F J Macartney

Nomenclature and classification of congenital heart disease.

At present there is no universally accepted nomenclature for congenital cardiac malformations. Much of the controversy results from failure to distinguish the structural connections of the heart from the morphology and spatial relations of its components. The confusion is compounded by an abundance of individual definitions, many of them speculative. The present article proposes a totally descriptive nomenclature. It describes in turn the connections of the cardiac segments, their morphology, their relations, and additional anomalies in any segment. Each step in the segmental approach is discrete. The overall effect is to force a succinct and comprehensive description of any cardiac malformation, no matter how complex.

Sequential chamber localization--logical approach to diagnosis in congenital heart disease.

A nomenclature is described for congenital heart disease employing sequential chamber localization. It is an eclectic system based in part upon the previous classifications of Van Praagh and Kirklin. It links together the atrial, ventricular, and arterial segments of the heart and then permits tabulation of associated anomalies. The atrial segment of the heart can exist as situs solitus, situs inversus, or situs ambiguus. Atrioventricular connexions can be concordant or discordant. In certain circumstances the terms concordant and discordant cannot be used. These are in the presence of primitive ventricle and in the presence of situs ambiguus. Alternative terms are described for these contingencies. Ventriculo-arterial connexions can be (a) normal; (b) transposition; (c) double outlet ventricle; or (d) single arterial trunk. These are defined as connexions; relations are relegated to secondary position. Associated anomalies are categorized in terms of venous return, atria, atrioventricular junction, ventricles, and great arteries. Controversial topics are discussed with regard to previous definitions.

Pulmonary atresia and intact ventricular septum: a revised classification.

The dismal outlook for patients with pulmonary atresia with intact ventricular septum may be related to associated right ventricular hypoplasia. Study of 32 autopsy specimens and 46 angiocardiograms of neonates with this lesion suggested that the cavitary hypoplasia was related to massive hypertrophy of the right ventricular wall. This hypertrophy was sufficient to obliterate the trabecular and/or infundibular portion of the ventricular cavity entirely in one‐third of the cases; this observation forms the basis for a revised classification of these hearts. Three autopsies and 14 angiograms of neonates with critical pulmonary stenosis were examined. Hearts with obliterated infundibular and trabecular cavities had thicker walls and smaller tricuspid valves, as estimated angiographically or at autopsy, than those in which the normal three portions of the ventricular cavity were represented.

Persistent truncus arteriosus: A study of 66 autopsy cases with special reference to definition and morphogenesis

Abstract Sixty-six hearts were examined in which a single arterial trunk, leaving the base of the heart through a single semilunar valve, supplied the aorta, pulmonary artery and coronary arteries. Careful attention was paid to the infundibular morphologic features in these hearts, and these were compared with findings in 24 hearts with single aortic trunk, pulmonary atresia and ventricular septal defect. It was concluded that the two anomalies represented morphologically discrete conditions. This was particularly true with regard to the disposition of the infundibular septum, the ventriculo-infundibular fold and the relation of the coronary arteries to the semilunar sinuses. Although it is theoretically possible for a heart with true persistent truncus arteriosus to have absence of the pulmonary trunk and right and left pulmonary arteries, it is argued that such hearts (“truncus type IV”) are best classified as pulmonary atresia with ventricular septal defect. It is also argued that hearts with a common arterial trunk supplied through discrete ventricular outflow tracts and two semilunar valves are best considered examples of aorticopulmonary window. It is suggested that persistent truncus arteriosus is best defined as that condition in which a single arterial trunk leaves the heart through a single semilunar valve and supplies the aorta, one or both pulmonary arteries and the coronary arteries.

Sequential segmental analysis of congenital heart disease.

1 Department of Paediatrics, Cardiothoracic Institute, Brompton Hospital, London, England; 2 Department of Pathology, Wilhelmina Gasthuis, University of Amsterdam, The Netherlands; 3 Department of Cardiology, Hospital for Sick Children, Toronto, Canada; 4 Thoracic Unit, Hospital for Sick Children, London, England; 5 Department of Pediatric Cardiology, Centro Ramon y Cajal, Madrid, Spain; 6 Institute of Child Health, University of Liverpool, Royal Liverpool Childrens Hospital, Liverpool, England; and 7 Department of Paediatrics, Guys Hospital, London, England

Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification.

Sixty patients with pulmonary atresia and intact ventricular septum (PA:IVS) presenting from 1970 to 1980 are reviewed. Three groups of patient are discussed: those with tripartite right ventricles, those with no trabecular portion to the cavity, and those with neither trabecular nor infundibular portions. The decrease in early mortality for neonates with PA:IVS since 1977 (one death in 15 patients) supports our current management policy of preoperative prostaglandin El infusion with transpulmonary valvotomy (for patients with an infundibular cavity) combined with a left modified Blalock‐Taussig shunt using a Gore‐Tex prosthesis. Tricuspid valve growth, estimated by serial angiograms in 12 patients, was greater if right ventricle‐to‐pulmonary artery continuity was established. Later definitive repair was attempted in nine patients, with two early deaths; five underwent right ventricular outflow tract reconstruction and four had modified Fontan procedures. Neonates with critical pulmonary stenosis are also discussed. Their neonatal mortality (nine deaths in 20 patients) was similar to that of comparable patients with PA:IVS, but their actuarial survival at 5 years (55%) was superior (36% at 5 years).

Two dimensional echocardiographic diagnosis of situs.

