James L. Wilkinson

Congenitally corrected transposition of the great arteries: Morphologic study of 32 cases

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries.Tis condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with inverted (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.

Univentricular atrioventricular connection: the single ventricle trap unsprung

tDepartment of Paediatrics, Cardiothoracic Institute, Brompton Hospital, London, UK; 2Thoracic Unit, Hospital for Sick Children, London, UK; ZEvelina Childrens Department, Guys Hospital, London, UK; 4Department of Pathology and Interuniversity Cardiology Institute, Academic Medical Centre, Amsterdam, The Netherlands; ~Department of Pediatric Cardiology, Hospital for Sick Children, Toronto, Canada; 6Paediatric Cardiologist, Hospital for Sick Children, Edinburgh, UK; 7Department of Cardiology, Freeman Hospital, Newcastle upon Tyne, UK; SDepartment of Paediatric Cardiology, Centro Ramon y Cajal, Madrid, Spain; 9Paediatric Cardiologist, Wessex Cardiac and Thoracic Centre, Southampton Western Hospital, UK; tODepartment of Radiology, University of Alabama in Birmingham, Alabama, USA; Department of Pathology, University of Padova, Italy; 2Paediatric Cardiologist, Royal Liverpool Childrens Hospital, UK; t3Department of Cardiothoracic Surgery, University of North Carolina, Chapel Hill, North Carolina, USA; Z4Division of Pediatric Cardiology, Childrens Hospital, Pittsburgh, Pennsylvania, USA

Is “tricuspid atresia” a univentricular heart?

SummaryIt is evident that the term “tricuspid atresia” is imprecise and can be used to describe hearts with widely varying morphology. It might be preferable if the term were reserved for one or other of these variants, probably that which is the most commonly encountered. In this anomaly there is total absence of the connection between the right atrium and the ventricular mass, and the ventricular morphology is that of a univentricular heart. Only if a detailed segmental analysis of the heart is performed can the distinction be made between this and other malformations in which the tricuspid valve is imperforate and in which any ventricular morphology can be encountered. Tricuspid atresia is usually, but not always, univentricular.

Captopril in treatment of infant heart failure: a preliminary report

We have studied retrospectively 18 infants who have received captopril for treatment of severe heart failure due to left-to-right shunts with pulmonary hypertension. Captopril has been administered in doses of up to 3.5 mg/kg/day (mean 2.47 mg/kg/day). Maintenance treatment with digoxin and frusemide was continued but potassium-sparing diuretics were stopped in most patients. The mean period of assessment was 19 days before and 27 days after commencing captopril. The mean daily weight gain before captopril was -7 g and after its introduction was + 13 g (P less than 0.001). There were statistically significant (P less than 0.05) falls in mean heart rate and respiratory rate and rises in plasma sodium concentration and feeding score. Plasma urea concentration fell but this did not reach statistical significance. Two patients suffered hypotension after increments in captopril dosage and subsequently had a rise in plasma urea and creatinine values. This adverse reaction may be linked to the presence of hyponatraemia. This preliminary report shows captopril may be useful in the control of severe heart failure in infancy.

Morphology of pulmonary atresia with ventricular septum in patients dying after operation

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.

Tricuspid atresia and univentricular heart

In our opinion the Comment offered by Bharati and Lev [2] restates the views that our article [I] set out to question. It does not consider the morphological evidence we presented against those views, or our d .d ative proposal. Thus, Dr. Bharati and Dr. Lev did not consider the distinction we made between an absent connection and an imperforate valve nor the distinction we made between a ventricle and a rudimentary chamber . They also did not consider the morphological similarity between the anterior chamber in classic tricuspid atresia and that in single ventricle, which is the cornerstone of our argument that the two anomalies are variants of univentricular heart . Bharati and Lev describe the artery marking the Posterior extent of the septum in tricuspid atresia as the posterior descending coronary artery . But the normal posterior descending coronary artery descends from the atrioventricular junction at the crag . The artery shown by Bharati and Lev in their IA, as also shown in our Fig . 5, does not descend from the crux . Our figure shows that this artery descends from the acute margin of the atrioventricularjunction and more resembles the normal right marginal artery than the posterior descending artery from a dominant right system . The artery should surely be judged relative to an external reference sept Point . We consider the position of artery and septum adjacent to the acute margin as evidence cotTOborating our belief that the septum in classic tricuspid atresia is a trabecular septum, similar traced Septum seen in single ventricle and illuslar n our Fig. 4. We do not believe this trabecuseptum is analogous to the posterior part of the septum found in the normal heart . In enngour article we presented evidence for considclassic tricuspid atresia and single ventricle as unventricular hearts . This evidence still awaits Pediatric Cardiology

Anatomy and conducting tissue in partial atrioventricular canal defects

Partial atrioventricular canal defects, or ostium primum ASDs, are in most respects very similar to complete atrioventricular canal defects in regard to both anatomy and conducting pathways.

Conducting tissue in complete atrioventricular canal malformations

In order to understand the abnormahties which occur in the conducting system in atrioventricular canal malformations, it is desirable first to review the normal features of the atrioventricular node, bundle of His and bundle branches.