Catheter embolization of pulmonary arteriovenous fistulas in an infant

Summary An infant with pulmonary arteriovenous fistulas is described. Surgical ligation of the fistulas and limited resection of paraenchyma from the right lung was followed at 11 months of age, by successful steel coil embolization of residual fistulas.     

Pulmonary arteriovenous malformation in the newborn: A familial case

Summary Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in the newborn with nine previously reported cases. Typical signs at presentation include cyanosis, murmur, and congestive heart failure. Abnormality on chest x-ray is common with cardiomegaly, oligemia, and focal pulmonary density. There is a male predominance, unlike older children and adults with PAVM. Familial cases of PAVM have been reported with Osler-Weber-Rendu syndrome. We report a familial case of PAVM presenting in the newborn.     

Micropulmonary arteriovenous fistula causing cyanosis

This article presents a young girl who came to the Department of Pediatrics, AIIMS, with cyanosis and was diagnosed to be having multiple pulmonary arteriovenous fistulae. The cardiovascular evaluation was normal and so was the respiratory evaluation. The desaturation did not improve with oxygen. The chest X-ray was also normal. The echocardiogram did not reveal any abnormality but agitated saline contrast echocardiography suggested an extracardiac right to left shunt which was confirmed on pulmonary angiography.     

Pulmonary artery to the left atrium fistula as an unusual cause of cyanosis in the newborn

Summary A fistula between the pulmonary artery (PA) and the left atrium (LA) is a rare form of congenital heart disease, 34 cases having been described in the literature, all from the right pulmonary artery (RPA). Only six cases were diagnosed in the newborn period. We report such a case, diagnosed at 1 day of age by Doppler echocardiography, with an unusual site of the fistula.     

Polysplenia with pulmonary arteriovenous malformations

Summary A patient with polysplenia syndrome, dextrocardia, left atrial isomerism, normal great vessel relationships, and no intracardiac shunts developed progressive cyanosis and clubbing. Pulmonary arteriovenous malformations (PAVMs) were diagnosed by angiography and confirmed by lung biopsy. Superior mesenteric arteriogram revealed hypoplasia of the intrahepatic portal vein branches and a portosystemic shunt. The possible etiologies of PAVMs are discussed.     

4岁3月龄女孩发绀4年余

4岁3月龄女性儿童,出生后即出现口唇发绀,1年前出现活动后气促,3个月前出现肢端发绀,伴明显杵状指/趾畸形。实验室检查示血红蛋白升高（178 g/L）、动脉血氧分压降低（37.7 mm Hg）。肺部CT平扫+增强见右下肺大片及多发小结节状边界清晰致密影,右下肺动脉增粗,右下肺静脉扩张。肺动脉磁共振血管成像检查显示巨大肺动静脉畸形。患儿确诊为先天性肺动静脉瘘,予介入下行肺动脉瘘栓塞术治疗。术后3个月随访,患儿气促、发绀等症状消失,活动耐量恢复正常,心率、血红蛋白浓度、红细胞计数、经皮血氧饱和度均恢复正常。     

Congenital pulmonary arteriovenous malformations

 We report the rare case of an 11-year-old boy who presented with intractable hypoxaemia and a pulmonary mass. Investigations revealed this to be a pulmonary arteriovenous malformation. It was entirely confined to the right middle lobe and was successfully treated via a right middle lobectomy. The relevant literature with respect to both diagnosis and treatment is reviewed. Accepted: 20 March 2000     

Congenital pulmonary arteriovenous fistula producing pulmonary arterial steal syndrome

Summary This report describes a congenital pulmonary arterial steal syndrome manifested as cyanosis and acidosis in a newborn. A fistulous connection between the right pulmonary artery and a large, anomalous right common pulmonary vein stole blood from the pulmonary arteries. The anomaly was suspected because of a pericardiac shadow on frontal and lateral chest films, substantiated by M-mode echocardiogram, confirmed at cardiac catheterization with angiocardiography, and analyzed at postmortem examination. Supported in part by a grant from The Rose M. Badgeley Charitable Trust     

Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy

OBJECTIVE: To describe outcomes of transcatheter embolotherapy (TCE) in children with pulmonary arteriovenous malformations (PAVMs). STUDY DESIGN: Chart and imaging review of all children (age 相似文献   

儿童颅内动静脉畸形的显微外科治疗

目的 总结显微手术治疗31例儿童颅内动静脉畸形的经验体会,探讨其治疗方案和手术注意事项.方法 我院自2003年6月～2007年6月对31例儿童颅内动静脉畸形的临床资料、治疗过程以及诊治结果进行回顾性分析.31例中有25例以脑内出血起病,5例以癫痫为主要表现,1例表现为轻度头痛.按照Spetzler-Martin分级,Ⅰ级2例,Ⅱ级12例,Ⅲ级10例,Ⅳ级5例,Ⅴ级2例.单纯手术治疗24例,栓塞后手术7例.结果 31例动静脉畸形团均全切除.6例治疗后出现一过性神经功能缺损或轻度新增神经功能缺损,1例患儿遗留偏瘫.无死亡病例.结论 显微手术切除术可以相对安全有效地治疗儿童颅内动静脉畸形,对于大型或位于功能区的颅内动静脉畸形(AVM),术前栓塞有助于提高疗效.     

