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Effect of pulmonary arterial hypertension specific therapy in the four clinical subgroups of patients with pulmonary arterial hypertension associated with congenital heart disease

Session Poster Session 4

Speaker Daniele Guarino

Congress : ESC Congress 2018

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Pulmonary Hypertension
  • Session type : Poster Session
  • FP Number : P3559

Authors : E Monti (Bologna,IT), A Rinaldi (Bologna,IT), F Dardi (Bologna,IT), M Palazzini (Bologna,IT), E Gotti (Bologna,IT), A Albini (Bologna,IT), E Zuffa (Bologna,IT), D Guarino (Bologna,IT), F Pasca (Bologna,IT), A De Lorenzis (Bologna,IT), A Cassani (Bologna,IT), M Orzalkiewicz (Bologna,IT), A Manes (Bologna,IT), N Galie' (Bologna,IT)

E. Monti1 , A. Rinaldi1 , F. Dardi1 , M. Palazzini1 , E. Gotti1 , A. Albini1 , E. Zuffa1 , D. Guarino1 , F. Pasca1 , A. De Lorenzis1 , A. Cassani1 , M. Orzalkiewicz1 , A. Manes1 , N. Galie'1 , 1University of Bologna, Department of Specialized, Diagnosticand Experimental Medicine – DIMES - Bologna/IT - Bologna - Italy ,

European Heart Journal ( 2018 ) 39 ( Supplement ), 731

Background: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a very heterogeneous disease. There are few published data on the effect of PAH-specific therapy in patients with PAH-CHD and they included only patients with Eisenmenger's syndrome (ES) and PAH after defect correction (DC).

Purpose: The aim of the study was to evaluate the effects of PAH-specific therapy in the 4 clinical subgroups of PAH-CHD patients: ES, PAH associated with prevalent systemic-to-pulmonary shunts, PAH with small/coincidental defects, DC.

Methods: From 1977 to December 2017 consecutive patients with PAH-CHD referred to our centre were included in the study. All patients underwent baseline clinical evaluation, six-minute walking distance (6MWD) and right heart catheterization. The same evaluations were performed before and 3–4 months after the beginning of a new PAH-specific drug initiated in our centre. Changes in 6MWD and haemodynamic parameters were analyzed using Wilcoxon signed-rank test and compared between the 4 clinical subgroups of PAH-CHD patients with Kruskal-Wallis test. Data are presented as median (interquartile range).

Results: 231 consecutive PAH-CHD patients (50% ES, 19% S/P, 6% SD, 25% DC) were enrolled. Patients with complex CHD were excluded from the analysis. Median follow-up was 117 (54–275) months. 102 patients began monotherapy (55 ES, 18 S/P, 7 SD, 22 CS) and 82 patients associated double combination therapy in our centre (44 ES, 10 S/P, 5 SD, 23 CS). Patients who received triple combination therapy were not analyzed because of the small size of the sample. Results are shown in the Table.

Conclusions: Initial monotherapy and double sequential combination therapy were effective in improving haemodynamic profile and exercise capacity in patients with PAH-CHD without any significant difference between the four clinical subgroups.

Table 1
Pre-MonoPost-Monop-valuep between 4 PAH-CHD clinical subgroups – MonoPre-DoublePost-Doublep-valuep between 4 PAH-CHD clinical subgroups – Double
6MWD (m)403 (340–477)444 (375–529)<0.0010.560451 (402–549)489 (426–559)<0.0010.922
RAP (mmHg)7 (5–10)8 (7–10)0.0620.1479 (6–11)8 (6–10)0.4720.899
mPAP (mmHg)70 (56–89)67 (54–83)<0.0010.57974 (60–88)69 (56–86)<0.0010.682
mBP (mmHg)87 (79–98)86 (78–94)0.3460.21484 (76–93)82 (76–90)0.0190.600
Pulmonary CI (l/min/m2)2.2 (1.6–3.2)2.7 (2–3.3)<0.0010.8192.4 (1.9–3.1)2.7 (2.3–3.5)<0.0010.792
PVR (W.U.)17.9 (10.6–24.4)13.3 (9.9–20.4)<0.0010.29114.5 (11–25.6)12.7 (8.4–20)<0.0010.349
SVR (W.U.)22.7 (18.4–32.3)20.8 (16.3–25.3)<0.0010.54720.2 (15.9–25.2)18.9 (14.7–24)0.0060.846
Legend: CI, cardiac index; mBP, mean blood pressure; mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; SVR, systemic vascular resistance 6MWD, 6 minute walking distance.

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