LAUSR

Pulmonary Hypertension (PH) represents infrequent, but unresolved paediatric issue. It leads to progressive right ventricular insufficiency, considerably declines children’s quality of life and causes premature death. Most cases of paediatric PH are associated with congenital heart disease (CHD). In recent years there has been significant progress in PH treatment, that describes this clinical case. Darya, 14 years old girl, has been observed by cardiologist since neonatal period with a CHD: right-formed right-located heart. Functionally uno-ventricle malformation (atresia of the tricuspid valve). Multiple ventricular septal defects (24 mm, 9 mm and 5 mm). Atrial septal defect (13 mm). Patent ductus arteriosus (6 mm). Heart failure (HF), class IV by NYHA, PH class III, atrial fibrillation. In the neonatal period dyspnea, nasolabial triangle cyanosis while feeding and poor weight increase took place. Patient`s mother refused from cardiac surgery in Moscow. The girl received hydrochlorothiazide, spironolactone, captopril, digoxin. Moderate signs of the HF and physical development delay were persisted. An increase in HF signs and hypoxemia was detected at the age of 1.5 years after respiratory infection (RI). Heart cavities catheterization with pulmonary artery (PA) blood pressure measuring and lung biopsy were performed in A.N.Bakulev cardiac surgery research institute. PH was diagnosed, therapy was corrected and surgical treatment in 6 months was recommended. Mother refused from surgery, the girl was treated by cardiologist. At the age of 6 cardiac re-catheterization has been carried out and the conclusion that surgical risk exceeds the risk of life without operation was given. Then the girl received nutritional support, digoxin, captopril, warfarin, spironolactone, furosemide constantly. Echocardiography was carried out in 2 years. But symptoms both of HF and PH were increased progressively. The end diastolic pressure (EDP) in LA increased from 21 mmHg at the age of 3 years to 49 mm Hg at 11 years. Sildenafil was additionally indicated in 2011. At the age of 11 years the girl was admitted to the hospital with bilateral segmental pneumonia associated with effusion pleurites, pericarditis with risk of cardiac tamponade, HF IV class NIHA, respiratory insufficiency II, PH class III, atrial fibrillation. Significant growth and weight deficiency and hypoxemia (SaO2 76%) were noted. On echocardiography EDP – 71 mmHg, left atrium – 71 mm (n=23 mm), left ventricle – 60 mm (n=37 mm), ejection fraction 45%, 6 min walk test – 290 m. Endothelin receptor antagonist bosentan was given in addition to conventional therapy. After 3 years PH signs without progression (EDP – 71 mmHg, 6 min walk test 390m).