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.     

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.     

Effect of low dose dopamine on hemodynamic and renal function in children

The purpose of the study was to investigate the effect of low doses of dopamine in children. Fourteen cases were studied after open heart surgery. Cardiac output and renal parameters were determined under baseline conditions and under continuous infusion of dopamine 2.5 and 5 micrograms/kg/min. During the control period cardiac index was 2.62 +/- 0.19 L/min/m2, renal plasma flow was decreased at 269 +/- 41 mL/min/1.73 m2, GFR was 86.6 +/- 9.2 mL/min/1.73 m2, and filtration fraction was elevated at 37.1 +/- 1.9%. Plasma concentration of aldosterone correlated with the filtration fraction. At 5 micrograms/kg/min dopamine increased significantly cardiac output, renal plasma flow, and to a lesser extent GFR, thus decreasing the filtration fraction. At 2.5 micrograms/kg/min dopamine, increased renal plasma flow only in patients older than 5 y and had no effect on the other parameters. The increase of cardiac output in response to dopamine was abolished by propranolol pretreatment. By contrast, the hemodynamic renal response to dopamine was not altered by beta-blockade. These results indicate that 5 micrograms/kg/min of dopamine could prevent renal failure after open heart surgery in children by increasing renal blood flow and attenuating renal compensatory mechanisms.     

Evaluation of vein of Galen arteriovenous malformation in newborns by two dimensional ultrasound,pulsed and colour Doppler method

Two babies with severe cardiac failure caused by a large arteriovenous malformation of the vein of Galen were evaluated with 2-dimensional ultrasound, pulsed and colour Doppler techniques. In both cases similar findings were recorded: dilated right cardiac chambers with high cardiac output in superior vena cava and ascending aorta; retrograde diastolic flow in descending aorta and continuous forward flow in arch arteries reflecting low resistance to flow in cerebral arteries; recirculation of microbubbles through the superior vena cava after passage unchanged through the cerebral malformation following contrast injection of saline into a peripheral vein or an arterial ombilical line. In addition, colour Doppler study of the brain clearly showed the malformation and the dilated straight sinus. Arteriovenous malformation of the vein of Galen can be noninvasively and easily detected by ultrasound studies of the heart and the brain.     

Ebstein anomaly as a rare cause of a non-immunological fetal hydrops: prenatal diagnosis using Doppler echocardiography

The Ebstein's malformation occurs in 0.5% of patients with congenital heart disease. The prenatal diagnosis of such a malformation in the 33rd week of pregnancy is reported. The fetal echocardiography was performed owing to a severe nonimmune hydrops fetalis. The typical distal displacement of the annular attachment of the tricuspid valve leaflets could be viewed in the apical four-chamber view. The application of the pulsed Doppler ultrasound enables the analysis of the cardial haemodynamics and thus the assessment of the severity and prognosis of the diagnosed malformation. In our case the prognosis was interdisciplinary estimated as being very poor (severe cardiac lesion with congestive heart failure already in utero); In the following days intrauterine death occurred. The autopsy confirmed all the prenatal findings.     

Malformation of the great vein of Galen with neonatal heart failure. Report of two cases

The clinical and patholgoical findings in two neonates with the malformation of the great vein of Galen are given. They both reported with serious neonatal heart failure suggesting congenital heart disease. In one of them cardiac catheterization revealed a foetal pattern of circulation causing cyanosis. A bruit and in one of them a thrill over the skull gave the clinical diagnosis of an intracranial arteriovenous aneurysm. They died 48 and 144 hours after birth in spite of medical treatment. The outlook for patients having malformation of the great vein of Galen and suffering neonatal heart failure treated conservatively, seems hopeless.     

MALFORMATION OF THE GREAT VEIN OF GALEN WITH NEONATAL HEART FAILURE Report of Two Cases

ABSTRACT. The clinical and pathological findings in two neonates with the malformation of the great vein of Galen are given. They both reported with serious neonatal heart failure suggesting congenital heart disease. In one of them cardiac catheterization revealed a foetal pattern of circulation causing cyanosis. A bruit and in one of them a thrill over the skull gave the clinical diagnosis of an intracranial arteriovenous aneurysm. They died 48 and 144 hours after birth in spite of medical treatment. The outlook for patients having malformation of the great vein of Galen and suffering neonatal heart failure treated conservatively, seems hopeless.     

