Airway spasm or collapse? An alternate mechanism of action for an important side effect of Sildenafil warrants consideration in patients with tracheobronchomalacia

Cohen et  al report that 2 of 269 pediatric study participants with pulmonary hypertension discontinued sildenafil owing to “airway spasm” described as “episodic desaturation associated with decreased air entry on physical examination, unresponsive to bronchodilators, and require(ing) aggressive baggi ng for resolution of symptoms.”1 Of note, 1 of the 2 patients referenced had severe tracheobronchomalacia. It was hypothesized that sildenafil caused edema in an already compromised airway, leading to episodic occlusion.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Letters to the Editor Source Type: research

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AbstractPulmonary hypertension (PH) is a complex and progressive disease characterized by increased pulmonary vascular resistance and pulmonary artery pressure, leading to pulmonary vascular remodeling, right heart failure, and ultimately death. Although several molecular pathways related to vascular remodeling have been reported, the mechanism behind this fatal disease with poor prognosis remains largely unclear. Long noncoding RNAs (lncRNAs) have been proven to be playing crucial roles in various biological processes, and emerging studies demonstrated lncRNAs as essential epigenetic modifiers involved in the onset and de...
Source: Heart Failure Reviews - Category: Cardiology Source Type: research
ConclusionsThis model for sildenafil in CDH patients shows that concentration-targeted sildenafil dosing of 0.4 mg/kg in 3 h, followed by 1.6 mg/kg/day continuous infusion achieves appropriate sildenafil plasma levels.
Source: European Journal of Clinical Pharmacology - Category: Drugs & Pharmacology Source Type: research
Pulmonary hypertension (PH) often complicates chronic left-sided heart failure, with a remarkable impact on quality of life, exercise capacity, and survival. PH in chronic left-sided heart failure (PH-LHD) is not only caused by backward transmission of pressures but also involves impairment of atrial function, inflammation, and vasoconstriction. Once the left atrium loses its reservoir capacity, usually pulmonary vascular resistances increase. Right atrial dilation commonly represents the first sign of PH-LHD, before right ventricle dilatation and systolic dysfunction develop, leading to right heart insufficiency, and ulti...
Source: Heart Failure Clinics - Category: Cardiology Authors: Source Type: research
We describe the case of a 3-year-old autistic boy with undiagnosed scurvy who was scheduled for cardiac catheterization. Immediately after induction, the patient became hemodynamically unstable, which worsened with administration of nitrous oxide. Cardiac catheterization revealed pulmonary hypertension, which dramatically improved with administration of vitamin C. Anesthesiologists should be aware that scurvy is more common than previously thought, even in developed countries and can cause unexpected circulatory collapse from pulmonary hypertensive crisis.
Source: A&A Case Reports - Category: Anesthesiology Tags: Case Reports Source Type: research
Conclusions:Cardiopulmonary complications are common in patients with scleroderma, one of which may be sleep-disordered breathing. In our cohort, approximately one-third of individuals with scleroderma had evidence of sleep-disordered breathing. Moreover, the rate of sleep-disordered breathing in our population of scleroderma patients was twice the rate of pulmonary hypertension and was approximately the same as the rate of interstitial lung disease. Future prospective studies are needed to further assess the role of sleep-disordered breathing in scleroderma clinical outcomes.Citation:Nokes BT, Raza HA, Cartin-Ceba R, Lyng...
Source: Journal of Clinical Sleep Medicine : JCSM - Category: Sleep Medicine Source Type: research
This study was designed to evaluate the regulatory roles of miR-200b in cor pulmonale. Cor pulmonary mouse model was built via monocrotaline injection of monocrotaline (MCT). The expression of miR-200b in the lungs, RV and left ventricle (LV) using real-time polymerase chain reaction. The transthoracic echocardiography was employed to determine the effects of miR-200b mimics and Gö6976 injection on MCT mice. The protein levels of protein kinase C α (PKCα), collagen, and fibronectin in the lung, RV, and LV in the mice with and without miR-200b mimics and Gö6976 injection were evaluated using western bl...
Source: Clinical and Experimental Pharmacology and Physiology - Category: Drugs & Pharmacology Authors: Tags: Clin Exp Pharmacol Physiol Source Type: research
CONCLUSION: In this high-risk cohort of patients with SSc, a positive EE may predict the future development of resting PH. In addition, a majority of patients may have a persistently positive EE for years without progression to resting PH. Finally, a consistently negative EE may identify patients at low risk for future PH. PMID: 31732551 [PubMed - as supplied by publisher]
Source: J Rheumatol - Category: Rheumatology Authors: Tags: J Rheumatol Source Type: research
Contributors : Christian Hiepen ; Jerome Jatzlau ; Petra KnausSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensBalanced TGF β/BMP-signalling is essential for tissue formation and homeostasis. While gain in TGFβ signaling is often found in diseases, the underlying cellular mechanisms remain poorly defined. Here we show, that the receptor BMPR2 serves as a central gatekeeper of this balance, highlighted by its deregulati on in diseases such as pulmonary arterial hypertension (PAH). We show that BMPR2-deficient cells express genes indicative for altered biophysical properties in...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
Publication date: Available online 13 November 2019Source: Pharmacological ResearchAuthor(s): Aline C. Oliveira, Elaine M. Richards, Mohan K. RaizadaAbstractPulmonary hypertension (PH) is classically considered a disease of pulmonary vasculature which has been the predominant target for drug development and pH therapy. Despite significant advancement in recent years in identification of new drug targets and innovative treatment strategies, the prognosis of pH remains poor, with median survival of 5 years. Recent studies have demonstrated involvement of neuroinflammation, altered autonomic and gastrointestinal functions and...
Source: Pharmacological Research - Category: Drugs & Pharmacology Source Type: research
Authors: Glöckl R, Schneeberger T, Boeselt T, Kenn K, Koczulla AR, Held M, Oberhoffer R, Halle M Abstract BACKGROUND:  Pulmonary hypertension (PH) is defined as an elevation of mean pulmonary-arterial pressure by> 20 mmHg at rest, which may lead to right heart failure. Physical exercise has not been regularly recommended for PH patients for fear of symptom deterioration or occurrence of exercise-induced adverse events. METHODS:  Three electronic databases were searched for randomized, controlled trials investigating exercise training in PH patients using the following keywords: "pulmo...
Source: Pneumologie - Category: Respiratory Medicine Tags: Pneumologie Source Type: research
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