Pulmonary artery pseudoaneurysm after percutaneous transluminal angioplasty in a pediatric patient

We report a case of a 4-year-old boy with tetralogy of Fallot, pulmonary atresia, and hypoplastic pulmonary arteries who presented with mild hemoptysis and upper respiratory infection 3 weeks following percutaneous transluminal angioplasty for pulmonary artery stenosis. While pneumonia was initially suggested on early plain radiographs of the chest, a large pseudoaneurysm of a right lower lobe pulmonary artery branch was subsequently diagnosed with CT and angiography. Received: 12 August 1996 Accepted: 24 March 1997     

Takayasu��s Arteritis Mimicking Unilateral Pulmonary Artery Agenesis in a Child With Severe Pulmonary Hypertension and Right Heart Failure: A Diagnostic Dilemma

Affliction of the pulmonary arteries in Takayasu’s arteritis is uncommon. Moreover the incidence of pulmonary artery involvement in this condition is often underestimated because of asymptomatic nature in most patients. Severe involvement may however present with pulmonary artery hypertension and hemoptysis, which may prove to be fatal. This case report describes a 9-year-old girl with severe pulmonary hypertension and right heart failure secondary to total occlusion of the right pulmonary artery. Detailed clinical examination and computed tomography (CT) angiography confirmed this diagnosis.     

Right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus

Pulmonary aplasia and bronchopulmonary foregut malformations in which a patent communication between the foregut and the pulmonary system is present are rare congenital abnormalities. Pulmonary aplasia associated with a pulmonary sling is an even rarer abnormality. We report a unique case of right pulmonary aplasia, aberrant left pulmonary artery, and bronchopulmonary sequestration with an esophageal bronchus diagnosed by multidetector helical CT.     

Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum

We report the case of a newborn with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation. He died several weeks after a Blalock-Taussig procedure because of a progressive stenosis of the main coronary artery. We present echocardiographic and dual-source CT images of the stenosis, with autopsy correlation. To our knowledge, CT images of this quality have never been reported in a newborn. This case illustrates the extreme difficulty in prognosticating the outcome for these patients and underlines the need for a detailed neonatal coronary mapping to assess right ventricle-dependent coronary circulation.     

Persistent unilateral white‐out in preterm infant

We would like to present a case of persistent right sided collapse in a preterm baby with an unexpected diagnosis. Baby X was born floppy requiring resuscitation and was intubated, ventilated and was transferred to NICU. There was decreased air entry on auscultation of right side of the chest, while the rest of the examination was normal. The chest X‐ray showed right side white‐out suggestive of collapse consolidation. She required significantly high ventilatory pressures to maintain saturations and chest X‐ray performed on day 4 remained unchanged with persistent right sided white‐out. The CT scan of the chest showed no definable right lung, right pulmonary artery or right main bronchus. The diagnosis of right lung agenesis with single pulmonary artery was made and baby was found to have more congenital malformations on further investigations, and later developed complications leading to palliative care.     

儿童先天性体动脉-肺动脉瘘4例并文献复习

目的总结临床表现为大量咯血的先天性体动脉-肺动脉瘘患儿的临床特点及诊治经 验。方法回顾性收集首都医科大学附属北京儿童医院2007年3月至2008年2月诊断为先 天性体动脉-肺动脉瘘4例患儿的临床资料,总结其临床表现、胸部X线片、胸部64 排CT增强扫描三维容积再现(3D-VR)、数字减影血管造影（DSA）、治疗及随访情 况。结果男1例,女3例,最大发病年龄为11岁,最小发病年龄为2个月,主要表现为咯 血。1例有杵状指,3例未见特异性体征。胸部X线片检查：3例未见异常,1例示肺 间实质浸润。胸部64排CT增强扫描3D-VR检查：1例提示支气管动脉迂曲,但未见 异常交通;1例导管栓塞治疗（TCE）后示右侧支气管动脉扩张迂曲,并与肺动脉 相通可能性大,考虑支气管动脉-肺动脉瘘;1例提示支气管动脉-肺动脉瘘;1例 未见异常。3例DSA提示为支气管动脉-肺动脉瘘,病变部位均位于右下肺,并行 TCE。随访至2009年2～5月,1例复发,表现为痰中带血,胸部64排CT增强扫描未 见异常,余3例未复发。3例怀疑为遗传性出血性毛细血管扩张症(HHT),1例考虑 为HHT高度危险者。结论体动脉-肺动脉瘘可造成大量咯血。胸部X线片一般无特异性表现,胸部64排 CT增强扫描3D-VR可显示病变部位,明确诊断需行DSA。可采用TCE治疗。TCE远期 效果应进行长期随访予以明确。     

