Mit der Neufassung der Mutterschafts-Richtlinien [2] wurden am 1. April 1995 drei Ultraschalluntersuchungen in die Mutterschaftsvorsorge aufgenommen und die damit verbundenen Screening-Leistungen (Mindestanforderungen) definiert. Mit diesem sog. 10 -20 -30 Schwangerschaftswochen-Screening sollte die Basis geschaffen werden, Auffälligkeiten früher und gezielter zu erkennen, um ggf. noch weitere diagnostische Maßnahmen in die Wege leiten zu können. Durch Konzentration von auffälligen Befunden in entsprechend ausgerichteten Pränatalzentren (3-Stufen-Konzept) [2] konnte innerhalb der letzten Jahre eine praxisrelevante Verbesserung der pränatalen Diagnostik erzielt werden [1]. Der Erfolg dieses Mehrstufenkonzeptes in der pränatalen Diagnostik ist gekennzeichnet durch eine fundierte Basissonographie, vorwiegend durch niedergelassene Frauenärzte und deren Zusammenarbeit mit qualifizierten Ultraschallspezialisten in pränataldiagnostisch ausgerichteten Zentren/Praxen.
Pulmonary hypertension (PH) is a progressive disease affecting patients across the life span.The pathophysiology primarily involves the pulmonary vasculature and right ventricle (RV), but eventually affects the left ventricular (LV) function as well. Safe, accurate imaging modalities are critical for diagnosis, serial monitoring, and tailored therapy. While cardiac catheterization remains the conventional modality for establishing diagnosis and serial monitoring, noninvasive imaging has gained considerable momentum in providing accurate assessment of the entire RV-pulmonary axis. In this state-of-the-art review, we will discuss the most recent developments in echocardiography, magnetic resonance imaging, and computed tomography in PH evaluation from pediatric to adult population.
While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD.
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