Congenital unilateral pulmonary venous atresia with pulmonary veno-occlusive disease in contralateral lung: An unusual association

Summary A patient presenting with the rare association of congenital unilateral pulmonary venous atresia in one lung and pulmonary veno-occlusive disease in the other is described. The patient first presented at the age of 3 &frac; years with anemia, hemoptysis, and pulmonary hypertension. After cardiac catheterization and angiocardiography, a diagnosis of atresia of the left pulmonary veins was made for which left pneumonectomy was done. Four years later the patient presented with right-sided congestive failure and radiologic evidence of right-sided pulmonary edema from which death resulted. At autopsy, the right lung showed changes of pulmonary veno-occlusive disease, while the major veins were not involved.     

A useful projection for demonstrating the bifurcation of the pulmonary artery

Summary The demonstration of the pulmonary bifurcation is important in order to exclude pulmonary branch stenoses. The origin of the right and left pulmonary arteries can be demonstrated in the anteroposterior plane if cranial angulation is used. Depending on the course of the left pulmonary artery, the origin of the left pulmonary artery may not be seen in spite of the maximal cranial angulation. On the lateral plane without tube angulation the origin left pulmonary artery is commonly superimposed on the origin of the right pulmonary artery. If maximum caudal angulation is added to the steep left anterior oblique view, not only the left, but also the origin of the right pulmonary artery can be seen.     

A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis

Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.     

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.     

肺动脉去神经治疗肺动脉高压研究进展

肺动脉去神经治疗(pulmonary artery denervation,PADN)可阻断局部肺动脉交感神经,降低肺动脉高压(pulmonary arterial hypertension,PAH)血流动力学参数,改善肺血管重塑及右心室心肌肥厚、纤维化,进而改善心室功能。前期基础实验已确定肺动脉交感神经在肺动脉内膜位置,并证实PADN对PAH动物模型安全有效,可能通过抑制交感神经系统和肾素-血管紧张素-醛固酮系统发挥作用。PADN已开展成人临床应用研究,取得良好临床效果。在此基础上正初步探索PADN在儿童PAH中应用的可能性。     

右侧剖胸与正中剖胸对小儿心脏手术后肺功能影响的比较

目的 对比研究右侧剖胸与正中剖胸对小儿心脏手术后肺功能的影响。方法随即选取40例行房、室缺修补手术的先天性心脏病患儿，随机分成正中剖胸组（n=20）和右侧剖胸组（n=20），分别在手术前1d、术后第1d、第7d、第14d以及出院后30d检测肺活量（VC）、用力肺活量（FVC）、第1s用力呼气量（FEVt）。结果术后第1d至术后第14d，两组肺功能明显下降，与术前相比差异有显著性（P〈0．05）。两组间比较，术后第1d至术后第14d，右侧剖胸组与正中剖胸组相比肺功能下降明显，差异有显著性意义意义（P〈0．05）。术后30d，两组肺功能恢复至术前水平，与术前相比差异无显著性意义。结论术后早期肺功能损伤右侧剖胸较正中剖胸明显，术后30d，肺功能均恢复正常。     

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.     

