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[Rastelli operation for complete transposition of the great vessels, ventricle septum defect and pulmonary atresis].

We report about a 6 year old patient with complete transpositon of the great arteries, a ventiricle septum defect, and pulmonary atresia, who was operated using Rastellis technique. A Hancock-prothesis of of 20 mm diameter was used for reconstruction of the outflow tract of the right ventricle and anastomosed distal with the pulmonary artery on the left side of the aorta. The proximal anastomosis was performed with the right ventricle after a longitudinal ventriculotomy. The intraoperative pressure recordings and the post-operative studies revealed a very good early result.

Blood Pressure↗

[Anatomically corrected transposition of the great vessels].

The paper is concerned with the description of a rare congenital heart disease: anatomically corrected malposition of major vessels in a 9-mos 24 day old girl. The diagnosis of this disease was shown on the results of angiocardiography, concomitant congenital heart diseases were described. This abnormality is characterized by common atrioventricular and ventriculovascular joints and inversion position of the major vessels, it is always attended by congenital heart diseases, such as an interventricular septal defect combined with pulmonary stenosis. Surgical intervention is aimed at the elimination of concomitant heart diseases.

Angiocardiography↗

[Results of Mustard's operation for transposition of the great vessels. Apropos of 81 cases of children who survived the intervention with a follow-up of from 1 to 6 years].

In a series of 81 children who survived to Mustard's operation, with a follow-up varying from 6 months to 6 years, there were 19% of secondary deaths, 19% of bad results, 29% of questionable results and only 33% of good results. The most frequent complications were: disturbances of cardiac rhythm and obstructions to pulmonary or caval venous return.

Adolescent↗

Left subclavian steal, interrupted aortic arch, complete transposition of the great vessels and single left ventricle.

Congenital subclavian steal consists of atresia of the proximal end of the subclavian artery, while the blood supply to the distal portion of the subclavian artery originates from the circle of Willis. It should be suspected in complicated malformations of the heart associated with marked hypoplasia or interruption of the aortic arch. Minimal differences in blood pressure determinations may be an important clue in the diagnosis of this anomaly. The diagnosis may be confirmed by angiography with careful attention to late arterial phase films.

Abnormalities, Multiple↗

Mitral systolic honk in a case of congenitally corrected transposition of the great vessels.

A woman suffering from TCGV associated with steno-insufficiency with a prolapse of left atrioventricular valve (tricuspid) and an insufficiency with a prolapse of right atrioventricular valve (mitral) presented a systolic honk which was to be registered in all the precordial area. The increase of this noise at the end of inspiration and after amyl nitrite, in the absence of any relevant change as to the prolapse of both atrioventricular valves, demonstrated that the honk originated from the right atrioventricular valve more than from the left one.

Adult↗

Left anterior and left posterior hemiblock in tricuspid atresia and transposition of the great vessels: observations and electrocardiographic nomenclature and electrophysiologic mechanisms.

A patient with tricuspid atresia and characteristic electrocardiographic features of counterclockwise and superiorly oriented frontal plane QRS loop (left anterior hemiblock) is presented. Operative intervention resulted in a clockwise and inferior rotation of the frontal QRS loop (left posterior hemiblock) without the development of complete left bundle branch block. This observation suggests that the electrocardiographic pattern of left anterior hemiblock may result from other mechanisms in addition to block of the left bundle branch fibers oriented toward the anterior part of the left ventricle. The case further suggests that electrocardiographic patterns of apparent A-V conduction defects may not be at all associated with true block in the A-V conduction system. Further, it emphasizes the fact that various electrophysiologic mechanisms may account for identical electrocardiographic patterns.

Child↗

[Palliative treatment of transposition of the great vessels. Indications and results. (author's transl)].

From 1973 to 1977, 93 infants (63 newborns) with d-TGA were treated with Blalock-Hanlon operation. All children had Rashkind atrioseptostomy at birth. 65 infants with isolated TGV were operated upon with two deaths (3%) and without any complication. 18 patients with TGV and VSD were treated with Blalock-Hanlon operation and Pulmonary Artery Banding and resulted in 5 deaths (28%). The other 10 cases had complex lesions. The overall operative mortality is 8.6%. The late mortality is 3.5%. The authors conclude that the Blalock-Hanlon operation is a safe procedure even in complex lesions. This kind of surgery does not seem to induce any arythmias but the authors did not perform any 24 hours ECG recording. This type of surgery makes the repair (Mustard operation) easier since the atrial septum is already removed.

