Second heart sound after pulmonary arterial banding operation.

After a pulmonary arterial banding procedure the phonocardiograms of 38 patients were correlated with haemodynamic and angiographic findings. Twenty-four patients had uncomplicated ventricular septal defect, 2 had single ventricle, 5 had transposition of the great arteries, 5 had atrioventricular canal defects, and 2 had coarctation of the aorta and ventricular septal defect. P2 was separated from A2 by less than 40 ms in 10 of the 11 patients with high pulmonary vascular resistance. Of 27 patients with nearly normal pulmonary vascular resistances and distal pulmonary artery pressures less than 50/20 mmHg (6-7/2-7 kPa), 24 had A2-P2 intervals of over 40 ms. A narrow A2-P2 interval with a satisfactory band was found in 2 patients with large right-to-left shunts. A2-P2 interval did not change appreciably with age, and this measurement is a useful guide as to the effectiveness of pulmonary artery banding by one year after operation. If this interval is less than 40 ms, repeat catheterization should be carried out as such patients may have persisting pulmonary hypertension and an increased pulmonary vascular resistance.     

Pulmonary vascular disease in complete transposition of the great arteries: A study of 200 patients

Lung specimens of 200 patients with transposition of the great arteries were examined microscopically for evidence of pulmonary vascular disease. In patients with an intact ventricular septum or a small ventricular septal defect, advanced pulmonary vascular disease was uncommon; only 9 of 107 such patients (8.4 percent) demonstrated greater than grade 2 (Heath-Edwards) pulmonary vascular disease. A persistent large patent ductus arteriosus appeared to promote progressive pulmonary vascular disease in this group since each of the five infants less than 1 year of age with grade 3 or 4 disease had this lesion. In contrast, pulmonary vascular disease was common in patients with a large ventricular septal defect; 37 of 93 patients (40 percent) with this defect had greater than grade 2 pulmonary vascular disease. Among patients more than 1 year of age, 26 of 35 (75 percent) had grade 4 disease. The catheterization data suggest that the calculated pulmonary vascular resistance may underestimate the degree of disease, probably by overestimating the pulmonary blood flow (Fick method). Pulmonic stenosis appeared to protect the lungs from progressive pulmonary vascular disease, and pulmonary arterial banding was protective when performed before age 6 months. Our studies indicate that a persistent large patent ductus arteriosus should be closed as early as possible in view of its association with advanced pulmonary vascular disease in these patients. In infants with a large ventricular septal defect, pulmonary arterial banding or corrective surgery with closure of the defect should be performed between the ages of 4 and 6 months to prevent progressive pulmonary vascular damage.     

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.     

Acute haemodynamic effects of nifedipine in patients with ventricular septal defect.

The haemodynamic effects of nifedipine were studied in 14 patients (aged 8-14 years, seven male and seven female) with ventricular septal defect with and without pulmonary hypertension. All underwent left and right heart catheterisation. In each patient the pressures and heart rate were measured and blood samples were taken for oximetry before and after sublingual administration of 10 mg nifedipine. In eight patients with ventricular septal defect without pulmonary hypertension (mean pulmonary artery pressure less than 20 mm Hg) nifedipine significantly reduced the mean aortic pressure and systemic vascular resistance, and significantly increased heart rate. The other haemodynamic indices did not change significantly. In six patients with ventricular septal defect complicated by pulmonary hypertension (mean pulmonary artery pressure greater than 20 mm Hg) nifedipine significantly increased systemic output, stroke volume, and heart rate, and significantly reduced systemic vascular resistance and the pulmonary to systemic flow ratio. The other haemodynamic indices did not change significantly. Nifedipine had a beneficial effect in patients with ventricular septal defect complicated by pulmonary hypertension. It reduced the left to right shunt and increased the stroke volume. This effect was not seen in patients with ventricular septal defect uncomplicated by pulmonary hypertension.     

