Transarterial and Transvenous Approach for Transcatheter Closure of a Large Coronary Artery Fistula with the Amplatzer Vascular Plug

Interventional occlusion of coronary artery fistulas has become a well-accepted alternative to surgical therapy. However, occlusion of high-flow lesions in children, necessitating implantation of large occluding devices, may be limited by the requirement of large delivery catheters. This report describes the interventional occlusion of a large coronary artery fistula in an 8-year-old girl. Complete occlusion was achieved by subsequent transvenous and transarterial implantation of two Amplatzer vascular plugs (AVP). The AVP is an interesting alternative for interventional occlusion of large coronary artery fistulas in children that can be deployed safely by rather small guiding catheters.     

Plain radiographic,nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia

Clinical and radiological findings of hepatogenic pulmonary angiodysplasia are reported in two cases. Myriad spidery pulmonary blood vessels are seen on plain radiographs and verified with right to left intrapulmonary shunting on pulmonary angiogram and pulmonary isotopic perfusion scan. Pathophysiology and differential diagnosis are discussed. We propose that the term "pulmonary angio-dysplasia" should include: 1) Pulmonary telangiectasia 2) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) 3) Classical discrete pulmonary arteriovenous fistula 4) Hepatogenic pulmonary angiodysplasia: pulmonary arteriovenous communications with right to left shunting secondary to hepatic cirrhosis.     

Large fetal pulmonary arteriovenous fistula: impact on pulmonary development

A case report of a patient with a large pulmonary arteriovenous fistula and valvar pulmonary stenosis is presented. The fistula was diagnosed prenatally and its effect on in utero cardiovascular growth and development documented. Due to concerns about massive intrapulmonary shunting potentially causing profound cyanosis after delivery, an EXIT (EX-utero Intrapartum Treatment) procedure was used to transfer the infant from placental to extracorporeal membrane oxygenation (ECMO) support. Severe pulmonary microvascular disease resulted in prohibitive pulmonary hypertension despite surgical ligation of the fistula. Prenatal and postnatal hemodynamic assessments of the fistula are presented and are compared to the pathologic findings.     

Clinical Improvement After Banding of a Pulmonary Branch Artery in a Symptomatic Patient with Osler–Rendu–Weber Syndrome

We report a symptomatic newborn with Osler–Rendu–Weber syndrome, multiple and diffuse pulmonary arteriovenous malformations, and right-to-left shunting in the left lung. Right-to-left shunting was significantly decreased by selectively banding the left pulmonary branch artery and clipping one large feeding vessel so that total resection of the left lung could be avoided.     

Morphological Studies of Pulmonary Arteriovenous Shunting in a Lamb Model of Superior Cavopulmonary Anastomosis

We sought to identify and characterize the abnormal vascular structures responsible for pulmonary arteriovenous shunting following the Glenn cavopulmonary shunt. Superior cavopulmonary shunt is commonly performed as part of the staged pathway to total cavopulmonary shunt to treat univentricular forms of congenital heart disease, however, clinically significant pulmonary arteriovenous malformations develop in some patients after the procedure. The causes of pulmonary arteriovenous malformations and other pulmonary vascular changes that occur after cavopulmonary shunt are not known. Using a juvenile lamb model of superior cavopulmonary anastomosis that reliably produces pulmonary arteriovenous malformations, we performed echocardiography and morphological analyses to determine the anatomic site of shunting and to identify the vascular structures involved. Pulmonary arteriovenous shunting was identified by contrast echocardiography in all surviving animals (n = 40) following superior cavopulmonary anastomosis. Pulmonary vascular corrosion casts revealed abnormal tortuous vessels joining pulmonary arteries and veins in cavopulmonary shunt animals but not control animals. In conclusion, unusual channels that bridged pulmonary arteries and veins were identified. These may represent the vascular structures responsible for arteriovenous shunting following the classic Glenn cavopulmonary shunt. Detailed analysis of these structures may elucidate factors responsible for their development.     

Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt

An infant with cyanotic congenital heart disease and polysplenia syndrome developed profound cyanosis within months of undergoing bilateral cavopulmonary anastomoses. Intrapulmonary shunting was diagnosed by contrast echocardiography with peripheral venous and selective pulmonary artery injection. Histopathology revealed abnormal, thin-walled vessels within the interstitium of the lung lobule. These vessels have not been reported previously and are likely to be the anatomic site of arteriovenous shunting. This case demonstrates that pulmonary arteriovenous fistulas (PAVFs) may develop rapidly after cavopulmonary anastomosis in young infants. It also illustrates the use of contrast echocardiography for following PAVF progression in these patients.     

