Arteriovenous Malformation of the Vein of Galen in a Newborn Diagnosed with Color Doppler Examination— A Case Report
Eltohami A. Eltohami, M.D., M.P.C.H. and
Andrej Robida, M.D.,
D.Sc.
DOHA, QATAR
Abstract A fourteen-day-old newborn infant presented with severe heart failure due to arteriovenous malformation of the vein of Galen. It was diagnosed by twodimensional, pulsed-wave and color Doppler echocardiographic imaging. The latter method showed the afferent vessel to be the anterior cerebral artery entering an aneurysm of the vein of Galen at its posterior aspect. Information for surgical anatomic definition appears to be adequately provided by color Doppler examination, which permits avoidance of preoperative angiography. Introduction Most infants with a large arteriovenous malformation (AVM) of the vein of Galen present with high-output heart failure early in the neonatal period. The heart failure is due to massive left-to-right shunting simulating structural heart disease. Clinical findings can be misleading, especially if a cranial bruit is not heard or not searched for. Various ultrasound modalities have proved useful in evaluation of AVM of the vein of Galen. 1-3 However, before surgical therapy, arteriography has been considered necessary to delineate location of the entry of feeder arteries into the aneurysm,4 although recent reports have indicated that color Doppler imaging could also precisely show the anatomy of these malformations.5-’ This report describes the color Doppler examination of a newborn infant with AVM of the vein of Galen accurately showing the location of the connection between branches of the feeder artery and the aneurysm.
From the
Department
of
Cardiology
and Cardiovascular
Surgery, Hamad General Hospital, Doha, Qatar
443
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
444
FIG. 1.A. Coronal plane of the head with ultrasound. Note dilated ventricles and cystic dilatation of the vein of Galen displayed in blue and mosaic (black arrow). The red and yellow colors denote the dilated feeder artery (white arrow). B. Saggital scan of the head. Dilated anterior cerebral artery is seen as it courses to the posterior aspect of the aneurysm (open arrows) of the vein of Galen (white arrow); 3 =dilated third ventricle.
Case
Report
This male infant of forty weeks of gestation weighing 3900 grams appeared normal at birth. At the age of fourteen days the parents noticed rapid and difficult breathing. On admission, his respiratory rate was 90 per minute and heart rate was 190 per minute. Peripheral arterial pulses were bounding in the upper limbs and neck while femoral artery pulsation was weak. The precordium was hyperactive. There was a gallop rhythm and grade 2/6 regurgitant systolic murmur was heard at the lower left sternal border. Blood pressure was 20 mmHg lower in the lower limbs than in the upper limbs. An intracranial bruit, not heard on admission, appeared after anticongestive therapy. The ECG revealed right atrial and biventricular hypertrophy. ST and T wave changes in the left precordial leads were noticed. Chest radiography showed a grossly enlarged heart with increased pulmonary vascularity. Echocardiography revealed the heart to be anatomically normal with mild tricuspid regurgitation, dilatation of all four heart chambers, and evidence of pulmonary arterial hypertension. There was right-to-left shunting across the foramen ovale. The superior vena cava, ascending aorta, and the innominate artery were all dilated, but the aortic isthmus was narrow. The pulsed-wave Doppler sample volume positioned in the transverse aortic arch showed systolic antegrade flow and diastolic retrograde flow. This flow pattern was noticed in the innominate artery as well. The transducer was then positioned over the arterior fontanelle, and moderate dilatation of the lateral and third ventricles was recorded. An echo-free area was noticed posterior to the third ventricle in the sagittal view, which was considered to represent an arteriovenous (AV) fistulous communication or aneurysm. Color Doppler imaging showed turbulence at the region of a feeder artery entering this aneurysm. In the saggital plane the anterior cerebral artery was dilated, and the flow in this artery was seen as red or as mosaic in the ascending limb, blue at the horizontal course of the vessel, and again mosaic in the descending limb of the artery due to turbulence (Fig. 1 A,B). Pulsed-wave Doppler interrogation revealed continuous flow along the course of the anterior cerebral artery, changing direction from flow toward the transducer in the ascending portion of
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
445
FIG. 2. Pulsed-wave interrogation with the same volin the aneurysm. Increased velocity of the continuous systolic-diastolic flow with decreased pulsatility index is shown.
