dimensions. However, after 12 months we observed an increase in left ventricular end-diastolic (from 47.1 4.9 to 50.8 4.5 mm) and end-systolic diameter (from 27.1 3.0 to 33.7 4.6 mm), as well as a reduction in septal (from 19.5 4.0 to 15.5 2.7 mm) and posterior wall thickness (from 14.0 2.2 to 12.9 1.3 mm) (p < 0.01 in all cases). Left ventricular end-diastolic and end-systolic volumes increased (from 106.4 26.9 to 123.1 28.7 ml and from 50.2 17.3 to 56.7 18.3 ml, respectively, p < 0.01 in both cases), without changes in left ventricular ejection fraction. The reduction in ventricular outflow tract pressure gradient at 12 months' follow-up correlated significantly with the increase in left ventricular end-systolic diameter (r = 0.63; p < 0.01). CONCLUSIONS: In patients with hypertrophic obstructive cardiomyopathy who underwent alcohol septal ablation, relief of ventricular outflow tract obstruction is associated with an increase in left ventricular chamber diameters and volume. These findings suggest that middle- and long-term ventricular remodeling and regression of hypertrophy occur in these patients, which may contribute to their clinical improvement.

Cardiomyopathy, Hypertrophic↗

Pre-operative evaluation with MR in tetralogy of fallot and pulmonary atresia with ventricular septal defect.

PURPOSE: To assess whether MR imaging could replace angiography in preoperative evaluation of patients with tetralogy of Fallot and pulmonary atresia with ventricular septal defect (VSD), especially since the surgical correction was done earlier than was previously the rule. MATERIAL AND METHODS: Fourteen patients with tetralogy of Fallot (n = 10) or pulmonary atresia with VSD (n = 4), mean age 7.5 +/- 4.4 months, were evaluated with angiocardiography and MR before definitive surgical correction. RESULTS: There was good diagnostic agreement between the two modalities when evaluating right ventricular outflow obstruction; 86% for valvular and 93% for supravalvular stenosis, but the agreement was somewhat lower for the subvalvular obstruction (57%). Surgery findings, however, were in favour of MR in 5 patients concerning the subvalvular right ventricular outflow tract obstruction. MR images identified all stenoses in the right and left pulmonary arteries, but overlooked one stenosis in the main pulmonary artery. MR could evaluate patency in all palliative shunts. CONCLUSION: Even in this young age group, MR imaging offers a good alternative to angiocardiography for the pre-operative evaluation of the right ventricular outflow tract, the main pulmonary artery and the proximal right and left pulmonary arteries, before definitive surgical correction of tetralogy of Fallot and pulmonary atresia with VSD.

Abnormalities, Multiple↗

[The role of transesophageal echocardiography (TEE) in assessment of left ventricular tract obstruction].

Twelve children with subaortic stenosis were analysed. Nine of them developed left ventricular outflow obstruction after surgery for congenital heart disease (VSD + IAA, VSD + DORV, VSD + TGA, VSD + CoA) and then developed an isolated form of primary stenosis. Both echocardiographic exams, TTE and TEE were performed in all patients. TTE was sufficient to assess isolated subaortic stenosis. In children after cardiosurgery, TEE was more reliable and provided more detailed visualisation of the stenosis and its relationship to surrounding structures.

Adolescent↗

Electrical activation of ventricles and interventricular septum in hypertrophic obstructive cardiomyopathy.

In 10 cases with proven left ventricular outflow obstruction, we studied the time sequence of activation at the epicardial surface, in the left ventricular wall, and in the interventricular septum by means of epicardial exploration and intramural needle electrodes carrying 10 to 20 terminals, during surgical exposure. A variable delay (15-40 msec) was found in subendocardial activation of the anterior paraseptal left ventricular wall, probably caused by a block in the anterior division of the left bundle-branch. Epicardial excitation is additionally retarded by the increased diameter of the left ventricular wall because of hypertrophy. Conduction velocity in the left ventricular wall and in the interventricular septum was found to be approximately normal: +/-45 cm/sec; activation of the hypertrophic interventricular septum starts at normal times and proceeds mainly from left to right, with a smaller contribution from right to left; both fronts meet to the right of the middle of the interventricular septum; total septal activation time is prolonged because of hypertrophy.

Adolescent↗

Accessory mitral valve tissue causing severe subaortic stenosis with dextrocardia in a premature newborn.

We report an unusual case of left-ventricular outflow obstruction caused by accessory mitral valve tissue associated with dextrocardia and ventricular septal defect in a seven-day-old, 2200 grams premature infant, who was referred with a heart murmur. The diagnosis was made by two-dimensional and Doppler echocardiography which demonstrated the accessory tissue as well as a 100 mmHg peak systolic gradient between the left ventricle and the aorta. Ten days after the presentation the infant underwent emergency surgery after respiratory arrest and recurrent episodes of syncope. The accessory mitral valve tissue and its fibrous extension were excised and the ventricular septal defect was closed. We believe that surgical treatment of patients with accessory mitral valve tissue should be performed early because of the possibility of acute deterioration. Combined aortotomy and interatrial approach is very helpful in evaluating the anatomy and the mitral valve function as well as delineating the tissue to be excised.

Aortic Valve Stenosis↗