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Biomedical subjects

F J Macartney

Publications and source records attributed to F J Macartney.

At least 73 records · Page 4Linked to original sources

Cross-sectional echocardiographic recognition of interruption of aortic arch between left carotid and subclavian arteries.

Interrupted aortic arch is a rare condition, usually lethal in early infancy without treatment. The only characteristic feature on conventional non-invasive investigation is peripheral pulse inequality, which indicates ductal construction, and therefore may be absent or transient and preterminal. We report the cross-sectional echocardiographic findings in seven patients with aortic arch interruption between the left carotid and subclavian arteries. Their ages were 1 day to 7 months (median 7.5 days). The arterial connection was concordant in four, double outlet right ventricle in two, and truncus arteriosus in one. In each case the ascending aorta was small in comparison to the pulmonary trunk. From the suprasternal approach the ascending aorta could be seen to terminate in the left carotid artery, and the ductus to continue smoothly into the descending aorta, with no vestige of an aortic arch linking its ascending and descending portions. The left subclavian artery was seen to arise distal to the ductus in all but one patient. All four patients with ventriculoarterial concordance had pronounced subaortic stenosis caused by posterior displacement of the infundibular septum. Cross-sectional echocardiography therefore provides the only accurate method of non-invasive diagnosis of this condition. It permits early treatment with prostaglandins to prevent ductal closure, a planned approach to cardiac catheterisation, and a further means of investigating the nature of subaortic stenosis in this condition.

Aorta, Thoracic↗

Suprasternal cross-sectional echocardiography in assessment of patient ducts arteriosus.

Ninety-four patients with patent ductus arteriosus, whose ages ranged from 28 weeks gestation to 8 years, were assessed by suprasternal cross-sectional echocardiography. A further group of 37 cases without a patent ductus arteriosus were assessed by the same technique. The appearances of the ductus via this approach varied according to the ventriculoarterial connections. Reliable assessment of patency was possibly in 87 cases. In seven cases where the ductal lumen was less than 2 mm in size, a false negative diagnosis was made. In the 37 patients without a patent ductus arteriosus, no false positive diagnoses were made. Suprasternal cross-sectional echocardiography enables a reliable assessment of ductal patency, provided the lumen falls within the range of lateral resolution of the equipment being used.

Aorta, Thoracic↗

Cross-sectional echocardiographic assessment of conditions with atrioventricular valve leaflets attached to the atrial septum at the same level.

Ninety two patients with both atrioventricular valves attached to the atrial septum, roofing a perimembranous inlet ventricular septal defect, were assessed by cross-sectional echocardiography. In the group, 42 had an isolated perimembranous inlet ventricular septal defect, 31 had atrioventricular discordance, nine an atrioventricular septal defect with intact interatrial septum, and 10 a straddling atrioventricular valve. In all but those with an atrioventricular septal defect the left atrioventricular valve had the appearance of a morphologically mitral valve. In the former lesion the atrioventricular junction was "sprung" and a cleft between the anterior and posterior bridging leaflets was identified in all. A straddling valve was identified by tensor apparatus from one atrioventricular valve in both ventricular chambers. Atrioventricular discordance was diagnosed by identifying the systemic and pulmonary venous atria and then assessing the morphology of the draining atrioventricular valves. Thus, with cross-sectional echocardiography, the constellation of abnormalities that give rise to lack of offsetting of the atrioventricular valves can be reliably identified.

Echocardiography↗

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.

Adolescent↗

Tricuspid atresia: analysis of coronary artery distribution and ventricular morphology.

There is still disagreement concerning the precise nature of the anterior ventricular chamber in "tricuspid atresia". Some argue that it is a right ventricle, while our own previous studies have suggested it is comparable to the outlet chamber seen in classical "single ventricle". We have compared the morphology of the anterior ventricular chambers in 48 examples of tricuspid atresia (absent right atrioventricular connection), 24 hearts with double inlet to the left ventricular chamber, and 15 hearts with pulmonary atresia and intact ventricular septum. Since there is further disagreement concerning the nature of the coronary artery which delimits the posterior extent of the ventricular septum in these hearts, we analysed the position of this vessel relative to external reference points on the atrioventricular junction. No significant difference was found with respect to the morphometry of hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet. In both groups, however, significant differences were shown between hearts with ventriculoarterial concordance and discordance. Coronary artery disposition was the same in both groups and different from that found in hearts with pulmonary atresia and intact septum. We conclude that the ventricular morphology is comparable in hearts with tricuspid atresia (absent right atrioventricular connection) and those with double inlet to a left ventricular chamber. The lesions are distinguishable by their atrioventricular connection which is nevertheless univentricular in both.

