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American Heart Journal

ventricular septum is uncertain. Balloon atria1 septostomy has been performed without complications in newborn infants with pulmonary or tricuspid atresia associated with ASA.i,2,4 Potential damage to the mitral valve appa- ratus was avoided by performing this procedure under the guidance of 2D echocardiography.

REFERENCES

1.

2.

3.

4.

5.

Freedom R, Rowe R. Aneurysm of the atria1 septum in tricuspid atresia: diagnosis during life and therapy. Am J Cardiol 1976;32:265. Sabn DJ, Allen HD, Anderson R, Goldberg SJ. Echocardio- graphic diagnosis of atria1 septal aneurysm in an infant with hypoplastic right heart syndrome. Chest 1978;73:227. Topaz 0, Feigl A, Edwards JE. Aneurysm of the fossa ovalis in infants: a pathologic study. Pediatr Cardiol 1985;6:65. Wolf WJ, Casta A, Sapire DW. Atria1 septal aneurysms in infants and children. AM HEART J 1987;113:1149. Reder RF, Yeh HC, Steinfeld L. Aneurysm of the interatrial septum causing pulmonary venous obstruction in an infant with tricuspid atresia. AM HEART J 1981;102:786.

Doppler findings in nonimmune hydrops fetalis and cerebral arteriovenous malformation

Walter Johnson, M.D., James M. Berry, Jr., RDMS, Stanley Einzig, M.D., Ph.D., and John L. Bass, M.D. Minneapolis, Minn.

Nonimmune hydrops fetalis has been reported in associa- tion with many conditions, including fetal congestive heart failure resulting from structural heart disease or dysrhythmia’ and systemic arteriovenous fistulas.2 The association of large cerebral arteriovenous malformation with severe congestive heart failure in infants has been well documented.3 To our knowledge, however, there are no reports of cerebral arteriovenous malformation associ- ated with hydrops fetalis. We report two infants with cerebral arteriovenous malformation who presented with fetal hydrops diagnosed in utero with two-dimensional ultrasound imaging. In addition, Doppler ultrasonography and contrast echocardiography performed after birth revealed important information about the circulation in such patients when patent ductus arteriosis (PDA) is present.

Fetal ultrasound examination was performed in both fetuses at 31 and 39 weeks’ gestation, respectively. In each fetus, there was marked polyhydramnios with increased placental thickness, fetal hydrocephalus, ascites with

From the Department of Pediatrics, University of Minnesota.

Supported in part by the Dwan Family Funds, Minneapolis, Minn.

Reprint requests: John L. Bass, M.D., Box 94, UMHC, Harvard Street at East River Road, University of Minnesota Hospitals, Minneapolis, MN 55455.

Fig. 1. Doppler recording from within PDA in infant with large cerebral arteriovenous malformation. A, Sam- ple volume (bracket) is positioned in PDA between pul- monary artery and descending aorta. B, Right-to-left shunt (negative flow) toward systemic circulation throughout cardiac cycle. DAo, Descending aorta; MPA, main pulmonary artery.

pleural and pericardial effusions, scalp edema, and cardio- megaly. In the older fetus, the right atrium and right ventricle were enlarged, and tricuspid regurgitation was detected with a maximal velocity of 3.8 m/set. The infants were delivered by cesarean section at 32 and 39 weeks, respectively. Clinical findings after birth were similar in both infants. Both had anasarca. Cranial bruits, a systolic precordial murmur, and hepatomegaly were present in each. Carotid pulses were increased. Chest roentgeno- grams showed cardiomegaly with increased pulmonary vascular markings. Cranial ultrasonography and com- puted tomography showed massive hydrocephalus with vascular structures suggestive of large cerebral arteriove- nous malformation. Results of maternal and infant anti- body screening were negative.

