Assessment of pulmonary hypertension in the pediatric catheterization laboratory: current insights from the Magic registry

Catheter Cardiovasc Interv. 2010 Nov 15;76(6):865-73. doi: 10.1002/ccd.22693.

Abstract

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.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Analysis of Variance
  • Anesthesia, General / adverse effects
  • Antihypertensive Agents
  • Blood Pressure*
  • Cardiac Catheterization* / adverse effects
  • Child
  • Child, Preschool
  • Familial Primary Pulmonary Hypertension
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / physiopathology
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Infant
  • Linear Models
  • Logistic Models
  • Predictive Value of Tests
  • Prospective Studies
  • Pulmonary Artery / physiopathology*
  • Registries
  • Risk Assessment
  • Risk Factors
  • Severity of Illness Index
  • Treatment Outcome
  • United States
  • Vasodilator Agents

Substances

  • Antihypertensive Agents
  • Vasodilator Agents