Hypertrophic obstructive cardiomyopathy. Late follow-up after ventricular myotomy-myectomy.

A series of 25 patients with hypertrophic obstructive cardiomyopathy, isolated or associated with minor anomalies, were operated upon using a modified Morrow's procedure. The indication for operation was based on either the presence of severe symptoms despite treatment with beta-blocking agents, or a significant peak gradient of over 50 mmHg even in asymptomatic patients. All patients survived and none was lost to follow-up. Long-term results were evaluated with 2-dimensional echocardiography. Surgical versus pharmacological treatment is discussed, with particular consideration given to paediatric patients.

Anatomy of the echocardiographic crux cordis in the evaluation of the spectrum of atrioventricular valve atresia.

The echocardiographic crux cordis is defined in normal subjects as the spatial interrelation between the atrial and ventricular septal planes and the plane of the atrioventricular (AV) junction. This crossroad can be visualized using apical, sub-costal, or precordial four-chamber echocardiographic projections. We studied this relationship in 33 patients with right AV valve atresia (29 with absent AV connection, 4 with an imperforate AV valve) and 14 with left AV valve atresia (9 with absent AV connection, 5 with an imperforate valve). An accurate diagnosis of AV valve atresia was successfully achieved in all patients, while the differentiation between an imperforate AV valve and absence of an AV connection was achieved in 45 out of 47 cases. Echocardiography demonstrated in some cases of absent AV connection, an arrangement which at first sight was analogous to that found in hearts with AV concordance and an imperforate AV valve. The differential diagnosis, therefore, must be based on the differing echocardiographic characteristics of the atretic atrial floor. In 31 out of 38 cases with absent AV connection it was possible to obtain simultaneous visualization of the ventricular septum and the atretic atrial floor. This proved helpful in planning the clinical management of these patients. The evaluation of crux cordis anatomy also differentiated AV valve atresia from other cardiac malformations with a similar presentation (valvular hypoplasia and common AV valve). It may prove the most reliable tool for the differential diagnosis of the spectrum of AV valve atresia.

[Echocardiographic evaluation of a single atrioventricular valve: problem of differential diagnosis]. / Valutazione ecocardiografica di una sola valvola atrioventricolare: problemi di diagnostica differenziale.

In the normal subject, the echocardiographic crux-cordis is defined by the spatial relationship between the plane of the interatrial and interventricular septa and the plane of the A-V valves. The bidimensional echocardiographic image of the crux-cordis may be obtained from various approaches: apical, subcostal, or precordial. In the present study we have assessed the usefulness of 2-D echocardiographic analysis of the crux-cordis in identifying the presence of a common A-V valve vs a single A-V valve (i.e. with atresia of the contralateral valve) in univentricular hearts (UH). We have studied 55 patients. Eight patients had a UH with a common A-V valve (6 with a rudimentary chamber, and 2 without a rudimentary chamber). Thirty-three patients were found to have atresia of the right A-V valve (29 with absent connection, and 4 with an imperforated valve), and 14 with atresia of the left A-V valve (9 with absent connection, and 5 with an imperforate valve). A correct differential diagnosis between common A-V valve and atresia of an A-V valve was achieved in 53 out of 55 patients through the direct visualization of the atretic atrial floor and by recognizing the origin of the leaflets of the one patent valve present. Of the patients with UH and common A-V valve, 5 presented a low interatrial septal defect, 3 a single atrium, 3 an interventricular septum which was clearly misaligned with the echocardiographic crux-cordis, in 5 an analysis of the crux-cordis did not permit visualization of a ventricular septal structure of which 3 resulted as false negatives.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pulsed echo-Doppler sensitivity and specificity for evaluation of mitral and aortic valve insufficiency (author's transl)]. / Sensibilita' e specificita' dell'ecocardiografia Doppler ad onda pulsante nella valutazione della insufficienza valvolare mitralica ed aortica.

