Cerebral arteriovenous malformation: prenatal and postnatal central blood flow dynamics

Using the Doppler technique, this study compared the prenatal and postnatal flow patterns of an infant with cerebral arteriovenous (AV) malformation. Fetal right ventricular end-diastolic dimension was 1.7 cm with right ventricular ejection equaling 66% of the combined cardiac output. Diastolic flow was reversed in the fetal aortic isthmus but forward-moving in the descending aorta, resulting in a watershed phenomenon. In the carotid arteries an increased forward diastolic flow was noted while the internal jugular vein exhibited a pulsatile arterial type of flow. The pulsatility index of the umbilical artery was normal. After birth and closure of the ductus arteriosus, the output from both ventricles was equally increased, and retrograde diastolic flow was then observed in the descending aorta. In conclusion, specific fetal features of cerebral AV malformation are increased right ventricular output probably related to the increased superior vena cava return, watershed phenomenon in the descending aorta causing the right ventricle to perfuse both the lower and upper body, and preserved umbilical circulation.     

单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 目的 探讨胎儿单纯性完全型肺静脉异位引流（TAPVC）的产前超声心动图特点,提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果,总结超声心动图特征。结果 8例单纯性TAPVC胎儿中,心下型2例,心内型1例,心上型5例,5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻;8例胎儿中,引产1例（心下型）,余7例均于出生后行手术治疗,其中1例术后死亡（心下型）,6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失,呈圆形或椭圆形,左心房光滑并多变小,在孕后期较为明显;②降主动脉与左心房间距离明显增大,多数在左心房后方可见一异常的腔隙（即共同肺静脉腔）,可显示左、右侧上升（心上型）或下降（心下型）的垂直静脉;③妊娠早期左、右心系统比值早期多正常,但妊娠中、晚期（孕26周后）可出现右心系统轻度扩大;④引流入冠状静脉窦时（心内型）,冠状静脉窦可有扩张;引流入上腔静脉时（心上型）,上腔静脉扩张;引流入肝内血管时（心下型）,肝内血管可有不同程度的扩张;⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时,TAPVC易被漏诊及误诊,应注意多角度、多切面扫查,孕晚期超声心动图检查可减少漏诊。     

Circulatory effects of fetal cardiac arrhythmias

Summary The circulatory consequences of cardiac arrhythmia and its compensatory mechanisms were examined in utero in 37 fetuses. A combined real-time and 2-MHz pulsed Doppler technique was used to measure blood velocity for waveform analysis and flow estimation in the descending thoracic aorta of the fetuses. The pattern of blood velocity in the inferior vena cava was studied to classify the arrhythmia. Despite severe cardiac arrhythmias the aortic blood flow was within normal limits in all but one fetus which had associated cardiac malformation and congestive failure. The rising slope and the peak value of the maximum aortic velocity were significantly increased in the postpremature beats and in fetuses with atrioventricular block. The results support the validity of Frank-Starling's law for the fetal myocardium.     

Vein of Galen aneurysmal malformation: a neonatal case with unusual evolution]

We report on a neonatal patient case with a cerebral vascular dilation consistent with a vein of Galen malformation diagnosed on an antenatal doppler ultrasound examination. Antenatal diagnosis was confirmed by fetal MRI scan. After delivery, cerebral MRI scan found the same dilatation and showed no cerebral parenchymal damage. The infant's condition was stable and particularly there was no congestive cardiac failure. Before discharge at ten days of age, clinical examination and particularly cardiac examination was normal. At 14 days of age, symptoms in relation to a congestive cardiac failure appeared. Echocardiography showed a high output cardiac failure with no cardiac malformation. Infectious laboratory tests were normal. Evolution was initially favourable after digitalo-diuretic treatment. Unfortunately, death occurred at 36 days of age due to intractable cardiac failure. This case highlights that, unusually, heart failure can occur long after the first days of life in cases of Galen vein aneurysmal malformation. The optimal time for vascular embolization in neonatal cases of Galen vein aneurysmal malformation is discussed.     