Acute haemodynamic effects of nifedipine in patients with ventricular septal defect

The haemodynamic effects of nifedipine were studied in 14 patients (aged 8-14 years, seven male and seven female) with ventricular septal defect with and without pulmonary hypertension. All underwent left and right heart catheterisation. In each patient the pressures and heart rate were measured and blood samples were taken for oximetry before and after sublingual administration of 10 mg nifedipine. In eight patients with ventricular septal defect without pulmonary hypertension (mean pulmonary artery pressure less than 20 mm Hg) nifedipine significantly reduced the mean aortic pressure and systemic vascular resistance, and significantly increased heart rate. The other haemodynamic indices did not change significantly. In six patients with ventricular septal defect complicated by pulmonary hypertension (mean pulmonary artery pressure greater than 20 mm Hg) nifedipine significantly increased systemic output, stroke volume, and heart rate, and significantly reduced systemic vascular resistance and the pulmonary to systemic flow ratio. The other haemodynamic indices did not change significantly. Nifedipine had a beneficial effect in patients with ventricular septal defect complicated by pulmonary hypertension. It reduced the left to right shunt and increased the stroke volume. This effect was not seen in patients with ventricular septal defect uncomplicated by pulmonary hypertension.     

Ventricular septal defect with increased pulmonary vascular resistance. Late results of surgical closure

The fate of 57 children who underwent surgical closure of ventricular septal defect in the presence of increased pulmonary vascular resistance was analyzed. Eighteen (32 percent) died at or immediately after operation. Seven late deaths, due to the Eisenmenger syndrome, occurred 1 to 7 years after operation. Follow-up studies, including cardiac catheterization, were performed in 25 survivors, 1 to 11 years (mean 5 years) after operation. In patients operated on after age 2 years, the degree of preoperative elevation of pulmonary vascular resistance largely determined the prognosis: The condition of patients with mild elevation (pulmonary vascular resistance less than one third the systemic level) returned to normal after operation, with one exception. Patients with more severe elevation (pulmonary vascular resistance more than one third the systemic level) most often showed progressive pulmonary vascular disease; the condition of a few remained unchanged and that of only one returned to normal after operation.Operative mortality rate was high in children with elevated pulmonary vascular resistance during the second year of life who were operated on before age 2 years, but pulmonary vascular resistance returned to normal in the three survivors. When pulmonary vascular resistance was increased in the first year of life, it generally decreased spontaneously (independently of operation); the early increase in these cases is believed to represent delayed maturation of the pulmonary vascular bed rather than pulmonary vascular disease.The overall results of surgery in ventricular septal defect associated with a high level of pulmonary vascular resistance are unfavorable and call for interruption of the natural history before significant pulmonary vascular obstructive changes have taken place; this can be achieved by surgical closure before age 2 years or pulmonary artery banding in infancy.     

Natural histories of atrial septal defect with pulmonary hypertension, and ventricular septal defect with pulmonary hypertension

A study of the natural history of 51 adult patients with atrial septal defect with pulmonary hypertension (ASD + PH) was performed. ASD + PH of less than 14 Um2 of pulmonary artery resistance (PVR) was considered an indication for surgery. The prognosis of surgically treated patients was favorable, but that of medically treated patients was poor. For patients with ventricular septal defect with pulmonary hypertension (VSD + PH), surgery was considered for pulmonary-systemic vascular resistance ratio (Rp/Rs) less than 0.5, and for patients under than 10 years and, ideally, under 2 years of age.     

Long-term outcome of patients operated for large ventricular septal defects with increased pulmonary vascular resistance

