[Acute aortic dissection and peripheral ischemia. Diagnostic and therapeutic problems. Remote control using nuclear magnetic resonance]. / Dissection aortique aiguë et ischémie périphérique. Problèmes diagnostiques et thérapeutiques. Contrôle à distance par résonance magnétique nucléaire.

This study concerns 7 cases of acute aortic dissection associated with visceral and/or lower limb ischemia. Only those cases are included which raised diagnostic and therapeutic problems. Patients were excluded who had purely angiographic involvement of an aortic branch and minor rapidly resolving ischemic syndromes. Five of the 7 patients presented type B (type III or distal) and 2 type A (type I or proximal) dissection. All patients received anti-hypertensor medical treatment. All but one had undergone surgery at least once at the acute stage. Five had been followed up and monitored by magnetic resonance imaging (MRI). One type A and 4 type B dissections were thus reviewed between the 15th month and the 9th year. Diagnostically, aortography was found to be inaccurate twice because of incomplete exploration of the thoracoabdominal aorta. Therapeutically, a case of intraoperative death occurred during replacement of the ascending aorta. Thus, out of the 6 patients who survived the acute stage, 4 are alive and asymptomatic, one has been lost sight of and the other died in year 5 after surgery for chronic dissecting aneurysm of the aortic arch. Among the 5 patients examined by MRI, 4 presented aortic ectasia, chronic dissecting aneurysm of the aortic arch and/or a descending aorta with a diameter between 45 and 65 mm. The patient with subnormal aortic diameter had his ascending aorta replaced (the follow-up period at this writing is only 27 months). Among 3 patients who were examined twice, one showed improvement after a year's interval, with a 5-mm increase in the caliber of the dissected aorta.(ABSTRACT TRUNCATED AT 250 WORDS)

[Left pulmonary artery sling associated with right pulmonary hypoplasia and ventricular septal defect]. / Artère pulmonaire gauche aberrante associée à une hypoplasie pulmonaire droite et á une communication interventriculaire.

The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.

[Magnetic resonance imaging in dissection of the aorta]. / Imagerie par résonance magnétique au cours des dissections de l'aorte.

In order to evaluate the usefulness of this new exploratory method in dissection of the aorta, 13 patients (10 men and 3 women, mean age 58 +/- 14 years) were examined by means of an 0.5 Tesla Magniscan 5000 nuclear magnetic resonance machine. Fifteen examinations were performed: 5 in patients with actual aortic dissection (type A 4 including one acute, type B1), 5 in patients previously operated upon for type A dissection and 5 for suspected acute aortic dissection unconfirmed by other examinations. Magnetic resonance imaging (MRI), synchronized with electrocardiography, was carried out using spin-echo techniques with 1 to 3 echoes and multiple contiguous sections along several planes. In the 5 patients with actual dissection the 2 channels separating the membrane could be demonstrated, thus providing a firm diagnosis (MRI proved superior to angiography in 2/5 patients). All 5 patients could be classified as either type A or type B. Extension to the cervical vessels was observed in 3 cases and the portal of entry was seen in one case, but the origin of the coronary arteries was never visualized. In the 5 patients previously operated upon MRI provided detailed analysis of the prosthesis of the supra-coronary ascending aorta implanted in all cases, of the aorta proximal and distal to that segment and of the periaortic mediastinum. By studying the signal from the false channel distal to the prosthesis information was obtained as to the presence in it (2 patients) or absence of circulating blood.(ABSTRACT TRUNCATED AT 250 WORDS)

[Atheromatous lesions of the proximal aorta. Severe complications of homozygous type IIa hypercholesterolemia in children]. / Les lésions athéromateuses de l'aorte proximale. Complications graves de l'hypercholestérolémie type IIa homozygote chez l'enfant.

Coronary lesions with atheromatous deposits occurring in later childhood characterize homozygous type IIa hypercholesterolaemia and condition the somber prognosis of a disease which affects one subject in a million. However, aortic lesions are constantly found, as shown by routine ultrasonographic and angiographic studies in these children. The walls of the proximal aorta are cardboard-like and thick, the origin of the aorta is narrow and the semilunar aortic valves are thickened. The valvular or supravalvular aortic gradient may be considerable; it is often progressive, but is sometimes stabilized or made regressive by medical treatments combined with plasmapheresis or porto-caval shunt. Aortoplasty or aortic valve replacement being difficult to perform in these patients, more aggressive therapeutic procedures, such as liver or heart transplantation, have been suggested. The last generation cholesterol-lowering drugs seem to offer some hope of success.

[Use of valved tubes in the treatment of ventriculo-pulmonary discontinuity. Long-term results]. / Utilisation des tubes valvulés pour le traitement des discontinuités ventriculo-pulmonaires. Résultats à distance.

Forty five patients, 2 months to 42 years of age, had valved tubes implanted for ventriculo-pulmonary discontinuity. The underlying malformations were: certain forms of Fallot's tetralogy (16 cases), transposition of the great arteries with ventricular septal defect and pulmonary stenosis (8 cases), truncus arteriosus (9 cases), double outlet right ventricle (6 cases), other malformations (4 cases). Hospital mortality (at 30 days) was 31% (14 cases). This was mainly due to technical difficulties related to the large number of palliative procedures and to irreversible pulmonary hypertension, present in 43% of cases with fatal outcomes. Thirty one patients were followed up for 6 months to 7 years (mean 3 +/- 1,6 years). There were 3 late deaths, one during reoperation for a residual shunt. All survivors underwent clinical assessment with hemodynamic control in 17 cases (15 routine control catheterisations). To date it has only been necessary to change one valved tube: a good result was obtained.