[Isolated intimal stenosis of the renal artery and curable arterial hypertension in Recklinghausen's disease. Association of hypoplastic stenosis of the aortic and pulmonary trunks and sigmoid aortic regurgitation]. / Sténose intimale isolée d'une artère rénale et hypertension artérielle curable dans la maladie de Recklinghausen.

The case of a patient with Von Recklinghausen's disease with multiple localisations, cardiac, aortic, pulmonary and renal artery is reported. Neurofibromatosis was confirmed by the presence of a "royal" tumour on the left elbow and of many "café au lait" spots of over 15 mm diameter. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta. There was no craniofacial dysmorphism or intellectual impairment. The karyotype was normal. The cardiovascular lesions comprised: - stenosis of the left renal artery resulting in renovascular hypertension. This was due to pure intimal hyperplasia; - supravalvular aortic stenosis with hypoplasia of the ascending aorta, severe aortic regurgitation with dilatation of the aortic ring without rheumatic valvular thickening; - supra valvular pulmonary stenosis which was atypical in site, extension and in the absence of post stenotic dilatation. The pressure gradient was mild (22 mm Hg). There was a striking similarity between the pulmonary and aortic lesions. This new syndrome may be classified amongst the genetic cardiocutaneous syndromes such as the Gorlin or "leopard" syndrome, Watson's syndrome and Noonan's syndrome. Unusual features are the absence of craniofacial abnormalities, normal intelligence, and the left-sided dominance aggravated by hypertension due to curable intimal stenosis of the renal artery.

[Spontaneous blocks by intra-atrial conduction disorders of reciprocating atrioventricular rates using a Kent's bundle]. / Arrêts spontanés par trouble de conduction intra-auriculaire de rythmes réciproques auriculo-ventriculaires utilisant un faisceau de Kent.

Paroxysmal tachycardia in Type A Wolff-Parkinson-White syndrome was due to a reciprocating rhythm involving a left lateral Kent Bundle in the retrograde direction. Spontaneous interruption of the reentry resulted not from block in the normal or accessory atrioventricular pathways but from an intraatrial conduction defect: delay in conduction between the left and right atria on intraatrial reentry terminated the tachycardia.

[Congenital arteriopathies and Leo Buerger's disease]. / Artériopathies congénitales et maladie de Leo Buerger.

A homogenous series of 7 consecutive cases of Buerger's disease were investigated and the congenital malformations of the upper and lower limb arterial trunks were classified. These malformations constitute the anatomical basis of Buerger's disease and define an autonomous disease entity. Total supra-malleolar interruption of the main lower limb arteries either by triple vessel agenesis or, more rarely, by femoro-popliteal or popliteal and tibio-peronal agenesis was observed. The appearances of the collateral circulation, already established in utero, are characteristic with long axial spiral-shaped arteries and dependant arterioles maintaining a precarious supply to the distal tissues which may be adequate for many years. The nautral history of the disease is directly related to the malformation of the congenital arterial system of the limbs. Proximal arterial malformative occlusion is the cause of early, inevitable trophic lesions; the spiral-shaped arteries and distal arterioles, which represent the maximal embryonic collateral circulation, limit the area of necrosis and favour healing.

[Arterial malformations and Takayashu disease]. / Malformations artérielles et maladie de Takayashu.

The authors report a recent series of 5 cases with Takayashu's disease and discuss the common pathological presentation of malformations of the arterial trunk, caused by disorderd development of the arterial system or by later segmental arrested development of the large trunks. The disease, which presents in the young adult as a diffuse inflammatory process, is probably auto-immune, but has its foundations in embryonic and foetal development.