Long-term results after atrial correction of complete transposition of the great arteries.

This study presents the late results for the first 104 consecutive patients surviving and atrial repair for transposition of the great arteries (TGA) between January 1971 and December 1978 (group 1). Mean follow-up was 12 years (range, 0.1 to 17.7 years). The actuarial survival rate at 18 years was 84.2% (70% confidence limits, 79% to 88%) for simple TGA and 93.7% (70% confidence limits, 84% to 97%) for complex TGA. Nine of the 11 deaths were sudden. Two (2.6%) of the 78 late survivors operated on for simple TGA are in New York Heart Association functional class III or IV versus 4 (26.7%) of the 15 survivors with complex TGA; the other patients are doing very well. To better assess long-term results, we report the findings for randomly obtained electrocardiograms, Holter monitor recordings, radionuclide angiographic studies, and cardiac catheterizations performed in 1987 in a larger group of 159 long-term survivors of atrial repair operated on at Ospedale Riuniti di Bergamo from January 1971 to December 1984 (group 2), which includes all of group 1. The findings confirm that the arterial switch repair is the procedure of choice for complex TGA and that there is a major incidence (approximately 10%) of systemic right ventricular dysfunction and rhythm disturbances after the atrial repair. On the other hand, our late survival rate at 18 years of 84% for simple TGA with 97.5% of the patients in functional class I is a result that should be kept in mind, especially in institutions where the arterial switch is a relatively new approach and presumably is a higher risk to cause early death.

[Atrioventricular connection anomalies associated with Fallot's tetralogy: bidimensional echocardiographic study]. / Anomalie di connessione atrioventricolare associate a tetralogia di Fallot: studio ecocardiografico bidimensionale.

From January 1979 to December 1981, 62 patients with Tetralogy of Fallot were studied by two-dimensional echocardiography (2-D Echo). In 10 cases various types of atrioventricular connection anomalies were found: type C atrioventricular canal in 8 cases, straddling tricuspid valve in 1 case, straddling and overriding tricuspid valve in 1 case. These associated anomalies usually are not suspected by means of clinical, electrocardiographic and radiological findings, whereas 2-D Echo appears to have a unique diagnostic value. Apical and subcostal four chamber views were the most helpful to detect the anomalies of the atrioventricular junction; in all cases with complete atrioventricular canal, the ostium primum atrial septal defect, the common atrioventricular valve and the inlet septal defect were clearly seen. In one case an overriding tricuspid valve was clearly demonstrated; in this and in another patient the straddling of the tricuspid valve could be only suspected, whereas its confirmation was obtained subsequently by angiography and surgery. A through 2-D echo evaluation of these anomalies is useful to select the most appropriate views for angiographic confirmation and to ensure optimal surgical planning.

[Tricuspid atresia: hemodynamic and surgical considerations (author's transl)]. / Atresia della tricuspide: considerazioni emodinamiche e chirurgiche

Recently some patients with tricuspid atresia underwent physiological correction, as indicated by Fontan, as an alternative to classical procedures. The aims of the hemodynamic and contrastographic study are illustrated and exemplified, in view of the type of surgery. 49 patients underwent palliative operations: 47 shunts and 2 banding. The most frequent procedure was the Waterston shunt. The total mortality was 10.2%, but no deaths occurred after the third month of life. Finally, the future and the limits of "total" correction are discussed.

[Left ventricle-right atrial communication. Surgical, hemodynamic and clinical aspects (author's transl)]. / Comunicazione ventricolo sinistro - atrio destro. Aspetti clinici, emodinamici e chirurgici

Anatomical, clinical radiographic and electrocardiographic aspects in 12 cases of left ventricle-right atrial communication are described. The usual severity and precocity of symptoms are outlined. At cardiac catheterization the level of the shunt was not found to be exactly correlated with the anatomic type of the defect; the mean QP/QS was 2,7 (1,4-5,8); only one case of pulmonary hypertension was detected and the mean RP/RS was 0,19 (0,06-0,83). Contrastographic aspects are emphasized and the importance of having two orthogonal projections is outlined. 9 patients underwent surgical repair with good results, except for the patient with pulmonary hypertension, who died soon after the operation: this patient shouldn't have been operated on. In all cases only right atriotomy was made, and the defect was closed by a dacron patch; no patient had a tricuspid plastic; one perforation and one cleft of the septal leaflet of the tricuspid valve were sutured directly. In view of the simplicity of surgical correction, wide indications are possible, considering, too, that there have been no reports on the spontaneous closure of the defect.

