Hypoplastic Left-Heart Syndrome: The First Description of the Pathophysiology in 1851; Translation of a Publication by Dr. Bardeleben From Giessen, Germany

Abstract

The term hypoplastic left-heart syndrome was first proposed by Noonan and Nadas 1 Noonan AJ Nadas AS The hypoplastic left heart syndrome: an analysis of 101 cases. Pediatr Clin North Am. 1958; 5: 1029-1058 Crossref PubMed Scopus (266) Google Scholar in 1958 and comprises a continuum of congenital cardiac anomalies characterized by underdevelopment of the aorta, aortic valve, left ventricle, mitral valve, and left atrium. The first published cases of aortic atresia were reported by Romberg 2 Romberg ELR On arctation and closure of the arteries in disease. Edinburgh Med Surg J. 1846; 65: 149 Google Scholar in 1846 and Canton 3 Canton Congenital obliteration of origin of the aorta. Transactions of the Pathological Society of London. 1848–1849/1849–1850; 2: 38 Google Scholar in 1849, and are more or less restricted to brief pathologic and anatomic descriptions. Neither pathophysiologic nor pathogenetic aspects were discussed. The account of Dr. Bardeleben, 4 Bardeleben K Obliteration of the left ostium arteriosum in the heart of a half-year old infant. in: Virchow R Reinhardt B Archiv für pathologische anatomie und physiologie und für klinische medicin (vol III). G. Reimer, Berlin, Germany1851: 305-312 Google Scholar written in 1851, was probably the first complete description of the clinical features, the pathologic-anatomic characteristics, and the pathophysiology of the hypoplastic left-heart syndrome. It includes an illustration of this malformation, a hypothesis on the etiology, and reflections on the symptoms. This historical publication documents the correct understanding of the circulation in this disorder, realizing that survival depends on a patent ductus arteriosus. Also, a differentiated theory on the development of cyanosis is provided. Pathogenetically, Dr. Bardeleben attributed aortic atresia not to a simple arrest of normal development, but to a pathologic process developing during intrauterine life. The precision and the clarity of the description and figures is striking. This account given by Dr. Bardeleben remains entirely valid even today. The following literal translation of the original manuscript gives the reader a sense of the original and gives credit to this early, superb, as yet unknown description (in the international literature) of a congenital heart disease.