Surgery of congenital cardiopathies without catheterization: initial experience with 141 consecutive cases

Our initial experience with surgical treatment of congenital heart disease in 141 consecutive cases without the need of hemodynamic study is described. Ages ranged between 1 day and 15 years; 39 cases under 1 months of age (23 in the first week of life), 38 between 1 and 12 months, and 64 above 1 year of age. Diagnosis were: ductus 32 cases, atrial septal defect 24, Fallot's tetralogy 14, aortic coarctation 13, complete AV canal defect 7, D-transposition of the great arteries 5, ventricular septal defect 6, pulmonary atresia with intact ventricular septum 5, aortic stenosis 3, tricuspid atresia 4, pseudotruncus 2, univentricular heart 2, atrial myxomas, 2, Fallot's like 3, other type of congenital heart defect 19 cases. There was an adequate correlation between eco-2D images and anatomic-surgical findings. In conclusion, we think that a great percentage of cases affected of congenital heart disease should be considered for surgery without the need of a hemodynamic and angiocardiographic study.     

Problems with radical corrective surgery after ascending aorta to right pulmonary artery shunt (Waterston's anastomosis) for cyanotic congenital heart disease.

Forty-five patients with Fallot's tetralogy or pulmonary atresia had total correction 2 to 6 years after palliative ascending aorta to right pulmonary artery anastomosis (Waterston's shunt). The operative mortality for total correction is increased in this group. Patients shown subsequently to have had a good anatomical correction died unexpectedly with high central pulmonary artery pressure, falling arterial PO2, low cardiac output, and progressive acidosis. The cause of this syndrome was difficult to determine. Histological examination of the lungs showed that severe pulmonary arteriolar thickening was not present. The factors apparently predisposing to these postoperative difficulties were kinking of the right pulmonary artery and/or lone perfusion of the right lung, remaining uncorrected for several years, in patients with pulmonary atresia or with Fallot's tetralogy with a shunt performed under the age of 3 years. In survivors, stenosis of the right pulmonary artery frequently occurred but was rarely severe. In view of these difficulties, early haemodynamic and angiocardiographic assessment is recommended in all patients with aorta to right pulmonary artery anastomosis, irrespective of the clinical result, in order to delineate the central pulmonary artery anatomy and assess perfusion of each lung; if kinking of the right pulmonary artery and inadequate perfusion of the central pulmonary arteries and left lung are demonstrated, early radical corrective surgery is recommended.     

Problems with radical corrective surgery after ascending aorta to right pulmonary artery shunt (Waterston's anastomosis) for cyanotic congenital heart disease.

Forty-five patients with Fallot's tetralogy or pulmonary atresia had total correction 2 to 6 years after palliative ascending aorta to right pulmonary artery anastomosis (Waterston's shunt). The operative mortality for total correction is increased in this group. Patients shown subsequently to have had a good anatomical correction died unexpectedly with high central pulmonary artery pressure, falling arterial PO2, low cardiac output, and progressive acidosis. The cause of this syndrome was difficult to determine. Histological examination of the lungs showed that severe pulmonary arteriolar thickening was not present. The factors apparently predisposing to these postoperative difficulties were kinking of the right pulmonary artery and/or lone perfusion of the right lung, remaining uncorrected for several years, in patients with pulmonary atresia or with Fallot's tetralogy with a shunt performed under the age of 3 years. In survivors, stenosis of the right pulmonary artery frequently occurred but was rarely severe. In view of these difficulties, early haemodynamic and angiocardiographic assessment is recommended in all patients with aorta to right pulmonary artery anastomosis, irrespective of the clinical result, in order to delineate the central pulmonary artery anatomy and assess perfusion of each lung; if kinking of the right pulmonary artery and inadequate perfusion of the central pulmonary arteries and left lung are demonstrated, early radical corrective surgery is recommended.     

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.     

Coarctation of the aorta and Fallot's tetralogy. Apropos of 2 cases

Two cases of the association of Fallot's tetralogy and coarctation of the aorta are reported. In both cases there was a right aortic arch. In addition, the second case had a cervical aorta and a left subclavian artery isolated from the aorta and vascularised by the left vertebral artery. The two children underwent complete repair: the first patient had a Waterston shunt followed by repair of the Fallot's tetralogy, followed by repair of the coarctation; the second patient first had a Gore-tex tube inserted from the ascending to the descending aorta and then underwent repair of the Fallot's tetralogy. The association of a coarctation with an obstructive lesion of the right heart is very rare; only two cases have previously been reported: one Fallot's tetralogy and one tricuspid atresia with pulmonary stenosis. Both cases also had a right aortic arch. This seems to go against the haemodynamic theory of coarctation: blood flow in the ascending aorta increases while flow in the pulmonary artery decreases. Coarctation could be caused by a partial involution of the terminal segment of the right primitive dorsal aorta as proposed in cases of coarctation with a right sided aortic arch. The second case also presented two uncommon features: a cervical aortic arch and a left subclavian artery isolated from the aorta.     

Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum

Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.     