[Situs inversus and dextrocardia. Analysis of the associated congenital heart diseases (author's transl)]. / Situs inversus con destrocardia. Analisi delle cardiopatie congenite associate.

13 cases of congenital heart diseases in patients having atrial situs inversus and dextrocardia, all studied by means of angiography, are described following an up to date segmental approach. The type of atrioventricular connection was concordant in the 11 biventricular hearts; double inlet in the 2 univentricular hearts. The mode was always via two atrioventricular valves. In two cases the atrioventricular flows realized a criss-cross condition, being horizontal the interventricular septum, superior the morphologically right ventricle and inferior the morphologically left ventricle. In none of the univentricular hearts was an accessory chamber identified; they were both considered to be of indeterminate type. Ventriculo-arterial connections were: concordant in 3 cases (23%), including one case of Tetralogy with pulmonary atresia; discordant in 3 cases (23%); double outlet in 7 cases *54%). The aortic valve was in the assumed position (left and posterior to the pulmonary valve) only in the 3 cases with concordant as a rule. A ventricular septal defect was actually found in all cases; in one it was associated with pulmonary vascular disease. Obstruction to the pulmonary flow (subvalvular, valvular or vascular) was ascertained in 85% of cases, whereas aortic obstruction was never found. Overall, important congenital heart malformations appear to be frequently associated with situs inversus and dextrocardia. The ventriculo-arterial junction is identified as the most affected point.

[Echocardiographic monitoring of atrial septostomy in the transposition of the great arteries]. / Monitoraggio ecocardiografico della settostomia atriale nella trasposizione delle grosse arterie.

In seven consecutive cases of transposition of the great arteries balloon atrial septostomy was performed under the sole echocardiographic guidance. The catheter, introduced into either the saphenous, femoral or umbilical vein, was advanced into the inferior vena cava, then into the right atrium, and, through the patent foramen ovale, into the left atrium. The correct location of the tip of the catheter within the left atrial body was easily identified by two-dimensional echocardiography, with a four chamber view from the subcostal approach. Under echocardiographic control alone the inflated balloon was then suddenly withdrawn into the right atrium according to the Rashkind's technique. The procedure was repeated 3 to 5 times. The balloon was inflated with 2 to 3.5 ml of saline solution. The septostomy was deemed to be adequate when the echocardiographic images revealed an atrial septal defect at least 5 mm in size and an increased flapping motion of the inferior rim of the atrial septum. With this technique the risks of complications due to an incorrect location of the catheter are minimized and the outcome of the procedure can be immediately evaluated by measuring the size of the atrial septal defect.

[Deep hypothermia for the correction of a congenital cardiac defect complete with atrioventricular block]. / Ipotermia profonda di superficie per la correzione di una cardiopatia complessa con BAV completo congenito.

The techniques of deep hypothermia for correction of congenital cardiac defects are well known in many cardiac centers and many cases can be treated even if there are pre-existing rhythm troubles. Our patient had the following diagnosis: left atrial isomerism, dextrocardia; left sided azygos continuation of inferior vena cava; left and right superior venae cavae, hepatic veins, pulmonary veins, all draining into a common atrium; d-loop with normally related ventricles; ventriculo-arterial concordance, small VSD. In addition complete a-v block was present. The patient, 40 days old and 3.4 kg., in heart failure, was paced with a temporary transvenous catheter at 130/min and, afterwards, catheterized. Surgery was undertaken three days post-catheterization using surface deep hypothermia. A Mustard operation, including enlargement of the new left atrium with PTFE (Goretex), was performed during total circulatory arrest. At the end, a permanent pacemaker was positioned in the abdomen and epicardial wires were left on the right ventricle. The postoperative period was uneventful and the patient is doing well 3 months latter. The interest of the case is that even in a complex congenital cardiac malformation with complete a-v block, the technique of surface deep hypothermia can be used because all the physiological parameters remain stable during the procedure in spite of the very low heart rate.

