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
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Coronary pathophysiology in idiopathic pulmonary arterial hypertension. [PDF]
JCI InsightBACKGROUND Idiopathic pulmonary arterial hypertension (IPAH) alters right ventricular size and function, curtailing life expectancy. Patients may experience angina and myocardial ischemia. However, the underlying mechanisms are poorly understood. METHODS
Boland E +11 more
europepmc +3 more sources
Asymmetrical Dimethylarginine in Idiopathic Pulmonary Arterial Hypertension [PDF]
Arteriosclerosis, Thrombosis, and Vascular Biology, 2005 Objective— We explored the potential role of the endogenous NO synthase inhibitor asymmetrical dimethylarginine (ADMA) in patients with idiopathic pulmonary arterial hypertension (IPAH). Method and Results— We correlated plasma
Jan Kielstein +2 more
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Pulmonary Circulation, 2019 Idiopathic pulmonary arterial hypertension is a rare and life-shortening condition often diagnosed at an advanced stage. Despite increased awareness, the delay to diagnosis remains unchanged.
David G. Kiely +10 more
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Idiopathic pulmonary arterial hypertension and co-existing lung disease: is this a new phenotype?
Pulmonary Circulation, 2020 Patients classified as idiopathic pulmonary arterial hypertension (defined as Group 1 on European Respiratory Society (ERS)/European Cardiac Society (ESC) criteria) may have evidence of minor co-existing lung disease on thoracic computed tomography.
Andrew J. Peacock +16 more
doaj +2 more sources
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
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
doaj +2 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
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
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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