Congestive heart failure secondary to cerebral arterio-venous fistula

Abstract

Arteriovenous fistula is a relatively rare cause of severe congestive heart failure in the newborn. An intracranial arteriovenous malformation associated with an aneurysm of the great vein of Galen is the most frequent of such malformations and, although well known, it remains a difficult diagnosis in the neonate because the clinical picture first presents with findings suggesting cardiac disease. The newborn with a vein of Galen aneurysm has volume and pressure overload on his myocardium; cyanosis is often present due to persistent fetal circulation; peripheral pulses are generally decreased in amplitude except in those arteries near the fistula where they are bounding, as an expression of a hyperdynamic status. A continuous murmur may be heard over the scalp, but this highly suggestive sign is often not present. The majority of newborns with intracranial arteriovenous fistula die very soon. Unfortunately, most of these infants are at first considered to have congenital heart disease and are, therefore, subjected to cardiac catheterization and angiography, with their well-known risks, before a correct diagnosis is made. Cross-sectional echocardiography, by demonstrating normal intracardiac anatomy, eliminates the need for an invasive investigation in a very sick neonate. Ultrasonography of the head will then provide a rapid and accurate demonstration of the aneurysm of vein of Galen. Cerebral angiography will complete the diagnosis in those cases in which a neurosurgical intervention is contemplated.

Vein of Galen malformation

“…Large arteriovenous shunts reduce the diastolic pressure within the aorta, leading to reduced coronary blood flow, which, coupled with the high ventricular pressure, can promote myocardial ischemia. 20,41 Flow reversal in the aorta can lead to renal hypoperfusion and subsequently to renal failure. Congestive heart failure developed prenatally can be detected on ultrasound and in severe cases can lead to multiorgan failure and irreversible brain damage.…”

Systemic arteriovenous fistula leads to pulmonary artery remodeling and abnormal vasoreactivity in the fetal lamb

“…We and other study groups have reported cases of systemic arteriovenous fistula diagnosed in the prenatal period that were associated with the development of PPHN in the postnatal period (9,13,15,26). These observations have led us to hypothesize that a systemic arteriovenous fistula by perturbating the fetal hemodynamics may affect the pulmonary vascular circulation leading to PPHN at birth.…”

Disseminated Coagulopathy Associated with Transtorcular Embolization of Vein of Galen Aneurysm in a Neonate

“…1,9 Recently, neuroradiologic techniques have improved the outcome for vein of Galen aneurysms. 4 Endovascular treatments include transarterial or transvenous embolization.…”

“…Finally, decreased afterload induced by the AVF increases the risk of myocardial ischemia. 1 Surgical management is often very difficult owing to the deep location of the malformation and the size of the patient. 2 Recently, endovascular embolization has emerged as the treatment of choice in neonates.…”

“…Neonatal presentation usually includes high-output congestive heart failure secondary to increased volume and pressure overload on the myocardium. 1 This condition is often refractory to medical management, and a more invasive therapeutic approach is required to reduce the vascular "steal" phenomenon. Because of the low vascular resistance within the arteriovenous fistula (AVF), the majority of left ventricular output might be delivered to the head instead of the descending aorta (hence the term "steal").…”