Direct entry of the right superior vena cava into the left atrium with aneurysmal dilatation and stenosis at its entry into the right atrium with stenosis of the pulmonary veins: A rare case

Summary This is the first autopsied case reported in the English language literature wherein the right superior vena cava entered both atria with obstruction of the entry into the right atrium, aneurysmal dilatation of the proximal part of the superior vena cava, and entry of the stenosed right upper pulmonary vein into the aneurysmal sac. The aneurysmally dilated right superior vena cava communicated directly with the left atrium. In addition, the remainder of the pulmonary veins, all of which were markedly stenosed, entered the left atrium. The embryogenesis of this unique malformation is briefly discussed. Aided by grant HL 30558-01 from the National Heart, Lung and Blood Institute of the National Institutes of Health, Bethesda, Maryland.     

Magnetic resonance demonstration in the newborn of generalized cerebral venous dilation with spontaneous resolution.

The authors present serial magnetic resonance (MR) images of an infant with cardiac failure and generalized cerebral venous dilation, which was initially misdiagnosed in the first week of life on cranial ultrasound as a vein of Galen malformation. At 3 months of age, repeat MR imaging demonstrated complete resolution of this marked cerebral venous distension with no evidence for cerebral injury. This case illustrates the value of MR in the identification of this disorder and its distinction from more serious conditions, such as vein of Galen malformation and venous sinus thrombosis. Complete resolution of the venous dilation and the lack of definable parenchymal injury suggest a good prognosis for this disorder.     

Aneurysm of the vein of Galen with neonatal cardiac insufficiency. Success of early neurosurgical treatment

The vein of Galen malformation is a rarely recognized cause of congestive heart failure in the newborn. This report describes a newborn who developed severe cardiac failure in the second week of life. An aneurysm of the vein of Galen was noted on the computerized tomography scan, confirming the clinical diagnosis of cerebral arteriovenous fistula. The vascular abnormality was well shown by cerebral arteriography. At the age of four weeks, surgery was carried out by clipping the afferent arteries. The patient is non a three years old infant with moderate developmental delay and hypotonia.     

An unusual cause of cardiac failure in a neonate

We report the case of a newborn admitted with signs of congestive cardiac failure with prominent and pulsatile cervical veins. Echocardiography showed a structurally normal heart, right-to-left ductal flow, and reversed diastolic flow in the proximal descending aorta. A computed-tomography scan of the head showed a vein of Galen arteriovenous malformation. This highlights the importance of considering an intracranial cause in the differential diagnosis of neonatal congestive heart failure.     

Doppler ultrasound and angiography of the vasculature of the liver in children after orthotopic liver transplantation: a prospective study

Despite the availability of Doppler ultrasound, angiography still forms part of the protocol for evaluating children after orthotopic liver transplantation (OLT) at our department. To investigate whether Doppler ultrasound is a reliable method for evaluating the patency of the hepatic artery, portal vein, inferior vena cava, and the anastomotic site of the portal vein in children after OLT, we performed a prospective study in which Doppler ultrasound was compared with angiography in 38 children with 40 transplants (ten examinations on clinical demand and 49 examinations according to protocol). Good correlation was found in relation to demonstrating a patent hepatic artery (sensitivity 96% and specificity 100%). Two false-negative Doppler ultrasound results were attributable to technical difficulties and rejection. For evaluating the patency of the portal vein, Doppler ultrasound agreed with angiography in 58 of the 59 examinations (98%). The one and only false-positive angiography result was explained by inadequate opacification. Doppler ultrasound visualized stenosis of the portal vein three times more often than angiography. In seven children, Doppler ultrasound findings suspicious of pathology of the inferior vena cava were confirmed using cavography or surgery. Doppler ultrasound proved to be a reliable technique for evaluating the patency of the hepatic artery, inferior vena cava, and portal vein and the anastomotic site of the portal vein.     

