Use of ATP-MgCl2 in the evaluation and treatment of children with pulmonary hypertension secondary to congenital heart defects.

Abstract

Background Pulmonary hypertension results in increased morbidity and mortality in children after surgical repair of congenital heart defects. Various vasodilators have been unsuccessful in providing preferential pulmonary vasodilation in these patients. Identification of a more preferential pulmonary vasodilator would improve the assessment, management, and outcome of these children. To determine whether ATP-MgCl2 is a preferential pulmonary vasodilator in children with pulmonary hypertension secondary to congenital heart defects, ATP-MgCl2 was administered during routine cardiac catheterization, and the effects were compared with tolazoline. In addition, ATP-MgCl2 was infused intravenously during episodes of postoperative pulmonary hypertension.Methods and Results During cardiac catheterization in 28 children, the effect of ATP-MgCl2 on the pulmonary artery

Nitric Oxide–Dependent Feedback Loop Regulates Transient Receptor Potential Vanilloid 4 (TRPV4) Channel Cooperativity and Endothelial Function in Small Pulmonary Arteries

“…The discovery of ATP as an endogenous activator of TRPV4‐eNOS signaling may be a crucial step towards deciphering the physiological and pathological roles of TRPV4 channels in the pulmonary circulation. Purinergic signaling is an essential component to the pulmonary vasculature,59, 60, 61, 62, 63 and ATP activation of TRPV4 channels represents a mechanism that may link physiological stimuli to the regulation of pulmonary vascular function. ATP can be released from sympathetic nerves during synaptic transmission, or can be released into the circulation by ECs, SMCs, or erythrocytes 25, 26, 64, 65.…”

Cardiac purinergic signalling in health and disease

“…Pulmonary hypertension is a serious problem in children with congenital heart defects. ATP-MgCl 2 (0.05, 0.1, and 0.2 mg of ATP/kg/min) was a safe and effective pulmonary vasodilator in children with pulmonary hypertension, secondary to congenital heart defects [816].…”

Pathophysiology and Therapeutic Potential of Purinergic Signaling

“…ATP is a rapidly acting hypotensive agent that compares favorably with sodium nitroprusside (Kien et al, 1987). ATP-MgCl 2 is a safe, effective, and preferential pulmonary vasodilator in children with pulmonary hypertension secondary to congenital heart defects; it has also been used for treating pulmonary hypertension after cardiac surgery (Brook et al, 1994). Red blood cells of humans with primary pulmonary hypertension fail to release ATP in response to mechanical deformation (Sprague et al, 2001).…”

“…Low-dose ATP exerts mitogenic actions on human pulmonary artery smooth muscle cells, which may be relevant to the pathophysiological basis of severe pulmonary hypertension (Zhang et al, 2004b). Pulmonary hypertension can be a problem in patients with chronic obstructive pulmonary disease, which also has other causes; it is a life-threatening condition, and intravenous ATP infusion produces a significant decrease in mean pulmonary arterial pressure and pulmonary vascular resistance without changing the mean systemic arterial pressure (Brook et al, 1994). ATP has been used to treat meconium aspiration-induced pulmonary hypertension in pigs (Kääpä et al, 1997).…”