Surgical Management of straddling tricuspid valve and associated defects is a complex problem. Between August 1992 and August 1995, 5 patients with major straddling of the tricuspid valve underwent a complete or partial biventricular repair. All patients had either an inlet ventricular septal defect (n=4) or a ventricular septal defect with an inlet component (n=1), Co-existing cardiac lesions included hypoplasia of the right ventricle (n=3), discordant ventriculoarterial connections (n=1), tetralogy of Fallot (n=1), and multiple muscular vetricular septal defects (n=2). At the time of presentation to our institution, two of these patients had previously been palliated in preparation for a Fontan procedure, having undergone construction of a bidirectional superior cavopulmonary shunt. One patient was referred specifically for a Fontan procedure. The tricuspid valve was repaired by transecting all of the straddling cords and reattaching them in the right ventricle or onto the right side of the patch used to close the ventricular septal defect. Associated procedures included closure of the septum in all patients, an arterial switch procedure in one, repair of tetralogy of Fallot in one, and construction of a bidirectional superior cavopulmonary shunt in one. There has been no early or late mortality. Complete heart block requiring insertion of a pacemaker occurred after surgery in three patients. At a median follow-up of 32 months, functional integrity of the tricuspid valve is well maintained, with only one patient having moderate tricuspid regurgitation. None of the patients are receiving any cardiac medication.

Construction of a modified Blalock-Taussig shunt using polytetrafluroethylene is frequently used for palliation in infants with complex cyanotic heart disease. This study was performed to assess angiographically the late patency rate of such prosthetic shunts. A modified Blalock-Taussig shunt had been constructed in 82 children and, of these, 63 (77%) patients had post-operative angiography and were included in this study. There were 29 males and 34 females, ranging in age from 1 day to 164 months. A second shunt was needed in 7 patients. Thus, 70 shunts were studied. Of the shunts, 16 were 4 mm in diameter, 28 shunts 5 mm in diameter, and 26 shunts 6 mm in diameter. Postoperative angiography was performed on 1 day to 132 months (median 39 months) after construction of the shunts. Acute post operative occlusion occurred in 2(3%) patients, who had 5 and 6 mm shunts, respectively. At the latest follow-up (median 39 ± 26 months), 6(9%) late occlusions were noticed. Overall patency was 89% for all the shunts, with 94% of the 4 mm shunts, 85% of the 5 mm and 88% of the 6 mm shunts being patent. Regression analysis showed a progressive reduction in diameter of the graft with time. This narrowing of the graft may have been related to the formation of a ‘neointima’ due to fibrous hyperplasia.

Aneurysm of the arterial duct is well described when presenting in infants and children. The case we present were detected by fetal echocardiogram in late pregnancy, indicating the congenital nature of this lesion and excluding widely accepted explanations of the pathogenesis. Spontaneous closure may occur more frequently than the literature suggests. We recommend a conservative approach in asymptomatic patients.

Anatomic study of 54 autopsied cases of tricuspid atresia revealed mitral valvar anomalies in 9 (16.7%). The morphologic findings in these 9 cases are presented and compared with 45 cases having a normal mitral valvar apparatus. The anomalies included a cleft valve in 2, parachute valve in 2, straddling valve in 3, and a valve with dual orifice in 2.Two of these cases had more than one anomaly. Data from echocardiography and cardiac catheterisation, when available, had failed to diagnose the anomalies during life. The anomalies were more commonly associated with discordant ventriculo-arterial connections (33.3% versus 13.3%, p < 0.01), were associated significantly with a large ventricular septal defect (33.3% versus 4.4%, p < 0.01), and with pulmonary hypertension (33.3% versus 2.2%, p < 0.01). Two of 3 post operative deaths among the 9 were due to hemodynamic consequences arising from the uncorrected mitral valvar anomaly (parachute valve in both ). Diagnosis of these anomalies prior to surgical correction is imperative, as their presence may necessitate repair or replacement of the mitral valve. Failure to do so may result in increased perioperative mortality, congestive cardiac failure and arrhythmias at a later date. Mitral valvar anomalies may be a contributory factor to the early onset of cardiac failure seen in patients with tricuspid atresia undergoing palliative shunts.

