Association of Hemodynamic Profiles With Wait-List Mortality in Children Listed for Heart Transplantation With Idiopathic Dilated Cardiomyopathy

The prognostic significance of intracardiac hemodynamics in children with advanced heart failure is unknown. The purpose of this study was to describe hemodynamic profiles in children with idiopathic dilated cardiomyopathy (IDC) listed for heart transplant (HT) and to assess their association with wait-list mortality. We identified all US children <18 years with IDC listed for HT during 2000 to 2010 with available pulmonary capillary wedge pressure (PCWP) and cardiac index (CIx) data. We excluded children on ventilator or mechanical support at listing. CIx >2.2 L/min/m2 (warm) and PCWP >18 mm Hg (wet) were used to define 4 hemodynamic profiles: warm-dry, warm-wet, cold-dry, and cold-wet. The primary end point was death on the wait-list or becoming too sick to transplant. Of 476 children analyzed, 248 (52%) children had PCWP >18 mm Hg and 300 (63%) had CIx >2.2 L/min/m2. Overall, 36% children were warm-dry, 27% were warm-wet, 12% were cold-dry, and 25% were cold-wet; 32 (6.7%) children reached the primary end point. In adjusted analysis, cold-dry (hazard ratio [HR] 3.5, 95% confidence interval [CI] 1.1, 11.5) and cold-wet (HR 3.2, 95% CI 1.2, 8.6) children were at higher risk of wait-list death versus warm-dry children, whereas warm-wet children were not (HR 2.3, 95% CI 0.8, 6.6). All groups were equally likely to receive HT and had similar 1-year post-transplant survival. In conclusion, in children with IDC listed for HT, those with low cardiac output at evaluation are at higher risk of wait-list mortality. Defining hemodynamic profiles may improve risk stratification of children with IDC listed for HT.