[Diagnosis and treatment of childhood cardiomyopathies]. / Diagnose und Therapie von Kardiomyopathien im Kindesalter.

Cardiomyopathies have either a primary (without associated anomalies) or a secondary origin. They are classified in three groups according to their anatomy and function: hypertrophic, dilated or restrictive. We review here the relevant diagnostic points of each type as well as their treatment. Restrictive cardiomyopathies, arrhythmogenic right ventricle, non compaction and Uhl's anomaly will not be dealt with in detail as they are very seldom in children.

Dilated cardiomyopathy and thrombo-embolism.

UNLABELLED: The purpose of this study was to investigate the incidence, outcome and prevention of thrombo-embolism in children with dilated cardiomyopathy. From 130 patients with dilated cardiomyopathy, 17 (14%) showed evidence of thrombo-embolism. Seven had initial cardiac thrombus, 7 exhibited initial embolus and in 3 thrombo-embolism was only diagnosed at autopsy. All 17 patients showed seriously impaired systolic function of the left ventricle with fractional shortening (FS) of 10 +/- 3%, range 5%-17%, as compared to those without thrombo-embolism with FS of 17% +/- 6%, range 5%-26% (P < 0.0001). Seven patients were treated with oral anticoagulants once thrombo-embolism had been diagnosed; one of them experienced a further embolic event as opposed to three out of four patients not treated with anticoagulants. CONCLUSION: All children with dilated cardiomyopathy and fractional shortening below 20% should be treated with prophylactic anticoagulative agents.

Effects of morphologic restenosis, defined by MRI after coarctation repair, on blood pressure and arm-leg and Doppler gradients.

Ten years after coarctation repair, 36 adolescents and young adults were studied in order to evaluate the relationship of anatomy at the resection site to blood pressure and arm-leg and Doppler gradients. The patients underwent magnetic resonance imaging (MRI), exercise testing, and continuous wave (CW) Doppler echocardiography. On MRI, residual narrowing at the resection site was measured as 1-(phi anastomosis/ phi descending aorta) and expressed as percent stenosis. Residual stenosis on MRI was negatively correlated with the leg pressure at rest (P = 0.0003) and during exercise (P = 0.002). Residual stenosis correlated positively with the arm-leg gradient at rest (P < 0.0001) and during exercise (P < 0.0001) and with the peak CW Doppler gradient across the anastomosis (P < 0.0001). However, residual stenosis was not related to the systolic blood pressure of the arm at rest or during exercise. The systolic arm pressures did not differ between patients with residual stenosis of less than 30% (group I), patients with residual stenosis of equal to or greater than 30% but less than 45% (group II), and patients with residual stenosis of equal to or greater than 45% (group III). In conclusion residual anatomic stenosis influences blood pressure in the legs, the arm-leg gradient, and the Doppler gradient across the anastomosis. Arm hypertension late after coarctation repair seems not to be related to residual stenosis, and the benefit of reintervention in these patients remains questionable.

Lung hypoplasia and severe pulmonary hypertension in an infant with double heterozygosity for spondyloepiphyseal dysplasia congenita and achondroplasia.

A rare instance of double heterozygosity for spondyloepiphyseal dysplasia congenita and achondroplasia is presented. Despite midface hypoplasia, thorax deformity and lung hypoplasia, the child survived the neonatal period. Severe pulmonary hypertension, already present at birth, led to right heart failure and death at the age of 1 year.

[Childhood treatment of paroxysmal supraventricular tachycardia using i.v. adenosine]. / Behandlung der paroxysmalen supraventrikulären Tachykardie im Kindesalter mit Adenosin i.v.

Paroxysmal supraventricular tachycardia is the most frequent significant arrhythmia in the pediatric age group, especially in the first year of life. In neonates and infants there are important limitations for the commonly used drugs such as verapamil and digitalis. In an open Swiss multicentre study we treated 19 children with a total of 29 episodes of tachycardia by means of adenosine i.v. as the drug of first choice. 76% of all the tachycardias were converted, whereas the success rate was 87% if only tachycardias with atrioventricular reentry were considered. The important advantage of adenosine lies in its very short half-life of about 15 seconds, which means that the rare relevant, and the more common mild, side effects are quite limited in duration. A major disadvantage are recurrences in about one third of cases. We conclude that adenosine is an efficient and safe treatment for paroxysmal supraventricular tachycardia in the whole pediatric age group including neonates and infants.

Tetralogy of Fallot and coarctation of the aorta: a rare combination and its clinical implications.

A neonate with tetralogy of Fallot and aortic coarctation is described. The clinical course was remarkable, with early congestive heart failure followed by rapidly progressive left ventricular myocardial thickening and the presentation of hypercyanotic spells. Primary repair of tetralogy without correction of coarctation was attempted unsuccessfully at 1 month of age. Death followed postoperative bradycardia, hypotension, and low cardiac output. Fetal and neonatal hemodynamics and the clinical implication of this rare combination of defects are discussed. Initial surgical intervention should relieve left-sided obstruction.

Aortic atresia with aortopulmonary window and interruption of the aortic arch.

The cross-sectional echocardiographic and postmortem appearances of the heart from a patient with the rare association of aortic valve atresia, aortopulmonary window, and interrupted aortic arch are described. Differentiation of this anomaly from truncus arteriosus with interrupted aortic arch is important.

Successfully treated mitral valve endocarditis in a neonate.

We report on a newborn with severe congestive heart failure where echocardiography was leading to the correct diagnosis and the treatment. Endocarditis in the newborn is a rare disease, and the diagnosis is possible from the echocardiographic demonstration of vegetations. It is therefore important to search for vegetations in the very ill newborn with cardiac symptoms, in order to start early antibiotic treatment independently of positive cultures and to avoid the usual fatal outcome of this disease.