Diastolic ventricular function after the Fontan operation.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A N Redington.
Explore the source record for details and available documents.
Severe mechanical haemolysis occurred in a seven month old infant after the insertion of a 17 mm Rashkind double umbrella device. The placement of a second device 23 days after the initial procedure abolished haemolysis.
OBJECTIVE: To determine the effects of residual pulmonary regurgitation on exercise tolerance after complete repair of tetralogy of Fallot. DESIGN: Prospective study of symptom free patients more than five years after complete repair. Graded exercise performance was measured with standard Bruce protocol. Maximal oxygen uptake and ventilatory anaerobic threshold were measured by respiratory mass spectrometry. Measurement of pulmonary regurgitant fraction was from pressure-volume loops constructed from measurements of right ventricular volume obtained from biplane angiograms and simultaneous pressures measured with a micromanometer. SETTING: Tertiary referral centre. PATIENTS: 16 patients were studied. Two patients had been excluded because of residual cardiac lesions or inadequate data from cardiac catheterisation. Four were later excluded because they failed to reach a respiratory quotient of greater than 1.0 during graded exercise. RESULTS: There was a significant negative correlation between the degree of residual regurgitation and both total duration of exercise and maximal heart rate achieved. Maximal heart rate and total duration of exercise were significantly lower in the patients than in normal controls. Patients with an abnormal maximal oxygen uptake (less than 85% of the predicted normal value) had significantly greater residual pulmonary regurgitation than those in whom oxygen uptake was normal. CONCLUSIONS: Impaired exercise capacity after complete repair of tetralogy of Fallot is directly related to the degree of residual pulmonary regurgitation. These data should be taken into account when deciding the optimal timing and nature of corrective surgery.
OBJECTIVE: To compare M mode derived indices of left ventricular performance obtained with transthoracic and transoesophageal echocardiography in children with congenital heart disease. DESIGN: Transthoracic and transoesophageal M mode echocardiograms were obtained under general anaesthesia before cardiac catheterisation. Recordings were digitised by dedicated software. Indices of cavity dimension and left ventricular wall dynamics were compared. PATIENTS: 16 unselected patients with congenital heart disease. RESULTS: Group data for simple measurements of ventricular dimension and wall thickness were similar with the two techniques and had acceptable coefficients of repeatability, but there were considerable individual differences. Correlation was poor with unacceptable repeatability for derived indices of cavity and ventricular wall dynamics. CONCLUSION: Both transthoracic and transoesophageal echocardiography can be used to obtain M mode derived indices of left ventricular performance but the resultant measurements are not directly comparable, presumably because of regional non-uniformity of function.
Whereas abnormalities of the morphological tricuspid valve are common in the setting of discordant atrioventricular connections, there are only a few postmortem reports of abnormalities of the mitral valve in this condition. This report describes two patients with discordant atrioventricular connections, in whom important abnormalities of the mitral valve were found during life by cross sectional echocardiography.
Explore the source record for details and available documents.
The cross-sectional echocardiographic and postmortem appearances of the heart from a patient with the rare association of aortic valve atresia, aortopulmonary window, and interrupted aortic arch are described. Differentiation of this anomaly from truncus arteriosus with interrupted aortic arch is important.
The development of abnormal flow has been described in patients with incoordinate motion of the ventricular wall. In all cases so far documented, these abnormal patterns of flow have been restricted to the ventricular cavity, and the atrioventricular valve remained closed. We identified a patient after a Fontan operation in whom the degree of ventricular mural incoordination was of such severity that the atrioventricular valve remained open and atrioventricular flow occurred for 140 msec after the onset of QRS complex. As far as we know, this phenomenon has not previously been described.
Pulmonary blood flow patterns were studied during the application of varying extrathoracic pressure in 2 patients after total cavopulmonary anastomosis. The application of negative extrathoracic pressure was associated with large increases in pulmonary blood flow, while positive extrathoracic pressure, caused retrograde flow away from the lungs. These preliminary observations suggest that negative extrathoracic pressure may be useful as a means of respiratory support in patients after right heart bypass procedures.
