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

Josef Gehrmann, MD; Thomas Krasemann, MD; Hans Gerd Kehl, MD; Johannes Vogt, MD, PhD
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*From the Children’s Hospital, Department of Pediatric Cardiology, Westfaelische Wilhelms-Universitaet Muenster, Muenster, Germany.

Correspondence to: Josef Gehrmann, MD, Children’s Hospital Department of Pediatric Cardiology, Westfaelische Wilhelms-Universitaet Muenster, Albert-Schweitzer-Strasse 33, 48149 Muenster, Germany; e-mail: JGehrman@uni-muenster.de



Chest. 2001;120(4):1368-1371. doi:10.1378/chest.120.4.1368
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The term hypoplastic left-heart syndrome was first proposed by Noonan and Nadas1 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 Romberg2 in 1846 and Canton3 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 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.

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