» Articles » PMID: 37427091

Factors Leading to Supranormal Cardiac Index in Pediatric Pulmonary Hypertension Patients Treated with Parenteral Prostanoid Therapy

Overview
Journal Pulm Circ
Publisher Wiley
Specialty Pulmonary Medicine
Date 2023 Jul 10
PMID 37427091
Authors
Affiliations
Soon will be listed here.
Abstract

Parenteral prostanoid therapy (PPT) can result in supranormal cardiac index (SCI; >4 L/min/m) in pediatric pulmonary hypertension (PPH) patients. We evaluated the incidence, hemodynamic factors, and outcomes associated with SCI in PPH. This retrospective cohort study included 22 PPH patients on PPT from 2005 to 2020. Hemodynamic profiles were compared between the baseline and 3-6 month follow-up catheterization in the SCI and non-SCI cohorts. Cox regression analysis examined time to composite adverse outcome (CAO; Potts shunt, lung transplant, or death) controlling for initial disease severity. SCI developed in 17 (77%) patients, of whom 11 (65%) developed SCI within 6 months. The SCI cohort was characterized by significant augmentation of cardiac index (CI) and stroke volume (SV) as well as reductions in systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR). Conversely, the non-SCI cohort had unchanged SV despite a modest rise in CI as well as persistent vasoconstriction. After median follow-up of 4.3 years (range 0.2-13 years), non-SCI patients were at significantly increased risk for the CAO (5/5: three deaths, two Potts shunts) compared with SCI patients (5/17: two deaths, three lung transplants; adjusted hazard ratio 14.0 [95% confidence interval: 2.1-91.3],  < 0.001). A majority of PPH patients developed SCI within 6-12 months of starting PPT and demonstrated lower risk of adverse outcomes compared with non-SCI patients. These data suggest that change in SVR and SV after 3-6 months of PPT may be early markers of therapeutic response and prognosis.

Citing Articles

Factors leading to supranormal cardiac index in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy.

Miles K, Critser P, Evers P, Cash M, Magness M, Geers E Pulm Circ. 2023; 13(3):e12264.

PMID: 37427091 PMC: 10323166. DOI: 10.1002/pul2.12264.

References
1.
Yung D, Widlitz A, Rosenzweig E, Kerstein D, Maislin G, Barst R . Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation. 2004; 110(6):660-5. DOI: 10.1161/01.CIR.0000138104.83366.E9. View

2.
de Man F, Tu L, Handoko M, Rain S, Ruiter G, Francois C . Dysregulated renin-angiotensin-aldosterone system contributes to pulmonary arterial hypertension. Am J Respir Crit Care Med. 2012; 186(8):780-9. PMC: 5104838. DOI: 10.1164/rccm.201203-0411OC. View

3.
van Loon R, Roofthooft M, Delhaas T, van Osch-Gevers M, Ten Harkel A, Strengers J . Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol. 2010; 106(1):117-24. DOI: 10.1016/j.amjcard.2010.02.023. View

4.
Moledina S, Pandya B, Bartsota M, Mortensen K, McMillan M, Quyam S . Prognostic significance of cardiac magnetic resonance imaging in children with pulmonary hypertension. Circ Cardiovasc Imaging. 2013; 6(3):407-14. DOI: 10.1161/CIRCIMAGING.112.000082. View

5.
Oudiz R, Farber H . Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am Heart J. 2009; 157(4):625-35. DOI: 10.1016/j.ahj.2008.10.029. View