Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care

Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period. We report on an infant who developed severe bilateral chylous pleural effusions with computed tomography (CT) features of pulmonary lymphangiectasis. She recovered spontaneously with supportive treatment only. We discuss aspects of her intensive care which might have contributed to the good outcome, and conclude that modern intensive care may allow spontaneous recovery in conditions described in the literature as having a very poor outcome.     

Congenital pulmonary lymphangiectasia: a case report of thoracic duct agenesis

We present a 17-year-old Caucasian male with congenital pulmonary lymphangiectasia and an absent thoracic duct. This patient is unique as he did not present with the disorder until age 9.5 years. Since his initial presentation he has had recurrent chylothoraces and has been treated symptomatically. We discuss the possible implications of his disorder as well as some of the limited treatment that is available.     

Association of three different congenital malformations in a same pulmonary lobe in a 5‐year‐old girl

We report the case of a 5‐year‐old girl with persistent chest X‐ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations. Pediatr. Pulmonol. 2010; 45:832–835. © 2010 Wiley‐Liss, Inc.     

Congenital pulmonary varix: Two case reports

Rationale:Patients with congenital pulmonary varix are asymptomatic and require no treatment, but the radiological characteristics of a pulmonary varix are similar to those of a pulmonary arteriovenous malformation, which requires treatment. Pulmonary angiography is useful for obtaining information about the dynamics of pulmonary blood flow to differentiate a pulmonary varix from a pulmonary arteriovenous malformation for the purpose of treatment planning. Two cases of congenital pulmonary varices that were differentiated from pulmonary arteriovenous malformations based on pulmonary angiography findings are presented.Patient concerns and diagnosis:The first patient was an asymptomatic 39-year-old man. Non-contrast-enhanced computed tomography performed as part of the treatment course for pneumonia showed pulmonary arteriovenous malformation in the right lung. Pulmonary angiography was performed and showed that it was a pulmonary varix. The second patient was an asymptomatic 23-year-old woman. As part of her regular health check-up, she underwent plain chest X-ray examination, which showed an abnormal shadow. Non-contrast-enhanced computed tomography was performed, and pulmonary arteriovenous malformation was suspected. However, contrast-enhanced computed tomography findings suggested that the patient had a congenital pulmonary varix rather than a pulmonary arteriovenous malformation. Pulmonary angiography was subsequently performed for diagnosis, and a pulmonary varix was confirmed.Interventions and outcomes:No treatment was administered to either patient. The first patient was followed up for four years, and the second patient for two years. Both patients had no symptoms or complications during the follow-up period.Lessons:Two cases of congenital pulmonary varices were reported. Information about the dynamics of pulmonary blood flow obtained by performing pulmonary angiography was effective in distinguishing between pulmonary arteriovenous malformation and congenital pulmonary varix.     

Type II congenital pulmonary airway malformation with primary ciliary dyskinesia in a 4-year-old child: A case report

A congenital pulmonary airway malformation (CPAM) combined with primary ciliary dyskinesia (PCD) has not been described in literature. Herein, we described the case of a 4-year-old boy who presented to us with recurrent productive cough and rhinorrhea for 2 years. High resolution computed tomography of the thorax revealed multiple, cystic, transparent shadows of different sizes near the posterior thoracic cavity in the lower lobe of the left lung. Thoracoscopic segmentectomy was carried out and histology confirmed a type II CPAM. Whole-exome sequencing revealed a compound heterozygous mutation (c.10568+1G>A, c.9484delG) in the DNAH11 gene associated with PCD that originated from the boy's mother and father, respectively. This report showed that when a child with CPAM presents with a productive cough and recurrent sinusitis, irrespective of situs inversus, PCD should be suspected. Genetic testing can aid in diagnosis.     

Pulmonary eosinophilic vasculitis in a neonate with congenital chylothorax

We report a case of congenital bilateral chylothorax, complicated by pulmonary eosinophilic vasculitis and transient eosinophilia. Excluding all known forms of eosinophilic pulmonary disease either histologically or clinically, possible mechanisms of its etiology are discussed. Treatment with corticosteroids was successful, and after a prolonged clinical course the patient recovered by age 2 years.     

肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄

目的 分析先天性肺静脉狭窄患儿的临床特点,探讨肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄的疗效.方法 回顾性分析2008年1月至2009年12月收治的4例先天性肺静脉狭窄患儿的临床资料,其中男3例,女1例,年龄2个月～2岁11个月.均行胸片、心电图和超声心动图检查,其中2例行心脏多排计算机断层扫描检查,以期明确诊断.4例患者共有7支肺静脉狭窄,右上肺静脉3例,右下肺静脉1例,左上肺静脉1例,左下肺静脉2例.狭窄部位均在肺静脉开口处,均合并其他心血管畸形：室间隔缺损3例,房间隔缺损1例,动脉导管未闭2例,主动脉缩窄1例,术前均提示重度肺动脉高压.术前根据病情予强心、利尿、扩血管及抗感染等治疗.完善术前准备后在全身麻醉、胸骨正中切口、深低温、体外循环下行肺静脉内膜剥脱术.结果 术后患儿血流动力学稳定;机械通气时间3.7（2～4）d,重症监护时间4.2（3～5）d;围术期无一例死亡.随访18～26个月,患儿均存活,心功能（纽约心脏协会）Ⅰ级3例、Ⅱ级1例,生长发育均正常.结论 先天性肺静脉狭窄常合并各种心内畸形,超声心动图、心脏多排计算机断层扫描检查对诊断帮助较大.肺静脉内膜剥脱术治疗先天性肺静脉狭窄患儿安全、有效,手术成功率高,近期随访疗效较好,对于婴幼儿先天性肺静脉狭窄不失为一种治疗选择.     

Hemodynamic effects of nifedipine and oxygen in children with pulmonary hypertension

Summary Fourteen patients, 2 to 20 years old were investigated. Two had primary pulmonary hypertension, 11 had congenital heart disease and post-tricuspid shunts, and 1, a 20-year-old patient, was investigated after he had undergone surgical correction of truncus arteriosus I. Pulmonary arterial pressure, pulmonary flow index, peripheral systolic blood pressure and heart rate were measured before, and several times after intrapulmonary injection into the pulmonary artery of 0.5 g nifedipine/kg. Six patients were given an additional dose of 1 g nifedipine per kilogram into the pulmonary artery and hemodynamic measurements were repeated. In eight children, receiving 100% oxygen via a breathing mask, nifedipine effects were compared with oxygen effects. After 10 minutes under oxygen, the same hemodynamics were determined as after nifedipine. In addition, in four of these children aortic pressure and arterial oxygen saturation were also measured. Maximal effects occurred within 4 minutes. 0.5 g nifedipine per kilogram caused a slight reduction in mean pulmonary arterial pressure (p<0.05), as well as increase in pulmonary flow index (p<0.005). However, no significant change in heart rate or in systolic blood pressure was observed. 1 g nifedipine per kilogram IP had almost the same effects. No adverse side effects occurred, besides mild headaches in one child. A comparison of nifedipine injected into the pulmonary artery with oxygen breathing in congenital heart disease combined with pulmonary hypertension, is reported for the first time. Nifedipine had a more pronounced and beneficial effect with a selective action on the pulmonary vascular bed.     

The efficacy of using pulmonary vein wedge pressure for the estimation of pulmonary artery pressure in children with congenital heart disease.