ume
the artery to flow away from the transducer in its descending part. The pulsatility index was lower than normal (Fig. 2). Computerized tomography (CT) of the head showed an aneurysm of the vein of Galen and confirmed the echocardiographic findings. Arteriography was planned prior to embolization or surgical treatment, but the infant died in intractable heart failure fifteen days after admission to the hospital. Discussion The clinical diagnosis of a cerebral AVM is difficult. A cranial bruit is not always present, and when present, it may not be of clinical significance, for it may be found in normal newborns or may occur as transmission of a loud heart murmur.’ It can mimic congenital heart disease, mainly those with large left-to-right shunt. A coarctation of the aorta can also be suspected because of weak femoral pulses and difference in systolic blood pressure between upper and lower limbs. Coarctation-like physiology could be explained by the runoff of the blood into the fistulous communication. 9&dquo;0 In our infant femoral pulses were faint and no cranial bruit was heard at initial presentation. AVM of the vein of Galen is an example of a truncal AV fistula where communication of a mature artery or arteries and vein without an intervening anastomatic channel occurs. Therefore, no resistance vessels are interposed between the arterial and venous side of the fistula, and this creates a low-resistance circuit. This is reflected as high-velocity turbulence and lower than normal pulsatility index when pulsed-wave and color Doppler are applied to investigate such
a
lesion. 5-~.&dquo;
Echocardiography in an infant with AVM of the vein of Galen reveals dilatation of the heart
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
446 chambers and large afferent and efferent vessels without structural heart disease. The ascending aorta and proximal feeder arteries are dilated. There is narrowing of the aortic isthmus as a result of diminished blood flow through it caused by runoff into the AV fistulas.’2’’3 Pulsedwave Doppler interrogation of the aortic arch proximal to the origin of the ductus arteriosus shows retrograde diastolic flow. Such a flow can also be found in feeder arteries and may be present in a small number of normal infants as well. However, in these normal infants, other echocardiographic and clinical signs are absent.’z Starc et al. 13 emphasized pulsed-wave Doppler mapping of the aorta beginning with the interrogation of the subaortic flow and continuing with the ascending aorta proximal to the innominate artery, transverse aortic arch proximal to the site of ductus arteriosus, postductal descending aorta, and finally, the thoracoabdominal aorta. Thus, aortic valve insufficiency, aorta-pulmonary window, coronary artery fistula, patent ductus arteriosus, or other systemic to pulmonary artery shunt can be differentiated. In our patient we found, in addition to classical two-dimensional and pulsed-wave Doppler signs suggesting extracardiac AV fistula, abdominal flow in the innominate artery, which together with its dilatation, identified it as the origin of the fistulous communication. Cerebral ultrasound examination was confirmatory. It showed a cystic lesion behind the third ventricle, and with color Doppler imaging we could follow the abnormal flow from the innominate artery into the right common carotid artery and into the aneurysm of the vein of Galen. The anterior cerebral artery entered the aneurysm at its posteroinferior aspects. Conclusion We share the opinion of others 1-7 that, in identifying the aneurysm and feeder vessel or vessels, color flow imaging is superior to two-dimensional, pulsed-wave Doppler and contrast echocardiography. It is also easier to apply and probably more accurate. It can replace CT scanning and will likely replace arteriography and digital subtraction angiography before an attempted surgical treatment because the definition of the location of the entry of tributary vessels into the aneurysm with color Doppler imaging is also possible.
Andrej Robida, M.D., D. Sc. Department of Cardiology and Hamad General Hospital P.O. Box 3050
Doha, Qatar
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
Cardiovascular
Surgery
447
References 1. Sivakoff M, Nouri S: Diagnosis of vein of Galen arteriovenous malformation by two-dimensional ultrasound and pulsed Doppler method. Pediatrics 69:84-86, 1982. 2. Stanbridge R, DeL Westaby S, Smallhorn J, et al: In-
3.
4.
5.
