Medical treatment of congestive cardiac failure from cerebral arteriovenous fistulas at bicêtre hospital.

SUMMARY: Arteriovenous fistulas create an increase in venous return and subsequent right heart overload. The result is right heart dilatation, pulmonary arterial hypertension, and increased pulmonary blood flow. Reduction in arterial diastolic pressure (as observed in arteriovenous shunt), an increase in end diastolic pressure (due to increased preload), a reduction in diastolic period (due to tachycardia) are all detrimental to myocardial perfusion and hence oxygen delivery and may precipitate left ventricular failure. Furthermore, the transition from a foetal circulatory pattern to an adult circulatory pattern is complex and both pulmonary and systemic circulations remain highly unstable during the first week after birth. This can explain a persistent transitional circulation with shunts through the ductus arteriosus, the foramen ovale and a pulmonary hypertension which worsen the systolic and diastolic wall stress. The clinical presentation depends on the size of the left-to-right shunt and its tolerance. The aims of symptomatic therapy are to improve oxygen delivery to the tissues and decrease tissue oxygen consumption. Measures that improve the patient's effective ventilation, arterial oxygen saturation, and hematocrit need to be the first steps. Diuretics are the first step for reducing preload. Cardiac output can be improved by increasing cardiac contractility with inotropic agents. Digoxin is the main agent used to increase myocardial contractility. However, its use in a hyperkinetic state due to arteriovenous fistulas remains controversial: pretreatment myocardial function indices may already be above normal, and there is no clear evidence that their further increase produces clinical benefit. Vasodilators are obviously contraindicated if systemic arterial pressure is low. If the failure cannot be controlled, embolisation of the lesion should be considered. The goal of this paper is to provide a rational approach to the treatment of circulatory failure due to cerebral arteriovenous shunts.

Liver T cell subsets and adhesion molecules in murine graft-versus-host disease.

Murine GVHD across multiple minor histocompatibility barriers (B10.D2 into irradiated BALB/c) results in cell-mediated destruction of bile ducts inside the liver. Similar changes are characteristic of hepatic GVHD in humans following BMT. We have defined the phenotypes of inflammatory cells and the accessory/adhesion molecules expressed in the liver between day 7-14 of murine GVHD. T cells (CD3+) comprised 65% of hepatic inflammatory cells. alpha-beta and gamma-delta cells accounted for 92 and 8%, respectively of hepatic T cells. The percentage of CD4+ cells (29%) was 3 times that of CD8+ cells (11%). Lymphocyte function-associated antigen-1 (LFA-1) was expressed by the majority of inflammatory cells. Thirty per cent of the cells were positive for Mac-1, a differentiation marker of macrophages, large granular lymphocytes, and natural killer cells. Expression of intercellular adhesion molecule-1 and major histocompatibility complex class II (IAd) molecules on bile duct epithelial and portal vein endothelial cells was induced during GVHD. These results suggest that hepatic GVHD is induced by donor alpha-beta T cells through mechanisms that may involve CD4:1Ad and LFA-1:ICAM-1 interactions.

Ultrasound diagnosis of thymic hemorrhage in an infant with late-onset hemorrhagic disease.

Thymic hemorrhage related to coagulopathy by vitamin K deficiency is a rare condition. We report a case in a 4-week-old boy presenting with acute onset of respiratory distress and anemia. Ultrasound examination of a compressive anterior mediastinal mass established the diagnosis of thymic hemorrhage and allowed us to follow resolution under medical treatment.

Hydrodynamics in vein of Galen malformations.

Forty-three patients with vein of Galen aneurysmal malformations (VGAM) referred to us for endovascular treatment between 1985 and 1990 and 335 additional cases published in the literature were reviewed with particular attention to the presence of ventricular enlargement and outcome after shunting. Hydrocephalus was the second most frequent symptom (46.8%); it is more frequent in infants (73%) than in children, adults (30%) or neonates (15%). Of the patients reported in the literature, 17.9% had undergone shunting. Within the shunted population there was an overall morbidity of 41% and a mortality of 10% (especially in the infant group). In our series 17 patients (39.5%) were shunted and a significant difference in the clinical outcome was noted between the shunted and the nonshunted group. Of the nonshunted patients, 66.6% were free of any neurological deficit or mental retardation and fewer than 5% presented with significant mental retardation. On the other hand, only 33.3% of the shunted patients had a favorable outcome and more than 15% developed significant mental retardation. Among the various causes of hydrocephalus in patients with VGAMs, such as obstruction of the aqueduct, subarachnoid hemorrhage, or ex vacuo hydrocephalus, high venous pressure may be of particular importance. In this article a physiopathological interpretation of the hydrodynamics in VGAMs is developed and a speculative explanation for CSF disorders related to ventricular shunting proposed. Treatment of hydrocephalus in VGAMs can be achieved through obliteration of the malformation or at least diminishing the venous pressure; surgical ventricular shunting does not have to be the first treatment of hydrodynamic disorders associated with VGAMs, especially in infants.

Vein of Galen malformation. Endovascular management of 43 cases.

Since 1984, 43 patients with true vein of Galen aneurysmal malformations have been referred to us and managed according to our patient selection, technique, and follow-up guidelines. Thirty-four were embolized transarterially with bucrilate (isobutyl cyanoacrylate) or enbucrilate (N-butyl cyanoacrylate) embolization. No cutdown or hypotension during or after the embolization was used and no balloon catheter was employed. Forty-seven percent of the children had a completely occluded lesion which was confirmed when the child was at least 6 months of age at the follow-up angiographic examination; 52.9% were found to be completely normal or only to have mild cardiac failure that could be treated medically or moderate macrocephaly without neurological symptoms or mental retardation. In the embolized group 5.8% died as a result of the wrong treatment (1 case) or poor timing of embolization 3 days after ventricular shunting (1 case). The overall mortality (embolized and non-embolized groups) in the neonatal children was 27.7% with a total of 18.6% for all ages. Complete morphological exclusion of the arteriovenous malformation was accomplished in 41.9%; 74.4% of all children referred are now clinically normal or present moderate mental retardation which is diminishing. There was 3% neurological morbidity in the embolized group (only following the venous approach) in 78 sessions and more than 100 arteries embolized. These results compare favorably with surgical or other techniques of arterial embolization (balloon or particles), as well as transvenous (transtorcular or transfemoral) embolization, where the morbidity and mortality are significantly higher and the late clinical evaluation is seldom satisfactory.(ABSTRACT TRUNCATED AT 250 WORDS)

Congestive cardiac manifestations from cerebrocranial arteriovenous shunts. Endovascular management in 30 children.

Since 1984 we have been involved in the management of 30 children who had cardiac manifestations secondary to cerebrocranial arteriovenous shunts. Aneurysm malformation of the vein of Galen was the most common vascular lesion observed (73% of cases). In 77% of the patients the cardiac symptoms were the main presenting complaint. Medical treatment and/or endovascular therapy were indicated, depending on the age of the patients and the severity of the cardiac manifestations. Following embolization, the cardiac symptoms resolved (73%) or improved (18%) in 1 or 2 sessions. Mortality in the embolized group was 9%, and transient nonneurologic morbidity occurred in one case. Overall mortality, including four patients rejected for embolization, was 20%. These results compare favorably with medical and/or surgical management, alone or combined. The technique, challenges, indications and contraindications of endovascular therapy are discussed. Embolization represents an effective adjunct treatment to control, improve or cure the congestive cardiac manifestations caused by cerebrocranial arteriovenous shunts.