Ultrasound detection of congenital Arteriovenous aneurysm of the great cerebral vein of Galen

Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis. Invasive methods such as cerebral angiography were avoided.Case histories, neuropathological findings, ultrasound method and results are presented. Pathogenesis, clinical signs, treatment and prognosis are discussed. With the ultrasound method presented, the nature and location of the vascular anomaly were demonstrable, thus additional higher risk diagnostic methods could be avoided or planned more purposefully.     

Which to measure,systemic or organ blood flow? Middle cerebral artery and superior vena cava flow in very preterm infants

AIM: To describe, in very preterm babies, postnatal changes in measures of middle cerebral artery (MCA) Doppler variables. To relate these peripheral measures to echocardiographic measures of systemic blood flow and ductal shunting, and to study their relation to subsequent intraventricular haemorrhage (IVH). METHODS: 126 babies born before 30 weeks were studied with serial echocardiography and cerebral and Doppler ultrasound of the MCA at 5, 12, 24, and 48 hours of age. Echocardiographic measures included superior vena cava (SVC) flow and colour Doppler diameter of the ductal shunt. MCA Doppler measures included mean velocity, pulsatility index (PI), and estimated colour Doppler diameter. RESULTS: MCA mean velocity increased whereas the PI decreased significantly over the first 48 hours. Babies with low SVC flow had significantly lower MCA mean velocity and estimated diameter than babies with normal SVC flow. There was no difference in PI. On multivariant analysis, the significant associations with MCA mean velocity were mean blood pressure (MBP), heart rate, SVC flow, and lower calculated vascular resistance. The significant associations with PI were larger ductal diameter and lower mean MBP. The significant associations with MCA diameter were higher SVC flow and lower calculated vascular resistance. After controlling for gestation, there was a highly significant association between lowest SVC flow and subsequent IVH but no association between IVH and lowest MCA mean velocity, estimated diameter, PI, or MBP. CONCLUSIONS: These data are consistent with the speculation that SVC flow is a reflection of cerebral blood flow. Low SVC flow is more strongly associated with subsequent IVH than cerebral artery Doppler measures or MBP.     

Congestive heart failure in a newborn infant with arteriovenous malformation of the vein of Galen: diagnosis using color-coded Doppler image echography

Arteriovenous malformations of the vein of Galen are rare disorders that may appear in the newborn period with severe congestive heart failure mimicking many intrinsic cardiac defects. Using combined two-dimensional ultrasound and color-coded blood flow mapping arterio-venous aneurysm of the vein of Galen and congenital atrial septal defect could be diagnosed in an newborn with congestive heart failure. In addition to the presented clinical value of the new two-dimensional color Doppler echography physiological aspects of intracranial arteriovenous fistula in infancy discussed.     

Cyanosis in a 9-month-old child after repair of total anomalous pulmonary venous return

A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.     

Superior vena caval thrombosis in a child: An uncommon presentation of idiopathic dilated cardiomyopathy

A nine year old girl who presented with a thrombosis of the right internal jugular vein and superior vena cava was found to be in cardiac failure and to have a dilated cardiomyopathy. After transient improvement she deteriorated and was accepted for cardiac transplantation. Following transplantation her condition has been much improved.     

Fetal breathing movements—A comparison of hard copy records produced by M-mode and Doppler ultrasound

To precisely measure the events of the fetal breath cycle it is necessary to have an accurate hard copy record. Two approaches to this have been made using either M-mode or Doppler ultrasound. It was the aim of this paper to compare these methods. Hard copy records of fetal breathing movements were produced by simultaneously recording movements of the fetal chest wall with M-mode and frequency spectral analysis of Doppler signals from either the fetal inferior vena cava or umbilical vein. Timing of the onset and end of inspiration was equally recognised by chest wall movement and changes in umbilical vein flow. Augmented flow in the fetal inferior cava began after the onset of “inspiration” and continued into “expiration”. This signal could be used for registering the occurrence of a breath but not for timing. Because of unequal'rotation and distortion of the chest wall, during fetal breathing movements, records of chest wall movement were considered less precise than those from the umbilical vein for timing the events of the fetal breath cycle. The umbilical vein flow velocity sonogram provides an accurate record to allow measurement of “inspiratory” time and total breath movement time.     

