Ebstein's malformation of the tricuspid valve is a rare but complex congenital cardiac lesion characterised by a variable degree of dysplasia and displacement of the proximal attachments of its inferior and septal leaflets from the true atrioventricular junction. The aim of our retrospective study is to report the risk factors for mortality, and to determine the clinical profile as seen in 52 cases diagnosed in our service between 1978 and 2002, concentrating in particular on the outcome for the neonatal patient. There were 26 females and 26 males, and the age at presentation ranged from 30 weeks gestational age to 46 years. We found 23 associated cardiac anomalies in 20 cases. Of the patients, 11 patients (21%) died. Actuarial survival at 30 years was 65%. Predictors of death included fetal or neonatal presentation, presence of associated defects, a grade within the Celermajer index of 3 or 4, and a cardiothoracic ratio equal to or greater than 65%. The diagnosis was made in the neonatal period in 24 patients, nine of whom died. Of the 15 survivors, only 4 are free of symptoms. Surgical treatment was undertaken in 9 patients, with a mortality rate of 33% without late deaths, with all the survivors being in good condition. The mean period of follow-up for the 41 living patients was 16.5 years. An arrhythmia of variable severity appeared during the evolution of 27 patients (66%). At present, only 7 cases are in the functional class III or IV of the grading system of the New York Heart Association, but 25 patients (61%) needed some medical treatment, meanly for arrhythmic events. Thus, fetal and neonatal presentation of Ebstein's malformation is associated with a poor outcome. Moreover, the echocardiographic appearance, marked cardiomegaly, and the presence of associated lesions are all risk factors for mortality. Arrhythmia and need of medical treatment are common in older children and adults. Survival after surgical treatment is associated with a good outcome.

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Between 1982 and 1989, nine patients with complete atrioventricular (AV) canal and tetralogy of Fallot underwent successful repair of both anomalies. Seven patients had Down's syndrome. One or more previous palliative shunts had been used in six patients. Associated cardiovascular lesions included persistent left superior vena cava (two patients), patent ductus arteriosus with marked stenosis of the left pulmonary artery (one patient). The diagnosis was confirmed by cardiac catheterization and cineangiography. Closure of the ventricular septal defect was performed through a combined right atrial and ventricular approach in each patient. The right ventricular outflow obstruction was relieved with the use of a transannular patch (4), right ventricular infundibular patch (4) or a valved external conduit (1). There were no hospital deaths. One patient died as a result of meningitis 2 years after repair. Progressive left AV valve incompetence developed in one patient requiring valve replacement. A single atrial approach may not provide the best exposure of the subaortic end of the defect. In this regard, we recommend an additional right ventriculotomy in order to ensure safe closure of the septal defect. Although an adequately shaped isolated patch could be sufficient to close the anterior VSD, we favour a separate patch to minimize the possibility of producing subaortic obstruction. A further method to avoid this subaortic stenosis consists of dividing the common anterior leaflet to the right towards the lateral extent of the infundibular septum.

Case report An asymptomatic II-year-old boy attended the Cardiac Division of the Navarra Hospital in February I969, for evaluation of an abnormal cardiac shadow. Five years before admission he was studied at another cardiac centre and a diagnosis of infundibular pulmonary stenosis with a systolic gradient of 20 mmHg was verified. On auscultation, a systolic murmur and a split second sound were heard in the pulmonary area. The x-ray examination of the thorax showed an abnormal cardiac shadow (Fig. I).The electrocardiogram revealed normal sinus rhythm. An rS pattern was observed in leads II, III, and aVF with very deep S waves, shifting the mean QRS axis to the left (-6o0) (Fig. 2). A Q wave was present in leads I, VI, V2, and V3 with 1 Address for reprints: Dr. E. de los Arcos, Department of Thoracic and Cardiovascular Surgery, Hospital de Navarra, Pamplona, Spain. S wave in I and all the praecordial leads. A small r' was present in lead V4R. The QRS interval was 0 I3 sec in lead II. The electrocardiographic changes were interpreted as left anterior hemiblock associated with right bundle-branch block.Withdrawal pressure tracings during right cardiac catheterization showed infundibular pulmonary stenosis with a systolic gradient of 8o mmHg. No abnormality was observed during left arterial retrograde cardiac catheterization.Right ventriculotomy under extracorporeal circulation revealed a hydatid cyst IO cm in diameter in the interventricular septum. The cystic adventitia was opened and the cyst was removed.The postoperative electrocardiogram (Fig. 3) showed QRS interval widening to o-I6 sec. The mean QRS axis was not modified but the degree of right bundle-branch block was increased.

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