Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis.

BACKGROUND: Congenital lung malformations (CLMs) are being detected more frequently during pregnancy. There is controversy regarding the optimal treatment for an asymptomatic child with prenatally diagnosed CLMs. Due to the paucity of information from clinical trials, we developed decision analytic models to compare two treatment strategies for such patients-elective surgical resection versus expectant management. METHODS: We built decision analytic models stratified by lesion size. We used data from English language literature identified through PubMed searches along with estimates from expert opinions of surgical colleagues. We analyzed results for two hypothetical asymptomatic 6-month-old children with CLMs; one has a large lesion occupying more than 50% of the involved lobe, while the other has a small lesion occupying less than 50% of the involved lobe. We used quality-adjusted life years (QALYs) to measure effectiveness. RESULTS: For an asymptomatic child with a small or large lesion, expectant management resulted in a small gain of 0.09 or 0.15 QALYs, respectively. Sensitivity analyses showed that surgical resection would be preferred if the probability of remaining asymptomatic was low. CONCLUSIONS: In contrast to current practice, expectant management may be a better alternative for asymptomatic children born with CLMs. More longitudinal studies are required to improve the accuracy of the model.

Utility of Fetal Cardiac Axis and Cardiac Position Assessment in Predicting Neonatal Respiratory Morbidity in Fetal Congenital Lung Lesions.

OBJECTIVES: To assess the diagnostic performance of the fetal cardiac axis (CA) and/or cardiac position (CP) versus the congenital pulmonary malformation volume ratio (CVR) in predicting any and severe neonatal respiratory morbidity in fetal congenital lung lesions. METHODS: This work was an 11-year retrospective cohort study. The sensitivity, specificity, positive predictive value, and negative predictive value of CA and/or CP assessment in prediction of respiratory morbidity were calculated before 24 weeks' gestation and between 24 and 32 weeks and compared to CVR cutoffs obtained from the literature. RESULTS: Fifty-three patients were included. CA and/or CP abnormalities were present in 45% and 38% of patients before 24 weeks and between 24 and 32 weeks and were significantly more common in left- versus right-sided lesions (60% versus 17%; P = .003). The sensitivity, specificity, positive predictive value, and negative predictive value of an abnormal CA and/or CP for any and severe respiratory morbidity were 0.67, 0.61, 0.33, and 0.86 and 0.8, 0.58, 0.17, and 0.97 before 24 weeks and 0.75, 0.73, 0.45, and 0.91 and 0.8, 0.67, 0.20, and 0.97 between 24 and 32 weeks, respectively. An abnormal CA and/or CP had higher sensitivity for any respiratory morbidity compared to the CVR at 0.5 and 0.8 cutoffs both before 24 weeks and between 24 and 32 weeks (P < .05). CONCLUSIONS: An abnormal CA and/or CP before 24 weeks and between 24 and 32 weeks has higher sensitivity for the detection of any respiratory morbidity at birth compared to the CVR at both 0.5 and 0.8 cutoffs. A normal CA and CP have a high negative predictive value for excluding any respiratory morbidity at birth both before 24 weeks and between 24 and 32 weeks.

Ultrasound assessment of congenital fetal lung masses and neonatal respiratory outcomes.

OBJECTIVE: Fetal congenital lung masses generate concern for compromised postnatal respiratory function. Congenital pulmonary adenomatoid malformation volume ratio (CVR) has been used to predict the risk of hydrops fetalis and need for antenatal intervention. This study investigates whether CVR could be used to predict neonatal respiratory outcomes. METHODS: The ultrasounds of fetuses diagnosed with a lung mass between 2005 and 2013 were reviewed. CVR was calculated at each ultrasound using the formula for a prolate ellipse divided by head circumference. The pregnancy outcome and information about NICU admission for respiratory insufficiency were collected. RESULTS: Forty-two fetuses were diagnosed with a lung mass during the study period. CVR prior to 24 weeks and between 24 and 32 weeks were associated with NICU admission (p < 0.0001 and <0.008, respectively). CVR increased up to 32 weeks and decreased thereafter for most subjects. The decrease in CVR beyond 32 weeks was larger for cases that required NICU admission (p = 0.002). For a CVR cut-off of <0.5, the sensitivity was 100%, the specificity 85.7%, and negative predictive value was 100% for regular nursery care. CONCLUSION: In pregnancies diagnosed with fetal lung masses, CVR predicted normal respiratory outcomes and need for NICU admission. This information may be helpful for delivery planning. © 2015 John Wiley & Sons, Ltd.

