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.     

Congenital pulmonary lymphangiectasis: Report of 11 Examples with Special Reference to Cardiovascular Findings

The features of 11 cases of congenital pulmonary lymphangiectasis dying during the neonatal period are presented. Seven were associated with total anomalous pulmonary venous drainage with constriction of the common pulmonary vein at its junction with the general venous system. The anatomical features are compared with 14 infants with total anomalous pulmonary venous drainage without pulmonary lymphangiectasis.Evidence from this series and from the published reports suggests that at least some examples of congenital pulmonary lymphangiectasis result from obstruction to the pulmonary venous return by anomalous pulmonary venous drainage or obstructive left heart anomalies. It is probable that congenital pulmonary lymphangiectasis will remain a bar to the successful surgical treatment in the newborn period of many patients with total anomalous pulmonary venous drainage.     

Doppler flow profiles in the right and left pulmonary artery in children with congenital heart disease and a bidirectional cavopulmonary shunt

Summary The systolic and diastolic Doppler tracings in the right and left pulmonary artery were analyzed in 10 patients with complex cyanotic congenital heart disease, aged 6 months to 12 years (median 3 years), after employment of a bidirectional cavopulmonary shunt. The postoperative interval ranged from 2 weeks to 1.7 years (median 1.3 years). In children with pulmonary atresia or severe pulmonary stenosis with minimal antegrade pulsatile pulmonary blood flow Doppler echocardiography confirmed a systolic and diastolic bidirectional shunt from the vena cava superior to both pulmonary arteries. In children with pulmonary stenosis, Doppler echocardiography confirmed a systolic shunt only to the right pulmonary artery and a diastolic bidirectional shunt into both pulmonary arteries. As the left pulmonary artery was perfused by the pulsatile transvalvular flow it was difficult to detect a concomitant systolic Glenn-related flow in those patients. Quantitative analysis of the diastolic Doppler tracings revealed a significant difference in the velocity time integral in the right and left pulmonary artery indicating a dominant right lung perfusion in diastole.Deceased     

肺静脉指数对肺血减少型先天性心脏病肺血管发育的评估

目的 探索能更准确反映肺血管发育及肺血流情况的指标,为外科手术方案的选择提供依据.方法 采用74例肺血减少型先天性心脏病心血管造影序列,测量左右肺动脉及4根肺静脉直径,分别计算Nakata指数,McGoon指数,肺静脉指数(PVI),分别与术后情况进行相关分析.结果 左、右侧肺静脉大小分别与左右肺动脉大小高度相关,左侧肺静脉与左肺动脉远端的相关性为0.73,左侧肺静脉与左肺动脉近端的相关性为0.72,右侧肺静脉与右肺动脉远端的相关性为0.67,右侧肺静脉与右肺动脉近端的相关性为0.71.PVI与术后监护时间,呼吸机维持时间,正性肌力药物用量的相关性分别为-0.51,-0.478和-0.693,均比Nakata指数,McGoon指数明显增高,能更准确的反映整个肺血管的发育情况.右室流出道重建术后无低心排组与低心排组的McGoon指数分别为1.97±0.58与1.36±0.51(t=2.347,P＜0.05),两组Nakata指数分别为(269±124)mm2/m2和(164±106)mm2/m2(t=2.218,P＜0.05),PVI分别为(273±125)mm2/m2和(152±77)mm2/m2(t=2.936,P＜0.01),低心排组肺血管值均明显小于无低心排组.当PVI小于180 mm2/m2时,术后易出现血流动力学不稳定,低心排,甚至死亡.结论 肺动脉、肺静脉发育彼此相关,PVI能更准确反映肺血管发育及肺血流情况的形态学指标,为外科手术方案的选择提供有效依据.     

High-altitude pulmonary edema with absent right pulmonary artery

High-altitude pulmonary edema potentially is fatal. Adults with unilateral absence of a right pulmonary artery are particularly susceptible to high-altitude pulmonary edema. The occurrence of high-altitude pulmonary edema was documented in a child with congenital absence of the right pulmonary artery. Improvement occurred only upon descent to low altitude. Physicians should be aware of this life-threatening condition in children ascending to high altitude, particularly in individuals with unilateral absence of a pulmonary artery.     

