[Utility of B-type natriuretic peptide in children]. / B-natriuretikus peptid meghatározás jelentosége gyermekkori szívbetegségekben.

UNLABELLED: Serum brain natriuretic peptide (BNP) has been reported to indicate ventricular dysfunction, however, in children it has not been studied yet in our country. PURPOSE: 157 BNP tests were performed in 107 children, on the one hand, to evaluate its clinical value, to assess LV or systemic RV function in patients with transposition of great arteries after Senning operation, on the other hand, to prove the relation between BNP, MRI and echocardiographic ventricular function parameters. PATIENTS' AGE: 4 months-20 years, mean 12.5 yrs. Group I: Senning patients, Groups II and III: patients with dilated or hypertrophic cardiomyopathy, Group IV: patients with aortic insufficiency. METHODS: BNP was determined using the electrochemiluminesce method (Elycsys-10 Roche). During the functional MRI Mass- Medis software RV LV EF, end-diastolic, end-systolic volumes were calculated. Echo M-mode, TEI index were calculated. RESULTS: BNPs were significantly as higher compared to normal in each group of patients. Group I: 318 +/- 285 pg/ml, p < 0.01, Group II: 7262 +/- 10970 pg/ml, p < 0.01, Group III: 1558 +/- 2765 pg/ml, p < 0.01, Group IV: 1076 +/- 2791 pg/ml, p < 0.00l, vs 58 +/- 31 pg/ml. BNP were negatively correlated with MRI RV EF (r: -0.51, p < 0.05) and showed good correlation with TEI index (0.43 +/- 0.18, p < 0.05). After 3 weeks of medical or surgical treatment BNP decreased significantly. 4 patients died during the follow-up period, these had the highest BNP levels in each patients group. CONCLUSIONS: BNP is a useful, prognostically valuable method in children to monitor ventricular function. BNP levels reflect the severity of the impairment of systemic RV function in Senning patients in whom a complex RV geometry is present causing the assessment of RV function more difficult, so we recommend BNP measurements as a longitudinal test in this patient group.

[Quantification of right and left ventricular function with magnetic resonance imaging after Senning operation for complete transposition of the great arteries]. / Mágneses rezonancia vizsgálat a jobb es bal kamra funkciójának megítélésére nagyér-transzpozíciós gyermekek Senning-mutétje után.

INTRODUCTION: The different non-invasive examinations do not result in unambigous results about the long-term determination of right ventricular function providing systemic circulation in children with transposition of the great arteries operated with Senning procedure. AIM: The goal of study was to determine the application of MRI for the observation of right and left ventricular morphology and function, for the recognition of the progression of the disease, and for the necessity of reoperation. METHODS: The authors have observed the morphology and function of the right and left ventricule by ECG triggered short axis MR pictures. For the analysis of MR pictures MASS 5.0 software was used. Authors have determined the end systolic and end diastolic volume, the ejection fraction, the mass, and the stroke volume of the right ventricule, the end systolic and end diastolic volume, ejection fraction, the mass, and the stroke volume of the left ventricle, and the shunt volume. The values of these data corrected to body mass have been calculated. PATIENTS: 176 Senning operations took place between 1980 and 1996. MRI exploration at 21 of the 118 analyzed patients were carried out. RESULTS: The right ventricular ejection fraction value at Senning operated patients is significantly different (49 +/- 9% vs. 70 +/- 4%) from those of healthy children (P < 0.01). Right ventricular stroke volume/m2 and right ventricular end systolic volume/m2 were significantly different compared to normal (43 +/- 10 ml/m2 vs. 48 +/- 7 ml/mn and 46 +/- 16 ml/m2 vs. 21 +/- 5 ml/m2, p < 0.05). Authors found also significant differences in left ventricular ejection fraction in Senning patients as compared to normal (60 +/- 9% vs. 70 +/- 6%, p < 0.01). CONCLUSION: MRI, which is a reliable method of objective determination of right ventricular function, became an available method in Hungary. According to the results, authors might presume that the development of right ventricular dysfunction is expected even among Senning operated children without clinical symptoms, so more frequent control is required. The pathological left ventricular parameters may be explained by ventricular interactions. Authors find MRI an important part of the complex follow-up protocol of Senning operations. MRI data extended by results of other non-invasive explorations are appropriate for the follow-up of right ventricular dysfunction.

