Publications by authors named "Sanne Boerman"
Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease and screening to detect pulmonary arteriovenous malformations (PAVMs) is important to prevent complications. In adults, transthoracic contrast echocardiogram (TTCE) is used to screen PAVMs. In children, a conservative screening method seems to be sufficient to rule out major PAVMs and prevent them from PAVM-related complications.
View Article and Find Full Text PDFTherapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH-specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts.
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- The study examines complications associated with balloon pulmonary angioplasty (BPA) in chronic thromboembolic pulmonary hypertension (CTEPH) patients, focusing on how pulmonary hemodynamics affect outcomes.
- Out of 87 patients treated with a total of 426 BPAs, complications were reported in 14% of procedures, primarily mild, with notable differences based on pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP).
- Patients with higher PVR (>6.6 WU) and mPAP (>45 mmHg) experienced significantly more complications and thoracic issues, emphasizing the relationship between poor pulmonary hemodynamics and increased risk during BPA.
Haploinsufficiency for Endoglin (ENG) and activin A receptor type II-like I (/ALK1) lead to the formation of weak and abnormal vessels in hereditary hemorrhagic telangiectasia (HHT). These cause epistaxis (nosebleeds) and/or gastrointestinal blood loss. In vitro in cultured endothelial cells, tacrolimus has been shown to increase ENG and ALK1 expression.
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