Hypertrabecular aspect of left ventricular myocardium: A possible complication of congenital hypothyroidism in a preterm infant

We report a case of hypertrabeculated left ventricular myocardium associated with congenital hypothyroidism in a preterm infant. The myocardical anomalies and long QT interval, absent at birth, appeared simultaneously to abnormal thyroid hormones and progressively improved until complete recovery during Levo-Thyroxine treatment. We speculate that thyroid dysfunction could be the potential cause of transient hypertrabecular aspect of the left ventricle.
  • [Show abstract] [Hide abstract] ABSTRACT: The French national neonatal screening program for congenital hypothyroidism (CH) was initiated in 1978. The purpose of this study was to ascertain the incidence of congenital extrathyroid anomalies (ETAs) among the infants with congenital hypothyroidism (CH) and to compare it with the Northeastern France Birth Defect Monitoring System data from 1979 to 1996. Among 129 CH infants on whom adequate data were available, 20 infants (15.5%) had associated congenital anomalies. Eight out of 76 infants with persistent CH had ETAs (10.5%) whereas 12 out of 53 children with transient hypothyroidism had ETAs (22.6%, p < 0.05). Some additional anomalies were considerably more common than in the general population. Nine infants had congenital cardiac anomalies (6.9%). This rises the question if teratogenic effects active during organogenesis may affect simultaneously many organs, including the developing thyroid, causing a relatively high percentage of CH infants with congenital ETAs.
    Article · Feb 1999
  • [Show abstract] [Hide abstract] ABSTRACT: To determine clear cut echocardiographic criteria for isolated ventricular non-compaction (IVNC), a cardiomyopathy as yet "unclassified" by the World Health Organization. The disease is not widely known and its diagnosis mostly missed. In seven out of a series of 34 patients with IVNC the in vivo echocardiographic characteristics were validated against the anatomical examination of the heart removed after death in four and due to heart transplantation in three patients. Four morphological criteria diagnostic for IVNC were found. (1) Coexisting cardiac abnormalities were absent (by definition). (2) A two layer structure was seen, with a compacted thin epicardial band and a much thicker non-compacted endocardial layer of trabecular meshwork with deep endomyocardial spaces. A maximal end systolic ratio of non-compacted to compacted layers of > 2 is diagnostic. (3) The predominant localisation of the pathology was to mid-lateral (seven of seven patients), apical (six), and mid-inferior (seven) areas. The pathological preparations confirmed the echocardiographic findings. Concomitant regional hypokinesia was not confined to the non-compacted segments. (4) There was colour Doppler evidence of deep perfused intertrabecular recesses. Four clear cut echocardiographic diagnostic criteria were established. It is suggested that the WHO classification of cardiomyopathies be reconsidered to include IVNC as a distinct cardiomyopathy.
    Full-text · Article · Dec 2001
    R JenniR JenniE OechslinE OechslinJ SchneiderJ Schneider+1more author...[...]
  • [Show abstract] [Hide abstract] ABSTRACT: Because of previous reports of myocardial dysfunction in adults and children with hypothyroidism, 12 newborns with congenital hypothyroidism were studied using standard electrocardiographic and echocardiographic techniques. The infants were between the ages of 7 and 28 days at the time of study. Five infants were athyrotic, and of these two had delayed skeletal maturation. Electrocardiogram revealed normal heart rates and QRS voltages in all children, while five had abnormal dome-shaped T-waves and absent ST-segments (Mosque sign). Echocardiograms were normal in all patients indicating no myocardial dysfunction or pericardial effusion. This study suggests that myocardial dysfunction is not present in the immediate newborn period of infants with congenital hypothyroidism. It is reasonable to conclude that early detection of congenital hypothyroidism by mass screening may prevent the cardiac affects of hypothyroidism as well as the other known sequelae. (C) Copyright 1986 Southern Society for Clinical Investigation
    Article · Aug 1986
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