Search

BACKGROUND--Vascular access for cardiac catheterisation of children and young adults who have had previous catheter procedures is often difficult. OBJECTIVE--To assess the influence of age at and type and technique of previous cardiac catheterisation on the ease of vascular access for subsequent study of paediatric and adolescent patients. SETTING--Tertiary referral centre for paediatric cardiology. PATIENTS--478 Consecutive patients aged 1 day to 19 years undergoing cardiac catheterisation over a 12 month period, including 131 patients who had had previous catheterisation(s). METHODS--Prospective study, recording for each patient the age, weight, diagnosis, vascular access (artery, vein, or both), vessels eventually catheterised, access time, total duration of the procedure, and details of any previous catheter studies. RESULTS--Of 131 patients who had had previous catheterisations, 80 (61%) had been studied once previously, 38 (29%) twice, and 13 (10%) on three or more occasions. The right femoral vessels were cannulated without difficulty in 72 cases (55%). Elective cannulation of left femoral vessels (because of scar tissue on the right side) or upper body vessels was undertaken in 18 cases (14%). Problems cannulating the right femoral vessels were encountered in 41 cases (31%); the vein was blocked in 29, the artery in six and the femoral veins were blocked bilaterally in six cases. The mean (SD) access time was significantly prolonged in these 41 children (41(18) v 21(13) minutes, p < 0.001) as was total duration of the procedure (116(31) v 94(34) minutes, p < 0.001). Children who had had a saphenous vein cut down as neonates subsequently had a blocked ipsilateral femoral vein in 10/15 cases (67%). Risk factors for problematic cannulation also included a higher number of previous catheterisation procedures. CONCLUSIONS--Difficulties with vascular access are common in children and adolescents who have had previous cardiac catheterisations. In those who have had cut down or multiple previous studies, elective entry to the left femoral vessels should be undertaken and procedure time may be prolonged. Such cases should therefore be performed under general anaesthesia. [ABSTRACT FROM PUBLISHER]

Balloon dilatation of the aortic valve was attempted in 34 consecutive children aged 16 months to 17 years (median 7 years), weight range 9-60 (median 22) kg. Previous surgical valvotomy had been performed in two patients (twice in one of them). The valve was not crossed in one patient. In the remaining 33 patients the pressure difference between the left ventricle and the ascending aorta during systole was reduced from 71 (30) to 28 (19) mm Hg. In 24 patients recatheterisation 2-19 (mean 9) months later showed gradients that were similar to those immediately after balloon dilatation (35 (20) v 31 (20) mm Hg). The two patients with the highest residual gradients immediately after balloon dilatation showed a spontaneous reduction in gradient at repeat catheterisation, whereas the patient who had twice had previous surgical valvotomy showed an increase in gradient from 37 to 99 mm Hg over nine months and required aortic root replacement. Balloon dilatation was repeated in two patients and this caused a further reduction in gradient. New aortic regurgitation occurred in nine (27%) patients (grade I, 8; grade II, 1) and aortic regurgitation was exacerbated (grade I to II) in two of the nine with pre-existing aortic regurgitation. External iliac artery avulsion occurred in one (3%) patient and two (6%) required intravenous streptokinase because the femoral artery became occluded. There were no other complications. Open valvotomy was performed in the child in whom the valve was not crossed, but no other child required aortic valve operation. Balloon dilatation of the aortic valve gave reasonable short term palliation and was well tolerated. It is an alternative to surgical valvotomy for initial palliation of congenital aortic stenosis in many children. [ABSTRACT FROM PUBLISHER]

The diagnostic and prognostic features of 44 episodes of infective endocarditis in 42 children with congenital heart disease were reviewed. Endocarditis occurred in 18 patients who had not had surgical correction or palliation of the defect (non-operated group). There were 26 episodes in 24 patients who had been treated surgically (operated group) (16 open and eight closed cardiac operations). Endocarditis occurred soon after open heart surgery in eight patients and as a late complication in the other 16. It recurred in two patients (operated group). Invasive monitoring and low cardiac output were consistent features in those patients who had endocarditis soon after open heart surgery whereas dental treatment was a common feature in non-operated cases and after closed cardiac operations. Late cases of endocarditis after open heart surgery had various microbiological features that were not typical of infection after dental problems. Gram positive infections occurred in non-operated patients and in those who had had closed cardiac operations. The group that had open heart surgery had infections caused by Gram positive, Gram negative, and anaerobic bacteria and fungi. Fever, anaemia, leucocytosis, and positive blood cultures were the only consistent findings. Vegetations were seen in nine of 12 patients at cross sectional echocardiography. All 12 (four non-operated, one closed, and seven open cases) needed acute surgical treatment. The mortality from infective endocarditis was 17% for non-operated cases, 0% for those who had had closed heart surgery, and 50% for those who had had open heart surgery. Infective endocarditis after open heart surgery differs from that in the other subgroups in terms of microbiology, source of infection, and outcome and its early diagnosis depends on a thorough investigation of minimal symptoms and signs. [ABSTRACT FROM PUBLISHER]

