Evaluation of preterm infants having bronchopulmonary dysplasia with echocardiography and serum biomarkers.

BACKGROUND AND OBJECTIVES: Pulmonary hypertension is frequent in infants with bronchopulmonary dysplasia. Echocardiography is easy to perform, non-invasive, and recommended by guidelines even though solely it is not enough. Catheterisation is gold standard but invasive, expensive, and not cost effective. Therefore, we aimed to assess to find out the role of biomarkers besides echocardiography in the diagnosis of pulmonary hypertension in preterm with bronchopulmonary dysplasia. METHODS: This study is done during the time period January 2016-2017. The diagnosis of pulmonary hypertension was assessed by echocardiography at 36 weeks later repeated at 3rd and 6th months. We also repeated biomarkers at 3rd and 6th months. The infants born ≤ 28 weeks in Erciyes University hospital who were diagnosed bronchopulmonary dysplasia were included. Infants with genetic syndromes, structural lung, and CHDs were excluded. Patients without bronchopulmonary dysplasia but having pulmonary hypertension due to other reasons and patients having echocardiograms without adequate images were excluded. RESULTS: At initial, 21/59 patients had bronchopulmonary dysplasia-pulmonary hypertension (Group 1), 21/59 had no bronchopulmonary dysplasia-pulmonary hypertension (Group 2), and 17/59 had bronchopulmonary dysplasia without pulmonary hypertension (Group 3). Systolic pulmonary artery pressure and pulmonary vascular resistance were found high in Group 1 (36 mmHg; p <0.001, 1.25 Woods Unit; p < 0.0017, respectively). Tricuspid annular plane systolic excursion values of Group 1 were low. Median serum kallistatin levels of Group 1 were lower than the other groups (230.5 (114.5-300.5) µg/ml; p < 0.005). During the study period, pulmonary hypertension of 14/21 bronchopulmonary dysplasia-pulmonary hypertension resolved, six patients in Group 3 developed pulmonary hypertension. However, there was no difference in the biomarkers of these six patients. CONCLUSION: In the diagnosis and the follow-up of pulmonary hypertension in bronchopulmonary dysplasia patients, besides echocardiography kallistatin, gelsolin, NT-probrain natriuretic peptide, homocysteine, and cystatin-C levels can be used. Further studies were required with large sample sizes.

Early onset marfan syndrome: Atypical clinical presentation of two cases.

Early onset Marfan Syndrome (eoMFS) is a rare, severe form of Marfan Syndrome (MFS). The disease has a poor prognosis and most patients present with resistance to heart failure treatment during the newborn period. This report presents two cases of eoMFS with similar clinical features diagnosed in the newborn period and who died at an early age due to the complications related to the involvement of the cardiovascular system.

Cardiovascular changes in children with coarctation of the aorta treated by endovascular stenting.

The aim of this study was to investigate the echocardiographic, biochemical short- and mid-term effects of the stenting procedure on left ventricular function, aortic stiffness, elasticity and systemic hypertension in children with coarctation of the aorta (CoA). Fifteen patients with native or recurrent CoA and 30 healthy controls who were sex and age matched were included in the study. The blood pressure values, echocardiographic measurements, elastic functions of ascending aorta and serum N-Terminal ProBNP (NT-ProBNP) levels were recorded prospectively before and at the first and sixth month after stenting. The mean arterial pressure recorded before stenting was 134.4±16.3 mm Hg; at the sixth month it was 115.5±9.5 mm Hg and in the control group it was 107.3±9.4 mm Hg. Although blood pressure levels were lower compared with the pre-stenting measurements (P<0.05), they were still significantly higher compared with the control group (P<0.05). Although a significant reduction was detected in the LVMIz at the end of the sixth month (50.4±14.3 g m(-2.7)) compared with the baseline (66.6±17.9 g m(-2.7); P<0.05), it was still higher compared with the control group (35.7±6.2 g m(-2.7); P<0.05). The baseline aortic elasticity (6.4±3.4 cm(2) dyn(-1) 10(-6)) was lower compared with the control group (10.0±1.7 cm(2) dyn(-1) 10(-6); P<0.05), and prestenting aortic stiffness was higher than that of the control group (5.6±1.6 dyn(-1) 10(-6); 2.5±0.45 dyn(-1) 10(-6); P<0.05). A statistically significant negative correlation was detected between the pressure gradient at the lesion site and aortic elasticity (r: -0.53, P: 0.04). Although resolution of the coarctation by endovascular stenting led to a reduction in the arteriopathy that had already begun before treatment, it was demonstrated that these children did not completely return to normal.