Year: 1960
Type: article
Abstract: The first account of mongolism (Down, 1866) made no reference to cardiac malformations as a feature of the disease, though mentioning that "the circulation is feeble."Ten years later, Fraser (1876) published the first necropsy report of a case of mongolism, noting a small heart with fene- strated aortic valves in a 40-year-old woman.At that time, Mitchell (1876), in his notes on the physical and mental condition of so-called "Kalmuc idiots" whom he had seen, did not mention cardiac defects.Nor did Ireland (1877), though several pages of his book are devoted to a descrip- tion of mongolism.In fact, 28 years were to pass after Down's original description before an association between congenital cardiac anomalies and mongolism was suggested.The credit for this belongs to Garrod (1894) who, in a series ofcases ofcardiac combined with other malformations, included an 18-month- old child with mongolism.Two years later, Smith (1896), in recommending thyroid treatment in mongolism, advised a cautious dose "as the heart is undersized and weak in these patients."Garrod's continued interest in the association was demonstrated at two 1898 meetings of the Clinical Society of London.At one, he (Garrod, 1898) showed a single case, and at the second, described 5 others (Garrod, 1899), and expressed the belief that a special connection existed between congenital heart disease and mongolism.At the latter meeting, Gossage (1898) drew attention to an additional case and Guthrie (1898) mentioned two more in whom he had found septal defects at necropsy.Thomson also (1898), in a paper read before the Edinburgh Medico-Chirurgical Society, observed that he had found congenital heart disease in three cases of mongolism.Sutherland (1899) reported that 5 of his 25 cases of mongolism had clinical evidence of congenital heart defects (in two confirmed at necropsy, one having a ventricular septal defect and the other a patent ductus), and Still (1899) observed that he had known congenital heart disease to be the cause of early death in mongolism.Neumann (1899), whom Cassel (1917) described as the first German author on mongolism, mentioned that 2 of his 13 cases of mongolism had congenital heart lesions.Neumann, whose interest in mongolism followed a visit to the Royal Albert Asylum in England, appears to be the only observer outside Britain to have noted the association of congenital cardiac lesions with mongolism before the present century.By then, this was established well enough for Sutherland (1900) to be able to list the frequency of congenital heart disease in mongolism as one of the features differentiating it from cretinism.Thereafter, the association was frequently noted and discussed.A considerable diversity of findings and opinions has been expressed both as to the incidence and the nature of congenital cardiac malformations in mongolism.We therefore thought it worthwhile to review published data, and to study a comparatively large number of cases of mongolism, comparing the findings with those in an adequate group of controls. SOURCE AND NATuRE OF PRESENT SERIESThe records and, where possible, the hearts of all cases of mongolism that came to necropsy at various periods at five different hospitals were examined.In order to collect as representative a 331 on
Source: Heart
DOI: 10.1136/hrt.22.3.331
Authors: J. M. Berg, L. Crome, N. E. France
Institution: Queen Elizabeth Hospital
Language: English
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W2012628832