Our experience with pulsed-Doppler echocardiography in mitral and aortic valve insufficiency is reported. The data resulting from the echo-Doppler analysis were compared to those found in angiocardiography. Left ventriculography was positive for mitral insufficiency in 19 patients and negative in 17 patients. Aortography was positive for aortic insufficiency in 22 patients and negative in 15 patients. The echo-Doppler analysis showed a specificity of 94% and a sensitivity of 86% for mitral valve insufficiency. A specificity of 83% and a sensitivity of 91% was found for aortic valve insufficiency in supersternal notch view. The location of the sample volume and the morphology of the flow profile for each individual valvulopathy are described which proved considerably useful in current diagnostic practice. The possible causes of false positives and negative are discussed. In our series, the data obtained through echo-Doppler analysis were found to be only quantitative and it was not possible to quantity the regurgitation.

Pulsed Doppler echocardiography for diagnosis of ventricular septal defects.

In order to assess the performance of pulsed Doppler echocardiography and to examine the factors affecting its reliability for the diagnosis of ventricular septal defects, 51 patients, in whom angiographic studies had recently been performed, were investigated before and after operation by this technique. The diagnosis was correct in all cases with left-to-right shunt, when the defect was located either in the upper muscular or in the infracristal portion of the septum, even when pulmonary hypertension or additional cardiac malformations were present. Pulsed Doppler echocardiography did not differentiate between supracristal ventricular septal defects and obstruction of the right ventricular outflow. Apical muscular defects were never observed in our cases. Defects of the inlet portion were not localised by the method. In cases with bidirectional shunt, pulsed Doppler echocardiography failed to provide any diagnostic clue. When a right-to-left shunt was present, only in one case with membranous pseudoaneurysm could pulsed Doppler echocardiography provide an exact description of the haemodynamics before and after operation. In 12 of 22 patients with tetralogy of Fallot, pulsed Doppler echocardiography showed flow tracings possibly related to the septal defect. The site of defect and the extent and direction of intracardiac shunting seem to be the main factors affecting its diagnostic performance in ventricular septal defects.

[Bidimensional echocardiography for pre- and post-surgical evaluation of atrio-ventricular canal defects (author's transl)]. / L'ecocardiografia bidimensionale nella valutazione pre- e post-chirurgica dei difetti del canale atrio-ventricolare.

Bidimensional echocardiography was employed for pre- and post-surgical evaluation of patients with Atrio-Ventricular Canal (AVC) defects. In 14 of the total 24 patients the echocardiographic images were compared with the actual anatomy at surgical inspection. In all cases bidimensional investigations provided essential clues toward diagnosis of AVC demonstrating the peculiar anatomic disorders (ostium primum, cleft anterior mitral cusp, anomalies of the left ventricular outflow tract). Moreover it made possible to distinguish between partial and complete forms of AVC demonstrating the degree of septal development and, where it present at the level of the interventricular septum, to show the split of the anterior cusp of the common AV valve into a mitral and tricuspidal components. In postoperative evaluations could be clearly visualized the following: the prosthetic patches, the thickened mitral anterior cusp (an indirect image of the sutured cleft) and, in the complete forms, the newly formed individual valvular ostia. Bidimensional echocardiography improved greatly the performance of ultra-sound investigation for patients with AVC.

[Two-dimensional echocardiography to evaluate anomalies of the great arteries (author's transl)]. / L'ecocardiografia bidimensionale nella diagnosi delle anomalie delle grndi arterie.

Two dimensional Echocardiography was employed to study 51 subjects with complex anomalies of the great arteries. Within a short period of time, they underwent also angiographic investigations and corrective surgery. The investigation was performed orienting the transducer according to two approaches. --Short-axis view: at the level of the origin of the great arteries, and from the basis of the ventricular septum. --Long-axis view: along the longitudinal axis of the heart. In all cases two dimensional Echocardiography could clarify the mutual interrelation of the great arteries, according to the classification by de La Cruz, and the relation between the great arteries and the ventricular septum. Only in two cases, where the dimensions of the ventricular septal defect were particularly large, the position of the great arteries relative to the septum itself could not be assessed.

Cervical aortic arch and a new type of double aortic arch. Report of a case.

A case of cervical aortic arch is reported. To the best of our knowledge, it is the first to be associated with a serious intracardiac anomaly. In addition, it is part of a new type of double aortic arch, caused by failure of reabsorption of both dorsal aortic roots and persistence of the fourth right and second (or third) left branchial arches.