儿童肺血管炎

肺血管炎是一组以肺血管壁及周围组织炎症细胞浸润为特征,伴肺血管壁损伤的疾病的总称,多为系统性疾病在肺部的表现之一,也可以是孤立性肺血管炎。儿童肺血管炎属少见病,其病因繁杂。因受累血管不同,肺血管炎的呼吸系统临床表现多样,可并发肺动脉狭窄、肺动脉瘤、肺动脉血栓、肺梗死、肺泡出血、肺动脉高压等。治疗目标包括控制血管炎症、处理肺部并发症。糖皮质激素仍是多数原发性肺血管炎治疗的一线药物,而联合恰当的免疫抑制剂治疗是改善预后的关键。近年,生物制剂的应用也得到了较好疗效。     

Progressive pulmonary venous obstruction and pulmonary interstitial fibrosis associated with tetralogy of Fallot

Summary Stenosis of individual pulmonary veins is a rare condition associated with a very high mortality. The present report is the first known case of progressive pulmonary venous obstruction associated with the tetralogy of Fallot. This clinical and anatomical study suggests that initial dynamic and possibly reversible occlusion occurs at the junction of the pulmonary vein and left atrium. Anatomical lesions in pulmonary veins then develop, possibly secondary to functional stenosis. The usually fatal outcome of these cases is due to the development of fixed pulmonary venous occlusion and possibly pulmonary interstitial fibrosis. The rapid progression of pulmonary venous stenosis strongly suggests that discrete veno-atrial stenosis should be surgically corrected as early as possible to prevent the irreversible diffuse pulmonary venous obstruction associated with an invariably fatal prognosis.     

肺动脉闭锁合并室间隔缺损104例诊断分析

目的　分析肺动脉闭锁合并室间隔缺损 (PA/VSD)的解剖类型及血流动力学改变 ,探讨其适宜的手术方式。方法　对 1992年 6月至 2 0 0 2年 5月在广东省心血管病研究所儿科住院的PA/VSD患儿共 10 4例 ,采用超声心动图结合心血管造影术 ,确定闭锁的部位、肺动脉的发育情况及血供来源。结果　右室流出道及瓣膜闭锁、有肺动脉总干 31例 ;肺动脉总干闭锁、左右肺动脉有汇合 5 0例 ;左肺动脉闭锁 10例 ,右肺动脉闭锁 8例 ;左右肺动脉均闭锁 5例。肺动脉的血供来源 :大的主肺动脉侧支血管 5 1例 ,动脉导管未闭 2 7例 ,多支小的侧支血管 2 6例。合并畸形有卵圆孔未闭、房间隔缺损、大动脉转位、完全性房室间隔缺损、右室双出口、镜面右位心、右旋心及左旋心。结论　合并室间隔缺损的肺动脉闭锁可发生在不同部位 ,肺动脉的血供来源多样化     

Leukotriene inhibition prevents and reverses hypoxic pulmonary vasoconstriction in newborn lambs

The effects of the leukotriene antagonist FPL 57231 on the circulation were studied in spontaneously breathing normoxic and hypoxic newborn lambs in order to evaluate the role of leukotrienes in the perinatal control of hypoxic pulmonary vasoconstriction. Hypoxia produced a 58% increase in pulmonary arterial pressure (p less than 0.05) and a 37% increase in pulmonary vascular resistance (p less than 0.05). Hypoxic pulmonary vasoconstriction was abolished by the prior infusion of FPL 57231. Pulmonary arterial pressure increased by only 8% and pulmonary vascular resistance decreased by 10% from normoxia. The infusion of FPL 57231 during hypoxia-induced pulmonary vasoconstriction reversed the increase in pulmonary arterial pressure (p less than 0.05) and pulmonary vascular resistance (p less than 0.05). The hypoxia-induced increase in cardiac output was maintained during the infusion of FPL 57231. Leukotrienes may play a significant role in the mediation of hypoxic pulmonary vasoconstriction. Leukotriene inhibition with FPL 57231 may be useful in the management of infants with persistent pulmonary hypertension syndrome.     

