The anomaly now known as the tetralogy of Fallot (TOF) was first described, insofar as is known, by Niels Stensen in 1671. He was also known in Latin as Nicolaus Steno. Stensen (1638–1686) was a Danish anatomist and naturalist from Copenhagen. He is said to have given up his medical career for the church, becoming the Bishop of Titiopolis in 1667. However, he remained a devoted…

What are anomalous muscle bundles of the right ventricle, also known as double-chambered right ventricle? As will be seen, these malformations really are anomalous infundibular muscle bundles. Statistics In the Congenital Cardiac Pathology Database of Boston Children’s Hospital, the diagnosis of anomalous muscle bundles of the right ventricle was made in 33 postmortem patients. The diagnosis of double-chambered right ventricle was made in 17 of our…

The malformation known as superoinferior (SI) ventricles is characterized typically by a superior morphologically right ventricle (RV), an inferior morphologically left ventricle (LV), and a relatively horizontal ventricular septum (VS). Rarely, the LV can be superior and the RV can be inferior. Our Cardiac Pathology database indicates that we have seen and studied 22 cases of SI ventricles out of a total series of 3216 autopsied…

Single ventricle means that one ventricular sinus, body, or inflow tract is present; in other words, one ventricular sinus, body, or inflow tract is absent. In single LV (morphologically left ventricle), the right ventricular sinus is absent (component 2, Fig. 17.1A ). Open full size image Fig. 17.1 (A) The normal right ventricular (RV) septal surface. (B) The normal left ventricle (LV) of septal surface. The…

In 1989, Dr. John Kirklin asked me how I thought ventricular septal defects (VSDs) should be described, named, and classified. Soto, Ceballos, and Kirklin were just about to publish their best thoughts on this important topic, and Dr. Kirklin asked me and my colleagues to do the same. Components of the Ventricular Septum The four main anatomic components that make up the normal ventricular septum are…

Now that we have considered tricuspid valve anomalies (see Chapter 13 ), mitral valve anomalies (see Chapter 14 ), and common atrioventricular (AV) canal (see Chapter 11 ), we are ready to consider how normally related and abnormally related great arteries are aligned and connected with the underlying ventricles, ventricular septum, AV canal, and AV valves. In other words, this chapter is about normally and abnormally…

What anomalies involve the mitral valve? Our database of cardiac pathologic conditions provides an answer that is summarized in Table 14.1 . TABLE 14.1 Mitral Valve Anomalies Mitral Valve Anomalies No. of Cases % of Series (n = 3216) a 1. Mitral atresia 177 5.50 2. Congenital mitral stenosis 201 6.25 3. Congenital mitral regurgitation 216 6.72 4. Congenital absence of mitral valve leaflets b 1…

How many anatomically different kinds of malformation can befall the tricuspid valve? Our database suggests that the answer is at least 12 ( Table 13.1 ). TABLE 13.1 Anatomic Types of Tricuspid Malformation Tricuspid Valve Anomaly No. of Cases Percent of Series ( n = 3216) 1. Tricuspid regurgitation, congenital 80 2.49 2. Tricuspid atresia 94 2.92 3. Ebstein’s malformation With tricuspid atresia (2) ∗ With…

Definition The morphologically right atrium (RA) opens into both the morphologically right ventricle (RV) and the morphologically left ventricle (LV) via two atrioventricular (AV) valves resulting in double-outlet right atrium (DORA), or via a common AV valve resulting in common-outlet right atrium (CORA). Anatomic Types of Double-Outlet Right Atrium/Common-Outlet Right Atrium Three anatomic types of DORA or CORA are known at the present time: (1) leftward…

Acknowledgment I would like to thank Silvio Litovsky, M.D., for his great help in analyzing the data on which this chapter is based. Definition The anomaly known as common atrioventricular canal (or atrioventricular septal defect) is characterized anatomically by incomplete separation of the atrioventricular canal into mitral and tricuspid canals. Hence, the atrioventricular canal is incompletely subdivided, or in common. The morphology and classification of these…

The anomaly now known as juxtaposition of the atrial appendages (JAA) was first described, to the best of our knowledge, by Thore in 1843 in an 11-day-old boy with left-sided JAA. Thore wrote that this neonate had “une anomalie du coeur” (an anomaly of the heart), which he described but did not name. The first paper concerning this malformation in the English literature was that of…

How common are interatrial communications —mostly the theostium secundum type of atrial septal defect and ostium primum type of atrial septal defect ( Figs. 9.1 and 9.2 )? Is atrial septal defect (ASD) a common and therefore statistically important problem, or is it a comparative rarity? As documented in Chapter 5 , interatrial communications are the second most frequent anatomic type of congenital heart disease found…

Definition The classic form of cor triatriatum (meaning “heart with three atria”) may be defined as a rare cardiac malformation in which the left atrium (LA) is subdivided into dorsal and ventral chambers by a fibromuscular diaphragm, the dorsal (posterior) chamber receiving the pulmonary veins, the ventral (anterior) chamber giving rise to the left atrial appendage and leading to the mitral valve, and the communication between…

Totally Anomalous Pulmonary Venous Connection/Drainage Definition Totally anomalous pulmonary venous connection (TAPVC) is an anomaly in which none of the pulmonary veins connect normally with the morphologically left atrium (LA). TAPVC used to be known as total anomalous pulmonary venous drainage. Then, Dr. Jesse Edwards and colleagues proposed that total anomalous pulmonary venous connection would be a more anatomically accurate diagnosis. This proposal seemed correct and…

We shall begin the specific anomalies section with systemic venous malformations because this book is organized in a venoarterial or blood-flow sequence—segment by segment, alignment by alignment, and connection by connection. The first question we must endeavor to answer is, What are the systemic venous anomalies? The answer to this question turns out to be stranger and more fascinating than anything one is likely to be…

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The segmental approach to the diagnosis of congenital heart disease is based on an understanding of the morphologic and segmental anatomy of the heart. The morphologic anatomy of the heart is summarized in Chapter 3 . The segmental anatomy of the heart is presented here. The segment-by-segment or step-by-step approach to diagnosis greatly simplifies the diagnostic problem posed even by the most complex forms of congenital…

An understanding of normal morphologic anatomy is basic to the accurate diagnosis of congenital heart disease. One of the diagnostic problems posed by complex congenital heart disease is that any cardiac chamber, valve, or vessel can be virtually “anywhere.” Consequently, the diagnostic identification of the cardiac chambers cannot be based on relative position (such as right sided or left sided) nor on function (such as venous…

In the human, an embryo may be defined as the developing organism from fertilization until the end of the second month of gestation, that is, from 0 to 60 days of life. The First Week of Life The salient events of the first week of life ( Fig. 2.1 ) are (1) ovulation, (2) fertilization, (3) segmentation, (4) blastocyst formation, and (5) the beginning of implantation.…

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