6月龄以下小婴儿危重型肺动脉瓣狭窄经皮球囊肺动脉瓣扩张成形术治疗的随访报告

目的 探讨经皮球囊肺动脉瓣扩张成形术(percutaneous balloon pulmonary valvuloplasty,PBPV)治疗新生儿和6月龄以下小婴儿危重型肺动脉瓣狭窄(critical pulmonary stenosis,CPS)的疗效以及早中期随访.方法 采用PBPV治疗34例日龄波动于13～175 d的新生儿以及小婴儿CPS,并进行了6个月～4年的临床随访.记录患儿PBPV前后右室收缩压(right ventricular systolic pressure,RVSP),肺动脉瓣跨瓣压差(transvalvular peak to peak systolic gradient,△P)以及PBPV术后采用心脏彩色多普勒动态监测的肺动脉瓣跨瓣压差的动态变化.结果 34例中32例(94%)完成PBPV术,1例术后2 h内死亡,2例心包积血,3例术中小球囊预扩张后换用合适球囊完成PBPV术,3例右室流出道痉挛,1例术后30 min右下肢股动脉搏动减弱.28例PBPV成功,RVSP由(96±28)mm Hg(1 mm Hg=0.133 kPa)下降至(49±20)mm Hg(下降49%,P＜0.01),△P由(89±25)mm Hg下降到(25±12)mm Hg(下降72%,P＜0.01),右室/主动脉收缩比值1.2±0.5下降到0.7±0.3(P＜0.01).随访6个月～4年,3例失访,5例(其中3例新生儿)因残余压超过40 mm Hg行第二次PBPV术,23例彩色多普勒心脏超声监测肺动脉瓣跨瓣压有进一步下降到(20±13)mm Hg,无明显的肺动脉瓣反流,无需进一步处理.结论 对于6个月以下小婴儿CPS,PBPV仍有效,安全的治疗方法并具有相对好的短中期疗效.

Abstract：

Objective To evaluate the effect and results of short and medium periods of follow-up of percutaneous balloon pulmonary valvuloplasty for critical pulmonary stenosis of neonates and infants under 6 months of age. Methods Between January 2002 and December 2008, 34 consecutive patients aged from 13to 175 days with critical pulmonary valvular stenosis underwent percutaneous balloon valvuloplasty. Patients records, catheterization data, angiograms and echocardiograms were reviewed. Patients were followed up for 6 months to 4 years ( mean 25.5 months ) by means of clinical examination and Doppler echocardiography.Results The pulmonary valvuloplasty was accomplished in 32 (94%) of 34 attempts. Immediately after dilation, right ventricular systolic pressure (RVSP) decreased from (96 ±28) mm Hg ( 1 mm Hg =0. 133kPa ) (49 ± 20 ) mm Hg ( P ＜ 0. 01 ), the transvalvular peak to peak systolic gradient (△P) decreased from (89±25) mm Hg to (25 ± 12) mm Hg (P ＜0.01 ), and the right ventricular/aortic systolic pressure ratio decreased from 1.2 ± 0. 5 to 0. 7 ± 0. 3 ( P ＜ 0. 01 ). One patient died because of cardiac tamponade following rupture of the pulmonary valve annulus, 2 patients developed pericardial effusion, 3 patients had infundibular spasm, 3 patients had a pre-dilation by small balloon and 1 patient had weakened femoral artery pollex. After a follow up period of 6 months to 4 years 3 of 31 patients lost to follow-up. Repeat valvuloplasty was performed in 5 patients (3 neonates), no patient required surgery, and the other 23 patients did not undergo further intervention, a mean peak systolic Doppler gradient of (20 ± 13) mm Hg was found and no significant pulmonary regurgitation was seen. Conclusions Percutaneous balloon pulmonary valvuloplasty was effective and safe for the treatment of critical pulmonary stenosis of neonates and infants under 6 months of age with good short and medium term results.     

Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum

Summary Fifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 65 months (range, 12–87 months).Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts.Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.     

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.     

Primary pulmonary artery sarcoma in two children

Summary The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.     