At present there is no reliable method of recognising atrial isomerism by two dimensional echocardiography. We therefore used two dimensional echocardiography to examine 158 patients including 25 with atrial isomerism and four with situs inversus. Particular attention was paid to the short and long axis subcostal scans of the abdomen. Using the position of the inferior vena cava and the aorta with respect to the spine it was possible to separate those with situs solitus from the others. Two false positives for abnormal situs had exomphalos. In situs solitus the aorta lay to the left of the spine and the inferior vena cava lay to the right. One patient with situs solitus and azygos continuation of the inferior vena cava also had inferior vena cava to right atrial connection. In the four patients with situs inversus the mirror image of the normal pattern was present. In nine patients with right isomerism the inferior vena cava and aorta ran together on one or other side of the spine. The inferior vena cava, anterior to the aorta at the level of the diaphragm, received at least the right hepatic veins (normal or partial anomalous hepatic venous connection). Of the 16 patients with left isomerism, 14 had azygos continuation of the inferior vena cava which was visualised posterior to the aorta in all but two. All patients with left isomerism had total anomalous hepatic venous connection to one or both atria via one or two separate veins. Two dimensional echocardiography therefore provides the means of detecting abnormal atrial situs and of diagnosing right or left isomerism in the great majority of patients, if not all.

Straddling and overriding atrioventricular valves: morphology and classification.

Abstract Fifty-seven hearts are described in which either the orifice or tension apparatus of an atrioventricular (A-V) valve was related to both sides of a septum in the ventricular mass. In most of the hearts both the orifice overrode the septum and the tension apparatus straddled the septum. In some hearts straddling of the tension apparatus was present in the absence of overriding of the anulus while in two hearts the anulus overrode in the absence of straddling. Hearts were observed in which the chamber receiving all of one valve and the straddling portion of the other valve had either right or left ventricular morphologic features, and in each type the chamber receiving only part of the straddling valve was found either to the right or the left. When the straddling valve was morphologically a tricuspid valve it always straddled the posterior part of a septum that never extended to the crux; when it was morphologically a mitral valve it always straddled the anterior part of a septum that did extend to the crux. This arrangement was found irrespective of the relations of the chambers. Four basic groups were therefore defined: straddling of the mitral and tricuspid valves in the setting of A-V concordance and discordance, respectively. However, the degree of override of the straddling valve was frequently such that the A-V connection present was double inlet ventricle rather than concordance or discordance. Indeed, in each group a series of anomalous hearts was found between the extremes of concordance or discordance and double inlet. In categorizing the A-V connections, these series were divided at their mid points. The hearts with double inlet connections were considered univentricular hearts and their chambers described accordingly. In each series hearts were found with unequally committed common valves that were virtually identical to the hearts with straddling right or left valves. They were therefore included in the study as were two hearts in which both right and left valves straddled. Examination of the conduction tissues in examples of each series showed that the position of the connecting node depended on whether or not the septum extended to the crux, anterior systems being found when it did not and either anterior or regular systems when it did, the latter variation depending on the A-V connection present. The study shows that straddling or overriding valves can be easily catalogued if attention is paid to the A-V connection and the morphologic features and relations of the ventricular chambers.

Cross-sectional echocardiographic diagnosis of systemic venous return.

To determine the sensitivity and specificity of cross-sectional echocardiography in diagnosing anomalous systemic venous return we used the technique in 800 consecutive children with congenital heart disease and whom the diagnosis was ultimately confirmed by angiography. Cross-sectional echocardiography was performed without prior knowledge of the diagnosis in all but 11 patients, who were recalled because of a known abnormality of atrial situs. The sensitivity of cross-sectional echocardiographic detection of various structures was as follows: right superior vena cava 792/792 (100%); left superior vena cava 46/48 (96%); bilateral superior vena cava 38/40 (95%); bridging innominate vein with bilateral superior vena cava 13/18 (72%); connection of superior caval segment to heart (coronary sinus or either atrium) (100%); absence of suprarenal inferior vena cava 23/23 (100%); azygos continuation of the inferior vena cava 31/33 (91%); downstream connection of azygos continuation, once seen, 21/21 (100%); partial anomalous hepatic venous connection (one hepatic vein not connected to the inferior vena cava) 1/1 (100%); total anomalous hepatic venous connection (invariably associated with left isomerism) 23/23 (100%). The specificity of each above diagnoses was 100% except in one infant with exomphalos in whom absence of the suprarenal inferior vena cava was incorrectly diagnosed. Thus cross-sectional echocardiography is an extremely specific and highly sensitive method of recognizing anomalous systemic venous return. It is therefore of great value of planning both cardiac catheterisation and cannulation for open heart surgery.

Percutaneous balloon valvuloplasty for pulmonary valve stenosis in infants and children.

Pulmonary valve stenosis was relieved by balloon dilatation during cardiac catheterisation on 27 occasions in 23 infants and children aged 7 days to 12 years, median 31 months (three aged less than 2 weeks). Pulmonary valve diameter was estimated by cross sectional echocardiography to assist in the choice of balloon size. Before dilatation the right ventricular systolic pressure ranged from 41 to 190 (median 92) mm Hg and was suprasystemic in 10 patients. There were significant reductions in the ratio of right ventricular to systemic systolic pressure and pulmonary systolic pressure gradients immediately after balloon dilatation. Twelve patients underwent recatheterisation (11 at six months and one at one week after balloon dilatation), which showed further improvement with significant reductions in right ventricular pressure or pulmonary valve gradient or both, particularly in the patients with the least satisfactory initial results. This improvement was attributed to resolution of the obstruction at infundibular level. Repeat pulmonary valve dilatation was successfully performed in four patients who had poor results after initial dilatation. Balloon pulmonary valvotomy appears to provide good short term and medium term relief of pulmonary valve stenosis and may obviate the need for surgery in many cases. An apparently poor immediate haemodynamic result does not preclude a good longer term result.