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Biomedical subjects

F J Macartney

Publications and source records attributed to F J Macartney.

At least 19 recordsLinked to original sources

Double-inlet ventricle presenting in infancy. II. Results of palliative operations.

The influence of palliation on survival was studied in 191 consecutive infants, presenting at under 1 year of age, with double-inlet ventricle (1973 to 1988, median follow-up 8.5 years). Palliative operations were performed on 154 occasions in 121 patients (63%). Survival after a systemic-pulmonary arterial shunt (n = 57) and banding of the pulmonary trunk (n = 35) was comparable (84% and 77% at 1 year, 62% and 45% at 5 years), but those who underwent repair of aortic arch obstruction fared worse (n = 18, 44% and 22% at 1 and 5 years, p less than 0.001). The remainder did not undergo an operation because of balanced physiology (n = 17, 9% of entire group), complex anatomy (n = 32, 15%), or irreversible low output (n = 19, 12%). Palliative surgery, overall, had a deleterious effect on immediate survival (greater than 1 month relative risk 6.6, p less than 0.001), but, in the survivors, medium-term outcome was improved (greater than 6 months, 0.68, p less than 0.05). This effect was most marked for those undergoing a systemic-pulmonary artery shunt (less than 1 month, 2.52; greater than 6 months, 0.43); by contrast, after banding of the pulmonary trunk, with or without additional repair of the aortic arch repair, medium-term risk was not altered (greater than 6 months, 1.13 and 0.91, respectively). These data will assist the clinician in making decisions concerning the management of infants with double-inlet ventricle and in the judicious use of palliative surgery.

Anastomosis, Surgical

Fetal damage despite low-phenylalanine diet after conception in a phenylketonuric woman.

A woman with phenylketonuria gave birth to an infant with a cardiac defect and microcephaly despite introduction of a low-phenylalanine diet 5 weeks after conception (3 weeks after the first missed period). In maternal phenylketonuria fetal damage leading to reduced brain growth and cardiac malformations probably occurs within a few weeks of conception.

Abnormalities, Multiple

Long-term sequential hemodynamic evaluation of right ventricular outflow tract reconstruction using a valve mechanism.

From May, 1972, to May, 1978, right ventricular outflow tract reconstruction was performed in 20 patients with congenital heart disease. A monocusp patch constructed entirely of glutaraldehyde-stabilized calf pericardium, was employed in 19 patients, and a composite conduit consisting of a three-cusp pericardial xenograft valve in a Dacron tube was used in 1 patient. There were 2 hospital deaths (10%) and no late deaths. The follow-up ranged from 5 to 75 months (mean, 55.7 +/- 4.6 months; total, 1,002 patient months). Eleven patients had completed 5 years of follow-up at the time of writing. Hemodynamic studies were performed in 10 patients at an average of 40 months after operation and the right ventricle-pulmonary artery systolic gradient was 9.3 +/- 1.7 mm Hg. This was not significantly different in 6 patients who had a second postoperative catheterization at 51.8 +/- 2.0 months following operation. Angiography showed fully mobile, thin valve cusps. These results compare well with those reported with other types of conduits, particularly with reference to relief of obstruction.

Adolescent

Surgical treatment of ventricular septal defect in infancy. Primary repair versus banding of pulmonary artery and later repair.

Results of primary closure of ventricular septal defects are compared with those of two-stage repair, with banding of the pulmonary artery followed by debanding and closure. Apart from the high incidence of unsatisfactory results after banding and a significant morbidity with the two-stage approach, the mortality for primary repair (2.4%) is considerably lower than that achieved with the staged repair (19.3%). Primary repair of ventricular septal defect is advocated for infants resistant to maximal medical treatment. A more flexible policy is adopted for patients with multiple ventricular septal defects and those with associated anomalies.

Child

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.

Coronary Vessels

Morphology and classification of atrioventricular defects.

Anatomical studies were made on 114 necropsy specimens of atrioventricular defects with atrioventricular concordance. The malformation is characterised by disproportion between the ventricular inlet and outlet dimensions and a malorientation of the aortic valve relative to the atrioventricular valve or valves. Associated with this there is a characteristic 'scopped-out' appearance of the muscular ventricular septum, gross abnormalities of the membranous components of the septum as compared with the normal heart, and narrowing of the aortic outflow tract. Hearts with these anatomical features can be divided into partial and complete forms depending on the morphology of the atrioventricular annuli. In the partial form the septal leaflets are conjoined to give separate mitral and tricuspid orifices, the conjoined leaflets being displaced into the ventricles and usually attached to the crest of the septum. In the complete form, anterior and posterior components of the 'septal' leaflets are separate, so that a single valve orifice connects the atrial to the ventricular chambers. Further subdivision of the complete form, apart from the morphology of the anterior leaflet, is dependent upon the presence or absence of an ostium primum atrial septal defect.

Aorta

Morphology and classification of complete atrioventricular defects.

Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved possible to subdivide the hearts, depending on the morphology of the valve leaflets. Five leaflets were distinguished by the commissural pattern and their insertion to major papillary muscles. They were a posterior bridging leaflet, right and left lateral leaflets, and right and left anterior leaflets. Subdivision was made on the basis of the disposition of the anterior leaflets. In six hearts the left anterior leaflet was committed to the left ventricle and the right anterior leaflet to the right ventricle, the commissure between them being on the crest of the ventricular septum. In 39 hearts there was minimal bridging of the left anterior leaflet so that it extended between the anterior papillary muscle of the left ventricle and the medial papillary complex of the right ventricle. In eight hearts the right margin of the left anterior leaflet was attached to an apical papillary muscle, while in 17 hearts it was attached to the anterolateral papillary muscle of the right ventricle. As the bridging of the left anterior leaflet increased, so the size of the right anterior leaflet decreased, but in all hearts both leaflets were identified. These findings were compared with previous classifications of complete atrioventricular defects.

Heart Atria

Angiocardiographic appearances of atrioventricular defects with particular reference to distinction of ostium primum atrial septal defect from common atrioventricular orifice.

Preoperative distinction between common atrioventricular orifice and ostium primum atrial septal defect may be difficult. To improve diagnostic accuracy, the right and left ventricle angiocardiograms were reviewed 'blind' in 92 patients with atrioventricular defects. The true diagnosis was known from necropsy or surgery in 60. Angiocardiograms had been obtained in various projections with or without craniocaudal tilt. Those features thought to distinguish between common orifice and ostium primum were coded, together with the ventricular systolic pressures. Computerised disciminant function analysis identified the following distinguishing features: (1) right ventricular systolic pressure; (2) immediate right ventricular outflow tract opacification from the left ventricle; (3) identification of the anterior attachment of the mitral component; (4) recognition of a single straddling atrioventricular orifice; (5) passage of contrast medium above or below the anterior or posterior bridging leaflets. Feature (3) indicates that in contrast to classic teaching the direct septal attachment of the mitral component does not contribute to the 'gooseneck' in complete atrioventricular defects. The significance of (4) and (5) is that they may be identified from right as well as left ventriculography, and are more likely to be identified in oblique than standard projections. Computerisation produced a correct diagnosis in 92 per cent of known cases, and determined precise probabilities of diagnosis in the remainder.

Angiocardiography

Parachute deformity of the tricuspid valve.

A parachute deformity of the tricuspid valve occurred in a heart with atrioventricular concordance, double outlet right ventricle, and straddling mitral valve. Although to the best of our knowledge parachute deformity of the tricuspid valve has not previously been reported, in this case its presence was insignificant in relation to the other lesions.

Heart Septal Defects, Ventricular

Anatomy of aortic atresia. Cases presenting with a ventricular septal defect.

The anatomy of 58 specimens of aortic outflow tract atresia was studied. All cases had situs solitus and levocardia, 37 had atrioventricular (AV) concordance, two had common inlet to a right ventricle and 19 had mitral atresia. The great arteries were normally interrelated in all cases. Fifty-one cases had an intact ventricular septum, while seven presented with a ventricular septal defect (VSD). Of the seven with VSD, in two it was associated with a common AV orifice draining exclusively into the right ventricle in the presence of a rudimentary left ventricular chamber. In one case a small VSD accompanied combined mitral and aortic atresia. In the other four cases the left ventricles and mitral valves were fairly normal in size; the VSD was subpulmonary in three cases, due to infundibuloventricular malalignment, and perimembranous in one. These last four cases are of particular interest since they could be amenable to surgical correction. Possible approaches to surgical treatment and morphologic features pertinent to them are described and discussed.

Aortic Valve

Univentricular heart of right ventricular type with double or common inlet.

Seventeen cases are described in which both atria connect directly to a chamber with right ventricular characteristics. The atria connected through separate atrioventricular valves in six hearts and a common valve in 11. All hearts had a posterior rudimentary chamber. The septum which separated it from the main chamber was directed to the crux of the heart. Ten hearts were from patients with atrial situs solitus and seven from patients with atrial situs ambigous. Arterial connections were concordant in three cases, had a double outlet from the main ventricular chamber in nine and single outlet of the heart in five. The patent artery always arose from the main chamber, with pulmonary atresia in three and aortic atresia in two. This and other studies indicate that double inlet atrioventricular connection does not predict the morphology of the main chamber. Although usually associated with a main chamber of left ventricular type, it may also be associated with a main chamber having right ventricular characteristics. Both types should be considered as univentricular hearts; the posterior chamber in hearts of right ventricular type are analogous to the anterior chamber in univentricular hearts of left ventricular type and are a rudimentary chamber rather than a hypoplastic ventricle. In the right ventricular form of univentricular heart, the trabecular zone of the rudimentary chamber is of left ventricular type.

Abnormalities, Multiple

Surgical technique to reduce the risks of heart block following closure of ventricular septal defect in atrioventricular discordance.

