Inhaled pharmacotherapies are promising treatment options for patients with pulmonary arterial hypertension (PAH) as they minimize extrapulmonary adverse effects. Recently, we developed a highly specific inhibitor (TPHi) of the serotonin synthesizing enzyme tryptophan hydroxylase 1, TPT-004. We hypothesized that repetitive nose-only inhalation of TPT-004 alleviates PAH and pulmonary vascular remodeling in the Sugen-hypoxia (SuHx) rat model.
View Article and Find Full Text PDFCoarctation of the aorta (CoA) is a potentially life-threatening congenital and obstructive anomaly of the distal aortic arch. After constriction of the ductus arteriosus, neonates may develop critical CoA in the isthmus area and present with severe left ventricular dysfunction or even cardiac failure. Low cardiac output and abdominal hypoperfusion (distal to the coarctation) may lead to metabolic derangements and clinical deterioration.
View Article and Find Full Text PDFChildren with left heart disease are at risk for developing pulmonary hypertension, initially secondary to pulmonary venous hypertension that can progress to include elevated pulmonary vascular resistance, known as combined pre- and postcapillary pulmonary hypertension. Elevated pulmonary vascular resistance may pose a risk to the right ventricle of a newly transplanted heart because of increased afterload and is an important consideration for heart transplant eligibility. However, the epidemiology, pathophysiology, optimal diagnostic and treatment approaches, and thresholds for pulmonary vascular resistance in pulmonary hypertension associated with left heart disease remain unclear because of lack of evidence, particularly in pediatrics.
View Article and Find Full Text PDFThe serotonin pathway has long been proposed as a promising target for pulmonary arterial hypertension (PAH)-a progressive and uncurable disease. We developed a highly specific inhibitor of the serotonin synthesizing enzyme tryptophan hydroxylase 1 (TPH1), TPT-001 (TPHi). In this study, the authors sought to treat severe PAH in the Sugen/hypoxia (SuHx) rat model with the oral TPHi TPT-001.
View Article and Find Full Text PDFEur Heart J Cardiovasc Imaging
November 2024
Cardiovascular magnetic resonance (CMR) imaging is recommended in patients with congenital heart disease (CHD) in clinical practice guidelines as the imaging standard for a large variety of diseases. As CMR is evolving, novel techniques are becoming available. Some of them are already used clinically, whereas others still need further evaluation.
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