[Is the right ventricle able to maintain the systemic circulation for a long time? The late results of the Senning operation for complete transposition of the great arteries]. / Képes-e a jobb kamra hosszú távon biztosítani a szisztémáis keringést? Nagyér-transzpozíciós betegeken végzett Senning-mutétek késoi eredményei.

UNLABELLED: The successful surgical management of congenital heart defects increased the number of such a type of defects, in which the morphological right ventricle has to provide the systemic circulation. Children with transposition of the great arteries operated with Senning procedure are good examples to investigate the long-term right ventricular function. Aim of our study was to determine the adequate non-invasive methods to assess the progression of the right ventricular dysfunction, to analyse the long-term surgical results and to determine the indication of reoperation. METHODS: Physical examination, electrocardiogram, 24 hours ECG, stress-testing, echocardiography, B-natriuretic peptide level measurement and magnetic resonance imagine were performed. PATIENTS: 176 Senning operations were carried out between 1980 and 1997. The follow-up period was 9-25 (mean 12.5) years and 83 children were included in our study. RESULTS: Significant correlations were found between the MRI RVEF, and echocardiographic TEI index, TDI and BNP results. Abnormal values were found in Senning patients compared to healthy children in MRI RVEF 55%, echocardiographic TEI index 0.33, in RV TDI systolic velocities 0.2 m/s, and B-natriuretic peptide levels 150 pg/ml. That means that right ventricular disfunction is expected in Senning children even without clinical symptoms. CONCLUSIONS: (1) BNP and echocardiographic TEI and TDI became available methods for the follow-up of right ventricular disfunction, and annual MRI is recommended. (2) Conversion of Senning to anatomic repair is considerable in selected young patients because the capacity of myocardium to respond to pressure overload decreasing with age. In older children and adults very close follow-up is necessary using biomarkers, echocardiography and MRI to decide the correct time of heart transplantation.

[Ross procedure instead of heart transplantation. Recovery potential of the dysfunctional myocardium in heart failure caused by congenital aortic valve insufficiency]. / Szívátültetés helyett ross-mútét. A diszfunkciós szívizomzat regenerációs képessége veleszületett aortabillentyú-betegség okozta szívelégtelenségben.

UNLABELLED: The authors report a case of a child who presented in advanced valvular heart failure secondary to aortic regurgitation. Due to social circumstances heart transplantation was not a viable option, therefore a high risk pulmonary autograft aortic root replacement (Ross procedure) was performed. Following surgery a delayed but rather gratifying myocardial recovery was observed. CONCLUSION: This case represents the preserved recovery potential of the pediatric myocardium in end stage heart failure.

[Aortic root replacement with pulmonary allograft (Ross procedure) in children. Early results]. / Veleszületett aortabillentyú-betegség kezelése aortagyökpótlással pulmonalis allografttal (Ross-mútét) gyermekkorban. Korai eredmények.

INTRODUCTION: For infants and children with congenital aortic valve disease root replacement with pulmonary allograft (Ross procedure) is the preferred method of choice. PATIENTS/RESULTS: The authors have successfully applied this operation in 12 children (age range from 2.5 to 17 years--mean 9 years, body weight from 12 to 58 kg--mean 46 kg), one of whom has also required a Konno extension for long segment left ventricular outflow tract obstruction. The operation was complicated by early postoperative endocarditis in one case, and the child required redo homograft root replacement on the ninth postoperative day. All patients, including this one survived, and are doing well at present. CONCLUSIONS: In the Hungarian literature this is the first report on the Ross and Konno procedure in children. On the basis of our excellent early results, Ross procedure is the method of choice in aortic valve disease in children.