小儿先天性心脏病介入治疗并发症的外科处理

目的 探讨先天性心脏病介入治疗后出现并发症而进行外科手术的适应证和手术时机.方法 1997年6月至2007年6月我院有28例先天性心脏病的患儿在介入治疗后出现并发症而需要外科手术治疗,其中男12例,女16例,年龄为8个月～14岁,平均为(8.9±3.4)岁,体重为9～43kg,平均为(22.7±2.1)kg;房间隔缺损12例,室间隔缺损8例,动脉导管未闭8例.介入治疗并发症包括有:堵闭器脱落12例,单纯残余分流10例(其中残余分流合并溶血3例),Ⅲ度房室传导阻滞4例,堵闭器脱落合并心包填塞1例,堵闭器被三尖瓣腱索缠绕1例.有23例在介入手术后当天进行急诊外科手术,5例于术后48h内手术.外科手术中,堵闭器取出术20例,房间隔缺损修补12例,室间隔缺损修补8例,动脉导管未闭缝闭术8例.结果 无住院死亡病例,经外科治疗后所有患儿均痊愈出院.术后复查心脏彩超无残余分流,而术前为Ⅲ度房室传导阻滞的4例患儿中,有3例恢复为窦性心律,1例转为Ⅱ度房室传导阻滞.结论 介入治疗术前必须有准确的诊断,并且选择合适的病例进行;出现并发症后应及时外科手术;对出现Ⅲ度房室传导阻滞的病例,经保守治疗无效后尽快选择外科手术.     

经导管介入封堵儿童期主肺动脉窗3例报告并文献复习

目的总结经导管介入封堵儿童期主肺动脉窗的经验及疗效。方法对3例儿童期主肺动脉窗予以经导管介入封堵,其中男2例、女1例,年龄分别为11个月、14岁及2岁。1例属非限制性缺损,2例属限制性缺损。1例采用逆向法(主动脉端至肺动脉端)通过缺损,2例采用顺向法;1例采用肌部室间隔缺损封堵器,2例采用动脉导管封堵器。结果 3例病例均成功封堵,2例无残余分流;1例有中量残余分流,1个月后复查无残余分流。3例均无左冠状动脉、肺动脉阻塞及主动脉或肺动脉反流。结论经导管介入封堵儿童期大的(非限制性或限制性)主肺动脉窗是安全、可行的。选择介入的主肺动脉窗需是中间型,无伴发其他先天性心脏病。介入治疗具有明显减少不适感、不需体外循环、无手术瘢痕及缩短住院时间等优点。     

Cerebral Hemorrhage Associated with Sildenafil (Revatio) in an Infant

A case of cerebral hemorrhage associated with sildenafil (Revatio) use in an infant is presented. Sildenafil is increasingly used in the treatment of primary and secondary pulmonary arterial hypertension and pulmonary arteriovenous fistula. In the reported case, sildenafil used to treat pulmonary arteriovenous fistula improved right-to-left shunting across the pulmonary fistula but resulted in cerebral hemorrhage. Cerebral hemorrhage, a previously reported complication of sildenafil, developed in an infant after a rapid increase in dose, to 4.7 mg/kg/day. Therefore, sildenafil doses must be increased only with care, and cerebral hemorrhage must be considered a potential complication.     

Interventional catheterization in children

In children, interventional catheterization is performed to treat congenital heart diseases: balloon atrial septostomy; balloon valvotomy for valvar pulmonary stenosis, valvar aortic stenosis, valvar mitral stenosis; balloon angioplasty for venous or arterial stenosis. Transcatheter vascular occlusion can be accomplished using a wide range of devices: transcatheter patent ductus arteriosus closure, embolisation of arteriovenous malformations, systemic-pulmonary collaterals and other extracardiac shunts. Intracardiac or intravascular catheters can be removed.     

Diffuse pulmonary arteriovenous fistula shown by contrast echocardiography and pulmonary angiography

We report an 11-year-old boy with diffuse pulmonary arteriovenous fistula (multiple telangiectasia type). Contrast echocardiography proved R to L shunting at the peripheral levels of the lungs. A definitive diagnosis was obtained by pulmonary arteriography.     

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。     

Transcatheter patent ductus arteriosus occlusion: evolution of techniques and results from the 1990s

OBJECTIVE: To review the evolution of transcatheter patent ductus arteriosus (PDA) occlusion techniques and results. METHODS: A single institution, retrospective review including all patients with intention to close a PDA from 1991 to 1998, with no exclusions. RESULTS: Rashkind occluder (n = 65), sideris double-button (n = 6), Cook detachable coil (n = 28) and Amplatzer ductal occluder (n = 4) were used. Successful implantation occurred in 99 of 103 patients. There was a need for a second transcatheter procedure to close residual ductal shunting in 12% of patients: Rashkind umbrellas (n = 8), double-button (n = 1), coils (n = 3). Eight patients (8%) required surgery, including 4 of 6 patients with the double-button occluder. CONCLUSIONS: The Rashkind occluder and the Sideris double-button device both had an unacceptably high rate of residual shunts requiring a second transcatheter procedure or surgical closure. Detachable coils and the Amplatzer ductal occluder have become the current technology of choice for transcatheter PDA closure with high success rates.     