Ectopic aortic origin of the right pulmonary artery in tetralogy of Fallot

A case is described wherein Fallot's tetralogy was associated with aortic origin of the right pulmonary artery. The left pulmonary artery was the continuation of the pulmonary trunk. Although a number of examples of Fallot's tetralogy associated with aortic origin of the left pulmonary artery are known, combination of Fallot's tetralogy and aortic origin of the right pulmonary artery is rare and has been described in only two cases previously.     

Hypertrophic myocardiopathy and tetralogy of Fallot. Study of 2 cases

The authors report two observations of concentric hypertrophic cardiomyopathy associated with Fallot's tetralogy in one case and with pulmonary atresia complicated by atrial septal defect in the other. The association of Fallot's tetralogy with hypertrophic cardiomyopathy is rare, as only 5 cases have been reported in the literature. In this connection, the mechanism of myocardial hypertrophy and the therapeutic problems posed by this association are discussed.     

Pulmonary atresia, "intact ventricular septum", and aortopulmonary collateral arteries.

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.     

Pulmonary atresia with an intact septum: indications for the neonatal surgical treatment guided by 2-dimensional and Doppler echocardiography

Twelve neonates with pulmonary atresia and intact ventricular septum underwent surgical treatment based on two-dimensional and Doppler echocardiography. Ten patients with adequate morphology of the right ventricular outflow tract portion shown by means of two-dimensional echocardiography underwent pulmonary valvulotomy and systemic-to-pulmonary shunt. Two neonates with atresia of the right ventricular infundibulum on two-dimensional echocardiography underwent systemic-to-pulmonary artery shunt without valvulotomy. In all patients the qualitative and quantitative echocardiographic diagnosis was confirmed at surgery and/or with the subsequent angiocardiographic study. Two-dimensional and Doppler echocardiography is a precise diagnostic method for planning surgical treatment in neonates with pulmonary atresia and intact ventricular septum.     

Transarterial pulmonary venous wedge arteriography in pulmonary atresia,ventricular septal defect,and intact atrial septum

Transarterial pulmonary venous wedge arteriography was performed in four cases of pulmonary atresia (PA), ventricular septal defect (VSD), and intact atrial septum. In two cases, only the left pulmonary artery was visualized by selective injection to the patent ductus arteriosus (PDA) or Blalock-Taussig anastomosis (B-T shunt), and the right pulmonary artery was opacified by transarterial retrograde pulmonary arteriography. In two other cases, with nonfunctioning B-T shunt, the pulmonary artery was not opacified by either aortography nor by injection into the B-T shunt. Transarterial pulmonary vein wedge arteriography visualized a confluent pulmonary artery. Transarterial pulmonary vein wedge arteriography was a useful and safe procedure for opacifying the pulmonary artery that could not be visualized antegradely in VSD, PA, and intact atrial septum.     

Operative balloon dilatation for pulmonary atresia with intact ventricular septum.

In six infants with pulmonary atresia and intact ventricular septum operative balloon dilatation was used to achieve continuity between the right ventricle and the main pulmonary artery as the initial procedure. Two of the six subsequently needed an aortico pulmonary shunt. All six are alive and well.     

Operative balloon dilatation for pulmonary atresia with intact ventricular septum

In six infants with pulmonary atresia and intact ventricular septum operative balloon dilatation was used to achieve continuity between the right ventricle and the main pulmonary artery as the initial procedure. Two of the six subsequently needed an aortico pulmonary shunt. All six are alive and well.     

Variations in clinical presentation of Fallot's tetralogy in infancy. Angiographic and pathogenetic implications.

Striking variability has been observed in the presenting features in infancy of patients subsequently shown to have tetralogy of Fallot. Some patients presented with severe cyanosis in the neonatal period while others had a systolic murmur and cyanosis only on crying. In these latter patients cyanosis became present at rest over the subsequent months. Yet others presented with episodic attacks of unconsciousness, and a final group presented with dyspnoea and heart failure, accompanying a left-to-right shunt. Angiography demonstrates corresponding variations in the anatomy of the ventricular outflow tracts. In the severely cyanosed patients, the conus septum was deviated so as to obstruct the pulmonary outflow tract, and was best visualized in the lateral projection. In the patients with increasing cyanosis or episodic attacks of unconsciousness, the conus septum again obstructed the pulmonary infundibulum, but was obliquely orientated, not being seen clearly on either lateral or frontal projections. The episodic attacks were considered to be related to infundibular spasm, as previously shown to occur in Fallot's tetralogy. In contrast, increasing cyanosis was believed to be related to hypertrophy of infundibular musculature. In the patients with an initial left-to-right shunt, the arteries were orientated side-by-side so that the conus septum was observed in the frontal projection. Again, subsequent cyanosis was related to ventricular hypertrophy producing outflow tract obstruction. These findings are interpreted in the setting of a recent study, indicating that Fallot's tetralogy is produced by rotation of the embryonic conus together with sinistro-anterior deviation of the conus septum.     

Variations in clinical presentation of Fallot's tetralogy in infancy. Angiographic and pathogenetic implications.