Heart Septal Defects, Ventricular↗

[Results of complete correction of various forms of transposition of the great vessels in the infant].

Over the past 3 years, a Mustard operation has been performed in 38 infants aged 30 days to 24 months, among them 29 less than one year of age. There were 27 TGV with intact ventricular septum, 8 TGV and VSD, (isolated in 3, and associated with PS in 2 and with coarctation of the aorta in 3), 2 TGV with isolated PS, and 1 TGV with aorta-pulmonary window. The operation was carried out under deep hypothermia, circulatory arrest and limited by-pass, using a pericardial patch in all cases but one. The operative mortality is 8% (3 cases) for the whole group, 7% in infants less than one year of age, and 3% for all TGV with intact ventricular septum. The long term results are reviewed, with a follow-up of 2 to 36 months (mean 17 months).

Aortic Coarctation↗

Cardiovascular response to exercise after the Mustard operation for simple and complex transposition of the great vessels.

After the Mustard operation, patients have reduced exercise tolerance, abnormal right and left ventricular responses to exercise and cardiac rhythm disturbances. The cardiovascular response to exercise was measured noninvasively in 19 patients from 4.5 to 20 years (mean 10.3) after operation. Mean work performed and maximal oxygen uptake for the group were substantially subnormal (42 +/- 23% and 59 +/- 18% [mean +/- 1 standard deviation] of the predicted values, respectively). Resting heart rate, blood pressure, systemic arterial blood oxygen saturation, cardiac index, stroke volume and systemic vascular resistance were not significantly different from control values. At maximal exercise, heart rate, systemic arterial blood oxygen saturation, cardiac index and stroke volume were significantly reduced in comparison with control values. After the Mustard operation, cardiovascular status at rest may be relatively normal, but during maximal exercise, marked abnormalities occur in nearly all indexes of cardiovascular function. Decreased cardiac output response to exercise is a result of decreased stroke volume response and, to a lesser extent, diminished heart rate. It is associated with abnormally increased total systemic vascular resistance.

Adolescent↗

[Atrioventricular and ventriculoarterial discordance (corrected transposition of the great vessels). Diagnosis with bidimensional echocardiography].

We present 15 patients with atrioventricular and ventriculoarterial discordance diagnosed with two-dimensional echocardiography from January 1973 to June 1991. The 15 had situs solitus and six dextrocardia. A different level of insertion of the atrioventricular valves worth from 0.77 +/- 0.3 cm, was observed in all the cases which didn't perimembranous inlet ventricular septal defect. Ten patients, presented septal insertion of one of the chords of the left atrioventricular valve, while, this insertion wasn't observed in the right atrioventricular valve. The ventricular morphology and trabeculation, permitted the identification of both ventricles in all the cases. The subxifoidea view resulted in being fundamental, for the examination of the ventriculoarterial connections. Ten cases (66.6%) had an obstruction in the pulmonary outflow tract (6 in the valve and 4 in subvalvular area) and nine (60%) ventricular septal defect (7 perimembranous and 2 muscular). We believe that the two-dimensional echocardiography gives enough information to arrive at the correct diagnosis of this cardiac malformation and of the associated anomalies. This information can be completed with the use of the color-flow Doppler.

Echocardiography↗

[Current status of Senning's physiologic correction of transposition of the great vessels. Personal experience].

From January 1978 to December 1985, 70 children affected by TGA have been operated with the Senning operation. Among the 70 cases, 64 were simple TGA and 6 were TGA + VSD. The mean age at operation was 6 months (range 2 day - 3 years). Of 64 cases with simple TGA, two (respectively of 2 days and 40 days) died with an operative mortality rate of 3.1% of 6 cases with TGA and VSD, one died with a mortality rate of 16.6%. The total mortality was 4.2%. Since 1981 in a continuative series of 47 patients there were no deaths. Our current policy is now the following: after the diagnosis of simple TGA by ECHO, if the Rashkind septostomy is successful, we perform Senning operation over 3 months; if the Rashkind is unsuccessful, we give prostaglandin (PGE1) for a long period (25-30 days) and in case we do a Blalock-Hanlon septectomy. In conclusion we think that in our hands, the policy followed in the treatment of simple TGA is justified by the results achieved but this policy does not prevent us from using different techniques if the results are better.

Follow-Up Studies↗