Large patent ductus arteriosus and interventricular communication associated with congenital absence of the pulmonary valve

A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.     

Effectiveness of Carvedilol for Congestive Heart Failure that Developed Long after Modified Fontan Operation

We report a case of a patient with severe heart failure after Fontan procedure in whom carvedilol was very effective. A 27-year-old man had intractable congestive heart failure due to severe ventricular dysfunction after Fontan operation. Central venous pressure was elevated to 29 mmHg. A right-to-left shunt was noted across a large collateral vessel between the innominate vein and the pulmonary vein. He was administered carvedilol (initial dose, 2 mg/day; maximum dose, 30 mg/day). Cardiac catheterization performed 1 year after carvedilol administration revealed a decrease in atrial pressure and improvement of ventricular function. He underwent a conversion operation to total cavopulmonary connection (TCPC) and ligation of a collateral vein communicating with the innominate and pulmonary veins. Carvedilol may be a legitimate treatment before TCPC conversion or heart transplantation for the high-risk group of patients with a failed Fontan circulation.     

Cardiac failure caused by arteriovenous malformation in the area of Galen's vein. Treatment by endovascular embolization

The authors report a case, in a 7-month-old girl, of cerebral arteriovenous malformation with a vein of Galen ectasia, complicated by a congestive heart failure and hydrocephalus. The clinical diagnosis was carried out by fontanelle auscultation and confirmed by: cerebral echography. D?ppler exploration, computerized tomography and cerebral angiography. A cerebral embolization permitted reduction of cardiac insufficiency and stabilisation of the hydrocephalus.     

Hepatic hemangioendothelioma: hemodynamic observations and treatment.

The clinical course of five infants with heptic hemangioendothelioma is reviewed. Hemodynamic abnormalities observed at cardiac catheterization included an increased cardiac output (range, 9.2 to 10.5 liters/min/sq m) with over 50% of this output being shunted through the arteriovenous malformation, an elevated right and left end diastolic pressure (8 to 10 mm Hg and 9 to 15 mm Hg respectively), a small systolic pressure gradient across the pulmonary outflow tract (4 to 6 mm Hg), and mild elevation of pulmonary artery pressure (range, 35/16 to 37/12 mm Hg). All four patients treated with prednisone improved while on therapy; three are well one to three years after cessation of therapy. One patient died from unrecognized congestive heart failure following discontinuation of prednisone and another before prednisone was started. We conclude that prednisone in high doses (2 to 4 mg/kg/day) with the addition of digitalis and diuretics for control of congestive heart failure is an effective treatment regimen for infants with hepatic hemangioendothelioma.     

Myocarditis and coronary dilatation in the 1st week of life: neonatal incomplete Kawasaki disease?

Acute heart failure in the early neonatal period is rare. Normally it is due to asphyxia, severe septicaemia, a congenital heart malformation or a viral myocarditis. Kawasaki disease (KD) as a cause of an neonatal myocarditis is not an established diagnosis. KD is a vasculitis of still unknown origin occurring predominantly in infants and preschool children. KD before the age of 3 months is rare. There are only few reports about KD in the 1st month. We present a newborn who showed the cardiac symptoms of KD in the 1st week of life with coronary dilatation and myocarditis. Conclusion The diagnosis of incomplete KD should be considered not only in infants but also in newborns with signs of myocarditis and coronary abnormalities. Therapy with gammaglobulins may prevent the sequelae of coronary involvement. Received: 29 April 1997 / Accepted: 21 November 1997     

Pulmonary arteriovenous malformation: An uncommon cause for cyanosis in the newborn

Pediatric Radiology - Pulmonary arteriovenous malformation is an uncommon cause of cyanosis and heart failure in newborns. A case is presented with review of the literature in order to emphasize...     

珠江三角洲新生儿转运网络工作报告

目的　评价珠江三角洲新生儿转运网络（ｎｅｏｎａｔａｌｔｒａｎｓｐｏｒｔｎｅｔｗｏｒｋ, ＮＴＮ） 十年工作及其效果。方法　介绍新生儿转运网络方法及分析有关效果的资料。结果　转运网络现有８７ 家医院;１０ 年共转运急危重症新生儿８ １２４ 例,转运途中死亡３５ 例,病死率为０．４３％ ;第三级新生儿监护病房（ＮＩＣＵ）收容８ ０８９ 例,共死亡３６４ 例,病死率为４．５０ ％ ,ＮＴＮ 医院中总死亡率为２．０７‰。结论　该ＮＴＮ 所实行的运转机制合理适用;促进了网络单位包括三级ＮＩＣＵ 的发展与提高;建立ＮＴＮ是降低新生儿死亡率的重要措施。     

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.     