Two cases of fatal idiopathic persistent pulmonary hypertension presented late in the neonatal period. Lungs were examined histologically by light and electron microscopy, and immunocytochemical studies were used to identify nerves. There was extension of medial smooth muscle distally along the arterial pathway so that most precapillary arteries had completely muscular walls, which in some cases completely obliterated the vessel lumen. Enlarged endothelial cells also contributed to the reduction in the size of the lumen. Nerve fibres accompanying muscular arteries were found in the alveolar region, more distal than is normal. The predominant neuropeptide was the vasoconstrictor tyrosine. Possible aetiological factors in persistent pulmonary hypertension of the newborn are increased muscularity of the peripheral pulmonary arteries antenatally, an increase in the number of vasoconstrictor nerves, or an imbalance in the production of leukotrienes and prostacyclins in the perinatal period.
Congenital aneurysms of the interventricular septum were found in a 29 year old man and his four year old son. Both were symptom free. In both, M mode and cross sectional echocardiography showed an aneurysm in the mid-muscular trabecular portion of the ventricular septum with considerable paradoxical motion of the aneurysmal segment. Otherwise the chamber dimensions, intracardiac structures, and cardiac function were normal for age. Congenital aneurysm of the interventricular septum is rare and these familial cases may be unique.
Interventional balloon catheterisation is now the recommended procedure for several congenital heart defects. The overall complication rate in the first 160 children (median age 3 years) to undergo balloon intervention (excluding balloon atrial septostomy) at the Brompton Hospital was 24% (14% excluding haemorrhage and including three deaths (1.9%]. Most complications were related to vascular problems at the site of cardiac catheterisation. Complications were more common in the younger patient, especially neonates, and also in procedures that were ultimately unsuccessful. Most complications were transient and usually had no long term effects. Improvements in equipment design and technical expertise may reduce morbidity from these procedures.
The pattern of pulmonary blood flow was studied in three patients after a total cavopulmonary shunt procedure. Doppler studies showed a phasic pattern of flow which varied with the respiratory cycle. Pulmonary blood flow was increased with normal inspiration, and was much augmented by the Mueller manoeuvre. This suggests that flow was occurring when a negative intrathoracic pressure was generated. During a brief Valsalva manoeuvre blood flowed away from the lungs. With a sustained Valsalva manoeuvre there was no spontaneous forward flow; instead there was low velocity pulsatile pulmonary blood flow that coincided with ventricular systole. Left ventricular cavity dimensions decreased, reflecting a considerably reduced pulmonary blood flow. Pulmonary blood flow after the total cavopulmonary shunt operation is critically dependent on changes in intrathoracic pressure. This has important implications in terms of the immediate postoperative management of these patients.
Five infants who underwent direct reimplantation or redirection of an anomalous left coronary artery from the pulmonary artery to the aorta were studied. There were no deaths from the procedure. Anastomoses were patent at a mean follow up of 33 months. Left ventricular angiograms before and after operation were digitised frame by frame. Analysis of wall motion showed considerable generalised hypokinesia with no change in cavity shape before operation. After reimplantation there was a significant improvement of global ventricular function as assessed by ejection fraction and end diastolic shape index. There was also a tendency for the shape index change to increase; this was compatible with better global systolic function. Three of the four patients who were restudied showed normal isometric and contour wall motion plots. One child, the oldest at the time of operation, still showed asynchronous onset of contraction and isovolumic relaxation. The procedure offers a low risk of mortality and a high rate of patency of the anastomosis. Improvement of ventricular function can be remarkable and its extent cannot be assessed simply by ejection fraction measurements.