肾上腺髓质素缓解低氧性肺动脉高压大鼠肺动脉中胶原堆积的研究

目的：观察长期应用肾上腺髓质素（adrenomedullin, ADM）对慢性低氧性肺动脉高压大鼠肺血管胶原代谢的影响,以探讨ADM对慢性低氧性肺血管结构重建的作用及其可能机制。方法：19只雄性Wistar大鼠随机分为对照组（n=6）、低氧组（n=7）、低氧+ADM组（n=6）。对于低氧+ADM组大鼠,通过微量渗透泵皮下持续给予ADM（300 ng/h）2周。低氧2周后,以右心导管法测定肺动脉平均压,检测右心室与左心室加室间隔比值,观测肺血管显微变化。用免疫组化法检测肺动脉中胶原I、胶原III和转化生长因子（TGF）-β的表达。结果：低氧2周后,大鼠肺动脉平均压明显升高(P<0.01),右心室与左心室加室间隔的比值明显增加(P<0.01),肺小血管肌化程度和肺动脉相对中膜厚度较对照组均明显增高（P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达增强。与低氧组大鼠相比,低氧+ADM组大鼠肺动脉平均压明显降低（P<0.01）,右心室与左心室加室间隔的比值明显下降（P<0.01）,肺小血管肌化程度和肺动脉相对中膜厚度较低氧组均明显降低（均P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达减弱。结论：外源性补充ADM可能通过抑制肺动脉TGF-β表达,减少肺动脉壁胶原异常堆积,对低氧性肺动脉高压和肺血管结构重建发挥调节作用。     

缺氧性肺动脉高压新生大鼠肺血管重塑的研究

目的：通过建立新生大鼠缺氧性肺动脉高压（hypoxia-induced pulmonary hypertension,HPH）模型,探讨HPH新生大鼠发病过程中肺血管重塑的变化。方法：将96只新生Wistar大鼠随机分为2组：缺氧组和常氧对照组,缺氧组建立新生大鼠HPH模型。分别于缺氧3、5、7、10、14、21 d 取缺氧组及对照组同日龄新生大鼠各8只测定其平均肺动脉压（mean pulmonary arteria pressure,mPAP）、右心室肥大指数（right ventricle hypertrophy index,RVHI）,血管重塑指标MT%、MA%,观察肺血管超微结构。结果：缺氧组大鼠在缺氧3、5、7、10、14、21 d mPAP持续上升,明显高于对照组（P<0.05）;随着缺氧时间的延长,差异更为显著。缺氧7 d后MT%、MA%、RVHI明显高于对照组（P<0.05）;随着缺氧时间的延长,差异同样更为显著。透射电镜显示,缺氧7 d后大鼠肺小动脉内膜增厚,内皮细胞增生、变性,细胞器增多;细胞外基质见胶原纤维沉积;出现肺血管重塑改变。结论：新生大鼠缺氧3～5 d肺动脉压力增高,处于肺血管痉挛阶段;缺氧7 d后肺血管出现重塑,右心室肥厚,出现不可逆变化;随着缺氧时间延长,上述变化加剧。     

Atrial natriuretic peptide concentrations in children with pulmonary hypertension: Correlation with hemodynamic measurements

Summary To define the relation between atrial natriuretic peptide (ANP) and hemodynamic parameters in children with pulmonary artery hypertension, we measured the ANP concentrations of the femoral vein, right atrium, pulmonary artery, left atrium and left ventricle, or femoral artery in 32 patients during right or left heart catheterization. There is a strong correlation among the ANP levels obtained from different locations (p<0.001), and these ANP levels are positively correlated with pulmonary arterial pressures and pulmonary resistance. Patients were divided into two groups according to their pulmonary arterial pressure. The group with pulmonary arterial systolic pressure higher than 31 mmHg had higher ANP levels than the group with pulmonary arterial systolic pressure lower than 31 mmHg. When patients were grouped according to their pulmonary resistance, ANP levels in the group with pulmonary resistance over 2 U/m² were higher than those in the group with pulmonary resistance lower than 2 U/m². In this study ANP levels showed a correlation with the right ventricular systolic pressure. A correlation was not seen between ANP levels and the flow ratios or the presence of shunt. The results of our study suggest that ANP should be considered an important factor in pulmonary hypertension, independent of other factors.     