Isolated congenital coronary arteriovenous fistula

During the last eight years a total of nine cases of isolated congenital coronary arteriovenous fistula (CAVF) have been diagnosed at our centre. Age of these patients ranged from 11 days to 22 years (mean 6.4±4.2 years). There were seven male and two female patients. Right and left coronary arteries were the sites of origin in seven and three cases respectively. One patient had two fistulae, one each originating from right coronary artery and obtuse marginal artery. The drainage sites were right ventricle in seven cases and right atrium in two. The pulmonary artery pressure was normal in eight cases and elevated in one case. The pulmonary artery to systemic flow ratio ranged from 1.1∶1–2.4∶1 in all except the 11 day-old-baby in whom it was 4∶1. Pulmonary vascular resistance (PVRI) was below 3.5 in all cases (range 1.0–3.5 units). Surgical ligation of fistula was done in four cases and two patients underwent coil embolisation of fistula. Follow-up is available in five out of eight patients including three cases in whom CAVF was closed. Two other young patients are being followed-up conservatively and all the patients are asymptomatic. CAVF can present at any age; majority of the cases have small left to right shunts and more commonly CAVF arise from right coronary artery. Surgical ligation of fistula is safe in older patients, non-surgical embolisation using coils may be an alternative to surgery in selected cases.     

Pulmonary Agenesis with Congenital Heart Disease

The combined anomalies of pulmonary agenesis and congenital heart disease are exceedingly rare and a highly lethal association. We describe four cardiovascular operations in three patients with this association. Open-heart surgery was performed in two patients, and palliative heart surgery was performed in two patients. One patient died 100 days after the operation because of severe tracheal stenosis and renal failure, but the other two patients are doing well. Surgical access through a thoracotomy, instead of a median approach, was adequate in all patients. Careful evaluation and a precise understanding of the anatomy are necessary for the surgical management of these patients.     

Endovascular treatment of pediatric intracranial arteriovenous shunt

Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high‐flow fistulas. In association with high‐flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient‐by‐patient basis, according to the unique physiological condition of the child.     

Coronary arteriovenous fistulae mimicking cardiovascular sequelae of Kawasaki disease

Summary A case of coronary artery dilatation in childhood, initially attributed to Kawasaki disease, was determined by careful serial echocardiography to be the result of congenital coronary arteriovenous fistulae.     

Pulmonary arteriovenous malformation in children

Although pulmonary arteriovenous malformations (PAVM) are relatively rare in children, they are important in the differential diagnosis of common pulmonary problems, such as hypoxemia, hemoptysis and dyspnea on exertion. We report the cases of two PAVM patients with different presentations and describe the treatment strategies.     

Diagnosis and Morphological Classification of Pulmonary Arteriovenous Fistula by 99mTc MAA

Pulmonary A-V fistula was diagnosed and classified morphologically based on the pattern of time-activity curves by ^99m Tc MAA. If the diameter of the fistula is large, macro-albumin easily reveals a shunt. If the iistula has the form of an “aneurysm,” the degree of shunt is generally slight.     

Regression of pulmonary arteriovenous malformations following heart transplantation

Pulmonary arteriovenous malformations (PAVMs) can occur following caval to pulmonary artery connection, Glenn and/or Fontan procedure, leading to severe cyanosis and exercise intolerance. It is unknown whether these abnormalities regress or persist following heart transplantation (HTx). Twenty patients with failed Fontan or Glenn procedures were screened for PAVMs prior to HTx by contrast echocardiography, selective pulmonary angiography, and pulmonary venous desaturation. Age at transplant, diagnosis, previous operations, time from Glenn to transplant, systemic oxygenation, hemoglobin level, and ventricular function were determined. The clinical course after HTx was characterized in three patients with significant PAVMs. Indications for HTx were exercise intolerance and severe cyanosis in one patient, and cyanosis and ventricular dysfunction in two. Pre-HTx, mean systemic saturation was 67%; mean pulmonary venous wedge saturation was 81%. Post-HTx, oxygen saturations were normal (> 96%) at 14, 40, and 180 days. Contrast echocardiography, performed 1 month to 3.3 yrs after HTx, showed no intrapulmonary shunting in two patients and minimal shunting in one. One patient suffered an embolic stroke from right-to-left shunting post-HTx. All patients are alive and well 35, 71, and 73 months post-HTx. In patients with single ventricle physiology, PAVMs are not an absolute contraindication to HTx. Heart-lung transplant may not be required for these patients.     

Innominate artery enlargement in congenital arteriovenous fistula with subsequent tracheal compression and stridor

To demonstrate that airway obstruction may be the first manifestation of a congenital fistula, a female newborn is reported who presented with increasing stridor during her first 23 months of life. Magnetic resonance imaging and Doppler echocardiography revealed an enlarged innominate artery with turbulent flow. Angiography demonstrated an arteriovenous fistula between the right subclavian artery and right subclavian vein and an abnormal origin of the right internal thoracic artery. Bronchoscopy showed a pulsating compression of the middle section of the trachea. Closure and division of the fistula and aortotruncopexy were performed. The stridor disappeared, and there was marked relief of the tracheal obstruction, confirmed by bronchoscopy. It is concluded that, a search for enlarged vessels is necessary in cases of airway obstruction.     

Bilateral coronary arteriovenous fistulas

Summary A 14-year-old girl with bilateral coronary arteriovenous fistulas (CAVFs) draining into the right ventricle (RV) is presented. Selective coronary angiography revealed involvement of both right and left coronary arteries in the fistulous formation, but communication into the RV was single.