Congenitally Malformed Hearts: Aspects of Teaching and Research Involving Medical Students

Abstract

To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. This article describes the experiences of the latest cohort of students on this programme, who undertook varying research projects using the Birmingham Cardiac Archive, with the guidance of Professor Anderson. The projects outlined include various aspects of isomerism, encompassing both the cardiac and abdominal manifestations, as well as details of congenitally corrected transposition of the great arteries and prenatally diagnosed right aortic arch and double arch. These studies all aimed to increase the knowledge base of their respective cardiac malformations and provide a basis for further research.

Keywords: abdominal heterotaxy; congenital heart defects; congenitally corrected transposition of the great arteries; double aortic arch; left isomerism of the atrial appendages; right aortic arch; right isomerism of the atrial appendages.

Figure 1

A superior view of the base of a heart with left atrial appendage isomerism, showing the external appearance of both appendages. The left-sided appendage, indicated by the green single-headed arrow, is small and tubular. The right-sided appendage, indicated by the red single-headed arrow, is tubular at its distal parts, but its proximal part is broad based, shown by the blue double-headed arrow. The pectinate muscle of both appendages was confined to the appendage (not shown in this image), confirming this as a heart with left atrial appendage isomerism. Orientation points displayed in an attitudinally correct fashion. AO, Aorta; PA, Pulmonary artery; RV, Right ventricle; LV, Left ventricle.

Figure 2

A left posterior view of a heart with left atrial isomerism, showing the juxtaposed morphologic left atrial appendages, indicated by the red single-headed arrows. Both appendages are tubular and had a narrow base to the atrium. Parts of the lungs, indicated by the blue single-headed arrow, have been moved to the right and superiorly to expose the appendages. This heart had been operated on in life, indicated by the black stitches in both appendages. Orientation points displayed in an attitudinally correct fashion. AO, Aorta; LV, Left ventricle.

Figure 3

Antero-superior view of the internal aspect of the common atrium and the common AV valve of a heart with left atrial appendage isomerism. The anterior wall of the atrium has been dissected and reflected superiorly and laterally. A septal strand on the posterior wall of the atrium is shown by the red single-headed arrow. The common AV valve, within the common AV junction, is shown by the double headed blue arrow.

Figure 4

Four Associated Anomalies of ccTGA. (A) Peri-membranous VSD with aorta overriding and tricuspid valve straddling, from the morphological left ventricle (mLV). (B) Septal leaflet is plastered to septum of morphological right ventricle (mRV). Dashed line shows normal atrioventricular junction and solid line shows apical displacement of septal leaflet. (C) Subpulmonary obstruction due to tissue tag of septal leaflet of tricuspid valve. (D) Ventricular hypoplasia of the mRV. MV, Mitral Valve; PV, Pulmonary Vein; TV, Tricuspid Valve; mLV, morphological left ventricle; mRV, morphological right ventricle; mLA: morphological left atrium.

Figure 5

Four Associated Anomalies of ccTGA. (A) Photo from mLV showing Peri-membranous VSD. (B) Photo from mRV showing Peri-membranous VSD. (C) Photo from mRV using mirror so the ventricle appears right sided. Geographies of VSD assessed as central. Star: VSD; MV, Mitral Valve; TV, Tricuspid Valve; mLV, morphological left ventricle; mRV, morphological right ventricle.