A case report of isolated absence of the right pulmonary artery: bronchofiberscopic findings and chest radiologic tracings over 9 years]

A 27-year-old man was admitted to hospital for the investigation of hemosputum. He is a serving member of the Japan Self-Defense Forces. Chest radiography showed absence of the right hilar component, and the ipsilateral hemithorax was smaller than that of on the left side. Pulmonary arteriography demonstrated complete absence of the right pulmonary artery. Right cardiac catheterization did not show any concomitant cardio-vascular malformation. Therefore, this case was diagnosed as an isolated absence of the right pulmonary artery. Bronchial arteriograms demonstrated showed vascular enlargement and proliferation of the right bronchial artery. Bronchofiberscopic examination showed obvious bronchial mucosal vascular enlargement with submucosal eruption and swelling of the right bronchi. We also re-examined the chest radiographs of this patient from the previous 9-years. Those revealed a chronological decrease of the right lung volume and an increase of the cardio-thoracic ratio. Isolated absence of the right pulmonary artery is generally considered to have preferable survival, but close observation is necessary to monitor the pulmonary hemodynamics.     

儿童先天性支气管动脉-肺动脉瘘的漏诊分析

目的:总结先天性支气管动脉-肺动脉瘘(DPS)非咯血患儿的临床诊治经验。方法:回顾性分析2009年7月至2013年11月间,在北京儿童医院行先天性心脏病(先心病)动脉导管未闭介入手术,诊断为DPS 31例患儿的临床资料,总结其临床表现、胸部X线片、大血管造影及随访情况。结果:男性16例,女性15例,年龄7个月~6岁,均为动脉导管介入手术中发现,术后21例仍有反复上呼吸道感染史。胸部x线片检查:8例未见异常,23例示右下肺内带肺纹理粗多。降主动脉造影均提示支气管动脉扩张、走行迂曲,呈扫帚状或网状,支气管动脉管腔内径≥2mm,在支气管动脉显影的同时有肺动脉同步显影,并可见支气管动脉与肺动脉管道状相交通,形成左向右分流。结论:PDA介入术中发现的细小先天性支气管肺动脉瘘,如无症状可随诊观察。     

A case of isolated absence of the right pulmonary artery]

A 40-year-old woman was admitted to our hospital for investigation of an abnormal chest roentgenogram. She had been well with normal exercise tolerance and had no history of pneumonia. The chest roentgenogram showed decreased vascularity of the right lung, ipsilateral small hemithorax, and shift of the mediastinum toward the right. In the right lung field, hazy and confluent micronodular shadows were seen, and the left lung was emphysematous and hyperlucent. Herniation of the left lung into the contralateral thorax was seen. Although lung perfusion scintigrams showed no detectable blood flow to the right lung, a krypton ventilation study revealed diminished but homogeneous ventilation of the ipsilateral lung. A bronchogram showed normal structure of the bronchi. Cardiac catheterization revealed normal pulmonary arterial pressure. A pulmonary angiogram showed no right pulmonary artery, and absence of other cardiovascular anomalies. Therefore, the diagnosis of isolated absence of the right pulmonary artery was confirmed. An aortic angiogram demonstrated collateral vessels originating from one of the bronchial arteries, the infradiaphragmatic artery, and the right coronary artery. Chest CT disclosed cystic changes in the right lung field.     

支气管动脉栓塞治疗肺结核大咯血31例

目的回顾性分析支气管动脉栓塞治疗肺结核合并大咯血。方法对31例肺结核大咯血在影像监视下行选择性支气管动脉造影后用明胶海绵颗粒进行栓塞。结果31例中右侧支气管动脉出血15例,左侧12例,还有4例为两侧支气管动脉共干。结论对肺结核合并大咯血内科保守治疗效果不佳又无条件进行外科手术者,应尽早行支气管动脉造影并栓塞。     

Idiopathic Dilatation of the Pulmonary Artery–A Case Report

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare anomaly presenting with enlargement of the pulmonary artery in the absence of an identifiable cause. The natural history of this silent but potentially life‐threatening disease can be unpredictable. We report a case of IDPA in an 80‐year‐old female with a massively dilated pulmonary artery.     