Traumatic heart block remains a major concern after ventricular septal defect (VSD) closure in patients with atrioventricular discordance. A technique of closing the VSD, placing the suture line on the morphologically right side of the septum without opening the systemic ventricle, is described. This was used in 13 consecutive patients. The VSD was closed through the right atrium in eight patients, the left ventricle in three patients, the right atrium and the left ventricle in one patient, and through the right ventricle in one patient with atrioventricular disordance and ventriculo-arterial concordance. All patients were in sinus rhythm preoperatively, two exhibited atrioventricular dissociation before intracardiac manipulation began, and 11 patients were in sinus rhythm postoperatively. No major arrhythmia could be attributed to the closure of the VSD.

Adolescent

Nomenclature of the univentricular heart.

A simple system for the analysis and classification of the univentricular heart is described. The basis of this approach is the use of a step by step sequential nomenclature with attention paid to the morphology, connexions and relationships of each cardiac segment in turn. Three major morphological types of univentricular heart are identified and these may be distinguished, by their characteristic trabecular patterns, into hearts of left ventricular type, right ventricular type and indeterminate type. With the exception of the last it is usual for a rudimentary second chamber to be present upon the use of a clear definition of "ventricle", depending on readily ascertainable anatomical characteristics. Each morphological type of univentricular heart may be associated with a wide variety of different connexions and intracardiac relationships and it is neither possible nor desirable to describe these in terms of a rigidly structured classification. Adherence to the simple semantic framework outlined would resolve much of the confusion and controversy which exists in relation to the univentricular heart.

Heart Septal Defects, Ventricular

Morphology and conducting tissue in univentricular hearts of right ventricular type.

22 specimens of univentricular hearts of right ventricular type were examined morphologically. 6 hearts were sectioned for study of the conducting tissue. All the specimens had rudimentary second chambers, which were frequently so small as to have been overlooked, both angiographically and at initial morphological study. The segmental connexions and intracardiac relationships varied widely. Examples were found with 2 atrioventricular valves, a common valve and with absence of either the right or the left atrio-ventricular connexion. In some cases straddling of an atrioventricular valve was observed. The rudimentary chambers always lay posteriorly but varied between a position to the left of the ventricle and one to the right. The septum between ventricle and rudimentary chamber ran to the crux of the heart in all but 2 instances. In most specimens both great arteries arose from the ventricle (double outlet ventricle), so that the rudimentary chamber was solely a trabecular pouch. 4 hearts had concordant arterial connections with the aorta arising from a posterior "outlet" chamber. Pulmonary atresia was found in 5 cases and aortic atresia in 2. The conduction system originated from a normally placed atrioventricular node in 4 out of 6 cases and was related to the trabecular septum in 5. The penetrating bundle usually descended onto the crest of the septum at the "crux" of the heart and bifurcated astride it. In 2 cases with an absent atrioventricular connexion the penetrating bundle originated from an abnormally placed node and in one of these specimens a sling of conducting tissue was identified with dual pathways.

Atrioventricular Node

Ventricular morphology in the univentricular heart.

The ventricle morphology is described in hearts in which the entire atrial inputs are committed to a single chamber in the ventricular mass, this being considered the criterion for definition of the univentricular heart. Such a definition does not exclude from the univentricular category hearts with two chambers in their ventricular mass providing that the second chamber does not receive a direct atrial input. Three types of ventricular morphology were identified: hearts with a main chamber of left ventricular pattern and a rudimentary chamber of right ventricular pattern; hearts with a main chamber of right ventricular pattern and a rudimentary chamber of left ventricular pattern and hearts with a sole chamber in their ventricular mass of indeterminate pattern. Hearts may also exist of right or left ventricular type without rudimentary chambers. Variation was noted in type and position of the rudimentary chambers. Chambers of either right or left ventricular type were found supporting either or both great arteries (outlet chambers), and also without a direct outlet (trabecular pouches). Right ventricular rudimentary chambers were usually anterior but could be right or left sided; left ventricular chambers were posterior but again could be right or left sided. Hearts with these ventricular morphologies possessed either double inlet atrioventricular connexions, or absence of the right or left atrioventriuclar connexion. In double inlet ventricles, cases were observed with two valves, a common valve, one imperforate valve, and with straddling atrioventricular valves. A straddling valve was also found in hearts with absence of one atrioventricular connexion. Further variation was found in the pattern of arterial connexions. It is concluded that ventricular morphology is an important feature of the iniventricular heart but must be considered in the context of other important and variable features.

Heart Septal Defects, Ventricular

Angiocardiography in the pre-operative evaluation of patients with univentricular hearts.

Diagnosis of the univentricular heart and recognition of its most important variations depends on the establishment of atrial situs, the demonstration of the type and mode of the atrio-ventricular connexion, the identification of the presence or absence of a rudimentary chamber, the establishment of its morphology, together with that of the main chamber, and classification of the arterial connexion. A logical means of demonstrating these features is presented, depending on bronchial visualization, one or possible two injections of contrast medium into the main chamber, followed if necessary by selective injection into the rudimentary chamber, and in exceptional cases, demonstration of the atrial septum by atrial injection. The advantagess of cranio-caudal tilt and oblique projections are discussed in detail.

Angiocardiography