Damus-Kay-Stansel procedure with ventricular septal defect enlargement

A full-term infant who had tricuspid atresia with transposed great arteries, a ventricular septal defect, subpulmonary stenosis with posterior malalignment of the conus septum, bicuspid pulmonary valve, and a high-takeoff left coronary artery was referred to our institution. The subpulmonary stenosis gradually progressed and cyanosis worsened. We successfully performed a Damus-Kay-Stansel procedure and a bidirectional Glenn shunt concomitant with ventricular septal defect enlargement. The conus septum was resected along with thick fibrous tissue through both semilunar valves (without ventriculotomy). Postoperative echocardiography demonstrated that both the ventricular septal defect and the subpulmonary space were enlarged effectively without semilunar valve regurgitation.

P719 Complex cyanotic congenital heart disease and its complications- it is never too late to treat

Noncardiac complications significantly contribute to the morbidity and mortality of adults with congenital heart disease (CHD), mainly because life expectancy and quality of life for those born with CHD have greatly improved in the last decades. Double-outlet right ventricle (DORV) with a subaortic ventricular septal defect (VSD) associated with subpulmonary stenosis is a complex cyanotic congenital heart disease from the spectrum of tetralogy of Fallot. We present the case of a young male who was diagnosed at the age of 18 with DORV with subaortic VSD (Figure A), subpulmonary stenosis (Figure C) and mitral valve malformation (anterior leaflet cleft (Figure B) and both leaflets prolapse) with secondary moderate mitral regurgitation (MR), associated with major aortopulmonary collaterals, without pulmonary hypertension. There was no surgical correction at that moment. At the age of 26 he presented with recurrent hemoptysis and embolisation of bronchial arteries was performed. In the same year, a cerebral MRI showed signs of multiple infratentorial and supratentorial ischemic strokes. One year later, he was diagnosed with brain abscess and otomastoiditis that were surgically managed; multiple microorganisms were isolated from the two sources (M. morganii, K. pneumoniae, P . aeruginosa) and the patient received prolonged antibiotic therapy. Two months later he presented with clinical, biological and echocardiographic signs of infective endocarditis (IE) and blood cultures confirmed the diagnosis of Candida albicans associated mitral valve IE (Figure D, E, F, G). Antifungal therapy was administered for one month, with the complete disappearance of the vegetation, but worsening of the mitral regurgitation and moderate left ventricular systolic dysfunction. Thus, the patient had a clear indication for mitral valve replacement together with complete repair of the cardiac malformation that seemed still feasible as the pulmonary arteries were well developed, there was no pulmonary vascular disease and the systolic function of the RV was normal. Correction of CHD was performed in March 2019 consisting of repair of the DORV (Figure H), mitral valve replacement (33 mm bileaflet mechanical valve) (Figure I), tricuspid valve repair with RVOT remodelling. Postoperative, the patient clinical status improved significantly and transthoracic echocardiography revealed a mild residual subpulmonary stenosis (Figure J) and normally functioning mitral prosthesis. There are frequent, various complications in the natural history of congenital heart diseases, especially unrepaired. This case illustrates how vicious the circle of complications can get in a case of unrepaired cyanotic cardiac malformation and the difficulty of breaking this circle. The correction of CHD is the best prevention method and should be performed whenever it is feasible, even at an adult age. Abstract P719 Figure.

Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries

Purpose: Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure. Methods: This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining. Results: Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions. Conclusion: The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations

It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with “la maladie bleu”, that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.

The essential echocardiographic features of tetralogy of Fallot

This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

The clinical anatomy of tetralogy of Fallot

It is now well over one hundred years since Arthur Louis Etienne Fallot showed that four discrete morphologic abnormalities co-existed in the majority of patients he had autopsied with “la maladie bleu”,1 or cyanosis as we now describe it. The lesions he identified were an interventricular communication, subpulmonary stenosis, biventricular origin of the aortic valve, and right ventricular hypertrophy. We now know that the combination of these anomalies had been recognised long before Fallot's epochal description. Indeed, it is Neils Stensen, the Danish monk who also described the parotid duct, who is usually acknowledged as being the first to describe the entity that we now call tetralogy of Fallot.2 Cases were certainly described by John Hunter,2 whilst with the benefit of hindsight, we can see an unequivocal example illustrated by the Baron von Rokitansky3 in his ground-breaking atlas (Fig. 1).