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.     

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 malformations

Pulmonary arteriovenous malformations rarely present in childhood. Two cases are presented in this report. Both the cases presented clinically with cyanosis and clubbing without a cardiac murmur. The second case had cerebral abscess in addition. Both the cases underwent a contrast-enhanced echocardiography which suggested the presence of pulmonary arteriovenous malformations. The first case also underwent^99mTc radionuclide scan and pulmonary angiography. The cases are being reported for their characteristic clinical features and for emphasizing the role of non-invasive modalities like contrast-enhanced echocardiography and radionuclide scan in reaching the diagnosis.     

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     

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 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.     

Post-biopsy renal arteriovenous fistula

A 6-yr-old boy developed progressively severe hypertension, which was unresponsive to medications, 1 week after percutaneous biopsy of his renal transplant. Renal angiogram revealed an arteriovenous fistula (AVF) in the lower pole at the site of the biopsy. Case findings and resolution after embolization are described, and the current literature is briefly reviewed.     

小儿先天性动静脉瘘的诊治

目的 探讨对小儿先天性动静脉瘘(congenital arteriovenous fistula，CAVF)的诊断与治疗。方法 本组共收治15例CAVF患儿，男6例、女9例，年龄8～14岁。对全部患儿行多普勒超声，动脉造影或MRA检查。发现病变局限者5例，病变范围广泛性者10例，病变大多发生于四肢。动脉造影显示：动静脉之间出现瘘支；动脉分支多而紊乱，可呈碗蜒扭曲状，病变多为弥漫性；静脉象早期出现。对11例患儿行手术治疗，其中4例局限性病变者行病灶切除。对7例病变广泛者行1或多次减量手术，方法是沿动脉主干切除瘘支与病变组织，对分流量大又无法切除的病变组织予以缝扎，以减少分流量。对5例患儿行栓塞治疗(其中2例为术前栓塞治疗以减少手术出血)。1例未治疗。结果 随访1～6年。5例局限性患儿中3例治愈，2例病情控制。10例广泛性病变中，5例病情好转或控制，5例病情仍继续发展。结论 影像学检查是CAVF诊断与治疗的依据。CAVF治疗困难。尤其对广泛性病变者，无论手术或栓塞治疗疗效均欠理想。但对小儿CAVF应尽早治疗，这样可控制病情发展．防止并发症发生。     

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。     

Asymptomatic spinal arteriovenous fistula presenting only as continuous murmur

Spinal arteriovenous fistula is extremely rare in children. Weakness and sensory disturbance in the lower extremities are the specific clinical presentations. Children, however, commonly have no subjective symptoms; in rare cases, a continuous murmur is the only physical finding. An 18‐month‐old boy was referred for evaluation of a continuous murmur audible at the back. He had no motor or sensory disorder; only a Levine 3/6 continuous murmur audible at the back was found. Echocardiography showed a structurally normal heart but indicated ascending continuous blood flow behind the aortic arch and dilatation of the innominate vein. We suspected spinal arteriovenous fistula, and it was visualized on computed tomography angiography. Spinal arteriovenous fistula was detected using only auscultation and echocardiography. Suspicion of this anomaly on careful auscultation and simple examination, and confirmation on detailed examination, even in the absence of motor or sensory disturbance, is important.     

Failure of color flow mapping to identify coronary artery to pulmonary artery fistula

Summary A case of coronary artery to pulmonary artery fistula presented with the typical clinical findings of asymptomatic precordial continuous murmur and small left-to-right shunt. Echocardiography with color flow mapping failed to demonstrate the fistula on three occasions, including one study performed after angiographic demonstration of the fistula site. This case illustrates the importance of angiography in patients with unexplained continuous murmurs, even when echocardiographic and color flow mapping results are normal.     

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.     

儿童肝-门静脉分支瘘血管造影诊断及其临床意义

目的　探讨儿童肝动脉 门静脉瘘的数字减影血管造影 (DSA)诊断及其临床意义。方法　回顾性分析 1997～ 2 0 0 1年 8例肝动脉 门静脉瘘患儿的肝动脉造影DSA图像 ,观察其特征性血管造影表现 ;通过与CT及超声图像比较 ,评价其对临床诊断及治疗的作用。结果　CT及超声诊断不明的 8例患儿经皮股动脉穿刺行肝动脉造影均能清晰显示 :肝动脉增粗 ,动脉期见大量的门静脉分支及主干显影。在DSA下导管顺利插入肝动脉 ,并注入聚乙烯醇颗粒进行扩充术 ,术后造影复查见肝动脉 门静脉瘘消失。结论　经皮肝动脉DSA对肝 门静脉分支瘘的诊断和治疗具有重要价值。     

新生儿先天性肝血管瘤伴动静脉瘘导致心功能不全1例北大核心CSCD

患儿女,3日龄,因生后气促3 d入院。患儿为胎龄36+2周早产儿。产前胎儿超声提示胎儿肝血管发育异常,生后即出现呼吸急促,需要呼吸支持。胸部X线检查提示心影增大,心脏彩超提示右心房和右心室增大,肝脏超声、腹部增强CT明确诊断为肝血管瘤伴动静脉瘘。19日龄时在全身麻醉下行肝动脉结扎术,术后心功能好转出院。基因检测显示ACVRL1基因变异,该变异来自患儿母亲。该文主要介绍1例由肝血管瘤伴肝动静脉瘘导致新生儿心功能不全病例,以及对该疾病的多学科诊疗。     

Mutant fetal hemoglobin causing cyanosis in a newborn

A well but cyanotic newborn was found to have a mutant gamma-globin chain, leading to a functionally abnormal fetal hemoglobin. A single amino acid substitution was found in a site consistent with known adult M hemoglobins. This patient showed no clinical evidence of cyanosis at 5 weeks of age as gamma-chain synthesis was replaced by beta-chain synthesis. A sibling born 20 months later was also cyanotic and the same mutant hemoglobin was found.     

Aneurysmal dilatation associated with arteriovenous fistula in a transplanted kidney after renal biopsies

AVF is a known complication of renal biopsy in both native and transplanted kidneys. A 20‐yr‐old woman with bilateral hypoplastic kidneys due to branchio‐oto‐renal syndrome had received living‐donor renal transplantation from her father at the age of 11. She had undergone allograft renal biopsies six times and all puncture sites were at the lower pole of her kidney from the first to the fifth biopsy. AVF with aneurysmal dilation (30 mm) had developed at the puncture site after the fifth biopsy. TAE was successfully performed with 11 platinum coils in the branch of the renal artery feeding the aneurysm. According to a review of the literature, the incidence of AVF is higher in transplanted kidneys than in native kidneys (7.5% vs. 2.1%) because transplanted kidneys, as single kidneys, are likely to be punctured repeatedly at the same site. When renal biopsy of a transplanted kidney is performed, previous biopsy puncture sites should be considered and the biopsy should be performed at a different site, if possible, to prevent the development of AVF.