小儿法乐四联症合并一侧肺动脉缺如的外科治疗

本文报告从１９９４年１月至１９９５年３月收治的１３６例法乐四联症（ＴＯＦ）中３例合并左肺动脉缺如（ａｂｓｅｎｃｅｏｆＬＰＡ），均行一期根治术，无一例死亡。男１例，女２例年龄４～１１岁。１例离断主肺动脉，近端缝闭，远端和右室切口间用同种异体主动脉带瓣管道（ＡＯ－ＶＨＣ）连接，另２例用自身心包片作右室流出道扩大补片。术后并发症主要是肺动脉高压、低氧血症，术后加强右心功能支持尤为重要。     

Hemoptysis following Mustard repair: a late complication

Hemoptysis was the presenting symptom in a 4-year, 11-month-old male who had had a Mustard operation for hemodynamic correction of transposition of the great vessels at the age of five months. Chest roentgenography demonstrated hyperlucency of the left lung and tomography showed compression and narrowing of left main stem bronchus. Angiography documented the absence of antegrade flow in the left pulmonary artery and no pulmonary venous drainage on the left. The left lung was supplied by bronchial collateral arteries which drained by retrograde filling of the left pulmonary artery. It is surmised that pulmonary venous drainage on the left was compromised at surgery and that the dilated main pulmonary artery compressed the left main stem bronchus. This combination promoted bronchial collateral ingrowth. Hemoptysis is a complication of enlargement of bronchial collateral vessels.     

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

Type II congenital pulmonary airway malformation in an esophageal lung

A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.     

CT肺动脉及支气管动脉联合造影对咯血患者的诊断价值

目的 探讨建立CT肺动脉(PA)及支气管动脉(BA)联合造影的方法及其对咯血患者的诊断价值.方法 连续纳入北京朝阳医院2007年6月至2009年7月36例中等量及以上咯血住院患者,其中男21例,女5例,年龄27 ～ 83岁,平均(56±14)岁.使用16排螺旋CT,静脉注射对比剂后,首先完成CT肺动脉造影(CTPA),延迟时间12～16 s,继之完成CT支气管动脉造影(CTBA),延迟时间26～28 s,经图像重建后对PA及BA进行评价.结果 36例患者CTPA检查发现7例PA异常(肺栓塞3例,PA畸形、原发性PA平滑肌肉瘤、PA边缘模糊及右下PA闭塞各1例).36例患者CTBA检查共显示37支右侧BA,其中11支迂曲增粗、40支左侧BA及3支非支气管体循环动脉,其中来源于腹主动脉的1支迂曲增粗.异常血管与肺部疾病关系密切.结论 利用16排螺旋CT技术,可进一步完成CTPA及CTBA检查,清楚显示异常的PA及BA,对咯血的诊断及治疗具有重要价值.     

Coexistence of Primary Sarcomatoid Carcinoma of the Right Ventricle and Absence of Right Pulmonary Artery

We describe the imaging features of a 48‐year‐old woman with primary sarcomatoid carcinoma originating from right ventricular outflow tract (RVOT) and isolated absence of right pulmonary artery (RPA). Computed tomography pulmonary angiography (CTPA) demonstrated the absence of RPA. Both transthoracic echocardiography (TTE) and CTPA revealed multiple masses filling and obstructing the RVOT. A palliative operation was performed on the patient, and the postoperative histopathology and immunohistochemical examination confirmed primary sarcomatoid carcinoma originating from the RVOT. The operation also confirmed the absence of RPA.     

A case of pulmonary veno-occlusive disease associated with systemic sclerosis

The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. Echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.     

肺动脉栓塞手术的麻醉处理

目的 :探讨肺动脉栓塞急诊手术的麻醉方法。方法 :对 5例巨大肺动脉栓塞急诊手术患者术前状态 ,手术方式 ,麻醉处理等情况进行分析 ;研究可行的麻醉方法。结果 :术后早期死亡 3例(6 0 % ) ,其中 2例死于不可控制的肺出血 ,1例死于肺动脉栓塞复发而猝死 ;治愈 2例。结论 :麻醉诱导需谨慎用药 ;必须施行双腔气管插管 ;在正性肌力药物支持下静滴扩血管药 ,改善右心衰竭 ;积极防治心律失常和支气管痉挛有益于术中心肺功能的维护。     

Unilateral absence of the pulmonary artery incidentally found after an episode of hemoptysis

A 29-year-old woman was admitted to our hospital because of hemoptysis and dyspnea. A chest radiographic study revealed an enlargement of the right lung hilum, and the left pulmonary artery could not be clearly visualized on contrast-enhanced CT scan. Both perfusion scintigraphy and pulmonary angiography confirmed the absence of the left pulmonary artery, and agenesis of the right upper pulmonary artery. Her clinical status has been stable for the previous two years of follow-up, even though she required long-term oxygen therapy. This is a rare case of a young woman with the unilateral absence of the left pulmonary artery incidentally found after an episode of hemoptysis.     

