Publications by authors named "Sophie-Guiti Malekzadeh-Milani"
Background: The primary genetic risk factor for heritable pulmonary arterial hypertension is the presence of monoallelic mutations in the gene. The incomplete penetrance of mutations implies that additional triggers are necessary for pulmonary arterial hypertension occurrence. Pulmonary artery stenosis directly raises pulmonary artery pressure, and the redirection of blood flow to unobstructed arteries leads to endothelial dysfunction and vascular remodeling.
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- Right heart catheterization (RHC) is a high-risk procedure for children with pulmonary arterial hypertension, and this study aims to clarify its purpose and assess how well noninvasive indicators align with hemodynamic outcomes.
- The study analyzed data from 71 untreated children with pulmonary arterial hypertension, identifying specific hemodynamic metrics, like pulmonary vascular resistance index and right atrial pressure, that were associated with negative outcomes such as death and transplantation.
- While noninvasive criteria can suggest changes in hemodynamics, about 70% of children who showed improvement still had risk factors when reassessed with RHC, underscoring the importance of repeat procedures for ongoing risk evaluation.
Aortic stenosis is a complex heart disease that involves the aortic valve and the left ventricle. Impairment of the left ventricle, abnormalities in its size, systolic and diastolic function determine the postnatal outcomes in the same way as the aortic valve. In the most severe forms, the left ventricle cannot provide systemic circulation at birth and the physiology is that of hypoplastic left heart syndrome.
View Article and Find Full Text PDFPurulent pericarditis is an exceptionally rare complication of pneumococcal pneumonia in infants but a rapidly fatal disease if left untreated. A previously healthy 4-month-old boy presented at our emergency department with a 10-day history of fever and non-productive cough. No signs of heart failure or cardiac friction rub were evidenced.
View Article and Find Full Text PDFBackground: In previous studies, an attenuated heart rate response to exercise has been noted in patients after surgical closure of atrial septal defect. The aim of this study was to compare the prevalence of chronotropic impairment after surgical and percutaneous closure of atrial septal defect.
Methods: Thirty-eight pediatric patients who underwent a surgical (group A, n = 18) or transcatheter closure (group B, n = 20) of atrial septal defect in our institution were prospectively included in the study.