Aortic atresia associated with the right atrial isomeric form of situs ambiguus.

A case is described which had the right isomeric form of situs ambiguus. A common atrial chamber was found with bilateral appendages of right morphology and bilateral superior venae cavae entered the chamber. It communicated through a common atrioventricular valve with a sole ventricular chamber of morphologically right ventricular type which gave rise anteriorly to the pulmonary trunk. There was aortic atresia, the coronary arteries and head and arm arteries being fed in retrograde fashion by a ductus arteriosus. It is emphasized that while aortic atresia is exceedingly rare in situs ambiguus of right isomeric type, it can exist. The case also exhibits the advantages to be gained from categorizing atrial situs on the basis of atrial morphology.

[Echocardiographic diagnosis of total anomalous pulmonary venous return]. / Diagnosi ecocardiografica di ritorno venoso polmonare anomalo totale.

Between March 83 and November 84, 20 patients with TAPVC were studied by cross-sectional echocardiography. The diagnosis was confirmed by catheterization, surgery or autopsy in each case. The anomalous drainage of the pulmonary veins was supracardiac in 10 patients, to the coronary sinus in 5, infracardiac in 4, mixed (supracardiac and infracardiac) in 1. The correct diagnosis was prospectively achieved in 17 patients (85); the echocardiographic examination could not show the precise site of the drainage in 2 (10); in only one patient (5) with right isomerism, double inlet indeterminate ventricle and severe pulmonary stenosis, the diagnosis of TAPVC infracardiac was missed. Seven patients (4 with TAPVC supracardiac, 3 to the coronary sinus) were sent to surgery without preoperative catheterization; the diagnosis was confirmed in all. Cross-sectional echocardiography proved to be a reliable diagnostic tool in the assessment of TAPVC. The patients in which the site of anomalous drainage is clearly identified by echo, can be sent to surgery without invasive investigation.

[Situs ambiguus and atrial isomerism. Angiographic analysis of associated congenital heart diseases]. / Situs ambiguus e isomerismo atriale. Analisi angiografica delle cardiopatie congenite associate.

Forty-seven cases of situs ambiguus have been analyzed angiographically: 24 had right atrial isomerism and 23 left atrial isomerism. The following criteria were considered for identification: auricular morphology; inferior vena cava: azygos continuation or connection in median position to the atrial cavity; bronchial anatomy; anatomy of the pulmonary arteries and their relation to the bronchi. In the group with right atrial isomerism we observed levocardia in 15 cases, dextrocardia in 7 and mesocardia in 2. Bilateral superior vena cava was identified in 13 cases. Inferior vena cava drained in most cases (21) in the middle portion of the atrial cavity; it was on the same side of the abdominal aorta in 20 cases. Pulmonary venous drainage was visualized in 19 patients: in 8 cases it followed the usual pattern of the total anomalous drainage, supracardiac (7) or infracardiac (1); in 2 cases mixed forms were found; in 9 cases the pulmonary veins entered directly the common atrial cavity. Common atrium was seen in 80% of the cases; in the remaining a huge atrial septal defect was present. The atrioventricular connection was double inlet in the 16 cases of univentricular heart; in all of them, and in additional 7 biventricular hearts, the mode was via a common atrio-ventricular valve; only in one case the atria connected to the ventricles through two distinct atrioventricular valves. The univentricular hearts in most cases (22) were, angiographically, of indeterminate type. The interventricular relationship was normal (left ventricle posterior and to the left) in 6 of the 8 biventricular hearts.(ABSTRACT TRUNCATED AT 250 WORDS)

Tetralogy of Fallot with absent pulmonary valve: definitive diagnosis by two-dimensional echocardiography.

A case is presented of a 27-day-old boy with Tetralogy of Fallot and absent pulmonary valve. The two-dimensional echocardiographic examination demonstrated: subaortic ventricular septal defect, overriding aorta, absence of the pulmonary valve with restrictive anulus, and massive dilatation of the pulmonary artery. These anatomic details were then confirmed by angiography and surgery. The echocardiographic features of Tetralogy of Fallot with absent pulmonary valve are typical, and allow the correct diagnosis to be made noninvasively. The haemodynamic investigation becomes obviously easier, and angiocardiography may be devoted to the study of possible additional defects, that are difficult to detect by echocardiography, like multiple ventricular septal defects, or peripheral pulmonary branch anomalies.