BACKGROUND: There is a paucity of data regarding the long-term outcome of patients operated for ventricular septal defect with severe pulmonary arterial hypertension and elevated pulmonary vascular resistance. METHODS AND RESULTS: We evaluated the long-term follow-up results of a selected cohort of patients with nonrestrictive ventricular septal defect and elevated pulmonary vascular resistance (>6 Wood units). Thirty-eight patients, median age 7.5 years (range 6 months-27 years), with nonrestrictive ventricular septal defect with severe pulmonary hypertension were operated between 1985 and 1996 at our institute. Preoperative pulmonary vascular resistance, ratio of pulmonary blood flow to systemic blood flow, and ratio of pulmonary vascular resistance to systemic vascular resistance were 7.63+/-1.8 Wood units, 1.9+/-0.48, and 0.41+/-0.12, respectively. The majority (68.4%) had perimembranous ventricular septal defect. Thirty patients (79%) had a good outcome and were asymptomatic at a mean follow-up of 8.7 years, with significant reduction in pulmonary artery pressures. Eight patients (21%) had a poor outcome, which included 5 immediate postoperative deaths, 1 late death and 2 surviving patients with persistent severe pulmonary arterial hypertension. There was no significant difference regarding hemodynamic parameters at baseline between those who had a good outcome and those who did not. Eleven patients with a preoperative pulmonary blood flow to systemic blood flow ratio of <2:1. who had a good outcome following surgery, underwent repeat catheterization at follow-up. There was a significant reduction in their mean pulmonary vascular resistance (8.03+/-1.4 v. 4.16+/-1.6 Wood units, p=0.001) and pulmonary vascular resistance to systemic vascular resistance ratio (0.41+/-0.12 v. 0.19+/-0.06, p=0.05). CONCLUSIONS: The late results of surgery on this selected group of patients with nonrestrictive ventricular septal defect with high pulmonary vascular resistance are encouraging. Operative correction of the ventricular septal defect should be actively considered in all children presenting with nonrestrictive ventricular septal defect with a significant left-to-right shunt, despite moderately elevated pulmonary vascular resistance. Even among older patients with ventricular septal defect and moderately elevated pulmonary vascular resistance, there is a specific group that does well after operation.     

Surgical management of coarctation of aorta with ventricular septal defect. Multivariate analysis.

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.     

Surgical management of the infant with coarctation of the aorta and ventricular septal defect.

Clinical and cardiac catheterization data were collected from 39 infants with coarctation of the aorta and ventricular septal defect, 31 of whom were initially managed only by surgical repair of coarctation. Data were analyzed to determine mortality, morbidity, outcome and factors that might predict survival or the need for septal defect closure. Of the eight patients who did not require surgical treatment before 3 months of age, seven underwent coarctation repair alone at a mean age of 2.3 years. Of the 23 infants managed with coarctation repair alone, before age 3 months, 9 needed no additional surgical treatment and 6 required early and 8 required late repair of the ventricular septal defect. Seven infants underwent coarctation repair and simultaneous pulmonary artery banding and one eventually required debanding after spontaneous closure of the septal defect. The overall mortality rate in this series was 10.3% (mean follow-up time 5.7 years). Of 39 infants, 16 (41%) never required a second operation for ventricular septal defect closure. For patients who had only coarctation or coarctation repair with pulmonary artery banding at less than 3 months of age, ventricular septal defect size was categorized as small (less than 0.5 cm/m2), moderate (less than 1 cm/m2) or large (greater than 1 cm/m2) on the basis of defect size at operative repair or echocardiographic or angiographic assessment. Defect size did not necessarily correlate with the need for operative repair.(ABSTRACT TRUNCATED AT 250 WORDS)     

Successful repair of an atrial septal defect associated with right to left shunting

An atrial septal defect was successfully repaired in a young woman despite the presence of pulmonary hypertension and right to left shunting. Before repair both isoprenaline infusion and 100% inspired oxygen produced significant falls in pulmonary artery pressure and pulmonary vascular resistance. A lung biopsy specimen at operation indicated a considerable decrease in the concentration of parenchymal pulmonary arteries and an absence of intimal fibrosis or medial hypertrophy. Pulmonary artery banding performed in infancy, as part of the management of a ventricular septal defect, may have contributed to the underdevelopment of the pulmonary vascular tree. The reduced number of pulmonary arteries is a possible explanation for the pulmonary hypertension.     

Successful repair of an atrial septal defect associated with right to left shunting.