Pulmonary arteriovenous fistula in the newborn: a case report of Rendu-Osler-Weber syndrome and a review of the literature.

In most instances, congenital arteriovenous fistula is only one manifestation of a more widespread abnormality; 60% of patients also have hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber syndrome). Among those with congenital pulmonary arteriovenous fistula, the diagnosis is made during infancy in only 15% of patients. We present a case of pulmonary arteriovenous fistula in a newborn and review the literature. This rare condition of newborns can be treated with different surgical procedures. Only 17 cases of newborn pulmonary arteriovenous fistula/have been reported, and only two of those had associated Rendu-Osler-Weber syndrome. The results of surgical procedures were good in most of these cases. We treated our case with lobectomy successfully.     

Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. A report of three cases.

Three children had an arteriovenous fistula between an aberrant vessel from the descending aorta and a normal pulmonary vein in the posterior basal segment of a lung. The affected lung was otherwise normally developed. Only a few similar cases could be found in the literature.     

Successful Embolization of Coronary Arteriovenous Fistula Using an Interlocking Detachable Coil

Although the efficacy and feasibility of coil embolization of coronary arteriovenous fistulas have been reported, the procedure may be complicated by migration of the coil into peripheral vessels or pulmonary arteries. We report two cases of successful coil embolization of such lesions using an interlocking detachable coil. This system can provide safer and more effective coil embolization in patients with coronary arteriovenous fistula.     

Postmortem demonstration of intrapulmonary arteriovenous shunting.

The measurement of the pressure required to drive a 2% solution of gelatin across the pulmonary vascular bed of a group of 49 infants dying suddenly at less than 44 weeks'' postnatal age, has shown the presence of low pressure arteriovenous shunting in 30 cases (61%). Injection of polymethylmethacrylate beads in three cases has demonstrated that the vessels that form the shunts are up to 60 microns in diameter. The presence of vessels allowing shunting at pressures as low as 0.1 kPa (1 cm H2O) could severely compromise the gas exchange function of the lung.     

Hereditary hemorrhagic telangiectasia. A case report with known cerebral involvement and cardiac catheterization data.

Numerous bleeding episodes, neurological signs and symptoms, and pulmonary arteriovenous malformations are well documented in reports of families with hereditary hemorrhagic telangiectasia. It is always assumed that these are due to the arteriovenous malformations. We are here reporting a case with positive cerebral angiographic findings of large arteriovenous communications, as well as catheterization data revealing pulmonary abnormalities secondary to the cerebral arteriovenous fistula, but no abnormalities within the pulmonary vasculature itself.     

先天性心脏病复合畸形的介入治疗

目的探讨介入治疗小儿先天性心脏病(CHD)复合畸形的可行性和疗效。方法对CHD复合畸形患儿10例施行联合介入治疗,其中房间隔缺损(ASD)并肺动脉瓣狭窄(PS)2例,室间隔缺损(VSD)并动脉导管未闭(PDA)4例,PDA并PS 2例,ASD并PDA 2例。结果7例1次治疗成功,余3例中2例分2次治疗,1例8个月患儿,因其在最初0.5年随访中发现VSD明显缩小,故先封堵了PDA,目前正在随访中。成功率100%,无严重并发症。经1个月-3年随访,患儿心脏缩小,心脏功能改善。结论在病例选择恰当情况下,对小儿CHD复合畸形进行介入治疗是安全、可行的。     

Interventioneller Verschluss eines persistierenden Ductus arteriosus

Background

A large persistent patent ductus arteriosus (PDA) in a symptomatic premature or newborn infant is to be regarded as an expression of the premature birth, which must per se be primarily treated. The PDA may persist, however, despite optimal neonatological therapy and specific pharmacological intervention, as a hemodynamically relevant connection between the aorta and the pulmonary artery stem. In this case the clinical symptoms depend on the extent of the shunt and pulmonary flooding or pulmonary pressure elevation and other concomitant phenomena may result. When preterm and newborn infants are clinically compromised by a large PDA, surgical closure is so far the only alternative to pharmacological treatment.

Aim

This study investigated whether interventional PDA closure is as effective and safe in preterm infants, full term infants and children weighing Material and methods In a retrospective analysis the experiences of two centers which performed interventional PDA closure in preterm and full-term infants and in children with body weight of Results A total of 68 children weighing ?95?%. Device-associated complications were low at 3?% and there were no mortalities.

Discussion

Interventional PDA closure in children (preterm and full term infants) with a hemodynamically relevant PDA and associated clinical symptoms represents a safe and efficient therapy option after the conservative options have been exhausted. The procedure can be applied safely and successfully in children weighing less than 6 kg and as little as 2 kg.