PT  - JOURNAL ARTICLE
AU  - Mario Gerges
AU  - Christian Gerges
AU  - Marie Lang
AU  - Irene Lang
TI  - Hemodynamic assessment of pulmonary hypertension in grown-up congenital heart disease
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - 2830
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/2830.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/2830.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - PURPOSE: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) is included in group 1 of the pulmonary hypertension (PH) clinical classification. The persistent exposure of the pulmonary vasculature to increased blood flow due to systemic-to-pulmonary shunts as well as increased pressure may result in a typical pulmonary arteriopathy that leads to an increase in invasively measured mean pulmonary arterial pressure (mPAP) ≥25mmHg at rest.METHODS: 3107 right and left heart catheterizations were analyzed. Diagnoses were validated on the grounds of patient histories, imaging, clinical data and patho-anatomic evidence (2369 complete data sets). 257 data sets were from patients with CHD.RESULTS: Underlying diagnoses were: pre-tricuspid defects in 172 patients, post-tricuspid defects in 38 patients and complex lesions in 47 patients. Of the 257 patients with CHD (38 were corrected), 141 patients had normal hemodynamics (“Non-PH” mPAP<25mmHg). Of the remaining 116 patients with PH (with wedge tracings missing or insufficient in 19 cases), 51 qualified as pre-capillary PH (CHD-PAH; PCWP≤15mmHg), 46 had CHD with elevated left ventricular filling pressures (CHD-PH; mPCWP>15mmHg; Table 1).View this table:Table 1. Hemodynamic data of patients with CHD and pulmonary hypertensionCONCLUSION: The data demonstrate that a significant proportion (almost 50%) of patients with PH in grown-up congenital heart disease suffer from post-capillary pulmonary hypertension.