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. [ABSTRACT FROM PUBLISHER]

Five patients with isolated clefts in the anterior leaflet of the mitral valve, unassociated with atrioventricular septal defects, are described. All had significant mitral regurgitation, with the cleft being the only abnormality in three. Two patients had an associated ventricular septal defect, one with a straddling right atrioventricular valve. Angiocardiography in four showed moderate regurgitation, but was not able to delineate the aetiology. Two dimensional echocardiography showed a constant defect in the anterior leaflet, pointing towards the left ventricular outflow tract. This differed from 30 cases with atrioventricular septal defects where the "cleft" pointed towards the interventricular septum and was situated between the anterior and posterior bridging leaflets. All cases with isolated clefts had surgical correction, with minimal residual regurgitation on follow-up examination in two cases. Our current policy in patients with uncomplicated isolated cleft involves non-invasive assessment of these children and surgical correction if the regurgitation is significant. [ABSTRACT FROM PUBLISHER]

Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred. Ventricular septal defect size was graded "blind" according to preoperative angiocardiographic and haemodynamic findings. Of 14 patients classified as having a large ventricular septal defect, necropsy and operative findings in eight showed defects 7 to 12 mm in diameter. Factors associated univariately with significantly increased mortality were young age, raised atrial and ventricular end-diastolic pressures, low weight, high admission blood urea, preoperative ventilation, and a large ventricular septal defect. All but the last two were also closely associated with each other. A jack-knifed discriminant function based upon ventricular septal defect size, blood urea, pulmonary venous oxygen content, and inferior caval oxygen saturation correctly predicted outcome in 78.9% of patients. Combinations of these four giving a probability greater than 0.9 of survival were rare with blood ureas above 8 mmol/litre. Raised blood urea was associated with low descending aortic pressure and subsequent dialysis. Only patients with a large ventricular septal defect stand to benefit from pulmonary artery banding at initial operation. Preoperative treatment to increase renal blood flow (prostaglandins, dopamine) may improve overall survival. [ABSTRACT FROM PUBLISHER]

In pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, there is a tendency for each collateral artery to be the sole supply to a particular region of lung. On injection into a collateral artery, however, "wash-out" of contrast medium by non-opacified blood from a second source is sometimes seen. Alternatively, contrast medium may faintly "wash-in" to an adjacent lobar artery supplied from a second source. "Wash-in" and "wash-out" therefore show that a duplicate blood supply exists. To assess the importance of these phenomena, we reviewed the angiograms of 50 patients with this condition. Duplicate pulmonary blood supply not resulting from surgical shunts was found in 25 patients (50%), and in 24 of 37 patients (65%) who had selective collateral injections. In one patient two collaterals anastomosed with each other. Thirty-nine instances of duplicate supply occurred in the remaining 24. The duplicate connection was to a region of lung connected to a central pulmonary artery in 29 of 39 instances (74%). Determination of how much of the peripheral pulmonary vasculature is connected to central pulmonary arteries greatly affects decisions about palliative and corrective surgery. These results show that unless "wash-in" and "wash-out" are specifically looked for, the information obtained from angiography may be erroneous. [ABSTRACT FROM PUBLISHER]

