Fergus J. Macartney

Anomalous origin of the left coronary artery from the pulmonary trunk. Potential for false negative diagnosis with cross sectional echocardiography.

Cross sectional echocardiography can identify anomalous origin of the left coronary artery from the pulmonary trunk. It has been suggested that identification of the left coronary artery arising from the aorta using this technique excludes the diagnosis. In three such infants the anomalous origin of the left coronary artery was identified in each by cross sectional echocardiography. In all three cases, however, an echo free linear structure apparently arising from the aorta, resembling a normal left coronary artery, was imaged. Anatomical sections in one patient, simulating cross sectional echocardiographic cuts, showed that this structure was almost certainly the transverse sinus of the pericardium. False positive cross sectional echocardiographic diagnosis of this condition is also possible because of the failure to image a normally arising left coronary artery. Thus identification of the anomalous origin of the left coronary artery from the pulmonary trunk appears to be the only reliable echocardiographic finding in this condition, and contrast cineaortography remains necessary in patients in whom the diagnosis is suspected clinically or electrocardiographically.

Doubly committed subarterial ventricular septal defect: new morphological criteria with echocardiographic and angiocardiographic correlation.

To gain a better understanding of the anatomy of doubly committed subarterial defects and its relation to findings at cross sectional echocardiography and angiocardiography, eight necropsy specimens from patients with this condition were examined, and preoperative echocardiograms and angiocardiograms from 313 patients with surgically or necropsy confirmed outlet defect were reviewed. Of these, 48 had doubly committed subarterial defects. Morphological review showed that doubly committed defects are roofed by the arterial valves in fibrous continuity because of lack of both the outlet septum and the septal aspect of the subpulmonary infundibulum. Angiocardiography had a lower sensitivity (50%) than echocardiography (95%) for diagnosis of doubly committed defects, but each was highly specific. In five (14%) of 35 available echocardiograms the arterial valves were normally offset, but in the remainder they were at the same level. The ventriculoarterial connection was concordant in 37/48 (77%), discordant in five (10%) of 48, and double outlet right ventricle in six (13%) of 48. Displacement of the fibrous raphe between the arterial valves in relation to the ventricular septum below was associated with outflow obstruction in 14 patients (pulmonary in nine and aortic in five). These features were readily identified by echocardiography. Thus echocardiography is not only a more accurate method than angiocardiography of recognising these defects, but also shows that the arterial pole of the heart is architecturally abnormal.

Analysis of atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 specimens with atrial isomerism.

We have analysed the atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 hearts with isomeric atrial chambers. Of the hearts, 32 had atrial chambers of bilateral right morphology while 11 had atrial chambers with bilateral left atrial characteristics. Among the hearts with right atrial isomerism, there were 13 biventricular hearts, all with ambiguous atrioventricular connection. Eight had a common valve and five had two atrioventricular valves. In the other 19 hearts, the atrial chambers were connected to only one ventricular chamber, 18 having double inlet ventricle through a common valve and the other having absence of the left atrioventricular connection. In these univentricular hearts, all possible types of ventricular morphology were found. The ventriculoarterial junction among these hearts with right isomerism showed great variation. In the hearts with left atrial isomerism, nine hearts had two ventricles and two were univentricular. The biventricular hearts all had ambiguous atrioventricular connection, six via a common valve and three via two valves. The two univentricular hearts both had double inlet via a common valve, one to a chamber of right ventricular type and the other to a chamber of left ventricular type. Both had rudimentary chambers of complementary pattern. The ventriculoarterial junction again showed much variation. Statistical analysis showed that pulmonary obstruction and a univentricular heart were both significantly more frequent in association with right compared with left isomerism. Significant differences were also noted in the two groups in terms of ventriculoarterial connections and infundibular morphology.

Cross sectional echocardiographic and angiocardiographic correlation in criss cross hearts.

Cross sectional echocardiography can provide accurate anatomical diagnosis in congenital heart diseases and therefore should be able reliably to identify criss cross hearts and enable the analysis of their sequential arrangement non-invasively. The cross sectional echocardiographic diagnoses in eight consecutive patients with this condition were compared with those made at cardiac catheterisation and cineangiocardiography (five retrospectively, three prospectively). The mean number of invasive studies required to reach the diagnosis was 1.9 (range 1-4). Complete anatomical diagnosis was achieved with cross sectional echocardiography in all patients, but identification of ventricular morphology was much more straightforward using cineangiocardiography. If the transducer was held steady in either a precordial or subcostal position and rocked anteriorly and posteriorly the characteristic crossing over of the ventricular inflows could easily be seen. In no plane was there normal parallel arrangement of ventricular inflows. A complete diagnosis should be possible in these patients using cross sectional echocardiography in experienced hands and at a single session in the cardiac catheterisation laboratory.

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.

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.

Diagnosis and management of parachute mitral valve and supravalvar mitral ring.