室间隔缺损合并肺动脉高压肺血管床功能状态评估指标的研究

目的探讨室间隔缺损合并肺动脉高压肺血管床功能状态的评估指标。方法对室间隔缺损合并重度肺动脉高压的患儿，于心导管术中应用酚妥拉明，将轻度全肺循环阻力（ＴＰＲ）增加的２７例与重度全肺循环阻力增加的１２例患儿的试验结果进行比较。结果两组患儿的单一肺动脉压降幅（Ｐｐ降幅）分别为２．３ｋＰａ（１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）和２．２ｋＰａ，Ｐ＞０．０５，差异无显著意义；而肺动脉压降幅（Ｐｐ降幅）／体循环压降幅（Ｐｓ降幅）的比值，试验前后肺动脉血氧饱和度变化值这二项指标的差异有非常显著意义（Ｐ＜０．０１）。Ｐｐ降幅／Ｐｓ降幅比值与全肺循环阻力的相关性良好［ｒ＝－０．８９９），Ｙ（ＴＰＲ）＝２６６８－１８９２Ｘ（Ｐｐ降幅／Ｐｓ降幅）］。结论用酚妥拉明作扩血管降压试验时，单一的肺动脉压降幅不能完全反映肺血管床的功能状态；而Ｐｐ降幅／Ｐｓ降幅比值，试验前后肺动脉血氧饱和度变化值这二项指标对室间隔缺损合并肺动脉高压患儿的肺血管床功能状态的评估，对手术适应证的选择具有一定指导意义。     

外源性二氧化硫对低氧性肺动脉高压大鼠肺动脉压力的影响

目的探讨外源性二氧化硫(SO2)对低氧性肺动脉高压大鼠肺动脉压力的影响。方法将大鼠分为对照组、低氧组和低氧加SO2组(予亚硫酸钠/亚硫酸氢钠)。对低氧和低氧加SO2组大鼠进行低氧处理21d。低氧处理后检测各组大鼠肺动脉平均压、收缩压及舒张压,分析外源性SO2对肺动脉压力的影响。结果与对照组比较,低氧组大鼠肺动脉平均压升高127.13%;与低氧组比较,低氧加SO2组大鼠肺动脉平均压降低24.0%。与对照组比较,低氧组大鼠肺动脉收缩压升高84.93%;与低氧组比较,低氧加SO2组大鼠的肺动脉收缩压降低23.10%。与对照组比较,低氧组大鼠肺动脉舒张压升高154.59%;与低氧组比较,低氧加SO2组大鼠的肺动脉舒张压下降不明显(P=0.065)。结论外源性SO2能显著降低低氧大鼠肺动脉平均压和肺动脉收缩压,而对肺动脉舒张压的影响不显著。     

Evaluation of Pulmonary Hypertension in a Child: Role of Computed Tomography

Unexplained pulmonary hypertension in pediatric patient is a diagnostic challenge. The natural history as well as management depends upon etiology of pulmonary hypertension. Despite newer insights in pathophysiology and management strategies, the outcome of pulmonary veno-occlusive disease remains dismal. The author’s report a case of 12-year-old girl who presented with severe pulmonary hypertension. Stepwise evaluation and CT angiographic assessment in the index case led to definitive diagnosis of pulmonary veno-occlusive disease. This case highlights the role of cardiac imaging in localising site of pulmonary hypertension. It is important for health care professionals involved in care of patients with pulmonary hypertension to make correct diagnosis of pulmonary veno-occlusive disease for better prognostication as well as to avoid therapeutic mishaps.     

Late Pulmonary Valve Replacement in Congenital Heart Disease Patients Without Original Congenital Pulmonary Valve Pathology

Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect (n = 3), complete atrioventricular septal defect (n = 1), and anomalous left coronary artery from the pulmonary artery (PA) (n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 ± 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease.     

Unusual diagnosis of partial left abnormal pulmonary venous return in neonate]

The authors report on a case of a newborn with partial anomalous pulmonary venous return of the lower left pulmonary vein. Diagnosis was performed during the introduction of a central venous catheter. The complications of partial anomalous pulmonary venous rely principally in the risk of endothelium damage and subsequent pulmonary arterial hypertension.     