An atrial septal defect was successfully repaired in a young woman despite the presence of pulmonary hypertension and right to left shunting. Before repair both isoprenaline infusion and 100% inspired oxygen produced significant falls in pulmonary artery pressure and pulmonary vascular resistance. A lung biopsy specimen at operation indicated a considerable decrease in the concentration of parenchymal pulmonary arteries and an absence of intimal fibrosis or medial hypertrophy. Pulmonary artery banding performed in infancy, as part of the management of a ventricular septal defect, may have contributed to the underdevelopment of the pulmonary vascular tree. The reduced number of pulmonary arteries is a possible explanation for the pulmonary hypertension.     

Optimal management of patients with complete atrioventricular septal defect

Controversy still persists as to whether patients with complete atrioventricular septal defect should be treated by primary total correction or a two-stage approach utilizing initial pulmonary artery banding. We believe a selected management program dictated by each individual patient's anatomy, presentation (size and preoperative functional class), and associated anomalies should be practiced, but with a strong preference toward initial total repair incorporating modern techniques to construct left and right atrioventricular valves, correct valvular incompetence, and close ventricular and atrial defects. Utilizing this management program, 23 consecutive patients (age 5 days to 51 months) with complete forms of atrioventricular septal defect were surgically corrected from July 1, 1984 through June 30, 1988. Fourteen patients were 12 months of age or less at the time of complete repair. Five patients (22%) had initial pulmonary artery banding, but only 2 were performed by us. Both had very extenuating circumstances making complete repair inappropriate. Only 2 of these 23 patients having complete repair died (hospital mortality 9%) and one had pulmonary artery banding 3 years previously. Of the 5 patients with initial pulmonary artery banding, 3 (60%) required right ventricular outflow tract reconstruction and/or repair of pulmonary artery bifurcation stenosis. We believe these good results support our continued practice of selective management of patients with complete forms of atrioventricular septal defect, but we maintain a strong preference toward initial complete repair.     

Surgical correction of truncus arteriosus type I

From 1976 through 1981, 8 corrective operations for truncus arteriosos communis type I have been performed. The patient's ages ranged from 2 months to 4 1/2 years; 2 of the children had previously undergone banding of the pulmonary artery. Intracardiac correction consisted in closure of the ventricular septal defect (VSD) and disconnection of the pulmonary trunk from the aorta with reconstruction of the right ventricular outflow tract using a valved Dacron conduit. One 3 1/2-year-old child died postoperatively because of right heart failure. In this child the pulmonary vascular resistance had risen to 13 U x m2 despite banding of the pulmonary artery in infancy. All other children have survived the operation without major complications and are in good condition. Postoperative follow-up (re-catheterization in 6 out of 7 survivors) showed a faultless function of the conduits in all instances. Persistence of pulmonary hypertension was ascertained in one patient. According to these findings, which are in agreement with the experience of others, it is concluded that primary correction of truncus arteriosus should be undertaken in early infancy prior to development of pulmonary vascular disease.     

Treatment of patients with transposition of great arteries and pulmonary vascular obstructive disease

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.     

Doppler echocardiography after anatomical repair of transposition of great vessels]

Forty seven patients who underwent anatomical repair of transposition of the great arteries were assessed by Doppler Echocardiography on average 16 months after surgery. Thirty three had transposition alone and 14 had an associated ventricular septal defect. Abnormal left ventricular function was observed in 4 patients (8%) and was associated with a preoperative left to right ventricular systolic pressure ratio less than that of patients with normal left ventricular function (0.76 +/- 0.22 vs 0.90 +/- 0.14 respectively, p = 0.10). Aortic regurgitation was detected in 19 children (40%). It was minimal in 18 cases and mild in the other case, in which a ventricular septal defect has been approached via the original pulmonary valves. Previous pulmonary banding before detransposition was a predisposing factor for postoperative aortic regurgitation. Thirteen patients had pulmonary pressure gradients of over 20 mmHg. The site of obstruction was usually the main pulmonary artery. The 3 cases in which the coronary orifices were closed with two patches instead of one had significant stenosis of the main pulmonary artery. Pulmonary regurgitation was detected in 38 patients (81%); it was minimal in 29 cases, mild in 5 cases and severe in 4 cases. Five patients (11%) had minimal mitral regurgitation and 16 (34%) tricuspid regurgitation. In the 22 cases who had serial echocardiographic evaluation with an average follow-up of 18 months, the postoperative abnormalities were characterised by their stability, with no significant progression of the regurgitant lesions. A reduction in pulmonary pressure gradient was observed in 5 patients and an increase in 1 patient (from 28 to 40 mmHg).(ABSTRACT TRUNCATED AT 250 WORDS)     