Double outlet right ventricle with atrioventricular discordance. Report on cases having situs solitus, dextrocardia, pulmonary stenosis and levomalposition of the aorta.

We describe six patients with situs solitus of viscera and atria, dextrocardia, atrioventricular discordance, ventricular septal defect, double outlet from the morphological right ventricle, pulmonic stenosis and levomalposition of the aorta. Four patients were male and two female; their age ranged from 3.5 to 31 years (mean 13.8 years). All had various degrees of disability, and presented with cyanosis, clubbing and high hematocrit levels. One patient had an atrio-ventricular block that varied from first to third degree; another patient showed intermittent junctional rhythm. At angiography the ventricular septum appeared to be almost perpendicular to the frontal plane in most cases, so that the anteroposterior projection resulted in a true axial view. One overriding left atrioventricular valve and one straddling right atrio-ventricular valve were demonstrated; no significant incompetence of either valve was observed. The ventricular septal defect was always single and related to the subpulmonary outflow. Pulmonic stenosis was valvular in every patient; an additional infundibular obstruction was present in one case. In two cases an additional stenosis was discovered at the supravalvular level. The left pulmonary branch was stenotic in one case; it was hypoplastic, with controlateral dilatation, in two cases; both pulmonary arteries were dilated in one case. The aorta was always to the left of the pulmonary artery, either anterior or side by side. Three patients were operated on in different Institutions: one had a pulmonic valvotomy at the age of six years; one, aged twenty, had a right Blalock-Taussig shunt; the third, with overriding left atrioventricular valve, underwent a modified Fontan operation at the age of thirty years with success.(ABSTRACT TRUNCATED AT 250 WORDS)

[Aortic coarctation and associated abnormalities in the first year of life. Echocardiographic diagnosis]. / Coartazione aortica e anomalie associate nel primo anno di vita. Diagnosi ecocardiografica.

Thirty-eight cases of "Aortic Coarctation Syndrome" presenting in the first year of life (66% under 3 months of age) were studied with cross-sectional echocardiography. Direct imaging of the coarctation was achieved in 75% of cases. Patent ductus arteriosus was present in 60% (80% before three months). Associated anomalies were present in 63%; VSD 29%, Aortic stenosis 16%, Mitral stenosis 16%, AV Canal 5%, Taussig Bing type of DORV 5%, Corrected transposition with VSD 3%, Ebstein anomaly 3%, Univentricular A-V connection 3%. The results were compared with angiographic and/or surgical and/or autoptic findings. The echocardiographic diagnosis proved to be very reliable in most cases. The policy of sending to surgery most neonates and infants with coarctation of the aorta without preoperative catheterization is discussed.

Recurrence after first cerebral infarction in young adults.

OBJECTIVE: We have investigated recurrence of stroke in a consecutive series of young adults, aged 16 to 45 years, after a first cerebral infarction. METHODS: From January 1, 1988 to December 31, 1996 we submitted those patients to a diagnostic protocol including angiographic, cardiological, and haematological investigations. The patients were followed at 6 month intervals up to December 31, 1998. RESULTS: We have evaluated and followed-up 135 patients, 71 men and 64 women, who were 3.99% of all the admitted stroke patients. At 12 months after stroke, 83 patients had returned to work, 40 patients were mildly to moderately handicapped, 4 were using a wheel-chair, and 8 had died. Follow-up was 26 to 123 months (mean 68.8). Recurrence of stroke, always of ischaemic nature, was seen in 15 patients (11.1%), 3 to 76 months after the first stroke (mean 27.4), for an annual incidence of 2.26%. Recurrence was significantly associated with Partial Anterior Circulation Syndrome and Haematological subtype of first stroke (respectively, P = 0.0209 and P = 0.0135, chi2 test), but not with age (< or = or > 35 years) or risk factors. Repetition of stroke was never fatal, but it caused heavy disability in 13 patients, 8 of whom had completely or nearly completely recovered after the first event. CONCLUSIONS: Our data suggest that recurrence of stroke is a major clinical problem also for the patients aged less than 45 years and that it might be more frequent with specific clinical syndromes and etiologic subtypes of first stroke.