Results of cardiac ultrasound examinations showed normal anatomic relationships with enlargement of the right atrium and right ventricle, as well as the superior vena cava (as compared to the inferior vena cava) and the

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Fig. 2. Doppler recordings from two infants with large cerebral arteriovenous malformation and PDA. A, Sample volume (bracket) is located in transverse aortic arch. B, There is a transient negative deflection, but retrograde (positive) flow away from PDA is recorded throughout cardiac cycle. AAo, Ascending aorta; DAo, descending aorta; RP.4. right pulmonary artery.

left innominate vein. The PDA was large in both. The atria1 septum deviated toward the left atrium. Left atria1 and left ventricular dimensions were normal. In one infant, the umbilical venous catheter had inadvertently been positioned in the left ventricle. Injection of saline solution produced an echo contrast effect that filled the ascending aorta to the right innominate artery and the left carotid artery. No contrast effect passed beyond the origin of the left carotid artery. Within 2 seconds, the contrast effect had returned to the superior vena cava. Doppler ultrasound recording from within the PDA showed right- to-left shunting throughout systole and diasole in both patients (Fig. 1). Flow through the transverse aortic arch was retrograde toward the carotid arteries during both systole and diastole (Fig. 2). The pattern of flow suggested by the Doppler and contrast ultrasound studies is dia- grammed in Fig. 3. Flow in the superior vena cava, recorded in one patient, had a normal velocity of 60

Systole Diastole

Fig. 3. Diagrammatic representation of patterns of flow found in two infants with large cerebral arteriovenous malformation and PDA. Right-to-left PDA shunt and retrograde flow in transverse aortic arch toward carotid arteries is present throughout systole and diastole. (Mod- ified from Spach MS, Serwer GA, Anderson PAW, Canent RV, Levin AR. Circulation 1980;61:11%)

cm/set. The infants were severely ill. Surgery was not attempted, and both died within 24 hours of birth. An autopsy was performed in each. An aneurysm of the great vein of Galen was found in one infant and an arteriove- nous malformation communicating with the right basal vein of Rosenthal in the other. There was massive cardio- megaly with right atria1 and right ventricular enlargement and a PDA in each. The only structural heart disease present was mild dysplasia of the tricuspid valve of the infant in whom fetal tricuspid insufficiency was de- tected.

Nonimmune hydrops fetalis has been reported with a wide variety of conditions that may result in fetal cardiac fai1ure.l However, we are aware of no reports of in utero demonstration of fetal hydrops resulting from a large cerebral arteriovenous malformation. A pattern of systolic and diastolic right-to-left shunting through the PDA associated with continuous retrograde flow through the transverse aortic arch was observed in both infants. This is similar to flow patterns described in patients with left ventricular outflow obstruction and rest.rictive interatrial communications, who presumably have markedly elevated pulmonary vascular resistance.4 Although elevated pulmo- nary vascular resistance cannot be exf.luded in these two infants, the flow patterns are best explained by markedly lowered systemic vascular resistance from the cerebral arteriovenous malformation.

The transverse arch and PDA flow patterns from contrast echocardiography and Doppler ultrasonography imply that the head, arms, and descending aorta are perfused by right ventricular outpul, whereas the left ventricle supplies only the head and arms. Combined with increased systemic venous return, this explains the right ventricular and right atria1 enlargement, reported in new- born infants with a large cerebral arteriovenous malforma- tion and a PDA. After the PDA closes. t,he left ventricle

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must supply the entire systemic circulation including the systemic arterial to venous shunt. Elevated systemic venous return persists, and biventricular enlargement results. The Doppler flow patterns in these two neonates support the angiographic and contrast echocardiographic findings in cases reported by Cummings and Snider et aL6

REFERENCES

1. Kleinman CS, Donnerstein RL, DeVore GR, Jaffe CC, Lynch DC, Berkowitz RL, Tamer NS, Hobbins JC. Fetal echocar- diography for evaluation of in utero congestive heart fail- ure-a technique for study of nonimmune fetal hydrops. N Engl J Med 1982;306:568.

2. Daniel SJ, Cassady G. Non-immunologic hydrops fetalis assocciated with a large hemangioendothelioma. Pediatrics 1968;42:828.

3. Gomez MR. Whitten CF. Nolke A. Bernstein J. Mever JS. Aneurysm2 malformation of the great vein of Galen causing heart failure in early infancy. Pediatrics 1963;31:400.

4. Bass JL, Berry JM, Einzig S. Flow in the aorta and patent ductus arteriosus in infants with ao.rtic atresia or aortic stenosis: a pulsed Doppler ultrasound study. Circulation 1986;74:315.

5. Cumming GR. Circulation in neonates with intracranial arteriovenous fistula and cardiac failure. Am J Cardiol 1980; 45:1019.

6. Snider AR, Soifer SJ, Silverman NH: Detection of intracrani- al arteriovenous fistula by two dimensional ultrasonography. Circulation 1981;63:1179.