The objectives of this study were to assess the accuracy of pulmonary vein wedge pressure (PVWP) in estimating pulmonary artery pressure (PAP) in various types of congenital heart disease, including single-ventricle physiology. The systolic, diastolic, and mean values of both PAPs and PVWPs were measured in 30 patients (a total of 46 points). Pulmonary artery pressure ranged from 13 to 74 (34 +/- 15) mm Hg in systole, 5 to 25 (13 +/- 6) mm Hg in diastole, and 6 to 48 (18 +/- 10) mm Hg in mean. As a whole, good correlations between PAPs and PVWPs were observed (systole, r = 0.70; diastole, r = 0.85; mean, r = 0.82; P < 0.0001). However, with an increase in PAP, the discrepancy between PAPs and PVWPs increased. When the mean PVWP was more than 18 mm Hg, the mean PVWP in 14 of 24 (58%) underestimated the mean PAP by up to 22 mm Hg (mean difference, -1.7 +/- 5.8 mm Hg). On the other hand, all of the patients with mean PVWPs less than 18 mm Hg (n = 22) had mean PAPs less than 18 mm Hg (r = 0.86; PAP = 1.11 x PVWP - 1.41; P < 0.0001), and the mean difference was -0.2 +/- 1.8 mm Hg. The mean PVWP can accurately estimate the mean PAP in children with congenital heart disease who have a mean PVWP less than 18 mm Hg.     

选择性肺动脉造影评价先天性心脏病肺动脉高压的初步分析

通过选择性肺动脉造影检查，了解肺血管病变程度。对４３例先天性左向右分流型心脏病病人行选择性肺动脉造影检查，通过分析肺循环时间、肺动脉终末单支血管计数、肺动脉分级、毛细血管像等指标，并与右心导管（４３例）和肺病理（７例）结果进行比较分析。结果显示：随着肺血管病变加重，肺循环时间延长；肺动脉终末单支血管计数、肺动脉分级减少；肺毛细血管象分布不均。结论：选择性肺动脉造影检查可以比较准确地判定肺血管病变程度。     

Aerosolized iloprost for pulmonary vasoreactivity testing in children with long‐standing pulmonary hypertension related to congenital heart disease

Background: In congenital heart disease with increased pulmonary blood flow and pressure, progressive changes in the vascular structure can lead to irreversible pulmonary hypertension (PH). Pulmonary hemodynamic parameters are used to determine whether surgical correction is no longer indicated. In this study, aerosolized iloprost was used to assess pulmonary vasoreactivity in children with long‐standing PH related to congenital heart disease. Methods: Children with long‐standing and severe PH secondary to congenital heart disease were included in this study. Various hemodynamic parameters were measured before and after iloprost inhalation (0.5 μg/kg), and vascular resistance was determined. Responders to the iloprost test were defined as those with a decrease in both pulmonary vascular resistance (PVR) and pulmonary‐to‐systemic vascular resistance ratio (R_p/R_s) of >10%. Results: Eighteen children aged between 7 months and 13 years with long‐standing and severe PH secondary to congenital heart disease were studied. Thirteen children had a positive response, resulting in a mean (± SD) decrease of PVR from 9.3 ± 4.6 to 4.6 ± 2.7 Wood U · m² (P < 0.001), and a mean decrease of R_p/R_s from 0.54 ± 0.37 to 0.24 ± 0.14 (P = 0.005). Conclusions: Iloprost‐induced pulmonary vasodilator responses vary among children with PH related to congenital heart disease. The use of inhale iloprost in the cardiac catheterization laboratory results in pulmonary vasoreactivity for some of these children particularly a reduction in PVR and the pulmonary‐to‐systemic vascular resistance ratio. © 2008 Wiley‐Liss, Inc.     

Cutting balloon angioplasty for children with small-vessel pulmonary artery stenoses.

Patients with complex congenital heart disease may have pulmonary artery stenoses that are either congenital or associated with scarring following surgical procedures. This study evaluates cutting balloon angioplasty for small-vessel pulmonary artery stenoses resistant to standard balloon angioplasty. Between October 1998 and December 1999, patients were enrolled in an FDA-approved compassionate-use protocol. During four catheterizations, there were seven lesions found resistant to standard balloon angioplasty (mean lesion diameter was unchanged: 1.8 mm +/- 0.8 mm to 1.9 +/- 0.8 mm). A cutting balloon was inflated twice in each of these lesions. Standard balloon angioplasty was then repeated. Final mean lesion diameter was increased significantly (1.9 mm +/- 0.8 mm to 3.8 +/- 1.3 mm; P 相似文献