Coronary Vessels↗

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.

Heart Defects, Congenital↗

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 E1 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).

Blood Pressure↗

Left-sided obstructive lesions in atrioventricular septal defects: an anatomic study.

Actual and potential factors that create an obstruction to the flow within the systemic side of the heart have been analyzed in 114 specimens with atrioventricular septal defects. Hearts with separate right and left atrioventricular orifices and with common atrioventricular orifice were included. Potential inlet obstruction was due to tethering of the "cleft" of the left atrioventricular valve to the septum in 17.5%, incomplete "cleft" in 8%, and deformed valve leaflets in 70% of cases. Potential factors predisposing to left ventricular outflow tract obstruction were seen in 70% of cases, 21% being due to abnormal position of the papillary muscle and the remainder due to intrinsic hypoplasia of the outflow tract. Anatomic factors producing unequivocal obstruction were seen in 29% of cases. Twenty-two percent had obstructions at the inlet level, of which 13% were due to malformations of the left atrioventricular valve or the left component of a common valve. The other 9% had overall inlet obstruction due to severe hypoplasia of the left ventricle itself. Unequivocal outlet obstruction was observed in 7% of cases, 5% due to abnormal disposition of papillary muscles in the outflow tract and 2% to aneurysm of the interventricular septal remnant. Although anatomic obstruction occurs in the minority of cases (29%), this report shows that potential obstruction must be taken into account when considering surgical repairs.

Heart Septal Defects↗

Infradiaphragmatic total anomalous pulmonary venous drainage: surgical treatment and long-term results.

Since 1971, 20 infants (1 day to 3 months old) with infradiaphragmatic total anomalous pulmonary venous drainage (TAPVD) have undergone repair at The Hospital for Sick Children, Great Ormond Street, London. All patients were seen with severe cyanosis (mean partial pressure of arterial oxygen, 35.8 mm Hg) and pulmonary edema. All had a persistent ductus arteriosus. Right-sided pressures were equal to or greater than systemic in 17 patients. During surgical repair, the confluence of the pulmonary veins was anastomosed to the left atrium. The descending vein was ligated in 11, divided in 4, and left open in 5 patients. Atrial septal defects were closed with a patch in 6 and sutured in 14. Eight patients died (40%). Acidosis on admission was significantly greater among nonsurvivors (mean pH, 7.29 +/- 0.02) than survivors (mean ph, 7.37 +/- 0.02) (p less than 0.05). Twelve survivors are well four months to 8 years after operation. All are asymptomatic and none requires cardiac medication. Six have undergone repeat catheterization, demonstrating normal pressures and no shunts. We conclude that the mortality of patients with infradiaphragmatic TAPVD depends mainly on the condition of the child on admission.

Blood Pressure↗

Changes in ventricular depolarization in patients in sinus rhythm following closure of ventricular septal defect associated with atrioventricular discordance.

Closing the ventricular septal defect in patients with atrioventricular discordance with sutures placed through the defect onto the morphologically right septal surface should avoid production of complete heart block. To discover whether this procedure otherwise affects conduction, standard electrocardiogram (ECG) were compared preoperatively and postoperatively in 11 such patients. Operation had lengthened the PR interval in one and shortened it in another. The QRS interval remained unchanged in 5 patients thought 2 of these showed minor changes in QRS configuration. Four patients showed QRS prolongation (increase greater than 20 msec) with delayed conduction towards the right ventricle and the initial QRS vector preserved, i.e., morphologically right bundle branch block. Two patients showed QRS prolongation with initial QRS vector alteration and delayed conduction towards the left ventricle, i.e., morphologically left bundle branch block. Repair through the morphologically left ventricle invariably caused bundle branch block. Repair through a right atriotomy caused bundle branch block in only 3 of 8 patients. Therefore closure of a ventricular septal defect by this method, particularly transatrially, need not affect conduction. Morphologically left ventriculotomy rarely if ever causes morphologically left bundle branch block. When present, morphologically right bundle branch block presumably results from interruption of the proximal right bundle.

