Assessment of pulmonary hypertension in the pediatric catheterization laboratory: Current insights from the magic registry

Objectives: To assess protocols, demographics, and hemodynamics in pediatric patients undergoing catheterization for pulmonary hypertension (PH). Background: Pediatric specific data is limited on PH. Methods: Review of the Mid‐Atlantic Group of Interventional Cardiology (MAGIC) collaboration PH registry dataset. Results: Between November 2003 and October 2008, seven institutions submitted data from 177 initial catheterizations in pediatric patients with suspected PH. Pulmonary arterial hypertension associated with congenital heart disease (APAH‐CHD) ( n = 61, 34%) was more common than idiopathic PAH (IPAH) ( n = 36, 20%). IPAH patients were older with higher mean pulmonary arterial pressures (mPAP) ( P < 0.01). Oxygen lowered mPAP in patients with IPAH ( P < 0.01) and associated PAH not related to congenital heart disease (APAH‐non CHD) ( P < 0.01). A synergistic effect was seen with inhaled Nitric Oxide (iNO) ( P < 0.01). Overall 9/30 (29%) patients with IPAH and 8/48 (16%) patients with APAH‐non CHD were reactive to vasodilator testing. Oxygen lowered pulmonary vascular resistance index (PVRI) in patients with APAH‐CHD ( P < 0.01). There was no additive effect with iNO but a subset of patients required iNO to lower PVRI below 5 WU·m 2 . General anesthesia (GA) lowered systemic arterial pressure ( P < 0.01) with no difference between GA and procedural sedation on mPAP or PVRI. Adverse events were rare ( n = 7) with no procedural deaths. Conclusions: Pediatric patients with PH demonstrate a higher incidence of APAH‐CHD and neonatal specific disorders compared to adults. Pediatric PH patients may demonstrate baseline mPAP < 40 mm Hg but > 50% systemic illustrating the difficulty in applying adult criteria to children with PH. Catheterization in children with PH is relatively safe. © 2010 Wiley‐Liss, Inc.