Superior vena cava flow: Role,assessment and controversies in the management of perinatal perfusion

The superior vena cava (SVC) is a large vein responsible for the venous return of blood from structures located superior to the diaphragm. The flow in the SVC can be assessed with Doppler ultrasound and can be used as a proxy for cerebral perfusion. Early clinical research studies showed that low SVC flow, particularly if for a prolonged period, was associated with short term morbidity such as intraventricular hemorrhage, mortality, and poorer neurodevelopmental outcomes. However, these findings have not been consistently reported in more recent studies, and the role of SVC flow in early management and as a predictor of poor long-term neurodevelopment has been questioned. This paper provides an overview of SVC assessment, the expected range of findings, and reviews the role of SVC flow as a diagnostic and monitoring tool for the assessment of perinatal perfusion.     

Doppler flow profiles in the right and left pulmonary artery in children with congenital heart disease and a bidirectional cavopulmonary shunt

Summary The systolic and diastolic Doppler tracings in the right and left pulmonary artery were analyzed in 10 patients with complex cyanotic congenital heart disease, aged 6 months to 12 years (median 3 years), after employment of a bidirectional cavopulmonary shunt. The postoperative interval ranged from 2 weeks to 1.7 years (median 1.3 years). In children with pulmonary atresia or severe pulmonary stenosis with minimal antegrade pulsatile pulmonary blood flow Doppler echocardiography confirmed a systolic and diastolic bidirectional shunt from the vena cava superior to both pulmonary arteries. In children with pulmonary stenosis, Doppler echocardiography confirmed a systolic shunt only to the right pulmonary artery and a diastolic bidirectional shunt into both pulmonary arteries. As the left pulmonary artery was perfused by the pulsatile transvalvular flow it was difficult to detect a concomitant systolic Glenn-related flow in those patients. Quantitative analysis of the diastolic Doppler tracings revealed a significant difference in the velocity time integral in the right and left pulmonary artery indicating a dominant right lung perfusion in diastole.Deceased     

Arteriovenous malformation of the great cerebral vein (of Galen) in a newborn

This is a report of a case with an arteriovenous (a.v.) malformation of the great cerebral vein. Applicable diagnostic procedures are reviewed. The initial diagnostic procedure in the approach to an a.v. malformation of the great vein of Galen is cerebral ultrasound.The findings in our patient are compared with those reported in the literature.     

Neuroimaging findings in neonates and infants from superior vena cava obstruction after cardiac operation

BACKGROUND: Extraventricular obstructive hydrocephalus may develop after superior vena cava obstruction, an uncommon complication after cardiac surgery. OBJECTIVE: To describe the neuroimaging findings in neonates and infants with superior vena cava thrombosis after cardiac surgery for congenital heart disease. MATERIALS AND METHODS: Between 1993 and 2001, 333 neonates and infants in our hospital underwent cardiac surgery, of whom 13 (3.9%) subsequently acquired superior vena cava syndrome. Eleven of these 13 children (7 boys, 4 girls) were evaluated by head ultrasound and computed tomography scans. RESULTS: One child had normal findings on head ultrasound, and 10 children had extraventricular obstructive hydrocephalus (EVOH). In 6 children, aggravation of the hydrocephalus was noted up to 11.4 months after cardiac surgery; in 3 of them, the hydrocephalus was shunted to the peritoneum. One child had thrombosis of the dural sinuses, and 1 had hemorrhagic infarction. Two children died during follow-up. CONCLUSION: EVOH is a common complication of superior vena cava thrombosis, and head ultrasound should be performed in all neonates and infants with superior vena cava thrombosis after cardiac surgery. Long-term follow-up is needed, as the hydrocephalus may worsen even months after surgery.     

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported – all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed. Received: 3 December 1996 / Accepted in revised form: 9 September 1997