Adolescent↗

How the left lung is perfused after ligating the left pulmonary artery in the pig at birth: clinical implications for the hypoperfused lung.

The left pulmonary artery and ductus arteriosus were ligated in 14 pigs at birth. Animals were sacrificed at intervals from 2 to 24 weeks of age. In the right lung the pulmonary artery and in the left, either the distal pulmonary artery, bronchial arteries or both were injected. The fixed lung specimens were studied by arteriography, dissection and microscopic examination of serial and random sections of lung tissue. The bronchial arterial circulation to, and within the right lung appeared normal and was similar to that described in the human lung. In the left lung, the bronchial arterial circulation hypertrophied rapidly during the first 2 weeks, and large anastomoses between pulmonary and systemic circulations were found at the same sites as in the normal pig lung. The position and structural characteristics of the anastomosing arteries is described in the different types of broncho-pulmonary connection. In most animals aged 16 weeks or more, peripheral bronchial arteries immediately proximal to the anastomotic sites, developed intimal and medial proliferation. The left lung continued to grow although in all animals it was small. The axial pulmonary artery and its branches became smaller with age. These findings help explain how the lung is perfused and grows in children with congenital heart disease and an acquired collateral pulmonary arterial circulation.

Animals↗

Determinants of systemic vascular resistance in children with congenital heart disease.

It is well known on the basis of acute experiments that changes in systemic arterial oxygen saturation, haemoglobin concentration and acid-base state produce changes in systemic vascular resistance. However, it is not known whether the same changes occur in the chronic state. In order to investigate this problem, we measured systemic vascular resistance in a population in whom chronic changes in these variables are common, namely 195 subjects with congenital heart disease. The significance of these factors was assessed by their ability to predict systemic vascular resistance in a multiple regression equation which also took account of the size of the patient. No matter how the size of the patient was allowed for, haemoglobin concentration, systemic arterial pH (or non-respiratory pH) and systemic arterial oxygen saturation were significantly (P less than 0.025) and positively correlated with systemic vascular resistance. We conclude that, as far as these factors are concerned, the mechanisms operating in the acute state continue to function in the chronic state.

Age Factors↗

Correction of isolated secundum atrial septal defect in infancy.

Although it is a common congenital cardiac lesion, secundum atrial septal defect (ASD) rarely presents in infancy. Its natural history in the first two decades of life is generally benign. Reports of symptomatic ASD in early childhood stress the place of conservative management, and recommend that the operation should be deferred until school age. Few accounts of the indications for surgery in infancy exist. We present our experience of 6 patients who failed to respond adequately to conservative treatment and required operation for intractable heart failure due to isolated secundum ASD in the first year of life. As their presentation and physical signs were not typical of ASD seen in later childhood, they presented problems both of diagnosis and management.

Cardiac Catheterization↗

Congenital fistula between left ventricle and coronary sinus.

The occurrence of a congenital fistula between the left ventricle and the coronary sinus has not previously been reported. A patient with this rare anomaly is described in whom the diagnosis was established during life and confirmed at operation and necropsy.

Child, Preschool↗

Rastelli procedure for transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. Early and late results in 41 patients (1971 to 1978).

Forty-one children with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction underwent a Rastelli operation between 1971 and 1978. A homograft valve preserved in an antibiotic solution and extended with A dacron tube was the conduit of choice. Alternatively, conduits with porcine heterografts or valves constructed from calf pericardium were used. They were positioned to the left of the aorta whenever possible. The intraventricular tunnel from the left ventricle to the aorta was constructed from Dacron velour. There were four early and seven late deaths. The last 13 consecutive patients have survived. Early deaths were related to unfavourable anatomy, conduit compression, and sepsis. Residual ventricular septal defects and postoperative infection were the main factors contributing to the late deaths.

Adolescent↗