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.     

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)     

Coarctation of the aorta in early infancy. Left ventricular outflow tract obstruction and its clinical implications

In order to clarify the clinical significance of left ventricular outflow tract obstruction, 11 infants with coarctation of the aorta and ventricular septal defect were subjected to cineangiography (or autopsy) analysis. Three infants without left ventricular outflow tract obstruction were salvaged by aortoplasty using a subclavian flap or a Dacron patch with concomitant pulmonary artery banding. The remaining 8 infants died surgically or nonsurgically, and were confirmed retrospectively to have had left ventricular outflow tract obstruction. It seems worthwhile to stress that the presence of an abnormal muscle bundle in the left ventricle can be demonstrated angiographically, especially by the axial technique. From our present study, though the number of cases examined is limited, we have an impression that left ventricular outflow tract obstruction may be a determining factor with regard to the appropriateness of pulmonary artery banding in the two-stage operation for infantile coarctation associated with a large ventricular septal defect.     

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.     

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band.

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.     

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.     

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.     

Successful surgical repair of aortic atresia associated with normal left ventricle

Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.     

Surgical correction of ventricular septal defects treated by banding the pulmonary artery. Apropos of 38 cases]

The authors report their experience with two-stage surgical treatment of severe ventricular septal defects in babies: initial banding of the pulmonary artery, followed by closure of the ventricular septal defect and removal of the band by an open-heart technique. Out of 162 operations to band the pulmonary artery, 90 were for cases of ventricular septal defect. The mortality was low (9.1%) when there was a ventricular septal defect with or without a patent ductus. It is considerably higher (31.4%) when there is a coincident coarctation of the aortic isthmus. So far 38 children have had the second operation, with 2 deaths. The authors have studied the surgical problems and the long-term results (6 months to 3 years follow-up) in the first 29 patients undergoing surgery. Although the results from this two-stage operation are good, when the authors take into account their current experience with one-stage closure of ventricular septal defects in the babies, they feel that this latter technique is to be preferred. Reservations must, however, be expressed when the child is very young (Weighing 3.5 kg or less), when there are multiple defects between the ventricles, and when the defect is severe, with a combination of VSD, patent ductus and coarctation of the aorta, and sometimes when the opertion must be carried out as an emergency.     

Concealed left atrial membrane: Pitfalls in the diagnosis of cor triatriatum and supravalve mitral ring

Cor triatriatum and supravalve mitral ring are forms of congenital left ventricular inflow obstruction produced by membranes within the left atrium. Typically, these defects occur as isolated anomalies with manifestations of pulmonary venous obstruction. Four children are presented whose left atrial membrane was associated with other significant cardiac defects, including, in one patient each, simple coarctation of the aorta, sinus venosus atrial septal defect, tricuspid atresia and complex coarctation of the aorta syndrome. The patient with the latter defect had undergone previous pulmonary arterial banding. None of these patients demonstrated significant pulmonary venous obstruction at cardiac catheterization. All patients had a normal value for either pulmonary arterial diastolic or pulmonary arterial wedge pressure. Three mechanisms explained the lack of pulmonary venous obstruction: (1) a large cross-sectional area of membrane openings, (2) an atrial septal defect that was confined to the pulmonary venous chamber and decompressed it by allowing blood to escape into the right atrium, and (3) decreased pulmonary blood flow. The diagnosis was facilitated by two dimensional echocardiography. Accurate diagnosis of left atrial membrane in the setting of other cardiac defects is of practical significance because pulmonary venous obstruction may occur after surgery for the associated defects.     

Morphological characterisation of ventricular septal defects associated with coarctation of aorta by cross-sectional echocardiography.

Cross-sectional echocardiograms of 18 neonates and infants with coarctation of the aorta and ventricular septal defect were retrospectively assessed. With a combination of subcostal and precordial cuts the site and anatomical relations of the defects were determined. In one case there was a muscular trabecular ventricular septal defect. Three had a malalignment defect with associated left ventricular outflow tract obstruction. In two there was a doubly committed subarterial defect with associated malalignment of the point of continuity between the aortic and pulmonary valves and the crest of the trabecular septum. Twelve cases had a perimembranous defect, with varying degrees of extension into the inlet, trabecular, or outlet septum. In this group 10 had associated aortic override, with varying degrees of left ventricular outflow tract narrowing. Abnormal insertion of the tricuspid valve was observed in 10 cases, such that it partly obscured the ventricular septal defect. Thus in most cases of coarctation with ventricular septal defect, the morphology of the ventricular septal defect and ventricular outflow tracts is such that left ventricular ejection is directed towards the pulmonary artery rather than the aorta.     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

Pulmonary arterial development in infants with large perimembranous ventricular septal defects associated with overriding of the aortic valve

Intracardiac anatomy and pulmonary vascular structure were studied in 13 cases having large perimembranous ventricular septal defects associated with overriding of the aortic valve, 12 of whom died aged 2 days-9 months. In all cases the ventricular septal defect and aorta were of the same size, but the diameter of the pulmonary orifice was usually greater. Quantitative morphometric techniques were used to study the lungs. Structural adaptation to extra-uterine life was shown to have occurred normally in the 4 patients who died as neonates. In the cases aged 2 months-4 years, there was an increase in arterial medial thickness and extension of muscle, while the intra-acinar arteries were larger than normal in size and normal in number. Intimal proliferation was seen in 1 case at 4.5 months, but such proliferation caused significant obstruction only in one child aged 4 years. These findings emphasize the rapidity with which structural adaptation to extra-uterine life can occur in the patient with a large unrestrictive ventricular septal defect. A rapid increase in muscularity can be seen by 2 months of age. Should banding of the pulmonary artery be required in patients with more complicated intracardiac abnormalities in whom it is desirable to achieve a normal pulmonary vascular bed, our findings encourage its early performance, particularly in those destined for a Fontan repair.     