Nitric oxide in preterm infant with pulmonary hypoplasia

Premature infants with hypoplastic lungs may have elevated pulmonary vascular resistance with right to left shunt across ductus arteriosus and/or foramen ovale. Inhaled nitric oxide (NO) being selective pulmonary vasodilator without significant effects on systemic circulation can potentially reverse this shunt. The authors herewith report a case of a premature infant with severe hypoxemic respiratory failure after preterm premature prolonged rupture of membranes leading to oligohydramnios and pulmonary hypoplasia that was treated successfully with NO and describe the neurodevelopmental outcome at 1 year of age.     

Partial abnormal pulmonary venous return in Turner syndrome

Three cases of partial anomalous pulmonary venous return, in one case combined with coarctation of the aorta and in another with discrete subaortic stenosis, are described in patients with Turner syndrome. In two of them the right and left superior pulmonary veins drained into the right superior vena cava and left innominate vein respectively. Remarkably, in all three cases the atrial septum was intact. We feel that this unique combination probably is not purely coincidental, but might rather be specific for Turner syndrome.Abbreviations AI aortic insufficiency - ASD atrial septal defect - PAPVR partial abnormal pulmonary venous return - SVC superior vena cava     

Anomalous origin of the left anteirior descending coronary artery from the pulmonary trunk associated with type B interrupted aortic arch

Summary An extremely rare congenital anomaly of the coronary circulation in a neonate with type B interrupted aortic arch is reported in which the left anterior descending coronary artery arose from the pulmonary trunk and the right and left circumflex coronary arteries from the aorta. An anomalous coronary artery was demonstrated by selective pulmonary angiography and then confirmed at necropsy.     

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.     

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     

右室减压术伴改良体肺分流术治疗室间隔完整型肺动脉膜性闭锁

目的 比较体外循环下行右心室流出道切开后跨肺动脉瓣补片术加改良体肺分流术(术式a)与经右室表面行肺动脉瓣球囊扩张术加改良体肺分流术(术式b)两种术式治疗室间隔完整型肺动脉膜性闭锁的临床效果.方法 2003年7月至2014年7月,共收治57例室间隔完整型肺动脉闭锁患儿,从中随机选出22例室间隔完整型肺动脉膜性闭锁患儿,其中11例患儿行术式a(a组),另外11例行术式b(b组),比较两组患儿的病死率、呼吸机辅助时间、CCU住院时间.术后随访6个月,监测出院当日、术后1个月、3个月、6个月的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉跨瓣压差、肺动脉瓣反流率(肺动脉反流束宽度与肺动脉瓣环内径的比值),比较两组患儿上述指标的差异.结果 a组患儿共死亡4例,b组患儿共死亡2例.a组患儿术后呼吸机辅助呼吸平均时间为(127.09±46.81)h、CCU平均住院时间为(7.91±2.5)d.b组患儿术后呼吸机辅助呼吸平均时间为(81.55±26.25)h、平均CCU住院时间为(4.82±1.08)d.两组比较,b组时间明显短于a组.出院当日,a组的肺动脉瓣反流率明显低于b组,两组间差异具有统计学意义(P＜0.01);a、b两组的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉跨瓣压差之间差异无统计学意义.术后1个月,a、b两组患儿的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉瓣反流率之间差异无统计学意义;a组患儿肺动脉跨瓣压差低于b组,差异有统计学意义(P＜0.05).术后3个月,a组患儿经皮氧饱和度明显高于b组,a组患儿肺动脉跨瓣压差明显低于b组,两组间差异具有统计学意义(P＜0.01);a组患儿三尖瓣Z值、McGoon比值均高于b组,两组间差异有统计学意义(P＜0.05);a、b两组间肺动脉瓣反流率之间差异无统计学意义.术后6个月,a组肺动脉瓣反流率低于b组,两组间差异有统计学意义(P＜0.05);a、b两组的经皮氧饱和度、三尖瓣Z值、McGoon比值、肺动脉跨瓣压差之间差异具有统计学意义(P＜0.01).结论 b组呼吸机辅助时间、CCU住院时间均短于a组,但术后3个月以后随访,a组患儿右心室及肺血管发育优于b组患儿.