The role of surgical therapy for pulmonary vein atresia in childhood

The outcome for children with pulmonary vein atresia has been historically poor. This report describes clinical outcomes after surgical treatment of primary and secondary pulmonary vein atresia. All patients undergoing surgery for pulmonary vein atresia at Children??s Hospital Boston from 1 January 2005 to 1 January 2009 were identified. The data available for analysis included demographic characteristics, findings based on cardiac catheterization and other imaging, clinical information, operative surgical intervention, and pulmonary vein histology. The study-specific information included the calculated number of atretic pulmonary veins as well as surgical interventions performed during the study period. The study end points included successful relief of pulmonary vein atresia, recurrence of pulmonary atresia or stenosis, overall pulmonary vein disease progression, and patient survival. A total of 16 patients with one or more pulmonary vein atresias were identified in the cardiac surgery registry database. Of these 16 patients, 5 had primary pulmonary atresia, and 11 had secondary atresia after repair of anomalous pulmonary venous drainage. Surgical recanalization of atretic pulmonary veins was achieved for more than 80% of the patients. At the most recent follow-up assessment, 7 (44%) of the 16 patients had recurrence of atresia. Five of the patients had development of new atresia in other previously healthy pulmonary veins. The median follow-up period was 4?months for primary and 17?months for secondary pulmonary vein atresia. The three main predictors of pulmonary vein disease progression after surgery were the presence of underlying lung disease (P?=?0.036), prematurity (P?=?0.035), and a history of recurrent pulmonary vein atresia. The overall mortality rate for the patients with primary or secondary pulmonary vein atresia was 56% at the most recent follow-up assessment (100% for the patients with primary vein atresia and 36% for the patients with secondary vein atresia). Conclusions: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. Recurrence of atresia can take place as early as a few months in the presence of underlying lung disease, prematurity, or history of recurrent pulmonary vein atresia. Further research is needed to identify mechanisms for attaining and sustaining pulmonary vein patency after surgery.     

Pulmonary hypertension in children: the twenty-first century

Pulmonary hypertension is an elevation in pulmonary artery pressure that is associated with a spectrum of diseases and causes. Its clinical severity and presentation are widely varied. The field of study has changed immensely over the past several years. Significant knowledge has been gained in the pathophysiology, genetics, and vascular biology associated with pulmonary hypertension. These discoveries have contributed to medical interventions that have improved outcomes associated with pulmonary hypertension. This article reviews pulmonary hypertension in children, focusing on idiopathic pulmonary hypertension. Because most information is associated with children who have this form of the disease, formerly classified as primary pulmonary hypertension, medical therapy is discussed with a focus on this patient group. Additional therapeutic concepts relevant to other causes of pulmonary hypertension are highlighted.     

Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: Case report and review of the literature

The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect is highly variable. Several cases have been reported in the literature in which a coronary artery to pulmonary artery fistula, alone or in combination with additional vessels, supplies the pulmonary circulation. We report a case of successful repair during early infancy, with unique hemodynamic, clinical, and anatomic features. The literature is reviewed.     

Iodopovidone Pleurodesis for Isolated Pulmonary Langerhan’s Cell Histiocytosis in a Two Year Old Child

Isolated pulmonary involvement in Langerhans Cell Histiocytosis (LCH) is rare in childhood. The authors report a 2-y-old boy who presented with acute history of fever, cough and respiratory distress; later developed pneumothorax; whose CT thorax showed diffuse pulmonary cystic lucencies bilaterally. Lung biopsy confirmed pulmonary LCH with CD1a positivity. Recurrent spontaneous pneumothoraces are common in patients of pulmonary LCH; which necessitates rapid intervention. The authors share their experience of successfully using iodopovidone in pleurodesis for repeated pneumothorax episodes in this child with rare diagnosis of isolated pulmonary LCH.     

Crisscross Pulmonary Artery With Double Aortic Arch: An Unusual Association

Anomalies of pulmonary artery origin are rare. Crisscross pulmonary artery origin is a rare benign anomaly characterized by the left pulmonary artery arising superiorly and to the right side of the right pulmonary artery. The condition is usually accompanied by a conotruncal anomaly. Here, we report a child with crisscross pulmonary arteries and a complete vascular ring formed by a double aortic arch, which was confirmed by computed tomography angiography. The child underwent surgical correction for relief of stridor.     

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.