Maturation of pulmonary input impedance spectrum in infants and children with ventricular septal defect

To determine whether pulmonary vascular disease can be detected in infants with ventricular septal defect (VSD) by the presence of an increase in the frequency of the impedance modulus minimum of the pulmonary input impedance spectrum, as has been implied for older children, spectra of 25 infants (2 years or younger) (group 1) were compared with spectra of 20 children (ages 2 to 7 years) (group 2). Groups were subdivided according to mean pulmonary artery (PA) pressure: those with moderate pressure levels (35 mm Hg or less, groups 1A and 2A) and those with high pressure levels (at least 40 mm Hg, groups 1B and 2B). Pulmonary vascular resistance, characteristic impedance and frequency of the modulus minimum were significantly lower in group 2A than in group 1A. The decrease in pulmonary vascular resistance and characteristic impedance with increasing age was consistent with body surface area increases; however, the shift in frequency of the modulus minimum could be more easily related to a decrease in the pulse wave velocity than to a shift in the primary reflection site. Pulmonary vascular resistance, characteristic impedance and the frequency of the first modulus minimum were comparable in groups 1B and 2B; however, none of the patients in group 1B had evidence of pulmonary damage, whereas 3 of 4 group 2B patients had microscopically apparent pulmonary vascular disease.(ABSTRACT TRUNCATED AT 250 WORDS)     

Treatment of patients with transposition of great arteries and pulmonary vascular obstructive disease.

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.     

Pulmonary artery morphology and hemodynamics in pulmonic valve atresia with ventricular septal defect before and after repair

Cardiac catheterization and angiography were performed in 22 patients with pulmonic valve atresia and ventricular septal defect to evaluate pulmonary morphology and hemodynamics before and after repair. In 12 of the 22, pulmonic valve atresia and ventricular septal defect were associated with major aortopulmonary collateral arteries, which were ligated in most. Mean postoperative pulmonary artery pressure (PAP) ranged from 9 to 92 mm Hg (mean 28 +/- 19) and pulmonary vascular resistance ranged from 1.1 to 35.2 U.m2 (mean 6.4 +/- 8.0). These data correlated (r = 0.89, p less than 0.001). The number of pulmonary artery subsegments connected to the central pulmonary arteries was 22 to 42 (mean 38 +/- 6). Univariate analysis revealed that the mean postoperative PAP correlated with the number of pulmonary artery subsegments connected to the central pulmonary arteries (r = -0.81, p less than 0.001), with mean postoperative PAP (r = 0.79, p less than 0.001), with the postoperative pulmonary artery area index of the right and left pulmonary arteries at prebranching (r = -0.76, p less than 0.001), and with the sum of the pulmonary artery areas after branching (r = -0.69, p less than 0.005). Pulmonary vascular resistance correlated with the number of pulmonary artery subsegments connected to the central pulmonary arteries (r = -0.85, p less than 0.001), with the mean preoperative PAP (r = 0.79, p less than 0.001), with the sum of the pulmonary artery areas after branching (r = -0.73, p less than 0.001), and with the postoperative pulmonary artery area index (r = -0.70, p less than 0.001). The incidence of pulmonary vascular resistance being less than 3 U.m2 was significantly higher in patients with greater than 36 pulmonary artery subsegments connected to the central pulmonary arteries and with a preoperative pulmonary artery area index greater than 0.5 (88%) (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)