Formalin infiltration of the ductus arteriosus was performed in 13 neonates with pulmonary atresia (three with ventricular septal defect, two with tricuspid atresia, and eight with intact ventricular septum, one of whom had Ebstein's anomaly) in an attempt to maintain duct patency. Nine patients had an additional retrograde pulmonary valvotomy and one a Blalock-Taussig shunt. The mean preoperative systemic PO2 was 31 mmHg and rose significantly to 46 mmHg after operation. However, the PO2 rose in only two of the four patients who had formalin infiltration alone. There were five early deaths and three had clinical evidence of duct closure. Four patients needed further surgery (systemic-pulmonary artery shunt) within eight days. Seven of eight surviving patients were shown to have a closed duct at repeat cardiac catheterisation (two to 13 months after operation). The eighth died before restudy. There were four late deaths, one following late reoperation and the other three within five months of early reoperation. These results suggest that formalin infiltration cannot ensure long-term duct patency. As short-term patency can be maintained more reliably by prostaglandin administration, we have abandoned the operation. [ABSTRACT FROM PUBLISHER]

In nine patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, the effect of a systemic-pulmonary anastomosis on the lung was studied by comparison of pre- and postoperative cine angiograms. Selective injections into the collateral arteries were performed in all patients and the source of blood supply to each bronchopulmonary segment was sought. Central pulmonary arteries were present in six children and absent in three, as confirmed at thoracotomy. After insertion of a shunt, central pulmonary arteries increased in size, but the intrapulmonary vessels with which they connected remained abnormally small and were frequently stenosed. In addition, in each case the central pulmonary arteries increased flow to only five to 11 segments of lung. In the absence of central pulmonary arteries, shunting to a hilar or a lobar pulmonary artery increased perfusion to five to nine segments of lung per case. In all these three cases the pulmonary arteries at lobar level showed aneurysmal dilatation proximal to a severe stenosis. Sixty-eight per cent of collateral arteries were stenosed. The findings suggest that in this anomaly, when the central pulmonary arteries are hypoplastic, the intrapulmonary branches are also hypoplastic, emphasising the need for early surgical intervention to increase blood flow while the lung still has growth potential. Further, one collateral artery may connect with at least as many bronchopulmonary segments as does a central pulmonary artery, and the peripheral intrapulmonary arteries with which it connects may appear at least as normal angiographically as do vessels connected to central pulmonary arteries. These observations suggest that segments of lung connected to collaterals alone should, and sometimes can, be connected to central pulmonary arteries, the aim being to produce a unifocal blood supply as a prelude to total correction. [ABSTRACT FROM PUBLISHER]

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. [ABSTRACT FROM PUBLISHER]

Determination of atrial situs is of cardinal importance in the analysis of complex congenital heart lesions, and is best predicted from bronchial situs. Previous methods for assessing bronchial morphology, however, are unsuited to the very young patient. To assess bronchial morphology, 100 consecutive patients under 18 months of age (medium 57.5 days) with suspected congenital heart disease were studied by high kilovoltage filtered beam radiographs, before cardiac catheterisation. This low radiation dose technique clearly defined bronchial anatomy in 95 patients. The lengths of the left and right main bronchi were compared and 10 cases (10%) had a ratio less than 1.5 suggesting bronchial isomerism. Discriminant function analysis based on tracheal width and bronchial length enabled clear distinction of right from left bronchi. Four patients had bilateral right and six had bilateral left bronchi. Four of these 10 cases died and had necropsy confirmation of the radiological diagnosis. Practical prediction about cardiac anatomy, particularly the systemic and pulmonary venous return, may be made when bronchial morphology is known. [ABSTRACT FROM PUBLISHER]

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. [ABSTRACT FROM PUBLISHER]

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. [ABSTRACT FROM PUBLISHER]

Twenty-two patients with transposition of the great arteries with or without ventricular septal defect and one with double outlet right ventricle, d-malposition, and severe pulmonary vascular obstructive disease were treated surgically. All were cyanosed and had very limited exercise tolerance. Preoperatively, systemic arterial oxygen saturation (SaO2) varied from 45 to 79% (mean 65), haemoglobin was 13 to 23 g/dl (mean 19). Pulmonary arteriolar resistance was 6.4 to 35 units m2 (mean 17). In the patients with a ventricular septal defect the Mustard operation was done without closure of the ventricular septal defect, and in the 3 patients with intact ventricular septum the Mustard operation was combined with creation of a ventricular septal defect. All patients survived the operation and improved. Postoperative SaO2 ranged from 75 to 96% (mean 89) and haemoglobin from 10.6 to 17.8 g/dl (mean 14.0). This improvement was significant (P less than 0.05). Five patients have had a postoperative cardiac catheterisation. The pulmonary arteriolar resistance remains high in all. Postoperative follow-up varies from 4 to 40 months (mean 14 months). So far there have been no late deaths and all patients remain improved. [ABSTRACT FROM PUBLISHER]

