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.     

Unusual cause of neonatal cyanosis

We present a case of a full-term female neonate who presented at 6 h of age with severe cyanosis and was partially responsive to oxygen supplementation. An echocardiogram showed an isolated congenital severe tricuspid valve insufficiency due to rupture of the papillary muscle of the anterior tricuspid valve leaflet. Magnesium sulfate was infused to lower the pulmonary resistance and thus enhancing the antegrade pulmonary blood flow. Ductal patency was secured by prostaglandin infusion thus providing an additional pulmonary blood flow through the ductus arteriosus.The above measures were adequate to stabilize the patient with no further deterioration or the need for other supportive measures such as Nitric Oxide therapy or extracorporeal membrane oxygenation (ECMO). Therefore, early diagnosis and adequate measures to improve the pulmonary blood flow are mandatory, important pre-operative measures in the management of these patients.     

PaO2 increases with coughing in patients with chronic lung disease

We considered if the cyanosis frequently observed during a cough attack in patients with chronic lung disease was due to worsening hypoxemia. To investigate the effects of cough on PaO₂, we measured arterial blood gases before and after a voluntary coughing period of 45 sec, in 11 patients with Interstitial Lung Disease (ILD) and 14 patients with Chronic Obstructive Lung Disease (COPD). All patients significantly increased (p < 0.05) their PaO₂ (COPD: from 49 ± 2 to 60 ± 2 mmHg; ILD from 44 ± 2 to 51 ± 3 mmHg, mean ± SD) and decreased their PaCO₂. We conclude that stable patients with COPD and ILD increase their PaO₂ with coughing most likely due to hyperventilation. The cyanosis observed could be due to peripheral circulatory effects of coughing.     

紫绀型先天性心脏病患者血红蛋白增高的临床意义

目的：探讨紫绀型先天性心脏病（CHD）患者血红蛋白增高的临床意义。方法：将91例紫绀型CHD按血红蛋白值随机分为A组（180-200g/L）、B组（200-220g/L）及C组（〉220g/L）,分析各组临床病理数据之间的相关性。结果：在血红蛋白增高患者中原发病为肺血减少型先天性心脏病的占81.32%。法洛四联症患者McGoon值与血红蛋白计数呈正性相关（P〈0.01）。A组体-肺侧支阳性例数较C组高（P〈0.01）。血红蛋白计数与红细胞压积、凝血酶原时间和国际化标准值呈正性线性关系（P〈0.01）,与血小板计数呈负性线性关系（P〈0.01）。19例患者尿蛋白异常,占21.11%,3组间尿蛋白检测结果无差异（P〉0.05）。结论：紫绀型先天性心脏病患者血红蛋白增高与原发病、肺血管和侧支情况有关,可伴有血液系统、凝血功能和肾功能异常。     

Multiple Intrapulmonary Arteriovenous Fistulas in Childhood

Pulmonary arteriovenous fistulas (AVFs) are a rare but recognized cause of cyanosis in childhood. Lesions may be acquired as in hepatopulmonary syndrome or they may be congenital, particularly in association with certain multisystem disorders. Large fistulas are more common than multiple small connections. Two cases, both boys, presenting in the first decade of life are described. ``Bubble' echocardiography was the most telling investigation and strongly suggested the presence of AVFs in both cases. Each patient then underwent cardiac catheterization, which demonstrated normal pulmonary artery pressure and diffuse pulmonary telangiectasis. Both patients were treated effectively with nifedipine and continue with this mode of therapy.     

新生儿青紫129例病因及临床特征分析

目的:探讨新生儿青紫的病因和临床特征。方法:回顾性分析湖北省中山医院新生儿重症监护室2007年1月-2008年12月病因明确的129例青紫患儿的临床资料。结果:(1)129例青紫新生儿中,中心性青紫101例(78.3%),其中以原发肺部疾病为首要病因,即肺源性青紫92例(71.3%),心源性青紫仅9例(7%);周围性青紫13例(10.1%);其他原因青紫15例(11.6%)。(2)129例青紫新生儿入院时连续监测经皮血氧饱和度(continuous trans-cutaneous oxygen saturation,TcSO2)并完善相关检查,根据病情给予不同方式的氧疗。氧疗前101例中心性青紫患儿TcSO2均低于85%,氧疗后76例肺源性青紫消失,TcSO2升至85%以上,余25例皮肤青紫未消失,TcSO2仍低于85%;氧疗效果不佳的25例中心性青紫新生儿中,13例为肺部疾患合并持续胎儿循环,9例为复合型先心病,3例为先天性膈肌发育异常。氧疗前13例周围性青紫患儿TcSO2均大于85%,8例氧疗后青紫略有减轻,5例为局部挤压伤停用氧疗。在15例其他原因青紫患儿中,氧疗对青紫发作现象无改善作用。结论:新生儿青紫的病因以原发肺部疾病为首位。氧疗是青紫治疗的基本方法,但氧疗的效果因病因的不同而有明显的差异。     

Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring associations of late gadolinium enhancement with clinical status and survival

Background

A relationship between myocardial fibrosis and ventricular dysfunction has been demonstrated using late gadolinium enhancement (LGE) in the pressure-loaded right ventricle from congenital heart defects. In patients with Eisenmenger syndrome (ES), the presence of LGE has not been investigated. The aims of this study were to detect any myocardial fibrosis in ES and describe major clinical variables associated with the finding.

Methods

From 45 subjects screened, 30 subjects (age 43 ± 13 years, 20 female) underwent prospective cardiovascular magnetic resonance with LGE to quantify biventricular volume and function as well as maximal and submaximal exercise during a single visit. Standard cine acquisitions were obtained for ventricular volume and function. Further imaging was performed after administration of 0.1 mmol/kg gadolinium contrast. Regions of LGE were evaluated qualitatively and quantitatively by manual contouring of identified areas, with total area expressed as a percentage of mass. Patients were followed prospectively (mean follow up 7.4 ± 0.4 years) and any deaths recorded. Patients with LGE findings were compared to those without.

Results

LGE was present in 22/30 (73%) patients, specifically in RV myocardium (70%), RV trabeculae (60%), LV myocardium (33%) or LV papillary muscles (30%), though in small amounts (mean 1.4% of total ventricular mass, range 0.16 – 6.0%). Those with any LGE were not different in age, history of arrhythmia, desaturation, nor hemoglobin, nor ventricular size, mass, or function. Exercise capacity was low, but also not different between those with and without LGE. Similarly no significant associations were found with amount of fibrosis. There were five deaths among patients with LGE, versus two in patients without, but no difference in survival (log rank =0.03, P = 0.85).

Conclusions

Myocardial fibrosis by LGE is common in ES, though not extensive. The presence and quantity of LGE did not correlate with ventricular size, function, degree of cyanosis, exercise capacity, or survival in this pilot study. More data are clearly required before recommendations for routine use of LGE in these patients can be made.     

Cardiac growth patterns in response to chronic hypoxia in a neonatal rat model mimicking cyanotic heart disease

OBJECTIVE:

Myocardial growth during fetal life is accomplished by the proliferation of myocytes. Shortly after birth, normal myocytes largely lose their capability to replicate. The present study aims to assess the effect of persistent postnatal hypoxia on myocardial growth patterns in an animal model mimicking cyanotic heart disease.

METHODS:

Sprague-Dawley rats were placed in a normobaric hypoxic environment at birth and oxygen levels were maintained at 10% (group H). Controls (group C) remained in room air. The animals were sacrificed and the hearts were harvested at one, four and eight weeks.

RESULTS:

Significant polycythemia developed in the hypoxic rats. There was a significant increase in indexed right ventricle (RV) and left ventricle (LV) masses compared with controls. Myocardial DNA concentrations were significantly increased in both ventricles of the hypoxic rats. For the RV, the increase in DNA content for group H was 135%, 132% and 112% that of group C values at one, four and eight weeks, respectively. RV and LV myocardial protein:DNA ratios were lower in the one-week- and four-week-old hypoxic rats, and significantly higher in the hypoxic eight-week-old rats. Polyploidy, hydroxyproline concentrations and dry:wet weight ratios were not significantly different in the LV and RV between both group H and group C animals.

CONCLUSION:

Cardiac mass increases in response to chronic hypoxia were greater in the RV than the LV. This increase was mainly due to myocardial proliferation in the first four weeks of life. Although the three groups of hypoxic rats had significant elevations in DNA concentration compared with controls, there was a shift from proliferation to hypertrophy after week 4 of life. The age of the myocyte appears to be the most important factor in triggering proliferation in this hypoxic animal model.