左冠状动脉异常起源于肺动脉的超声心动图诊断价值

目的:探讨左冠状动脉异常起源于肺动脉(ALCAPA)的超声心动图诊断价值。方法:以CTA和手术证实的16例ALCAPA患儿为研究对象,回顾分析其超声心动图表现特征。结果:16例中,超声首诊11例,误诊5例,5例均误诊为心内膜弹力纤维增生症(EFE),复诊检出3例,余2例最后经CTA检出,超声首诊正确率69%。其主要超声表现为:左冠状动脉主干异常开口于肺动脉;右冠状动脉扩张;左心室扩大;心内膜、二尖瓣腱索及乳头肌及回声增强。彩色多普勒显示左冠状动脉内血流为逆向灌注,肺动脉内舒张期见异常血流进入;室间隔可见异常交通循环血流信号。结论:ALCAPA具有特征性超声心动图表现,超声是早期诊断的重要手段。     

Mitral valve prolapse causing severe mitral regurgitation in a patient with absent right pulmonary artery

A 33-year-old man was investigated for dyspnea on exertion and the presence of a pansystolic murmur. Physical examination revealed dextrocardia confirmed by chest radiograph, which also showed oligemic right lung field. Subsequent cardiac catheterization revealed secundum atrial septal defect, persistent left sided superior vena cava, and severe mitral valve prolapse causing severe mitral regurgitation with pulmonary hypertension. The right pulmonary artery was absent. It is the first report of the association between severe mitral valve prolapse and absent right pulmonary artery.     

红细胞分布宽度与简化肺栓塞严重指数关系的临床研究

目的通过探讨红细胞分布宽度(RDW)与简化肺栓塞严重指数(s PESI)的关系来预测急性肺栓塞危险程度。方法将90例急性肺栓塞患者分为s PESI低危组和高危组,比较两组患者在基本资料、血常规、凝血功能、肝肾功能、胸部影像学及心脏彩超等方面的差异。结果 s PESI高危组与低危组比较,在心率、收缩压、凝血酶原时间、国际标准化比值、中性粒细胞、RDW、累及主肺动脉及右心室增大等方面,差异有统计学意义(P0.05);logistic单因素回归分析发现收缩压、INR、RDW、累及主肺动脉、右心室增大与s PESI分组有关(P0.05);logistic多因素回归分析发现累及主肺动脉是影响s PESI分组的独立危险因素(P0.05)。结论 RDW在s PESI高危组与低危组差异有显著性,但不是预测s PESI分组的独立危险因素,对急性肺栓塞危险程度的判断有一定价值。     

A case of idiopathic bronchial artery aneurysm connecting to the pulmonary artery]

Case. A 54-year old woman was found to have an abnormal shadow in a regular checkup chest X-ray film. We suspected a bronchial artery aneurysm based on a contrast-enhanced chest CT. Aortography, bronchial arteriography and pulmonary arteriography showed a bronchial artery aneurysm, 2 cm in diameter, connecting the right bronchial artery, inferior phrenic artery, and pulmonary artery. Bronchoscopy revealed dilatation and hypervascularity of moniliform submucosal vessels below the right truncus intermedius. She underwent bronchial artery embolization several times, but new feeding vessels developed each time. We considered this case required surgical resection. Bronchoscopy after aneurysmectomy revealed decrease of moniliform submucosal vessels. Histological examination revealed a three-layer structure leading to both the pulmonary and bronchial arteries. Conclusion. We diagnosed idiopathic bronchial artery aneurysm connecting to a pulmonary artery.     

A case of racemose hemangioma of a bronchial artery complicated with atrial septal defect and localized peripheral ground-glass opacity]

We report a case of racemose hemangioma of the right bronchial artery complicated with an atrial septal defect (ASD) and ground-glass opacity localized in the right upper lung lobe. A 42-year-old woman was admitted for the pre-operative assessment of ASD. Aortography and selective angiography revealed that the right bronchial artery was markedly dilated and convoluted, draining into the right intercostal arteries and the right upper pulmonary vein. The patient was suffering from exertional dyspnea, but produced no bloody sputum. Since videobronchoscopy showed normal mucosal findings without bronchiectasis, primary racemose hemangioma of the right bronchial artery was diagnosed, and was treated by ligation of the bronchial artery and patch-closure of the ASD. The ground-glass opacity observed preoperatively in the periphery of the right upper lung lobe vanished after surgery. This opacity was considered to have been localized pulmonary congestion due to augmented hydrostatic pressure in the right upper pulmonary vein, induced by increased pulmonary blood flow through the ASD and the drainage route of the racemose bronchial artery into right upper pulmonary vein.     

Pulmonary artery occlusion from tuberculous lymphadenopathy in a child

Occlusion of the pulmonary artery is a rare complication of mediastinal tuberculosis. We report on a 10-year-old girl who presented with a tuberculous pericardial effusion in whom subsequent imaging showed a totally occluded right pulmonary artery from tuberculous lymphadenopathy. Diagnosis was confirmed by polymerase chain reaction from a lymph node biopsy. Failure of medical therapy necessitated surgical reconstruction of her right pulmonary artery. Postoperatively she has normal perfusion of the right lung and normal lung function.