Congenital obstruction to left atrial flow can originate at or near the junction of pulmonary veins and left atrium (pulmonary vein stenosis), within the left atrium (cor triatriatum), immediately above the mitral valve (supravalvular stenosing ring), or within the mitral apparatus (mitral stenosis) ( Fig. 9.1 and Box 9.1 ). Pure or relatively pure forms of each defect are emphasized in this chapter, although a variety…

Historical Notes In 1760, the Prussian anatomist Johann Friedreich Meckel characterized coarctation of the aorta as an “extraordinary dilatation of the heart which came from the fact that the aortic conduit was too narrow.” Saul Jarcho’s historical papers underscored the accuracy of early accounts of this congenital malformation. Coarctation of the aorta Anatomic considerations Coarctation of the aorta is typically located near the aortic attachment of…

Historical Notes The bicuspid aortic valve was first identified in the early 16th century by Leonardo da Vinci in his remarkable Anatomical, Physiological, and Embryological Drawings released by Dover Publications in a facsimile edition. Thomas Peacock recognized the tendency for bicuspid aortic valves to become stenotic in his On Malformations of the Human Heart (1858). Congenital aortic stenosis Normal aortic valves are composed of a connective…

Historical Notes More than a century elapsed before Karl von Rokitansky applied the term corrected to a hitherto undescribed form of transposition of the great arteries: The left atrioventricular valve and the left-sided ventricle resembled the usual right atrioventricular valve and right ventricle. The aorta is positioned somewhat left and anterior…. The right-sided ventricle… is finely trabeculated, as usually seen in the left-sided ventricle. The venous…

Historical Notes Congenital absence of the pericardium was recognized by M. Realdus Columbus in 1559, and by Matthew Baille in 1793, but it was not until 1959 that the anomaly was clinically diagnosed in a chest x-ray. The pericardial abnormality varies from a localized defect to complete absence. The sternum, abdominal wall, pericardium, and part of the diaphragm arise from somatic mesoderm. Morphogenesis of congenital defects…

Historical Notes Congenital complete atrioventricular block was recognized in 1901 when Morquio described familial recurrence with Stokes-Adams attacks and death in childhood. In 1908, van den Heuvel published the electrocardiogram of a patient with complete heart block and syncopal episodes that dated from infancy. Thirteen years later, White and Eustis described slow fetal heart rate; at birth, the electrocardiogram disclosed complete heart block. Davis and Steche…

Historical Notes Dextrocardia in situs inversus was known to the anatomist-surgeon Marco Aurelio Severino in 1643 and was one of the first recognized congenital malformations of the heart. Nearly a century and a half elapsed before Matthew Baillie’s account of “complete transposition in the human subject, of the thoracic and abdominal viscera, to the opposite side from what is natural.” In 1958 Thomas Peacock indicated that…

Practicing cardiology remains a powerful experience for the clinician. The ability to look, feel, and listen remains the most highly rewarding foundation of the diagnostic capability, nowhere more evident than in the assessment of subjects with congenital heart disease. The stethoscope is a lasting symbol of healing professions. Invented in 1816 by Laënnec in France, the word “stethoscope” originates from the Greek “stethos,” meaning “chest,” and…

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