Assessing pulmonary arterial hypertension in infants with severe chronic lung disease of infancy: a role for a pulmonary artery catheter?

The treatment of pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy (CLDI) is becoming commonplace. However, an optimal approach to the monitoring of this treatment has not been clearly established, and data suggest that such therapy may not be without risk. This study assessed the feasibility and safety of pulmonary artery catheter (PAC) placement and its role in the management of PAH associated with CLDI. The medical records of 12 infants with CLDI requiring chronic mechanical ventilation who underwent PAC monitoring were reviewed. Data analyzed included demographics, hemodynamic data, PAH pharmacological therapy, respiratory support, echocardiographic data, sedation level, complications related to PAC use, and mortality. In this analysis, PAC placement and monitoring was found to be feasible, appeared safe, and was associated with the ability to wean inspired oxygen, decrease sedation, and titrate PAH therapy without untoward effect. However, no definitive conclusions can be drawn from this report given its small sample size and uncontrolled, retrospective design. It is hoped that these data will renew interest in PAC monitoring for CLDI and foster prospective study where its true value can be ascertained.

Multiomics endotyping of preterm infants with bronchopulmonary dysplasia and pulmonary hypertension-A pilot study.

Pulmonary hypertension associated with bronchopulmonary dysplasia is a severe complication of preterm birth resulting in high mortality of up to 50% within the first 2 years of life. There is a direct relationship between bronchopulmonary dysplasia severity and incidence of associated pulmonary hypertension. However, it is challenging to clinically characterize severe bronchopulmonary dysplasia with and without pulmonary hypertension and there is need for better understanding of the two entities. Our main objective is to identify markers to help understand biological processes and characterize infants with pulmonary hypertension associated with bronchopulmonary dysplasia using tracheal aspirates. We conducted an unbiased multiomic analysis of tracheal aspirates via microRNA (miRNA) polymerase chain reaction arrays, RNA sequencing, and mass spectrometry proteomics in preterm infants with severe bronchopulmonary dysplasia with and without pulmonary hypertension (n = 46). Our pilot study analysis revealed 12 miRNAs (hsa-miR-29a, has-miR-542-3p, has-miR-624, has-miR-183, hsa-miR-501-3p, hsa-miR-101, hsa-miR-3131, hsa-miR-3683, hsa-miR-3193, hsa-miR-3672, hsa-miR-3128, and hsa-miR-1287), 6 transcripts (IL6, RPL35P5, HSD3B7, RNA5SP215, OR2A1-AS1, and RNVU1-19), and 5 proteins (CAPS, AAT, KRT5, SFTPB, and LGALS3BP) with significant differential expression in preterm infants with severe lung disease with pulmonary hypertension when compared with infants with severe lung disease but no pulmonary hypertension. Pathway analysis of the integrated multiomic expression signatures revealed NFkB, VEGF, SERPINA1, IL6, and ERK1/2 as target molecules and cellular development, cellular growth and proliferation, and cellular movement as key affected molecular functions. Our multiomic analysis of tracheal aspirates revealed a comprehensive thumbprint of miRNAs, mRNAs, and proteins that could help endotype infants with severe lung disease and pulmonary hypertension.

Cardiac tumor in juvenile onset Behçet's disease: case report and review of the literature.

Behçet's disease is a chronic multisystemic inflammatory disorder characterized by orogenital ulcerations, uveitis, and occasional cardiac involvement. This report describes an atypical presentation of pediatric Behçet's disease with pulmonary emboli and a cardiac mass. A 16-year-old boy with a 2-year history of oral ulcers presented with weight loss, fever, joint pain, and a large tumor adhering to the free wall of the heart's right ventricle. Surgical biopsy demonstrated endomyocarditis with thrombus formation and led to the diagnosis. At the 2-year follow-up evaluation, intracardiac thrombus and pulmonic emboli had not recurred. Symptom control with immunosuppressive therapy was good.