6.
tracranial arteriovenous malformation with aneurysm of the vein of Galen as a cause of heart failure in infancy : Echocardiographic diagnosis and results of treatment. Br Heart J 49:157-162, 1983. Mardini MK, Lazarte R, Huntington D, et al: Diagnosis of cerebral arteriovenous malformation by contrast two-dimensional and Doppler ultrasonography of the head after saline injection in the umbilical arterial line. Am Heart J 114:450-454, 1987. Raimondi AJ: Pediatric Neurosurgery. New York: Springer Verlag, 1987, p 321. Schranz D, Jungst BK, Golla G, et al: Herzinsuffizienz bei einem Neugebornen mit arteriovenous Malformation der Vena Galeni: Diagnose mittels farbcodierter Doppler-Bild-Echographie. Monatsschr Kinderheilkd 136:47-49, 1988. Vaksmenn G, Decoulx E, Mauran P, et al: Evaluation of vein of Galen arteriovenous malformation in newborns by two dimensional ultrasound, pulsed and colour Doppler method. Eur J Pediatr 148:510-512,
1989.
7.
Schorf J: Colour Doppler imaging of arterimalformation of the vein of Galen in a newborn. Neuroradiol 32:60-63, 1990. Knudson RF, Alden ER: Symptomatic arteriovenous malformation in infants less than 6 months of age. Pediatrics 64:238-241, 1979. Walker WY, Mullins CE, Knovick GC: Cyanosis, cardiomegaly and weak pulses. A manifestation of massive congenital systemic arteriovenous fistula. Circulation 29:777-781, 1964. Deverall PB, Taylor JFN, Sturrock GS, et al: Coarctation-like physiology with cerebral arteriovenous fistula. Pediatrics 44:1024-1028, 1969. Lindegaard KF, Grolimund P, Aaslid R, et al: Evaluation of cerebral AVMs using transcranial Doppler ultrasound. J Neurosurg 65:335-344, 1986. Musewe NN, Smallhorn JF, Burrows PE, et al: Echocardiographic and Doppler evaluation of the aortic arch and brachiocephalic vessels in cerebral and systemic arteriovenous fistulas. J Am Coll Cardiol 12:1524-1535, 1988. Starc TJ, Kongrad E, Bierman FZ: Two-dimensional and Doppler findings in cerebral arteriovenous malformation. Am J Cardiol 64:252-254, 1989.
Deeg KN,
ovenous
8.
9.
10.
11.
12.
13.
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
Eltohami A. Eltohami, M.D., M.P.C.H. and
Andrej Robida, M.D.,
D.Sc.
DOHA, QATAR
Abstract A fourteen-day-old newborn infant presented with severe heart failure due to arteriovenous malformation of the vein of Galen. It was diagnosed by twodimensional, pulsed-wave and color Doppler echocardiographic imaging. The latter method showed the afferent vessel to be the anterior cerebral artery entering an aneurysm of the vein of Galen at its posterior aspect. Information for surgical anatomic definition appears to be adequately provided by color Doppler examination, which permits avoidance of preoperative angiography. Introduction Most infants with a large arteriovenous malformation (AVM) of the vein of Galen present with high-output heart failure early in the neonatal period. The heart failure is due to massive left-to-right shunting simulating structural heart disease. Clinical findings can be misleading, especially if a cranial bruit is not heard or not searched for. Various ultrasound modalities have proved useful in evaluation of AVM of the vein of Galen. 1-3 However, before surgical therapy, arteriography has been considered necessary to delineate location of the entry of feeder arteries into the aneurysm,4 although recent reports have indicated that color Doppler imaging could also precisely show the anatomy of these malformations.5-’ This report describes the color Doppler examination of a newborn infant with AVM of the vein of Galen accurately showing the location of the connection between branches of the feeder artery and the aneurysm.
From the
Department
of
Cardiology
and Cardiovascular
Surgery, Hamad General Hospital, Doha, Qatar
443
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
444
FIG. 1.A. Coronal plane of the head with ultrasound. Note dilated ventricles and cystic dilatation of the vein of Galen displayed in blue and mosaic (black arrow). The red and yellow colors denote the dilated feeder artery (white arrow). B. Saggital scan of the head. Dilated anterior cerebral artery is seen as it courses to the posterior aspect of the aneurysm (open arrows) of the vein of Galen (white arrow); 3 =dilated third ventricle.