Striking variability has been observed in the presenting features in infancy of patients subsequently shown to have tetralogy of Fallot. Some patients presented with severe cyanosis in the neonatal period while others had a systolic murmur and cyanosis only on crying. In these latter patients cyanosis became present at rest over the subsequent months. Yet others presented with episodic attacks of unconsciousness, and a final group presented with dyspnoea and heart failure, accompanying a left-to-right shunt. Angiography demonstrates corresponding variations in the anatomy of the ventricular outflow tracts. In the severely cyanosed patients, the conus septum was deviated so as to obstruct the pulmonary outflow tract, and was best visualized in the lateral projection. In the patients with increasing cyanosis or episodic attacks of unconsciousness, the conus septum again obstructed the pulmonary infundibulum, but was obliquely orientated, not being seen clearly on either lateral or frontal projections. The episodic attacks were considered to be related to infundibular spasm, as previously shown to occur in Fallot's tetralogy. In contrast, increasing cyanosis was believed to be related to hypertrophy of infundibular musculature. In the patients with an initial left-to-right shunt, the arteries were orientated side-by-side so that the conus septum was observed in the frontal projection. Again, subsequent cyanosis was related to ventricular hypertrophy producing outflow tract obstruction. These findings are interpreted in the setting of a recent study, indicating that Fallot's tetralogy is produced by rotation of the embryonic conus together with sinistro-anterior deviation of the conus septum.     

Complete atrioventricular canal associated with conotruncal malformations: anatomical observations in 13 specimens.

Conotruncal anomalies associated with atrioventricular (AV) canal defects are more common than is generally appreciated on clinical grounds. Among 39 specimens of AV canal malformations, 13 (33%) presented with conotruncal abnormalities: a complete form of AV canal has been observed in all. 5 cases exhibited visceral situs solitus, 5 situs ambiguus with asplenia and 3 situs ambiguus with polysplenia. In the first group, conotruncal anomalies were tetralogy of Fallot in 3 cases, bilateral conus with double outlet right ventricle (DORV) in 2, one with subpulmonary ventricular septal defect (VSD) and the other with doubly commited VSD. Survival in these patients was relatively longer (average 20 mth) and the clinical course was mainly determined by the degree of the pulmonary outflow obstruction: surgical correction should have been feasible in these cases. Patients with situs ambiguus, both with asplenia and polysplenia, had further severe cardiovascular malformations associated with AV canal which led to early death (average survival 12 days): anomalous pulmonary and systemic venous return and univentricular hearts. In the latter patients, tetralogy of Fallot, bilateral conus with DORV and pulmonary atresia were the conotruncal malformation. Retrospectively, in no case of the last category a complete repair had been accomplished. All but one specimen presented the complete form of AV canal with 'free floating anterior leaflet' and hypoplastic anterior tricuspid component. This hypoplasia could be interpreted as missing conal tissue in the development of the anterior tricuspid cusp. For this leaflet a dual embryological origin, both from the dextro-dorsal conal ridge and the right lateral AV cushion, is suggested.     

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? A 5th aortic arch with a systemic-to-pulmonary arterial connection

Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.     

Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum

In this prospective study, 27 consecutive neonates suspected to be suffering from pulmonary atresia and intact ventricular septum underwent detailed two-dimensional echocardiographic examination before cardiac catheterization. Of the 27 neonates 25 had pulmonary atresia and intact ventricular septum and the remaining 2 had "functional pulmonary atresia" secondary to severe Ebstein's anomaly of the tricuspid valve. In all 25 neonates with pulmonary atresia and intact ventricular septum, the diagnosis and right ventricular morphology based on the tripartite approach were correctly established by echocardiography. The associated Ebstein's anomaly in two babies with pulmonary atresia and intact ventricular septum was also correctly identified by echocardiography. Among the five babies who had a sinusoidal-coronary artery communication, echocardiography demonstrated the fistula in one and provided clues for its diagnosis in two others. In the 25 neonates with pulmonary atresia and intact ventricular septum, the echocardiographic dimensions of their tricuspid anulus, right ventricular infundibulum and main pulmonary artery correlated well with the angiocardiographic measurements (r greater than 0.8). The results of this study suggest that, in the management of neonates with pulmonary atresia and intact ventricular septum, preoperative evaluation by echocardiography is usually sufficient and cardiac catheterization should be reserved for selected cases.     

Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)     

Importance and potentials of angiocardiographic study in the diagnosis of congenital heart defects]

Contemporary requirements to angiocardiographic examination in congenital heart diseases are discussed. It is shown that patients with high pulmonary hypertension should be examined by means of left ventriculography and aortography which permit the diagnosis of intraventricular septal defect combined with patent ductus arteriosus, common arterial trunk and open atrioventricular canal. Auxiliary methods of examination in such a commonly encountered anomaly as Fallot's tetralogy and atresia of the pulmonary artery are described, which make it possible to judge the condition of the pulmonary artery. The necessity is demonstrated for performing a two- and multiserial examination in complete and corrected transposition of the major vessels, dual origin of the aorta and pulmonary artery from the right ventricle, and in cases with a solitary ventricle. The role of angiocardiography in appraising the volume of cardiac cavities in patients with congenital heart diseases is noted.