Four pairs of thoracopagus twins have been described. Cardiac catheterisation was performed in all the cases. Angiocardiographic and necropsy findings suggest that the most common abnormality was some form of univentricular heart. The communication between the 2 hearts was at atrial level in 2 cases. Separation was performed in 1 of these cases but only 1 of the twins survived for 14 hours after operation. It is suggested that full cardiac catheterisation with selective angiocardiogram is essential before separation is considered. Identical heart rates were observed in each pair and there was invariably a major communication between the hearts of the twins. [ABSTRACT FROM PUBLISHER]

Between May 1971 and December 1975, 39 infants had operations for correction of total anomalous pulmonary venous drainage. Fourteen of the 39 patients were under 1 month of age at the time of operation. Twenty-four patients had supracardiac, 7 intracardiac, and 6 infracardiac total anomalous pulmonary venous drainage, and 2 had a mixed type. The overall hospital mortality was 36 per cent. There have been no late deaths. The improvement in survival rate in this series is attributed to: (1) earlier recognition and prompt referral, (2) an aggressive approach to diagnosis involving complete cardiac catheterisation and angiocardiography, (3) vigorous preoperative care, (4) early complete correction including construction of a large anastomosis and enlargement of the left atrium when indicated, and (5) intensive postoperative management paying particular attention to fluid balance and treatment of pulmonary complications. Operative mortality was highest in patients with total anomalous pulmonary venous drainage directly to the superior vena cava, and in those with infradiaphragmatic drainage of whom all had pulmonary venous obstruction. Mortality was not closely related to age, body weight, or severity of pulmonary hypertension. [ABSTRACT FROM PUBLISHER]

Progressive cyanosis after banding of the pulmonary artery in infancy occurred in a child with transposition of the great arteries and a ventricular septal defect, and a Blalock-Taussig shunt operation had to be performed. At the time of correction a segment of pulmonary artery between the left ventricle and the band was found to be completely occluded so that continuity between the left ventricle and the pulmonary artery could not be restored. A Rastelli type of operation was not feasible as the ventricular septal defect was sited low in the muscular septum. Therefore, in addition to Mustard's operation, a Dacron conduit was inserted from the left ventricle to the main pulmonary artery to relieve the obstruction. Postoperative cardiac catheterization with angiocardiography indicated a satisfactory haemodynamic result. The patient remains well 11 months after the operation. This operation, a left ventricle to pulmonary artery conduit, may be used as an alternative procedure in patients with transposition of the great arteries, intact interventricular septum, and obstruction to the left ventricular outflow, if the obstruction cannot be adequately relieved. [ABSTRACT FROM PUBLISHER]

Cardiac catheterization data from 54 investigations after Mustard's procedure were examined to study the influence of the operation on pressure events in the atria, great veins, and pulmonary circulation. Systemic venous atrial pressure tracings were characterized by a rapid, sharp 'y' descent. Pressure gradients between the venae cavae and systemic venous atrium were invariable, whether or not vena caval pathway obstruction was present, the 'y' trough and 'a' wave gradients being greater than the mean gradient. Pulmonary venous atrial pressure tracings were not different from normal except when tricuspid regurgitation was present. It is suggested that the baffle effectively reduces the size and compliance of the systemic venous atrium, but influences the pulmonary venous atrium to a lesser degree. The systolic pressure gradient from the left ventricle to pulmonary artery was decreased postoperatively, suggesting that it may be flow-related; the greatest changes were seen in the group with preoperative ventricular septal defect. The ratio of pulmonary: systemic vascular resistance did not change significantly after operation, and it is suggested that both the pre- and postoperative values were higher than normal. Examination of the left ventricular or pulmonary arterial mean pressure postoperatively should raise the suspicion of a complication, e.g. pulmonary venous obstruction or tricuspid regurgitation. [ABSTRACT FROM PUBLISHER]

Aortic atresia occurring with complete transposition of the great arteries (ventriculoarterial discordance) has not previously been reported. A patient with this condition is described, who is alive and relatively well at the age of 6 years. This survival contrasts conspicuously with that of patients with the far commoner situation of aortic atresia with normally connected great arteries. It is suggested that this difference in longevity is a result of the greater ability of the left ventricle to supply both the systemic and pulmonary circulations. [ABSTRACT FROM PUBLISHER]