Case
Report
This male infant of forty weeks of gestation weighing 3900 grams appeared normal at birth. At the age of fourteen days the parents noticed rapid and difficult breathing. On admission, his respiratory rate was 90 per minute and heart rate was 190 per minute. Peripheral arterial pulses were bounding in the upper limbs and neck while femoral artery pulsation was weak. The precordium was hyperactive. There was a gallop rhythm and grade 2/6 regurgitant systolic murmur was heard at the lower left sternal border. Blood pressure was 20 mmHg lower in the lower limbs than in the upper limbs. An intracranial bruit, not heard on admission, appeared after anticongestive therapy. The ECG revealed right atrial and biventricular hypertrophy. ST and T wave changes in the left precordial leads were noticed. Chest radiography showed a grossly enlarged heart with increased pulmonary vascularity. Echocardiography revealed the heart to be anatomically normal with mild tricuspid regurgitation, dilatation of all four heart chambers, and evidence of pulmonary arterial hypertension. There was right-to-left shunting across the foramen ovale. The superior vena cava, ascending aorta, and the innominate artery were all dilated, but the aortic isthmus was narrow. The pulsed-wave Doppler sample volume positioned in the transverse aortic arch showed systolic antegrade flow and diastolic retrograde flow. This flow pattern was noticed in the innominate artery as well. The transducer was then positioned over the arterior fontanelle, and moderate dilatation of the lateral and third ventricles was recorded. An echo-free area was noticed posterior to the third ventricle in the sagittal view, which was considered to represent an arteriovenous (AV) fistulous communication or aneurysm. Color Doppler imaging showed turbulence at the region of a feeder artery entering this aneurysm. In the saggital plane the anterior cerebral artery was dilated, and the flow in this artery was seen as red or as mosaic in the ascending limb, blue at the horizontal course of the vessel, and again mosaic in the descending limb of the artery due to turbulence (Fig. 1 A,B). Pulsed-wave Doppler interrogation revealed continuous flow along the course of the anterior cerebral artery, changing direction from flow toward the transducer in the ascending portion of
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
445
FIG. 2. Pulsed-wave interrogation with the same volin the aneurysm. Increased velocity of the continuous systolic-diastolic flow with decreased pulsatility index is shown.
ume
the artery to flow away from the transducer in its descending part. The pulsatility index was lower than normal (Fig. 2). Computerized tomography (CT) of the head showed an aneurysm of the vein of Galen and confirmed the echocardiographic findings. Arteriography was planned prior to embolization or surgical treatment, but the infant died in intractable heart failure fifteen days after admission to the hospital. Discussion The clinical diagnosis of a cerebral AVM is difficult. A cranial bruit is not always present, and when present, it may not be of clinical significance, for it may be found in normal newborns or may occur as transmission of a loud heart murmur.’ It can mimic congenital heart disease, mainly those with large left-to-right shunt. A coarctation of the aorta can also be suspected because of weak femoral pulses and difference in systolic blood pressure between upper and lower limbs. Coarctation-like physiology could be explained by the runoff of the blood into the fistulous communication. 9&dquo;0 In our infant femoral pulses were faint and no cranial bruit was heard at initial presentation. AVM of the vein of Galen is an example of a truncal AV fistula where communication of a mature artery or arteries and vein without an intervening anastomatic channel occurs. Therefore, no resistance vessels are interposed between the arterial and venous side of the fistula, and this creates a low-resistance circuit. This is reflected as high-velocity turbulence and lower than normal pulsatility index when pulsed-wave and color Doppler are applied to investigate such
a
lesion. 5-~.&dquo;
Echocardiography in an infant with AVM of the vein of Galen reveals dilatation of the heart
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
446 chambers and large afferent and efferent vessels without structural heart disease. The ascending aorta and proximal feeder arteries are dilated. There is narrowing of the aortic isthmus as a result of diminished blood flow through it caused by runoff into the AV fistulas.’2’’3 Pulsedwave Doppler interrogation of the aortic arch proximal to the origin of the ductus arteriosus shows retrograde diastolic flow. Such a flow can also be found in feeder arteries and may be present in a small number of normal infants as well. However, in these normal infants, other echocardiographic and clinical signs are absent.’z Starc et al. 13 emphasized pulsed-wave Doppler mapping of the aorta beginning with the interrogation of the subaortic flow and continuing with the ascending aorta proximal to the innominate artery, transverse aortic arch proximal to the site of ductus arteriosus, postductal descending aorta, and finally, the thoracoabdominal aorta. Thus, aortic valve insufficiency, aorta-pulmonary window, coronary artery fistula, patent ductus arteriosus, or other systemic to pulmonary artery shunt can be differentiated. In our patient we found, in addition to classical two-dimensional and pulsed-wave Doppler signs suggesting extracardiac AV fistula, abdominal flow in the innominate artery, which together with its dilatation, identified it as the origin of the fistulous communication. Cerebral ultrasound examination was confirmatory. It showed a cystic lesion behind the third ventricle, and with color Doppler imaging we could follow the abnormal flow from the innominate artery into the right common carotid artery and into the aneurysm of the vein of Galen. The anterior cerebral artery entered the aneurysm at its posteroinferior aspects. Conclusion We share the opinion of others 1-7 that, in identifying the aneurysm and feeder vessel or vessels, color flow imaging is superior to two-dimensional, pulsed-wave Doppler and contrast echocardiography. It is also easier to apply and probably more accurate. It can replace CT scanning and will likely replace arteriography and digital subtraction angiography before an attempted surgical treatment because the definition of the location of the entry of tributary vessels into the aneurysm with color Doppler imaging is also possible.
