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Biomedical subjects

S Kleinert

Publications and source records attributed to S Kleinert.

At least 19 recordsLinked to original sources

Clinical, electrocardiographic, and histologic correlations in children with dilated cardiomyopathy.

OBJECTIVE: To determine whether presenting electrocardiography is related to histologic findings and clinical outcomes in children with dilated cardiomyopathy. BACKGROUND: Lymphocytic myocarditis is an important cause of childhood dilated cardiomyopathy, the outcome of which is unclear. The results of non-invasive investigations are often used to infer the presence or absence of lymphocytic myocarditis. METHODS: Thirty-four children, presenting acutely with dilated cardiomyopathy, underwent both early electrocardiography and endomyocardial biopsy. The parameters examined included heart rate, PR, QRS, and corrected QT intervals, R-wave voltages in Leads V(1) and V(6), S-wave voltages in Leads V(1) and V(6), and sum of SV(1) and RV(6). We expressed measurements as Z scores, based on published normal values for age and gender. RESULTS: A total of 15 patients had lymphocytic myocarditis on endomyocardial biopsy (Group I), and 19 had non-specific histologic findings (Group II). We did not distinguish the 2 groups by age, time to endomyocardial biopsy, or duration of follow-up. Group I patients had significantly smaller R-wave Z scores in Leads V(1) and V(6), and combined S in V(1) and R in V(6) Z scores (p < 0.02 for each). The positive and negative predictive values of an R-wave amplitude in V(6) < 5th percentile were 75% and 65%, respectively, for the diagnosis of lymphocytic myocarditis. An R-wave amplitude in V(6) > 95th percentile had a positive and negative predictive value of 80% and 63%, respectively, for the diagnosis of idiopathic dilated cardiomyopathy. Survival and freedom from late cardiac dysfunction were more common among Group I patients compared with Group II (p <or= 0.02 for both). CONCLUSION: Myocardial histology cannot reliably be inferred from the presenting electrocardiogram. Survival and outcome for children with lymphocytic myocarditis is better than for those with non-specific histology.

Biopsy↗

Pulmonary vascular resistance and reactivity in children with end-stage cardiomyopathy.

Pulmonary vascular resistance (PVR) and reactivity were compared in 63 children with end-stage cardiomyopathy (CM) referred for cardiac transplantation. Diagnostic category of CM was the sole determinant of PVR. Compared with other patients, children with restrictive CM were younger at diagnosis and had a significantly higher pulmonary vascular resistance index (PVRI). Children with a baseline PVRI of up to 11.8 units per meter squared (U.m(2)) who showed reactivity underwent successful orthotopic cardiac transplantation.

Adolescent↗

Finale.

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Female↗

Pulmonary valve annulus grows after balloon dilatation of neonatal critical pulmonary valve stenosis.

BACKGROUND: Neonates with critical pulmonary valve stenosis often demonstrate small or hypoplastic right ventricular structures. Relief of the obstruction enhances forward flow across the right ventricle and reduces its pressure load. Growth of the right ventricle and especially of the pulmonary valve annulus was evaluated after balloon dilatation. METHODS: Ten consecutive neonates with critical pulmonary valve stenosis who underwent balloon valvuloplasty were studied by serial echocardiography to assess growth of right ventricular structures at follow-up. RESULTS: The mean diameter of the pulmonary valve annulus increased from 6.1 +/- 1.4 mm to 12.6 +/- 3.5 mm (z scores from -2.9 +/- 1.0 SD to - 1.3 +/- 1.2 SD, p < 0.0001) after a mean follow-up period of 2.7 +/- 2.0 years. The mean diameter of the tricuspid valve annulus increased from 12.9 +/- 3.8 mm to 19.0 +/- 3.1 mm; however, the respective z score did not change significantly (from 0.5 +/- 2.4 SD to -0.5 +/- 1.0 SD). Right ventricular cavity size was hypoplastic in four patients initially and normal in all patients at latest follow-up. CONCLUSIONS: Balloon dilatation of critical pulmonary valve stenosis encourages catch-up growth of the pulmonary valve, and surgery may be avoided even in a hypoplastic pulmonary valve annulus.

Catheterization↗

Anatomic features and surgical strategies in double-outlet right ventricle.

BACKGROUND: The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality. METHODS AND RESULTS: Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years. CONCLUSIONS: Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.

Adolescent↗

Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression.