Utility of electrocardiogram in the assessment and monitoring of pulmonary hypertension (idiopathic or secondary to pulmonary developmental abnormalities) in patients≤18 years of age.

Electrocardiograms have utility in disease stratification and monitoring in adult pulmonary arterial hypertension (PAH). We examined the electrocardiographic findings that are common in pediatric PAH and assessed for correlation with disease severity and progression. We retrospectively identified patients aged≤18 years followed at a single institution from January 2001 to June 2012 with catheterization-confirmed diagnosis of idiopathic PAH and PAH secondary to pulmonary developmental abnormalities. Patients with an electrocardiography performed within 60 days of catheterization were included. Primary and secondary outcomes are the prevalence of abnormal electrocardiographic findings at the time of catheterization and the association between electrocardiographic and hemodynamic findings and electrocardiographic changes with disease progression on follow-up catheterization, respectively. Of the 100 electrocardiography-catheterization pairs derived from the 46 patients identified, 93% had an electrocardiographic abnormality: 78% had right ventricular hypertrophy (RVH) and 52% had right axis deviation (RAD) for age. In patients with idiopathic PAH, the presence of RVH and RAD correlated with pulmonary vascular resistance and transpulmonary gradient. RAD and RVH on baseline electrocardiogram was associated with an increased risk of disease progression on subsequent catheterization (odds ratio 11.0, 95% confidence interval 1.3 to 96.2, p=0.03) after adjusting for PAH subgroup. The sensitivity, specificity, and positive and negative predictive values of RAD and RVH on baseline electrocardiogram for disease progression were 92%, 48%, 33%, and 95%, respectively. In conclusion, electrocardiographic abnormalities are common in pediatric PAH. RAD and RVH on electrocardiogram were associated with worse hemodynamics, whereas their absence is suggestive of a lack of disease progression.

Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?

Preoperative pulmonary vascular disease remains an important risk factor for death or right-heart failure in selected children undergoing two-ventricle repair, single-ventricle palliation, or heart transplantation. Preoperative criteria for poor outcome after operation remain unclear. The purpose of this review is to critically assess both the historic and current data and make recommendations where appropriate. An extensive literature search was undertaken in October 2009. Data were analyzed by an expert multidisciplinary team and recommendations were made by consensus. PubMed was searched in October 2009. Data were analyzed and recommendations were made by consensus of a multidisciplinary team. In patients with suspected pulmonary vascular disease anticipating a two-ventricle repair, although preoperative testing via cardiac catheterization with vasodilators is reasonable, the preoperative parameters and the precise values of these parameters that best correlate with early and late outcome remain unclear. Further investigation is warranted in selected populations, such as the growing group of children with congenital heart disease complicated by chronic lung disease of prematurity, and in the developing world where patients may be more likely to present late with advanced pulmonary vascular disease. In patients with a functional single ventricle, there is growing evidence that mean pulmonary artery pressure of >15 mm Hg may be associated with both early and late mortality after the Fontan operation. The relationship of preoperative pulmonary hemodynamics to early and late morbidity remains to be defined. There most likely is a level of preoperative pulmonary vascular disease that puts an individual patient at increased risk for death or severe cyanosis after a bidirectional cavopulmonary anastomosis. It remains unclear, however, how to best assess this risk preoperatively. The limitations in obtaining an accurate assessment of pulmonary vascular disease in the complex single ventricle are discussed. In children awaiting cardiac transplantation with elevated pulmonary vascular disease of >6 U.m and/or transpulmonary gradient of >15 mm Hg, heart transplantation is deemed feasible in most transplant centers if the administration of inotropes or vasodilators can decrease the pulmonary vascular disease to <6 U.m or transpulmonary gradient to <15 mm Hg. In patients with preoperative pulmonary vascular disease, there may be contributing factors to the pulmonary vascular disease, such as the specifics of the cardiac lesion (atrioventricular valve regurgitation, low cardiac output), parenchymal and/or airway issues, and/or individual genetic predisposition. Amelioration of any reversible factors before operation and optimization of their management in the preoperative and postoperative period are recommended.

[High-resolution computed tomography (HRCT) of the pediatric lung]. / Hochauflösende Computertomographie der Lunge bei Kindern und Jugendlichen.

High-resolution computed tomography (HRCT) of the lung is a very valuable method in the evaluation of children with acute and chronic lung disease due to the high spatial resolution and precise display of anatomy and pathology without superposition. The following publication will describe an optimized HRCT technique in order to reduce dose, explain various HRCT pattern and explain typical pediatric lung diseases.