The results of investigation in 5 children with congenital deformities of the mitral valve region characteristic of the parachute mitral valve complex were reviewed. Three patients had parachute mitral valve, i had supravalvar mitral ring, and i had both these lesions. Associated anomalies included ventricular septal defect, double outlet right ventricle, subaortic stenosis, bicuspid aortic valve, and coarctation of the aorta. Clinical presentation depended on which abnormality was dominant. However, all patients had clinical and haemodynamic evidence of mitral valve disease: stenosis in 4 patients and incompetence in i. In i patient the single, hypertrophied papillary muscle obstructed left ventricular ejection, mimicking hypertrophic obstructive cardiomyopathy. Parachute mitral valve produced a specific angiocardiographic abnormality, afilling defect during diastole shaped like an egg-timer in the lateral left ventricular angiogram. This was produced by non-opacified blood posterior to the mitral leaflets and chordae tendineae converging towards the filling defect produced by the enlarged, single papillary muscle. Supravalvar mitral ring produced a thin, crescentic, filling defect between the left atrium and ventricle, seen in the lateral view during opacification of the ventriclefrom the atrium. Careful preoperative assessment of the relative importance of these multiple anomalies allowed successful surgical correction of the significant lesions in the 4 patients operated on. Of 3 patients with parachute mitral valve, 2 required mitral valve replacement. In i of these a supravalvar ring was also excised. In the other patient with supravalvar mitral stenosis, excision of the ring alone relieved mitral obstruction adequately.

Long-term results of the “Palliative” mustard operation

A long-term follow-up study after 41 palliative Mustard operations for transposition of the great arteries and pulmonary vascular obstructive disease is presented. The operations were performed from 1973 to 1980. Mean pulmonary arteriolar resistance was 13.96 +/- 6.7 Um2. A ventricular septal defect was not closed in 34 patients; in 7 it was created at operation. There were three hospital and two late deaths. Survivors were followed up for 3 to 10 years (mean 76.7 +/- 22.8 months). Before operation 4 children were in New York Heart Association functional class IV and 33 were in class III. Mean hemoglobin concentration was 19.43 +/- 3.14 g/dl and arterial oxygen saturation was 63.44 +/- 11.29%. After operation 18 patients were in functional class I, 17 in class II and only 1 in class III. Hemoglobin level decreased to 14.19 +/- 2.3 g/dl and arterial oxygen saturation improved to 89.12 +/- 7.25%. Cardiac catheterization in 21 survivors confirmed no change in pulmonary artery pressure and resistance. Effective pulmonary blood flow improved from 1.39 +/- 0.39 before to 2.6 +/- 0.78 liters/min per m2 after operation. The palliative Mustard operation carries a low risk and provides excellent symptomatic improvement up to 10 years in patients with transposition of the great arteries and pulmonary vascular disease.

Effects of oxygen administration, bicarbonate infusions, and brief hyperventilation on patients with pulmonary vascular obstructive disease.

and correction ofacidosis werestudied in16patients inwhomcongenital heart disease was associated with pulmonary vascular disease. Onbreathing 100percentoxygenthele was asignificant fall inpulmonary vascular resistance from218± 4.6to12.93-6units/M2 (P< 0.001), with arise inpulmonary blood flow from4-4 ± 0-6to8-8 ± 2-01/min perm2(P< 0025) andafall inpulmonaryartery pressurefrom #67.8 ± 2.8 to618± 40mmHg(P< 0025). Thechanges occurring onsodium bicarbonate adninistra;tion in6patients didnotreach levels ofsignificance, butthesize ofeach individual responsewasclosely correlated withtheresponsetooxygenadministration. Nosignificant changes occurred either on voluntaryhyperventilation or,inthesystemic circulation, with anyintervention. However, systemic vascular resistance waspositively correlated withpulmonary vascular resistance (P< 0.01). Asaresult ofthis, though agewascorrelated positively with both pulmonaryartery mean pressure(P< 0025) andvascular resistance (P< 0.025), itwas notcorrelated withtheratio ofpulmonary tosystemic resistance. Since pulmonaryvascular disease isprogressive, these results castsomedoubt onthevalidity ofresistance ratio asa measureofitsseverity. Patients withcongenital heart disease, particularly whenthisinvolves communications between the pulmonary andsystemic circulations atventricular orgreatarterial level, orconcordant (complete) transposition ofthegreatarteries, areatriskof developing pulmonary vascular obstructive disease. Thisinturnisamajor determinant ofbothimmediateandlate surgical mortality forcorrection ofthe defect (Blount andWoodwark, 1960;Goodwin, 1961; Kirklin, 1965;Cartmill etal., 1966;Mairet al., 1974, 1976). Thedegree ofpulmonary vascular obstructive disease canbeassessed directly before attempted correction bylungbiopsy (Heath etal., 1958b), though this hasdistinct limitations (Adams etal., 1957; Wagenvoort etal., 1967). A promising, butasyetuntested, newtechnique isinhalation and injection ofradioactive nitrogen (13N) (McKenzie

Late hemodynamic results of fascia lata reconstruction of the right ventricular outlet

Eight patients were catheterized between 1.2 and 2.4 years after reconstruction of the right ventricular outflow tract with autologous fascia lata. Whereas the immediate post-bypass pressures had demonstrated a maximum gradient of 15 mm. Hg across the fascial valve at the time of recatheterization, this varied from 55 to 142 mm. Hg (mean 83 mm. Hg). Right ventricular systolic pressure varied between 80 and 160mm. Hg (mean 106 mm. Hg). All but one patient had clinical evidence of pulmonary incompetence. Selective angiography with injection into the right ventricle and pulmonary artery demonstrated shrunken, thickened, immobile valve cusps with an abrupt stenosis of the fascial tube or a diaphragm across it. Re-operation has been done in five patients. In each, the graft tube functioned satisfactorily, but at the site of the valve, no leaflets were visible, and the orifice of the conduit narrowed abruptly to 6 to 10 mm. in diameter. It is concluded that autologous fascia lata is unsuitable for reconstruction of the right ventricular outflow tract.