BACKGROUND: The true incidence and prognosis of myocarditis in children with acute dilated cardiomyopathy (DCM) at presentation remains uncertain. This study examines the incidence of lymphocytic myocarditis in a consecutive cohort of children with acute DCM at presentation and outcome after dual therapy immunosuppression with cyclosporine and steroids. METHODS: Twenty-nine consecutive children with acute DCM underwent early endomyocardial biopsy. Children with "definite" myocarditis comprised group I (n = 9) and were treated with cyclosporine and prednisolone. Group II (n = 2) had "borderline" myocarditis, and group III (n = 18) nonspecific histologic findings. Outcome was assessed by echocardiographic measurement of left ventricular end-diastolic dimension and fractional shortening, with follow-up endomyocardial biopsy in group I subjects. RESULTS: Myocardial inflammation with or without myocardial necrosis (groups I and II) was present in 38% of all cases. There were no initial clinical, electrocardiographic, or echocardiographic features to distinguish patients in group I from patients in group III. At presentation, the mean +/- SEM left ventricular end-diastolic dimension and fractional score-Z scores of group I patients were 4.6 +/- 1.7 and -5.1 +/- 0.8, respectively, compared with 0.8 +/- 0.3 and -0.9 +/- 0.4, respectively, at withdrawal of immunosuppression (p < 0.001 for both). Both of these parameters did not differ significantly from normal controls at least follow up. Two group I patients had a biopsy-proven relapse after withdrawal of therapy that responded to reinstitution of immunosuppression. At latest follow-up, all nine group I patients had regained normal left ventricular function compared with four of 18 group III patients (p < 0.001). CONCLUSION: Lymphocytic myocarditis is frequent in children with dilated cardiomyopathy and cannot be predicted from noninvasive investigations. The use of cyclosporine and steroids is associated with a favorable outcome, and a controlled trial of dual therapy immunosuppression in children is therefore warranted.

Anti-Inflammatory Agents↗

Treatment of critical pulmonary valve stenosis by balloon dilatation in the neonate.

Critical pulmonary valve stenosis represents an emergency, and immediate treatment is mandatory. The purpose of this study was to evaluate the immediate and medium-term results of pulmonary valve dilatation. We report 18 neonates in whom pulmonary valvuloplasty was attempted. The procedure could be accomplished in 14 patients. The angiographically determined diameters of the pulmonary and tricuspid valve at the time of procedure were 5.6 +/- 1.5 mm and 14.0 +/- 5.4 mm. The mean Doppler gradient decreased from 71 +/- 27 mm Hg to 27 +/- 14 mm Hg. Perforation of the right ventricular outflow tract was the major complication in three patients with one fatal event. Infusion of prostaglandin E1 could be discontinued 1 to 5 days after the procedure. On follow-up three children required a second balloon dilatation with good results. Seven patients monitored for more than 9 months with a mean follow-up time of 34.4 +/- 16 months had a residual gradient of 11.6 +/- 6.7 mm Hg. In spite of a hypoplastic pulmonary valve annulus in seven of the patients, results were good and surgery could be avoided.

Catheterization↗

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis.

Early intervention is necessary in neonates with critical aortic stenosis. The advent of alternate therapy, particularly balloon aortic valvuloplasty, requires a reappraisal of the traditional surgical approach, including the efficacy of initial transvalvar gradient reduction and freedom from recurrence of obstruction in the longer term. This report describes a series of 33 consecutive infants who underwent surgical aortic valvotomy in the first month of life. The hospital mortality was 18 percent with a 5-year probability of survival of 66 percent (90 percent CI, 50 percent to 79 percent). Fourteen reinterventions, nine reoperations and five balloon dilatations, were required at a median age of 0.8 years (range 9 days to 6 years). Three patients died after reintervention (one early and two late). The median follow-up time was 5.8 years (range 0.2 to 14 years). At last follow-up Doppler investigation the average Doppler mean and peak gradients were 34 mm Hg and 18 mm Hg, respectively. Open valvotomy in neonatal aortic valve stenosis allows the use of an appropriate surgical approach with low initial mortality and satisfactory 5-year freedom from reintervention.

Aortic Valve Stenosis↗

Alveolar epithelial lining fluid cellularity, protein and endothelin-1 in children with congenital heart disease.

This study applied bronchoalveolar lavage (BAL) to children with congenital heart disease (CHD) prior to elective cardiac catheterization (n = 48), to determine the influence of pulmonary blood flow and viral infection on the alveolar epithelial lining fluid (ELF) concentration of leucocytes, protein and endothelin-1 (ET-1). Lower respiratory tract (LRT) viral infection was defined as either a positive immunofluorescence for virus, or a virus cultured from the bronchoalveolar lavage fluid (BALF). Haemodynamic status was determined at cardiac catheterization. Normative data for BALF, but not ELF parameters, were obtained from 26 asymptomatic, noninfected normal children undergoing elective surgery. In the absence of LRT infection, the BALF macrophage, lymphocyte and neutrophil differential in CHD was not significantly different from the normal controls. In CHD, both increased pulmonary-to-systemic flow ratio (Q'p/Q's) and increased pulmonary artery-to-left ventricular pressure ratio PAP/LVP were associated with a decrease in ELF protein (rs = -0.59; p < 0.0001; and rs = -0.50; p < 0.0001 respectively). A respiratory virus was isolated from the BALF in 8 (17%) of CHD children. Virus isolation was associated with an increased ELF total protein (p < 0.05 vs no infection), a decreased alveolar macrophage differential count (p < 0.01), and an increased neutrophil differential count (p < 0.05). ET-1 was detected in the BALF of 83% of the noninfected CHD children compared to only 23% of the controls (p < 0.001). ELF ET-1 concentrations did not correlate with haemodynamic status in CHD, but were up to 100 times higher than paired plasma levels. We conclude that, in congenital heart disease, both lower respiratory tract viral infection and increased pulmonary blood flow and/or pulmonary vascular pressure influence the alveolar milieu. High alveolar epithelial lining fluid concentrations of endothelin-1 occur in congenital heart disease, but the stimulus for pulmonary endothelin-1 production is unclear.

Bronchoalveolar Lavage Fluid↗