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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

AVMs are congenital malformations of central nervous system blood vessels. The majority of lesions become symptomatic prior to age 40. Hemorrhage and epilepsy are the most frequent presenting symptoms. Disabling headache, transient, progressive, and permanent neurological deficit, heart failure, hydrocephalus, macrocephaly, and intellectual impairment may also occur. Long-term mortality is approximately 18%, and morbidity leading to disability occurs in 30% of patients. Aggressive management when possible is indicated.
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PMID:Natural history and pathophysiology of arteriovenous malformations. 54 27

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)
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PMID:Vein of Galen malformation. Endovascular management of 43 cases. 179 15

High-flow cerebral arteriovenous fistulae are well known to present in the neonatal period with macrocephaly, cranial bruits and high-output cardiac failure. This report describes a newborn infant with such a clinical presentation, who had unilateral megalencephaly without macroscopic arteriovenous shunts. Ultrasound Doppler examination of the carotid and cerebral vessels showed diastolic flow, or a decreased pulsatility index, consistent with decreased intracranial vascular resistance. The ipsilateral cerebral arteries and veins were markedly enlarged at angiography and at post-mortem examination, but there was no arteriovenous malformation. The infant expired from high-output cardiac failure and hypoglycemia. It is postulated that the high-output cardiac failure was due to increased blood flow through the enlarged, dysplastic cerebral hemisphere.
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PMID:Unilateral megalencephaly associated with neonatal high output cardiac failure. 214 45

We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, these lesions are the most aggressive ones for the maturing brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology.
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PMID:Nongalenic cerebral arteriovenous malformations in neonates and infants. Review of 26 consecutive cases (1982-1992). 762 85

Central nervous system vascular disorders in the neonate comprise structural anomalies or malformations of arteries and veins and physiologic alterations of cerebral blood flow, which can mimic structural vascular disease. Clinical , imaging, and transcatheter therapeutic aspects of neonatal cerebral vascular malformations are described. Symptomatic high-flow vascular malformations characteristically present with cardiac failure and associated systemic problems in the neonate, whereas infants typically present with macrocephaly and hydrocephalus and older children with hemorrhage, developmental delay, or focal deficits. Neonatal cerebral hemorrhage is typically primary or associated with immaturity, parturitional trauma, or coagulopathy. Likewise, cerebral ischemic lesions are more likely secondary to hypoxic, ischemic events than to thromboembolic or structural cerebrovascular occlusive disease. The role of the current noninvasive imaging modalities in each of these clinical problems is reviewed and illustrated.
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PMID:Neonatal central nervous system vascular disorders. 940 72

Large dural arteriovenous malformations (DAVMs) accompanied by cardiac failure usually carry a poor prognosis with a high risk of morbidity and death. The authors report on the case of a male neonate with a massive DAVM who presented at birth with macrocephaly and high-output cardiac failure. The child initially underwent treatment with surgical clipping of the large main feeding artery. Hydrocephalus, thought to be due to venous hypertension, developed when the boy was 8 months old. The condition resolved after interventional embolization treatment. The patient did not require placement of a ventriculoperitoneal shunt. At 21 months of age, the child had near normal development without any focal neurological deficits.
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PMID:Developmental outcomes for neonatal dural arteriovenous fistulas. 1927 8

Sotos syndrome is an autosomal dominant condition characterized by pre- and postnatal overgrowth (tall stature and macrocephaly), a typical facial appearance, advanced bone age, and developmental delay. The syndrome is caused by mutations or deletions of the nuclear receptor binding SET domain protein 1 (NSD1) gene, which encodes a histone methyltransferase implicated in the regulation of chromatin. Left ventricular noncompaction (LVNC), also called left ventricular (LV) hypertrabeculation, is a rare disorder classified as a primary genetic cardiomyopathy by the American Heart Association. This condition is characterized by an altered myocardial wall due to arrest of embryonic compaction of the loose interwoven meshwork that makes up the fetal myocardial primordium. The cardiac manifestations of this cardiomyopathy are variable, ranging from an absence of symptoms to a progressive deterioration in cardiac function, with heart failure, arrhythmias, and systemic thromboemboli. We describe two unrelated patients who had LVNC, as based on echocardiographic findings, and Sotos syndrome, as based on physical features and molecular analysis. To our knowledge, the literature contains no previous reports of concomitant LVNC and Sotos syndrome. In the light of these two cases, we suggest that patients with Sotos syndrome be evaluated for LVNC cardiomyopathy when being screened for heart defects.
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PMID:Left ventricular noncompaction in Sotos syndrome. 2148 93

Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high-flow fistulas. In association with high-flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient-by-patient basis, according to the unique physiological condition of the child.
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PMID:Endovascular treatment of pediatric intracranial arteriovenous shunt. 2760 22