Andrej Robida, M.D., D. Sc. Department of Cardiology and Hamad General Hospital P.O. Box 3050
Doha, Qatar
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
Cardiovascular
Surgery
447
References 1. Sivakoff M, Nouri S: Diagnosis of vein of Galen arteriovenous malformation by two-dimensional ultrasound and pulsed Doppler method. Pediatrics 69:84-86, 1982. 2. Stanbridge R, DeL Westaby S, Smallhorn J, et al: In-
3.
4.
5.
6.
tracranial arteriovenous malformation with aneurysm of the vein of Galen as a cause of heart failure in infancy : Echocardiographic diagnosis and results of treatment. Br Heart J 49:157-162, 1983. Mardini MK, Lazarte R, Huntington D, et al: Diagnosis of cerebral arteriovenous malformation by contrast two-dimensional and Doppler ultrasonography of the head after saline injection in the umbilical arterial line. Am Heart J 114:450-454, 1987. Raimondi AJ: Pediatric Neurosurgery. New York: Springer Verlag, 1987, p 321. Schranz D, Jungst BK, Golla G, et al: Herzinsuffizienz bei einem Neugebornen mit arteriovenous Malformation der Vena Galeni: Diagnose mittels farbcodierter Doppler-Bild-Echographie. Monatsschr Kinderheilkd 136:47-49, 1988. Vaksmenn G, Decoulx E, Mauran P, et al: Evaluation of vein of Galen arteriovenous malformation in newborns by two dimensional ultrasound, pulsed and colour Doppler method. Eur J Pediatr 148:510-512,
1989.
7.
Schorf J: Colour Doppler imaging of arterimalformation of the vein of Galen in a newborn. Neuroradiol 32:60-63, 1990. Knudson RF, Alden ER: Symptomatic arteriovenous malformation in infants less than 6 months of age. Pediatrics 64:238-241, 1979. Walker WY, Mullins CE, Knovick GC: Cyanosis, cardiomegaly and weak pulses. A manifestation of massive congenital systemic arteriovenous fistula. Circulation 29:777-781, 1964. Deverall PB, Taylor JFN, Sturrock GS, et al: Coarctation-like physiology with cerebral arteriovenous fistula. Pediatrics 44:1024-1028, 1969. Lindegaard KF, Grolimund P, Aaslid R, et al: Evaluation of cerebral AVMs using transcranial Doppler ultrasound. J Neurosurg 65:335-344, 1986. Musewe NN, Smallhorn JF, Burrows PE, et al: Echocardiographic and Doppler evaluation of the aortic arch and brachiocephalic vessels in cerebral and systemic arteriovenous fistulas. J Am Coll Cardiol 12:1524-1535, 1988. Starc TJ, Kongrad E, Bierman FZ: Two-dimensional and Doppler findings in cerebral arteriovenous malformation. Am J Cardiol 64:252-254, 1989.
Deeg KN,
ovenous
8.
9.
10.
11.
12.
13.
Downloaded from ang.sagepub.com at University of Manitoba Libraries on June 14, 2015