OBJECTIVE: To examine left ventricular function after surgical resection of subaortic stenosis during childhood. DESIGN: Left ventricular performance was measured non-invasively in all patients who responded to an invitation for formal assessment. SETTING: Outpatient study, tertiary referral centre. PATIENTS: Twenty three (12 male and 11 female) patients (age range 3 to 31 years) of 43 consecutive patients with fixed subaortic stenosis undergoing surgical resection between 1975 and 1989 reattended for formal assessment 16 months to 15 years (median 4 years 4 months) after operation. MAIN OUTCOME MEASURES: Left ventricular dimension, left ventricular wall thickness, left ventricular Doppler inflow velocities, and left ventricular diastolic pressure (measured from apexcardiograms). Results were compared with those in controls individually matched for age and sex. RESULTS: All patients were symptom free. Left ventricular cavity dimensions were normal, as was the mean fractional shortening. Posterior wall thickness tended to be greater in the patients and there was a significant increase in septal thickness. Normalised peak rate of posterior wall thinning was significantly lower in the patients and the isovolumic relaxation time was significantly shorter. Doppler inflow velocity measurements showed that early diastolic mitral flow acceleration time was normal but deceleration time was significantly shorter in the patients. The ratio of mitral flow in early diastole (E) to E plus mitral flow in late diastole (A) was significantly higher in the patients and in two patients there was complete absence of A wave flow despite large A waves on the apexcardiogram. CONCLUSIONS: Systolic function was well preserved in patients after operation for subaortic stenosis. A restrictive pattern of left ventricular filling was common, however, and presumably reflected a response to the chronic pressure load and to surgery in the paediatric heart.
OBJECTIVE: To document whether the act of respiration influences pulmonary blood flow in patients after the Fontan operation. DESIGN: Prospective study in which patients acted as their own controls. SETTING: Supraregional paediatric cardiology centre. PATIENTS: Sixteen patients who had undergone atriopulmonary anastomosis (Fontan operation) for the treatment of congenital heart disease. INTERVENTION: Doppler assessment of pulmonary artery flow patterns with a simultaneous respirometer, electrocardiogram and phonocardiogram. RESULTS: Total (mean (1SD) ) forward pulmonary flow was 63.6 (35)% higher during inspiratory cardiac cycles than during expiratory ones. CONCLUSIONS: The act of breathing provides an additional energy supply to pulmonary blood flow in patients after the Fontan operation.
OBJECTIVE: To assess whether regional abnormalities of ventricular function are present in patients after the Fontan operation and to explore the implications of any such abnormalities for ventricular filling. DESIGN AND PATIENTS: Prospective study in which 25 patients after the Fontan operation were compared with 25 healthy controls and 12 patients with a univentricular atrioventricular connection, before the Fontan operation. INTERVENTIONS: Doppler echocardiography, with simultaneous electrocardiogram, phonocardiogram, and respirometer. RESULTS: Isovolumic relaxation time was significantly longer in patients after the Fontan operation than in normal children (p less than 0.001) or the preoperative patients (p = 0.001). Systolic intraventricular flow was detected in 60% of patients after the Fontan operation and in 42% of preoperative patients. After the Fontan operation 80% of patients showed intraventricular flow during isovolumic relaxation compared with 8% of normal children and none of the preoperative patients. CONCLUSIONS: Incoordinate ventricular relaxation is common after the Fontan operation. This may have important implications for ventricular diastolic filling, pulmonary blood flow, and cardiac output in these patients.
OBJECTIVE: To study regional wall motion of the systemic ventricle in patients after the Fontan operation. DESIGN AND PATIENTS: Systemic ventricular angiograms of 15 patients after the Fontan operation and of 11 unoperated patients with a univentricular atrioventricular connection were digitised frame by frame. Strict criteria for abnormal wall motion were used so that minor abnormalities were not considered. RESULTS: Incoordinate contraction of the ventricular wall was found in five of the 11 patients before and in four of the 15 patients after the Fontan operation (NS). Only three of the 11 patients before the Fontan operation, showed incoordination of ventricular relaxation, but incoordinate relaxation was seen in 12 of the 15 patients after operation (p less than 0.01). CONCLUSIONS: Whereas incoordination of ventricular contraction was common in patients with a univentricular atrioventricular connection, before or after the Fontan operation, incoordinate relaxation of the ventricular wall was a common consequence of the Fontan operation and was rare in patients before operation.