Six-Minute Walk Test as a Predictor for Outcome in Children with Dilated Cardiomyopathy and Chronic Stable Heart Failure

Developmen

Absent pulmonary valve syndrome with intact ventricular septum and patent ductus arteriosus: report of two cases and a short review of the literature.

Item does not contain fulltextWe describe two patients who both presented shortly after birth with congestive heart failure due to an absent pulmonary valve and patent ductus arteriosus. Diagnostic evaluation revealed in both cases an aneurysmatic dilation of the pulmonary vascular tree and an abundant left-to-right shunt over a large patent ductus arteriosus. Ventricular septal defects were not detected. Early surgical closure of the patent ductus arteriosus improved the hemodynamic situation so that implantation of a homograft valve could be delayed

Resection of a cardiac aneurysm in an infant with anomalous origin of the left coronary artery from the pulmonary trunk.

Contains fulltext : 57512.pdf (publisher's version ) (Closed access)We describe an infant with an anomalous left coronary artery arising from the pulmonary trunk leading to myocardial ischaemia and development of an apical aneurysm of the left ventricle. Clinical presentation in general is based on myocardial hypoperfusion resulting in ischaemia and infarction. When presenting in infancy, however, then the features, as in our patient, may be tachypnea, dyspnea, failure to thrive and irritability, especially during feeding. Then, again as in our patient, it is possible to miss the definitive diagnosis, which is made by echocardiography and cardiac catheterization. Reimplantation of the anomalous left coronary artery into the aorta usually results in improvement of left ventricular function over time. In our patient, the myocardial infarction was complicated by formation of an aneurysm, and reimplantation alone was not sufficient to restore cardiac function. Resection of the aneurysm greatly improved the hemodynamics

Cardiac resynchronization as therapy for congestive cardiac failure in children dependent on chronic cardiac pacing.

Contains fulltext : 49941.pdf (publisher's version ) (Closed access)Three patients with heart failure after chronic right ventricular apical pacing were treated with resynchronization. Biventricular pacing was used for two patients, and the other was treated with left univentricular pacing. In all patients, we observed a dramatic improvement of left ventricular dimension, function, and clinical state. We conclude that biventricular or left ventricular pacing is superior to right ventricular apical pacing in children who are pacemaker-dependent

Myocardial infarction in a neonate with left-sided congenital diaphragmatic hernia.

Contains fulltext : 79830.pdf (publisher's version ) (Closed access)We present a case study of a newborn girl with a left-sided congenital diaphragmatic hernia and a myocardial infarction (MI). The occurrence of MI in newborns has been associated with cardiac malformations and abnormalities of the coronary arteries or thromboembolization. In our patient, echocardiography revealed left ventricular dysfunction, persistent pulmonary hypertension and an inferolateral MI. Within 24 hours cardiac function and oxygenation did not improve and the baby died. Autopsy showed massive MI, a small aortic valve and ascending aorta, and hypoplasia of the distal aortic arch and isthmus. An accessory spleen was also present. The present report underlines the importance of cardiac evaluation in patients with congenital diaphragmatic hernia

Cardiac resynchronization therapy for mitral systolic anterior motion in a child.

Contains fulltext : 52993.pdf (publisher's version ) (Closed access)We describe the successful use of cardiac resynchronization therapy for treatment of mitral valve systolic anterior motion with left ventricle outflow tract obstruction after re-excision of a subaortic membrane and septal myectomy in a 12-year-old child. In the recovery phase, a total atrioventricular block persisted. Therefore a permanent atrioventricular pacing system was implanted

Pericarditis as complication of appendicitis.

Contains fulltext : 59250.pdf (publisher's version ) (Closed access)Pericarditis as a complication of appendicitis is a rare event. In a 25-year period we encountered two pediatric cases with this severe complication due to (a)typical presentation of appendicitis resulting in small bowel obstruction, intraabdominal abscesses, constrictive pericarditis, and purulent pericarditis

Early cardiac involvement in children carrying the A3243G mtDNA mutation.

The phenotypic spectrum of the mitochondrial A3243G DKA mutation is highly variable, particularly when occuring in childhood. In contrast to the classical presentation in adulthood (MELAS syndrome; mitochondria! myopathy, encephalopathy, lactic acidosis and stroke-like episodes) children show a different pattern of symptoms, often without the typical encephalopathy or psychomotor regression. We present six children carrying the A3243G mtDNA mutation with a heteroplasmy above 50 % in muscle tissue. The age of diagnosis ranged from 2 weeks up to 14.5 years. The clinical presentation was rather non-specific including muscle weakness, developmental delay and epilepsy. In this small pediatric group we detected presymptomatic cardiac involvement in five out of six children already at an early stage of disease. The cardiac pathology included cardiomyopathy and biventricular hypertrophy with rhythm disturbances (for example long QT-syndrome). The observed cardiac changes do not always increase the risk of cardiac deterioration; however, two of our patients died early on. CONCLUSION: We hypothesize that the A3243G mutation might be underdiagnosed, as patients could suffer from an unexplained cardiac death before the diagnosis is made. We advise performing regular repeated ECGs and echocardiography in all children carrying a A3243G mtDNA mutation independently from the presence of cardiac symptoms

Extravascular lung water measurement using transpulmonary thermodilution in children.

Contains fulltext : 80712.pdf (publisher's version ) (Closed access)OBJECTIVE: Measurement of extravascular lung water (EVLW) may be useful in the treatment of critically ill children and can be performed at the bedside using the transpulmonary thermodilution technique (TPTD). There are currently no data to verify the accuracy of these measurements in (small) children. We compared the results of TPTD measurement with the clinical gold standard transpulmonary double indicator dilution (TPDD) measurement in young children. DESIGN: Prospective clinical study in children. SETTING: Catheterization laboratory of a university hospital. PATIENTS AND METHODS: Twelve children (<2 yrs or <12 kg) under general anesthesia. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Measurements were performed using injections of ice-cold indicator (saline or dye) through a central venous catheter. Mean cardiac index was 3.91 L/min/m, mean intrathoracic blood volume index (ITBVITPDD) was 614.9 mL/m, and mean extravascular lung water index (EVLWITPDD) was 11.7 mL/kg. The correlation coefficient between EVLWITPDD and EVLWITPTD is 0.96 (95% confidence interval: 0.87-0.99; p < 0.0001). Bland-Altman analysis for EVLW measurements showed a mean bias of 2.34 mL/kg (18.13%) and limits of agreement +/-2.97 mL/kg (19.78%). The difference between measurements via the right atrium compared with the femoral vein was 2.8% for cardiac output, 8.2% for global end-diastolic volume, and 0.1% for EVLW. CONCLUSION: Clinical measurement of EVLW in young children can be performed using the TPTD with the injection catheter inserted in the femoral vein. Further studies are needed to clarify the clinical value of these measurements