Arteriovenous malformation of the vein of Galen in children

Arteriovenous malformation of the vein of Galen (VGM) is a unique vascular anomaly in children. Its extra parenchymal location allows for aggressive endovascular treatment. Depending on the age of the child, the clinical presentation of VGM varies from congestive heart failure to hydrocephalus. The deleterious consequences to the brain depend on many factors, the most important of which is the volume of blood shunting through the malformation and away from the brain parenchyma (“steal” phenomenon). High flow in a fetus with a developing brain will lead not only to brain ischemia but also to multiple organ failure. At the other end of the spectrum, a small malformation can be well tolerated. Treatment should be tailored to each individual case. Received/accepted: 20 December 1996     

儿童冠状动脉疾病32例临床分析

目的探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿（不包括复杂心脏畸形合并冠状动脉异常者）的临床资料,分析临床表现、辅助检查、诊断方法和治疗。结果32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁。经ECG、超声心动图、64排CT和心导管造影检查明确诊断。左冠状动脉异常起源于肺动脉10例(31.2%),8例以心力衰竭起病,其中长期误诊为“心内膜弹力纤维增生症”4例,“扩张性心肌病”1例,“二尖瓣重度关闭不全”2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例(40.6%),9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例(18.8%),出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例(9.4%),表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重。结论儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键。     

Worsening renal function in children hospitalized with decompensated heart failure: evidence for a pediatric cardiorenal syndrome?

OBJECTIVES: The purpose of this study was to determine the incidence of renal insufficiency in children hospitalized with acute decompensated heart failure and whether worsening renal function is associated with adverse cardiovascular outcome. DESIGN: Prospective observational cohort study. SETTING: Single-center children's hospital. PATIENTS: All pediatric patients from birth to age 21 yrs admitted to our institution with acute decompensated heart failure from October 2003 to October 2005. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Acute decompensated heart failure was defined as new-onset or acute exacerbation of heart failure signs or symptoms requiring hospitalization and inpatient treatment. We required that heart failure be attributable to ventricular dysfunction only. Worsening renal function was defined as an increase in serum creatinine by > or = 0.3 mg/dL during hospitalization. Sixty-three patients (35 male, 28 female) comprised 73 patient hospitalizations. Median age at admission was 10 yrs (range 0.1-20.3 yrs). Median serum creatinine at admission was 0.6 mg/dL (range 0.2-3.5 mg/dL), and median creatinine clearance was 103 mL/min/1.73 m2 (range 22-431 mL/min/1.73 m2). Serum creatinine increased during 60 of 73 (82%) patient hospitalizations (median increase 0.2 mg/dL, range 0.1-2.7 mg/dL), and worsening renal function occurred in 35 of 73 (48%) patient hospitalizations. Clinical variables associated with worsening renal function included admission serum creatinine (p = .009) and blood urea nitrogen (p = .04) and, during hospitalization, continuous infusions of dopamine (p = .028) or nesiritide (p = .007). Worsening renal function was independently associated with the combined end point of in-hospital death or need for mechanical circulatory support (adjusted odds ratio 10.2; 95% confidence interval 1.7-61.2, p = .011). Worsening renal function was also associated with longer observed length of stay (33 +/- 30 days vs. 18 +/- 25 days, p < .03). CONCLUSIONS: These data suggest that an important cardiorenal interaction occurs in children hospitalized for acute decompensated heart failure. Renal function commonly worsens in such patients and is associated with prolonged hospitalization and in-hospital death or the need for mechanical circulatory assistance.     

Aortic Runoff as a Sign of Intracranial Arteriovenous Malformation: Report of Two Cases

Background

Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion.

Case Presentation

We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranial arteriovenous malformation and misdiagnosis of congenital heart disease.

Conclusion

Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation and had the major role in early diagnosis.     

Nonimmune Hydrops Fetalis in the Liveborn: Series of 32 Autopsies

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.