study of some biochemical parameters of pulmonary hypertension in children with congenital and rheumatic heart disease

ABSTRACT

The study included 36 cases with congenital heart disease [CHD] all of them had left to right shunt [17 cases of them had pulmonary hypertension [PHT], 30 cases with valvular rheumatic heart disease [RHD] [15 of whom had PHT]. Ten healthy normal age-matched children were taken as control for each group. Full history and physical examination, pulse oximetry, standard 13-lead ECG and detailed echocardiographic examination were done for every patient and control case. Chest radiograph posterantrior view in erect position was done for all patients. Erythrocytic sedimentation rate, C-reactive rotein and antistreptolysin-O titer were done for patients with RHD. All cases and controls had the following done plasma level of endothelin-1 [ET-1], angiotesin-II [ANG-II], nitric oxide [NO] and serum level of angiotesin-converting enzyme [ACE] activity. The mean peak pulmonary pressure [PP] in patients having CHD and PHT was 54.35 +/- 9.69 mm Hg, while it was 61.73 +/- 11.55 mm Hg in those with RHD and RHT. The mean peak PP in the group of patients having CHD without PHT was 22.64 +/- 4.11 mm Hg. It was significantly higher than in those with RHD without PHT [9.41 +/- 1.98 mm Hg, P<0.05] and than controls [4.12 +/- 1.25, P<0.01]. It was observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in all cases with CHD [3.42 +/- 0.77, 77.21 +/- 29.14, 81.27 +/- 37.3, 107.2 +/- 47.65, P<0.001], cases with PHT [3.76 +/- 0.81, 94.1 +/- 21.1, 101.4 +/- 33.8, 136.2 +/- 37.9, P<0.001] OR without PHT [2.92 +/- 0.6,61.36 +/- 27.5, 63.28 +/- 16.5, 85.36 +/- 22.6, P<0.5 and 0.01] than in their controls [1.86 +/- 0.6, 34.07 +/- 10.7, 19.15 +/- 10.9, 54.1 +/- 32.1]. It was observed that the mean level of ET-1, NO, ANG-II and ACE were significantly higher in cases with CHD with PHT than in those without PHT [p<0.05 for each]. In the group of patients with RHD, it was observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in all cases [3.89 +/- 1.4, 78.46 +/- 59, 70.35 +/- 37.4, P<0.01 and 0.001] and cases with PHT [4.9 +/- 1.0, 100.5 +/- 32, 95.7 +/- 36.8, 121 +/- 40.9, P <0.001] than in controls [1.55 +/- 0.5, 33.47 +/- 13.0, 22.68 +/- 11.1, 53.27 +/- 32.4]. It was also observed that the mean levels of ET-1, NO, ANG-II and ACE were significantly higher in cases with than in those without PHT [2.6 +/- 0.8, 56.5 +/- 18.0, 44.96 +/- 12.7, 63.3 +/- 37] [P<0.01]. It was observed that the mean levels of ET-1, ANG-II and ACE were significantly higher in cases with CHD having heart failure [3.8 +/- 0.73, 106.8 +/- 50.37, 155.3 +/- 65.66] than those without heart failure [3.26 +/- 0.7, 69.88 +/- 25.1, 91.85 +/- 32.8], P<0.05 and 0.001. There was statistically significant positive correlation between PP in all the studied patients and serum level of ET-1, NO, ANG-II and ACE [P<0.001]. A significant positive corrlation was found between serum level of ET-1 and NO in all the studied cases [P<0.05]. A significant positive correlation was also found between serum level of ANG-II and ACE in all the studied cases [P<0.03]. ET-1 and NO production is increased in cases with PHT secondary to CHD with left to right shunt and to RHD and its production correlates with the level of PP. It could be also concluded that the level of ANG-II and ACE activity is increased in cases with PHT secondary to CHD with left to right shunt and to RHD and its production correlates with the level of PP. The use of NO, NO donors, prostacyclin and ET-1 antagonists should be considered in the management of pulmonary hypertension secondary to hyperkinetic arterial PHT due to CHD with left to right shunt and in the management of venous pulmonary hypertension secondary to RHD. The long-term effect of such drugs on the pathological process in such cases should be studied. Still early intervention in cases with hyperkinetic arterial PHT due to CHD with left to right shunt before the end of the first year of life is highly recommended. It may be also suggested that the use of ACE inhibitors could be useful to prevent the effects of ANG-II on the pulmonary vascular pathology