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Clinical observation

In order to focus attention on this problem of pediatricians, pediatric cardiologists and pulmonologists, we present an interesting clinical case of PAH on the background of a combination of BPD and congenital heart disease in a child with EBMT at birth.

A female child A., from the 1st pregnancy, which proceeded with the threat of termination in the first and second half, was born from 1 premature birth at the 26th week. gestation, by operation “cesarean section”, with a mass of 870 g and a length of 34 cm, with an Apgar score of 4/6 points.

From the first hours of her life, the girl had a serious condition with the manifestation of respiratory distress syndrome. Resuscitation measures were carried out using mechanical ventilation, oxygen therapy, surfactant replacement therapy, and a couvez regimen. On the 2nd day of life, a hemodynamically significant open ductus arteriosus (OAI) was detected, 3 doses of ibuprofen were used intravenously according to the usual scheme, without a positive effect.In the dynamics of manifestations of heart failure increased. In this regard, the child received cardiac glycosides, dopaminomimetics, β-adrenomimetics (dopamine, digoxin, dobutamine). With this therapy, the condition stabilized somewhat, although it remained severe. Persistent respiratory failure of the III degree was noted, anemia (Hb 102 g / l), hematuria were noted from the 3rd day, fine bubbling rales and crepitus were heard in the lungs on both sides. On the 8th day of life, radiological changes were revealed in the form of a bilateral decrease in pneumatization due to gentle infiltrative shading in both lungs with an increase in the bronchovascular pattern due to the interstitial component, leukocytosis (21.0 × 109 / L), there was a negative dynamics in blood tests per 1 th week life (increased leukocytosis, ESR, decreased hemoglobin).

Chest x-ray (direct projection) on the 80th day of life. (From the archive of E.S. Truntsova)
The diagnosis was made: Neonatal pneumonia, bilateral, focal, severe, stage of infiltration. DN III degree. Microbiological culture from the throat and endotracheal aspirate revealed Klebsiella pneumoniae. In addition, in a child with a burdened obstetric history, umbilical arteritis and phlebitis, anemia, pneumonia, signs of SIRS (thrombocytopenia, violation of thermoregulation, inflammatory changes in the blood) were recorded, which suggested its management as threatened by neonatal sepsis. Due to many diseases, the girl received complex therapy (including antibacterial) and continued to stay on mechanical ventilation for a long time (FiO2 0.45; f 40 in 1 min; pir. 19 mm of age; SaO2 90–92%) . Periodically, attempts were made to lower the parameters of mechanical ventilation, however, due to persistent hypercapnia (pCO2 55 mmHg) and hypoxia (pO2 24 mmHg), acidosis according to the acid-base blood composition (lactate 1.1 mmol / l; ССОО3 38.2 mmol / l; BE 12.3 mmol / l), low oxygen saturation values ​​could not extubate the child.

On the 28th day of life, in accordance with a set of criteria, the diagnosis was made: Bronchopulmonary dysplasia of premature infants, a new form, of a severe degree. The main point for the diagnosis was the need for oxygen with a concentration of more than 21% on the 28th day of life, oxygen dependence in 36 weeks. post-conceptual age indicated a severe course of BPD. On a CT scan, the main signs of BPD were identified – fibrous cords, frosted glass, bullous swelling (Fig. 2). With regard to the disease, the child received therapy with inhaled glucocorticosteroid (IHC) budesonide at a dose of 1.0 mg / day, muco- and bronchodilator therapy through a nebulizer (ambroxol, fenoterol, ipratropium bromide).

Computed tomography of the chest. Sections with signs of BPD: fibrous strands, frosted glass, bullous swellings. (From the archive of E.S. Truntsova)
At the age of 2.5 months. life confirmed diagnosis: Congenital heart disease (CHD): OAP. On ECHO-KG: OAP 3,5 mm with the discharge of blood into the pulmonary artery with a gradient of 4 mm RT. Art., aneurysm of the interatrial septum (AMP) and an open oval window of 3.5 mm with a discharge of blood from right to left, high PAH 89 mm RT. Art. with expansion of the right atrium (PP).

Echocardiographic picture of the expansion of the right atrium. (From the archive of Professor N.S. Cherkasov)
The ECG showed signs of PP overload. Radiographic: signs of enlargement of the right heart. In the hemogram: Hb anemia of 90 g / l, Er 3.88 × 1012 / l, CP 0.9, white blood cell without signs of inflammation. Blood glucose was 5.0 mmol / L, creatinine 51.5 mmol / L, ALT 0.5 μmol / L, AST 0.94 μmol / L. At the age of 3.5 months. In the Federal Center for Cardiovascular Surgery (Astrakhan), an operation was performed for OAP (ligation). The early postoperative period was uneventful.

Regarding the revealed pathology of CHD + PAH, the child received complex therapy, including oxygen therapy, diuretics (spironolactone under the control of creatinine and potassium), sildenafil (at a dose of 3.5 mg × 4 r. / Day), digoxin at a maintenance dose (10 mg / kg / day).

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