No abstract

Background: Critical coarctation of the aorta is the most common congenital heart disease

Background: Alteration in lung function is present in children with pulmonary arterial hypertension (PAH). We aimed to study the pulmonary function indices obtained by spirometry in pediatric patients with PAH, and to correlate them with B-type natriuretic peptide (BNP) and echocardiographic parameters. Methods: Thirty-one children with PAH were enrolled in this study, of those 26 patients (83.87%) associated shunt defect and 5 patients (16.12%) were diagnosed with idiopathic PAH. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC, peak expiratory flow rate (PEFR), forced expiratory flow at 25–75% of the pulmonary volume (FEF 25–75) were evaluated by spirometry and correlated with BNP and echocardiographic parameters that reflect right ventricle function. Results: Restrictive pattern of pulmonary function was present in 51.6% (n = 16) of all PAH children. There were significant correlations between BNP with FVC (p = 0.001), FEV1 (p = 0.001), and FEV1/FVC (p = 0.001). Serum BNP level was significantly increased in the group of patients without shunt. Of those echocardiographic parameters that reflect right ventricle function, we found that TAPSE significantly correlated with PEFR (r = −0.47, p <0.01), and with FEF 25–75 (r = −0.39, p <0.01). Conclusions: Deterioration of the pulmonary function indices are correlated with BNP and echocardiographic parameters, markers of RV dysfunction. Being easy and reliable tests, pulmonary functions can be introduced among the follow-up tools in children with PAH.

Background: Critical aortic coarctation is defined as the severe narrowing of the isthmic aortic lumen, representing a neonatal cardiac emergency, part of the congenital heart diseases with duct-dependent systemic circulation. Aim of the study: To assess the correlation between transthoracic echocardiography and computed tomography angiography (CTA) in the measurement of aortic diameters in a group of newborns diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch. Material and method: We performed a retrospective study on neonates diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch between January 1, 2015 and March 1, 2017. The studied parameters were diameters of the aorta at the level of the aortic annulus, coronary sinuses, sinotubular junction, ascending aorta, proximal and distal aortic arch, and the aortic isthmus. Measurements were obtained by transthoracic echocardiography and thoracic CTA. Results: Fifteen newborns diagnosed with duct-dependent aortic coarctation and/or associated hypoplastic aortic arch were included in this study. There was no statistically significant difference between the two imaging methods, the T test highlighting differences only between the measurements of the aortic annulus (p <0.016) and coronary sinuses (p <0.008). The patients included in the study associated other cardiovascular abnormalities: persistent ductus arteriosus (100%), atrial septal defect (100%), aortic arch hypoplasia (80%), bicuspid aortic valve (73.3%). Conclusions: These methods reveal important information on the anatomy of the cardiovascular malformation and its impact on the clinical and paraclinical status of the patient, being fundamental for establishing an optimal therapeutic approach.

Unilateral pulmonary artery agenesis is a rare congenital anomaly, that may develop in isolation, or in association with other congenital cardiovascular anomalies, such as tetralogy of Fallot, septal defects, right-sided aortic arch, or pulmonary atresia. Left-sided pulmonary artery agenesis is less frequent than the right-sided one. Diagnosis of unilateral pulmonary artery agenesis can be difficult. We report the case of a 15 year-old boy who presented with reduced exercise tolerance, shortness of breath and cyanosis. He was diagnosed with left pulmonary artery agenesis, associated with subaortic-ventricular septal defect, right-sided aortic arch, and severe pulmonary arterial hypertension (PAH), that precluded the surgical repair. Pulmonary vasodilator therapy was initiated in this case. The mortality rate of this rare anomaly is high due to its complications. It is essential to establish an early and correct diagnosis, in order to provide adequate treatment and prevent complications in this disease.