Historical Notes Coronary is derived from the Latin coronarius (pertaining to a crown) translated from the Greek stephanos (wreath) that refers to the crown-like or wreath-like arrangement of arteries that encircle the heart. This chapter deals with the coronary circulation—arterial and venous—including morphogenesis, the normal coronary arteries, and the congenital anomalies as classified in Box 29.1 . BOX 29.1 Classification of Congenital Anomalies of the Coronary…

Historical Notes In 1952, Lev called attention to congenital hypoplasia of major components of the left side of the heart. In 1958, Noonan and Nadas referred to these malformations as the hypoplastic left heart syndrome . At the severe end of the spectrum, the aortic and mitral valves are atretic, and the left ventricle is virtually nonexistent. , At the mild end of the spectrum, the…

Historical Notes In 1897, the British Medical Journal published a necropsy description of congenital pulmonary arteriovenous fistulae and, four decades later, the anomaly was recognized in a living subject. In 1865, Babington called attention to familial epistaxis, and in 1876, Legg described recurrent epistaxes and cutaneous telangiectasia in three generations. Twenty years later, Rendu published his classic description of familial epistaxes and telangiectasia (cutaneous angiomata) of…

Anomalous vena caval connections comprise a wide range of malformations that vary from minor to major, and that occur in isolation or with coexisting congenital heart disease. Persistent left superior vena cava occurs in 0.3% of the general population and in 4.3% of congenital heart disease. A left-sided inferior vena cava occurs in 0.2% to 0.5% of the general population. (This chapter focuses on isolated anomalous…

Historical Notes Truncus arteriosus was recognized in 1798, and the clinical and necropsy findings were described in 1864. Humphreys summarized the reports up to 1932, and Lev and Saphir critically reviewed published accounts during the following decade. The malformation accounts for approximately 1% to 2% of cases of congenital heart disease at necropsy, and approximately 0.7% to 1.2% of all congenital cardiac malformations. Anatomical considerations Truncus…

Historical Notes In 1797, Matthew Baillie called attention to “ a singular malformation in which the pulmonary artery arises from the left ventricle and the aorta from the right ventricle .” Seventeen years later, John Farre used the term transposition to characterize Baillie’s singular malformation. Anatomical considerations Each great artery is placed across the ventricular septum: positio means placed, trans means across . The term transposition…

Historical Notes In 1858, Thomas Peacock described hearts in which: The auricular sinuses are separated by a more or less complete septum, and there are generally two auriculoventricular apertures, while the ventricle is either wholly undivided or presents only a very rudimentary septum. The arteries which are given off are usually two in number—an aorta and a pulmonary artery. These cases correspond to hearts that consist…

Historical Notes Tricuspid atresia was described in 1817, but almost a century elapsed before the great arterial relationships were defined. Because of the morphologic heterogeneity of the malformation, “manifold anatomic combinations can result in this haemodynamic arrangement.” , Anatomical considerations Incidence has been estimated at 0.06 per 1000 live births with a prevalence of 1% to 3% of congenital heart disease. , Substantial variation exists in…

Historical Notes Thomas Peacock, in his first edition of Malformations of the Human Heart , wrote: … the orifice or trunk of the pulmonary artery is entirely impervious. A case of this description was described by John Hunter in 1783 . The child was born at the eighth month, was very livid … and died in convulsions on the thirteenth day. The pulmonary artery was found…

Historical Notes Antonio Maria Valsalva, anatomist and pathologist, was born in 1666 in the historic Emilia-Romagna region of northern Italy. The sinuses that bear Valsalva’s name consist of three small outpouchings in the wall of the aorta immediately above the attachments of each aortic cusp ( Fig. 20.1 ). In 1839, James Hope published an account of a ruptured congenital aneurysm of a sinus of Valsalva:…

Historical Notes Congenital coronary arterial fistula was described by Krause in 1865 and confirmed by Abbott in 1908 and by Trevor in 1912. Anatomical considerations Coronary arterial fistulas are the most frequent functionally significant congenital malformations of the coronary circulation, comprising 14% of all congenital coronary artery anomalies and 0.2% to 0.4% of all congenital cardiac defects (see Chapter 29 ). The right and left coronary…

Historical Notes In 1886, St. John Brooks described two cases of “an abnormal coronary artery arising from the pulmonary artery.” The diagnosis was subsequently called into question when the abnormal communication was attributed to a coronary arterial fistula. The seminal report of Bland, White, and Garland in 1933 referred to Maude Abbott’s case of a 60-year-old woman with anomalous origin of the left coronary artery from…

Historical Notes In 1593 Giambattista Carcano, Professor of Anatomy in Pavia, an ancient town in northern Italy, described the ductus arteriosus in his book on the great cardiac vessels of the fetus. However, Leo Bottali came to be associated with the arterial duct—the duktus arteriosus persistens —even though he misapplied the term to the foramen ovale. It was not until Karl von Rokitansky’s handbook of 1844…

Historical Notes The earliest report that alluded to double outlet right ventricle was published in French in 1703. Ninety years elapsed before an English language publication appeared. In 1793, John Abertheny, an assistant surgeon at St Bartholomew’s Hospital in London, described “partial transposition” of the great arteries, and in 1898, Karl von Vierordt called double outlet right ventricle partial transposition to signify that the aorta was…

Historical Notes Ventricular septal defects with obstruction to right ventricular outflow encompass a wide range of anatomic malformations and their physiologic and clinical expressions. Non-restrictive ventricular septal defects occur with pulmonary stenosis that varies from mild to severe to complete (pulmonary atresia). Restrictive ventricular septal defects occur with pulmonary stenosis that varies from mild to severe . This chapter focuses on Fallot tetralogy , which is…

Historical Notes In 1879, Henri Roger wrote, “ A developmental defect of the heart occurs from which cyanosis does not ensue in spite of the fact that a communication exists between the cavities of the two ventricles and in spite of the fact that admixture of venous blood and arterial blood occurs. This congenital defect, which is even compatible with a long life, is a simple…

Historical Notes In 1769, Giovanni Battista Morgagni described pulmonary stenosis with a patent foramen ovale, and in 1848 Thomas Peacock described Contraction of the Orifice of the Pulmonary Artery and Communication Between the Cavities of the Auricles by a Foramen Ovale. Pulmonary stenosis with reversed interatrial shunt has been called the trilogie de Fallot. Right ventricular outflow obstruction resides in a stenotic mobile dome-shaped pulmonary valve…

Historical Notes Galen was aware of the foramen ovale and its normal postnatal closure since 100 years AD. It was not until 1513 that Leonardo da Vinci characterized it, writing, “ I have found from left auricle to right auricle the perforating channel .” Leonardo’s account of a true atrial septal defect is believed to be the first record of a congenital malformation of the human…

Historical Notes In June 1864, a 19-year-old laborer was admitted to the All-Saints Hospital in Breslau (now Wroclaw), Poland, where he died 8 days later. Wilhelm Ebstein, then an assistant physician at All-Saints, performed a postmortem examination the following day and subsequently wrote a scholarly account of the clinical and necropsy findings entitled, On a Very Rare Case of Insufficiency of the Tricuspid Valve Caused by…

Historical Notes Dome-shaped pulmonary valve stenosis was described in 1761 by John Baptist Morgagni. In 1941, McAlister Gregg, an Australian ophthalmologist, described a relationship between maternal rubella and congenital abnormalities in offspring. What came to be known as the rubella syndrome consists of stenosis of the pulmonary artery and its branches with patent ductus arteriosus. , Subinfundibular stenosis or double-chambered right ventricle was described by Thomas…