Double outlet right ventricle: clinical spectrum and prognosis.

Patients with double outlet right ventricle can be separated into four distinct groups. In the largest (Group III), patients have a subaortic ventricular septal defect and pulmonary stenosis and clinically resemble patients with tetralogy of Fallot. In the next largest group (Group I), patients have a subpulmonry ventricular septal defect and no pulmonary stenosis and clinically resemble children with D-transposition of the great arteries and a ventricular septal defect. These patients have a high rate of coarctation of the aorta leading to early congestive heart failure, and their overall prognosis is poor. In the next largest group (Group II), patients have a subaortic ventricular septal defect and no pulmonary stenosis. Their presentation is similar to that of children with a large ventricular septal defect and pulmonary hypertension. In the smallest group (Group IV), the ventricular septal defect is uncommitted. Survivors in this group also clinically resemble children with a large ventricular septal defect and pulmonary hypertension. When present, coarctation of the aorta and severe mitral valve abnormalities greatly influence the prognosis in double outlet right ventricle. Although the prevalence of associated cardiac abnormalities is large, asplenia, polysplenia, chromosomal abnormalities and other congenital noncardiac abnormalities occur in only 12.5% of patients with double outlet right ventricle.     

Neonatal anatomical repair of transposition of great vessels associated with atrial septal defect. Apropos of 42 cases

The good results obtained by anatomic correction of simple transposition of the great arteries (TGA) in the neonatal period have incited some surgical teams to widen the indications to neonates with TGA associated with ventricular septal defect (VSD). The classical management of these patients is a two stage procedure: banding of the pulmonary artery followed by detransposition, which carries a certain risk. Between January 1985 and June 1990, 42 neonates with TGA and VSD underwent a combined procedure consisting in anatomic correction of the TGA and closure of the VSD. The average age of these patients was 16 days, and the average weight was 3.3 kg. Ten patients had coarctation and 6 underwent a complete one stage correction by an anterior approach. The surgical technique consisted in closing the VSD from the right atrium in 20 patients, from the right ventricle in 11 patients and from the pulmonary artery in 11 patients, associated with detransposition of the great arteries and coronary artery reimplantation. Three children died in the preoperative period (7.1%). In two cases, death was related to malposition of the coronary artery. The third fatality was the result of haemorrhage. There has been one late death three years after surgery. Four patients have been reoperated for stenosis of the right ventricular outflow tract (1 case), recurrence of coarctation (2 cases) and stenosis of the superior vena cava (1 case) and have survived. All patients were followed up for an average period of 26.4 +/- 19 months. They are all in the NYHA Class I without treatment. One patient has mild aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Obstructions to pulmonary ejection and restrictive interventricular septal defects

The association of a restrictive ventricular septal defect with a pulmonary tract obstruction is rare and may cause diagnostic and therapeutic problems. The authors report their experience of such an association in 10 children. 3 had a tetralogy of Fallot (1 death), 4 a double-outlet right ventricle (2 deaths), and 3 a coarctation of the aorta with ventricular septal defect and pulmonary artery banding (no death). Prognosis depends on the type of malformation. It has improved with years because of sooner recognition of these malformations and better surgical technics and post-operative care.     

Coarctation of aorta with special reference to infants. Long-term results of operation in 126 cases.

A review of 126 cases of coarctation of the aorta confirms the need for surgical resection in infants with intractable congestive cardiac failure. The high association with additional cardiovascular abnormalities in patients presenting in early infancy contributes significantly to the mortality. Patients with large ventricular septal defects and coarctation of the aorta are at risk and may require pulmonary artery banding at the time of resection of the aortic coarctation. Long-term complications included restenosis (18 cases) and persistent hypertension (10 cases). In order to prevent persistent hypertension, it is suggested that elective resection of the coarctation be done at 1 year of age.     

Surgical treatment of ventricular septal defect in infancy. Primary repair versus banding of pulmonary artery and later repair.

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.     

Periarterial abscess: another cause of cyanosis after pulmonary artery banding.

Twenty seven months after pulmonary artery banding a boy aged two and a half developed rapidly progressive cyanosis. A periarterial abscess was found at the site of the band. Repair of the pulmonary artery and closure of the ventricular septal defect were complicated by profoundly low cardiac output and prolonged dependence on a ventilator. This near fatal complication would have been avoided by primary closure of the ventricular septal defect.