We evaluated 6 patients with an anomalous subaortic left brachiocephalic vein. All cases were associated with some form of congenital heart disease: 4 patients had coexistent tetralogy of Fallot, one had congenitally corrected transposition, and the other an atrial septal defect with azygos connection of the inferior caval vein. Our incidence of an anomalous subaortic left brachiocephalic vein in patients with congenital heart diseases was 1.1% (6/527). It was observed in 4 out of 117 patients with tetralogy of Fallot (3.4%). Doppler echocardiography was useful in establishing the diagnosis. Furthermore, both digital subtraction angiography and magnetic resonance imaging demonstrated well the anomalous course of the vein, and were useful in making an accurate diagnosis.

We studied echocardiographically 17 children with depletion of carnitine due to treatment over 1–3 years with antibiotics containing pivalic acid. When the children were depleted of carnitine, the left ventriclar posterior wall was significantly thickened in diastole when compared to a reference group. Six months later, after normalisation of the concentrations of carnitine, the left ventricular posterior wall was significantly thinner and did not differ from the reference group. We conclude that drugs containing pivalic acid, when given for several months, decrease the stores of carnitine to levels where cardiac involvement occurs.

The clinical course, noninvasive findings and outcome of 25 infants and children with idiopathic dilated cardiomyopathy were reviewed retrospectively to identify factors predictive of outcome both at diagnosis and at short-term follow-up. Patients, stratified by clinical status at last visit, were assigned to groups encompassing those who were asymptomatic and receiving on medication, those patients who were symptomatic or needed medication to control symptoms, and those who were dead, awaiting heart transplantation, or had undergone transplantation. Older age at diagnosis was strongly associated with poor clinical outcome (p=0.005), as was the presence of arrhythmias at the time of diagnosis (p=0.008) and at short-term follow-up-(p=0.003). Echocardiographic studies at diagnosis failed to predict eventual clinical outcome, although patients who ultimately recovered and did not need medications tended to demonstrate improvement in or resolution of echocardiographic abnormalities within 6 months of diagnosis.

The availability of echo Doppler cardiography enables monitoring of the natural course of congenital aortic valvar stenosis more adequately than before. Between 1986 and 1993, 129 children with such stenosis were examined echocardiographically, 83 of them over a prolonged period with repeated studies of at least one per year. The pressure gradient between the left ventricle and ascending aorta, as well as the left ventricular wall thickness, were measured. When the pressure gradient detected increased to more than 60 mmHg, the patients were treated by balloon valvoplasty or surgical valvotomy. The last examination prior to intervention was taken as the final measurement.

We found a significant increase (≥10 mmHg) in the measured pressure gradient in 40 of the 83 patients (48%). The onset of severe stenosis was not observed at a specific age, rather the stenosis was found suddenly to increase in severity at any age. The gradients measured in children younger than 2 years of age, however, increased twice as rapidly as those measured in the older children. A high gradient was not always accompanied by evidence of left ventricular hypertrophy.

Based on our findings, we suggest that, since a sudden increase in severity of aortic valvar stenosis might be expected, the pressure gradient should be measured once every 6 months in children younger than 2 years of age and once a year after that age

Patients undergoing the modified Fontan procedure may develop low cardiac output postoperatively. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate the influence of triiodothyronine supplementation. Ten consecutive patients under-going the Fontan procedure were administered intravenous triiodothyronine (0.4 mcg per kg) following surgery. Clinical outcome and thyroid hormone profiles were assessed and then compared to a previous series of patients undergoing the Fontan procedure who had not received triiodothyronine supplementation. Both groups initially demonstrated marked decreases in serum free triiodothyronine levels. The group which received triiodothyronine supplementation demonstrated a more rapid return of serum triiodothyronine levels to baseline [259±17 vs 121±15 pg/dl (p<0.05) on the fifth postoperative day and 336±18 vs 178±12 pg/dl (p<0.05) on the eighth day]. In addition, patients receiving supplemental triiodothyronine demonstrated more rapid recovery of total triiodothyronine, free thyroxine, total thyroxine and thyroglobulin levels. The group which received triiodothyronine supplementation had a shorter length of hospital stay [9±2 vs 14±3 (p<0.05)] as compared to patients who did not receive exogenous triiodothyronine. The results of this study demonstrate that triiodothyronine supplementation aids in the recovery of thyroid hormone levels following Fontan procedure. This endocrinologic finding correlated with improved clinical outcome.