先天性血管环12例诊断与治疗分析

摘要目的探讨先天性血管环的临床特征、诊断与治疗。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诊断为先天性血管环患儿的临床特征、影像学检查、诊断和治疗。结果12例先天性血管环患儿进入分析,男3例,女9例。就诊年龄1个月至21岁,<1岁7例。反复咳喘11例,喂养困难2例,青紫3例,心脏杂音10例。12例超声心动图示主动脉双弓4例、右位主动脉弓伴左位动脉导管未闭1例、肺动脉吊带7例,其中10例伴其他心脏畸形。10例64排CT气道重建示主气管狭窄5例、左主支气管狭窄1例,右侧气管狭窄3例,食管狭窄2例;2例纤维支气管镜示主气管狭窄1例,右主支气管狭窄1例。7例行手术治疗,根据血管环的病理类型选择术式,包括2例切断双主动脉弓中的次弓、1例切断右位主动脉弓伴随的左位动脉导管、4例左肺动脉移植术。1例术后死亡,6例术后3～6个月呼吸道症状逐渐好转,生长发育改善。结论儿童中反复出现原因不明的咳喘等呼吸道症状者,应考虑到先天性血管环的可能,超声心动图联合多排CT可明确诊断,及早手术解除气管狭窄是改善预后的关键。     

Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience

Our aim was to evaluate the feasibility of using computed tomography (CT) to define the pulmonary artery anatomy in patients with tetralogy of Fallot and pulmonary atresia (TOF-PA). We retrospectively reviewed 110 patients with TOF-PA between 1995 and 2008. Those who received cardiac catheterization and surgery within 3 months of their CT examinations were enrolled. Based on Dr. Somerville's classification, the pulmonary arterial pattern was determined, including identifiable pulmonary trunk (type I), the presence of both left and right pulmonary arteries without trunk (II), only left or right pulmonary artery present (III), and absent intrapericardial pulmonary arteries (IV). The accuracy of both imaging modalities was evaluated with operation findings as the golden standard. The effective radiation doses and adverse events were also recorded. In the 64 eligible patients (median age, 23 months), CT and catheterization demonstrated accurate pulmonary arterial morphology in 60 (60/64) and 53 (53/64) TOF-PA patients, respectively. Thirty-two of 35 type I patients were correctly identified by CT, whereas 26 were correctly identified by catheterization (p?=?0.03). Of the 20 type II TOF-PA patients, 19 were diagnosed by CT, whereas 18 were diagnosed by catheterization. CT and catheterization both successfully defined six type III and three type IV patients. The median calculated radiation doses caused by CT and catheterization were 4.5 and 5.6 mSv, respectively (p?>?0.05). CONCLUSIONS: For patients with TOF-PA, CT could accurately delineate pulmonary arterial morphology with the same level of accuracy as cardiac catheterization. Therefore, CT can be considered a reasonable diagnostic alternative for such patients.     

The use of ultrasound in the diagnosis of a large thymic cyst

We present a case of pulmonary sling associated with tracheobronchial stenosis, and with hypoplasia of the right lung and right pulmonary artery. Radiologic studies showed evidence of pulmonary sling and hypoplasia of the right lung; associated hypoplasia of the right pulmonary artery was also present, but not recognized initially. Narrowing of the distal trachea and right main bronchus was present, not due to compression by the anomalous left pulmonary artery, but to congenital stenosis of the trachea and right main bronchus associated with complete cartilaginous tracheobronchial rings.     

儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.     

Berry Syndrome: A Possible Genetic Link

Berry syndrome comprises a rare combination of heart defects that includes aortopulmonary window, interrupted aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery. We report the case of a neonate confirmed to have Berry syndrome by transthoracic echocardiogram and computed tomography (CT). This neonate had the additional finding of an aberrant right subclavian artery arising from the descending aorta. A single-stage repair was successfully performed when the infant was 7 days of age. Genetic testing showed a 102-kb deletion within chromosome band 9p24.2; this deletion has not been previously linked to congenital heart defects. Berry syndrome can be diagnosed accurately by transthoracic echocardiogram and CT. There may be an underlying genetic etiology, and this possibility warrants further investigation.     

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced,reversible, left-sided pulmonary hypertension

Summary An infant with scimitar syndrome, absent right pulmonary artery, and systemic blood supply to the right lung presented in severe cardiac failure. Cardiac catheterization revealed suprasystemic pressure of the left pulmonary artery and a high pulmonary vascular resistance. Right-sided pneumonectomy abolished cardiac failure and normalized both pulmonary artery pressure and resistance. Pure volume load affecting one lung—as in this case through absence of the right pulmonary artery plus additional left-to-right shunt from a systemic collateral—can lead to pulmonary hypertension. Early operative intervention can reverse this process and prevent pulmonary vascular disease.     

Lung cysts following pulmonary artery operations: Diagnostic and therapeutic implications

We describe the case of a child suffering from congenital cyanotic heart disease — double outlet right ventricle (DORV) with transposition of great vessels (TOGV). She underwent a left Blalock-Tausig (BT) shunt at one month of age followed by a Glen procedure with left pulmonary artery augmentation at six months. Following the second procedure she developed extensive cyst formation in the upper lobe of the left lung and pneumothorax. She was managed by intercostal drainage of the pneumothorax. The cysts were observed and on a CT scan X-rays taken at one month and six months no cysts were seen. This case illustrates the occurrence of pneumatoceles after pulmonary artery manipulation, their proclivity for causing pneumothoraces and involution on follow-up. Cysts noted in such a setting should be monitored carefully and followed up to resolution.     

