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Pathogenic Glomulin Gene Variant in a Patient with Idiopathic Pulmonary Arterial Hypertension: A Novel Association Case Report [PDF]
ReportsBackground and Clinical Significance: Idiopathic pulmonary arterial hypertension is a rare disorder, often linked to genetic predisposition. Canonical pulmonary arterial hypertension genes such as BMPR2, KCNK3, and TBX4 are well described, but novel ...
Ilias E. Dimeas +5 more
doaj +2 more sources
Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey [PDF]
Pediatric Reports, 2023 Idiopathic pulmonary arterial hypertension (IPAH) is an uncommon and severe disease. We report the case of a 7-year-old boy investigated for cardiac murmur and exercise intolerance.
Tanguy Dutilleux +4 more
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Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension? [PDF]
Curr Opin Pulm Med, 2023 Purpose of review Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and treatment strategies for SSc-associated PAH (SSc-PAH) are informed by those used to treat patients with idiopathic PAH (IPAH).
Khan SL, Mathai SC.
europepmc +3 more sources
Pulmonary Artery Aneurysm in Longstanding Idiopathic Pulmonary Arterial Hypertension. [PDF]
Dtsch Arztebl Int, 2023 Hinze CA, Park DH, Bollmann BA.
europepmc +3 more sources
Cardiac sympathetic dysfunction in pulmonary arterial hypertension: lesson from left-sided heart failure [PDF]
Pulmonary Circulation, 2019 Sympathetic nervous system hyperactivity has a well-recognized role in the pathophysiology of heart failure with reduced left ventricular ejection fraction.
Valentina Mercurio +10 more
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Idiopathic pulmonary arterial hypertension [PDF]
Disease Models & Mechanisms, 2010 Despite improved understanding of the pathobiology of pulmonary arterial hypertension (PAH), it remains a severe and progressive disease, usually culminating in right heart failure, significant morbidity and early mortality. Over the last decade, some major advances have led to substantial improvements in the management of PAH.
Amy L, Firth +2 more
+6 more sources
Reverse Potts for the Treatment of Severe Idiopathic Pulmonary Hypertension in Children [PDF]
Brazilian Journal of Cardiovascular Surgery, 2023 Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment.
Marcelo Frederigue de Castro +5 more
doaj +1 more source
Pulmonary hypertension associated with neurofibromatosis type 2
Pulmonary Circulation, 2021 Although precapillary pulmonary hypertension is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown.
Hirohisa Taniguchi +9 more
doaj +1 more source
Pulmonary Circulation, 2020 Platelet-derived growth factor is one of the major growth factors found in human and mammalian serum and tissues. Abnormal activation of platelet-derived growth factor signaling pathway through platelet-derived growth factor receptors may contribute to ...
Kang Wu +21 more
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Nature Communications, 2021 Idiopathic pulmonary arterial hypertension is a rare and fatal disease with a heterogeneous treatment response. Here the authors show that unsupervised machine learning of whole blood transcriptomes from 359 patients with idiopathic pulmonary arterial ...
Sokratis Kariotis +34 more
doaj +1 more source