[Diagnostic algorithms and features of clinical course of congenital heart diseases in infants with lung anomalies].

The paper discusses the influence of lung anomalies on the progress of congenital heart diseases (CHD) in infants, as well as diagnostic value of radiography and ultraspeed computed tomography (CT). The four-year experience of the authors includes preoperative examination of 178 infants with CHD (mean age 5.8 +/- 0.6 months, mean weight 5.2 +/- 0.3 kg). Lung anomalies were determined in 24 patients (13.5%) on plain films and in 85 patients (47.8%)--by means of CT. The stenosis of main bronchi in combination with lobar emphysema and lung hypoplasia were found to be the most frequent and severe predictors of respiratory disorders. The obtained data suggest that CT examination may be recommended in infants with CHD for precise diagnostics of associated lung anomalies and further prevention or early management of respiratory complications.

[Myocardial fatty dystrophy in patients with Fallot tetralogy]. / Zhirovaia distrofiia miokarda u bol'nykh s tetradoi Fallo.

Fatty degeneration (FD) was studied histochemically and ultrastructurally in surgical biopsies from 147 patients aged 22 days to 40 years with Fallot's tetralogy. FD was most pronounced in patients aged 3 to 6 years when the main part of cardiomyocytes completed the program of ontogenetical growth and started growing as hypertrophic mature cells. An increase of FD is considered as manifestation of an initial stage of hypertrophy of mature cardiomyocytes.

[The use of positron emission tomography for noninvasive diagnosis of mitochondrial dysfunction and assessment of myocardial compensatory reserve in children with cardiomyopathies]. / Neinvazivnaia diagnostika mitokhondrial'noi disfunktsii i otsenka kompensatornykh vozmozhnostei miokarda u detei s kardiomiopatiiami s pomoshch'iu pozitronnoi émissionnoi tomografii.

BACKGROUND: Recent experimental and clinical studies have shown that cardiomyopathy (CMP) can be associated with disorders of oxidative metabolism in cardiomyocyte mitochondria. These disorders are connected with deficit of enzymes and complexes of electron transfer chain, and can constitute pathogenetic base of the disease. AIM. To study myocardial metabolism in children with cardiomyopathies using positron emission tomography (PET). METHODS: PET studies were carried out in 17 patients (pts.) aged 3-13 years (mean age 7,5-/+3,1) with CMP: hypertrophic (HCMP, n=4), dilated (DCMP, n=9), after radical correction of tetralogy of Fallot (TF, n=4). The dynamic study with [1-11C]-acetate was performed for evaluation of Krebs cycle activity. Rate constant (kmono) and [11C]-activity elimination half-time (T1/2) were calculated using myocardial time-activity curve. Myocardial perfusion was assessed with [13N]-ammonia, glucose metabolism - with [18F]-fluorodeoxyglucose. RESULTS: Patients with HCMP: left ventricular (LV) perfusion was normal, Krebs cycle activity - decreased; glucose utilization was increased in comparison with normal level in 2, and was normal in 2 other children. Patients with DCMP: LV perfusion was normal; Krebs cycle activity in LV was decreased in 6 and normal - in 3 pts. Overall 2 pts. with CMP had focal perfusion defects. More pronounced decrease of Krebs cycle activity in these regions compared with other LV parts implied development of ischemic injuries. Decrease of glucose utilization in these fields indicated that the injuries were irreversible. Patients with TF: 3 pts. had normal perfusion and Krebs cycle activity; 1 patient with severe hemodynamic disorders had decreased perfusion, glycolysis and Krebs cycle activity in both ventricles indicative of the presence of myocardial dystrophic changes. CONCLUSION: Despite normal perfusion children with cardiomyopathy had decreased Krebs cycle activity (mitochondrial dysfunction). Decrease in oxidative metabolism could be compensated by increased glycolysis.