Isolated Unilateral Agenesis of the Pulmonary Artery: Surgical Repair With an Artificial Graft

This report describes a 3-month-old Japanese boy with a diagnosis of isolated unilateral agenesis of the proximal right pulmonary artery with severe pulmonary hypertension. One-stage reconstruction of the right pulmonary artery was performed without cardiopulmonary bypass. The hilar right pulmonary artery and the distal main pulmonary artery were joined by anastomosis to an artificial ring graft. The boy's postoperative course was uneventful, and the pressure in the pulmonary artery was within the normal range. Although mild right pulmonary artery stenosis remained, the authors' therapeutic strategy may provide a clinically important option for isolated unilateral agenesis of the pulmonary artery.     

Scimitar syndrome with absence of the right pulmonary artery: a case report

We report a two-year-old female child with scimitar syndrome associated with absent pulmonary artery but with normal pulmonary artery pressure although several collaterals originated from the abdominal aorta to the right lung. To our knowledge, this is the fifth case with an absent pulmonary artery. Our case also had microphthalmia, an association not previously described in scimitar syndrome.     

Coarctation of the Left Pulmonary Artery: Effects on the Pulmonary Vasculature of Infants

At autopsy, two infants had unsuspected coarctation of the left pulmonary artery (CoLPA), which was produced by an extension of ductal tissue into the wall of the left pulmonary artery. The first case, a 4-month-old girl, also had a ventricular septal defect and an anomalous branching pattern of the innominate arterial trunk. Pulmonary arterial hypertensive changes were noted in the right lung. In contrast, the left lung showed thin-walled pulmonary arteries. The second case, a term female newborn, had exhibited severe unexplained respiratory distress since birth. Histologic sections of the right lung showed dilated pulmonary arteries with thinned media, whereas the left lung showed a persistent fetal arterial pattern. It is believed that the peripheral pulmonary arterial changes are age-dependent and associated with asymmetric blood flow between the right and left pulmonary arteries. CoLPA is a rare pulmonary artery defect, and early diagnosis of this abnormality is important.     

Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

目的 探讨单侧肺动脉异常起源于升主动脉（AOPA）的产前超声心动图特征，提高对本病产前诊断的准确率。方法 回顾性分析2012年6月1日至2018年10月30日经首都医科大学附属北京安贞医院（我院）产前诊断的6例AOPA胎儿的超声心动图特点，总结该病胎儿期的超声心动图特征。结果 5例胎儿为右肺动脉异常起源于升主动脉（AORPA），其中3例为孤立型AORPA，2例合并Berry综合征；1例为左肺动脉异常起源于升主动脉（AOLPA），同时合并先天性肺动脉瓣缺如综合征。6例胎儿均为近端型AOPA，其中2例胎儿引产（1例AOLPA，1例合并Berry综合征）。出生的4例患儿中，1例Berry综合征患儿于出生后63 d因反复肺炎、心衰死亡；2例行AORPA根治手术，术后均恢复良好；1例刚出生，仍在随访中。近端型AOPA胎儿超声心动图特征：①多切面显示正常的肺动脉主干分叉处“八”字型结构消失,正常的肺动脉分支内径增宽；②在三血管气管切面(3VT)及左室长轴切面二维及彩色多普勒显像（CDFI）可显示升主动脉发出一粗大血管向肺内走行，该血管发出位置靠近主动脉瓣左后或右后侧壁的升主动脉；③ CDFI显示异常肺动脉分支血流孤立，与主肺动脉缺乏确切连接；④频谱多普勒显示向肺内走行的血管为肺动脉血流频谱；⑤不合并其他心内畸形时，心内结构正常。结论 超声心动图可对胎儿期的近端型AOPA做出准确的诊断，3VT及左室长轴切面对诊断近端型AOPA至关重要。     

Aberrant left pulmonary artery and tracheal abnormality

A case of left pulmonary artery sling and tracheobronchial tree malformation is reported in a 3 month-old infant. Acute respiratory presenting symptoms required mechanical ventilatory support. The malformation was suspected because of ventilatory support difficulties. Barium oesophagogram showed an anterior indentation, fiberoptic bronchoscopy and tracheobronchography showed a severe tracheal stenosis from a right bronchus lobaris superior to the carina. This was worsened by the right side compression of trachea end part, due to the abnormal left pulmonary artery as demonstrated by pulmonary angiography. A surgical left artery transposition relieved lateral compression and allowed weaning of ventilatory support. After a 27 month-follow-up, the girl's respiratory status is satisfactory.     

Intrapericardial Bronchogenic Cyst Overriding the Pulmonary Artery

We describe a case of a 3-year-old child with an intrapericardial bronchogenic cyst arising from the pericardium overlying the pulmonary artery found incidentally at the time of elective repair of a sinus venosus atrial septal defect with partial anomalous pulmonary venous return. A cystic mass overriding the right pulmonary artery was resected and diagnostic studies revealed multiple loculated cysts lined by respiratory mucosa with cartilage and smooth muscle in the wall consistent with a bronchogenic cyst. The cyst was completely resected and postoperative recovery was uneventful.