The Fontan operation was initially designed to provide a “physiological correction” of tricuspid atresia, but its indications have been extended to more complex forms of congenitally malformed hearts. In selected patients with two adequately sized ventricles, but highly complex intra cardiac anatomy which makes a biventricular repair more hazardous and which would require multiple reoperations, a univentricular repair using Fontan's principle has been proposed. Between 1978 and 1992, a modified Fontan operation was carried out in 34 such patients. Of the patients. 13 had double outlet right ventricle and 21 had transposed great arteries. Anomalies of ventricular loop were present in 14 patients. A non-committed ventricular septal defect was found in 26 whilst multiple defects were present in 10. All patients had anomalies of the atrioventricular valves, with straddling of the tricuspid valve,11 and/or of the mitral valve11, and abnormal tricuspid5, or mitral4 cordal attachments. Two patients had mild tricuspid hypoplasia, 1 had mitral hypoplasia and 3 had a cleft mitral valve. Fourteen patients had anomalies of both atrioventricular valve but only 6 had mild atrioventricular valvar incompetence. Pulmonary stenosis or atresia was seem in 26. All the patients had at least one or more (mean 2.35) intracardiac anomalies that preculded a biventricular repair. The Fontan operation was performed at a median age of 4.75 years. There were 4 early (11.4%, 70% CL: 5.1–19.8) and 2 late deaths, with a 4 year actuarial survival rate of 79.6±8.1%. Univariate analysis did not reveal any risk factor for early of late death. Follow-up at a mean interval of 3.6 ±2.6 years was available in all but 1 survivor. There were 6 early and 2 late reoperations, and 3 patients required implantation of a pacemaker. In conclusion, the modified Fontan operation can provide satisfactory early and mid term results in patients with two ventricles but with complex intracardiac anatomy which precludes a biventricular repair.

In 3 patients with isomeric morphologically left atrial appendages, univentricular atrioventricular connection, concordant ventriculoarterial connections, bilateral superior caval veins, with the left one draining via the coronary sinus, together with absence of any communicating vein, interruption of inferior caval vein with drainage via a right-sided (n=2) or left-sided (n=l) azygos vein, the hepatic venous blood was rerouted via the large coronary sinus into the pulmonary arterial circulation. In a fourth patient with similar pathology, having interruption of the left-sided inferior caval vein with drainage to the left-sided superior caval vein via a left-sided azygos vein and a large communicating vein, the pathway from the left superior caval vein to the coronary sinus was correspondingly small. An extracardiac conduit was therefore constructed between the hepatic veins and the left pulmonary artery so as to reroute the hepatic venous blood into the pulmonary arterial circulation. At a mean follow-up of 8.5 months, all patients are clinically well and none of them have developed pulmonary arteriovenous malformations. To avoid the latter complication in Fontan physiology, especially in the setting of an interrupted inferior caval vein with drainage via the azygos vein, we believe that it is preferable to reroute the hepatic venous blood into the pulmonary circulation.

We report experimental use of a new device for transcatheter closure of atrial septal defects. The device was implanted in surgically created atrial septal defects in two pigs. The devices were easy to implant, and the procedures were short. Both devices were implanted correctly in the desired position, and closed the defect completely. No procedural complications nor problems were encountered. The technique is certainly worth trying in the clinical setting. The device appears to be particularly favourable for use in children because it can be implanted through a 7 French sheath.

A 5-week-old male, born with an aneurysm of the arterial duct forming part of a vascular ring, underwent successful surgery without cardiopulmonary bypass. From our search of the literature, it seems, that this combination of rare congenital malformations has not previously been reported.

A 3-year-old boy developed acute pulmonary edema following 6 months of recurrent fevers and thrombocytosis. Echocardiography demonstrated moderate-to-severe insufficiency of both mitral and aortic valves. Angiography and intravascular ultrasound were used to diagnose this unusual presentation of Takayasu's arteritis. To the best of our knowledge, this is the first histological demonstration of acute and chronic inflammatory changes in the mitral valve in these circumstances.