Guido Oppido

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries

OBJECTIVE To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). METHODS A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. PATIENT POPULATION Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9±1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61±2% vs 62±6% of group II, p=1.0). RESULTS The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p=0.04) and logistic regression (p=0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p=0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p=0.1). CONCLUSIONS The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.

Surgical treatment of congenital mitral valve disease: Midterm results of a repair-oriented policy

OBJECTIVE Management of congenital mitral valve disease is challenging because of a wide morphologic spectrum, frequent associated lesions, and small patient size. We evaluated the results of a repair-oriented policy. METHODS All consecutive patients with congenital mitral valve disease who underwent surgery between 1996 and 2006 were studied retrospectively. Patients with atrioventricular canal, atrioventricular discordance, or ischemic regurgitation were excluded. RESULTS During this period, 71 children (median age 2.9 years, range 3 days-20.8 years) underwent surgery. All but 1 underwent primary mitral valve repair. Twenty-two (30%) were younger than 12 months. Associated cardiac lesions were present in 45 children (63%) and were addressed concurrently in 35; previous cardiac procedures had been performed in 17 patients (24%). Mitral incompetence was predominant in 60 (85%) and stenosis in 11 (15%). During a median follow-up of 47.8 months (range 2-120 months), 14 patients underwent 17 mitral reinterventions: 14 repairs and 3 replacements. After 60 months, overall survival was 94% +/- 2.8%; freedoms from reoperation and prosthesis implantation were 76% +/- 5.6% and 94% +/- 3.6%, respectively. There were 4 deaths, and all survivors remain in New York Heart Association class I or II with moderate (6 patients) or less mitral dysfunction. CONCLUSION Surgical repair of the congenital mitral valve can be successfully performed with low mortality, satisfactory valvular function at midterm follow-up, and acceptable reoperation rate while obviating risks associated with valvular prostheses. Suboptimal primary repair was significant predictor for reoperation but re-repair was often successful.

Impact of pulmonary regurgitation and right ventricular dysfunction on oxygen uptake recovery kinetics in repaired tetralogy of Fallot

Patients with repaired tetralogy of Fallot (ToF) featuring severe pulmonary regurgitation (PR) and/or right ventricular (RV) dysfunction have reduced exercise tolerance.

Cleft mitral valve without ostium primum defect: anatomic data and surgical considerations based on 41 cases

BACKGROUND Cleft mitral valve without an ostium primum defect, referred to as isolated cleft mitral valve, has been the subject of many reports; yet its morphology and operability remain incompletely understood. METHODS The anatomic findings in 36 postmortem cases, five explanted hearts, and relevant clinical data constitute the material basis of this report. Cardiac catheterization data were available in 29 cases and two-dimensional echocardiograms in 13 cases. RESULTS Twenty cases had normally related great arteries with subpulmonary conus. Of these cases 4 (20%) had tetralogy of Fallot and 1 had tricuspid atresia. Twenty-one cases had abnormal ventriculoarterial relationships with subaortic or bilateral conus resulting in transposition in 16 (76%) and double-outlet right ventricle in 5 (24%). In the cases with normally related great arteries, the morphology of the ventricular septal defect and the mitral cleft were similar to those of the more complete forms of atrioventricular canal defects. The mitral cleft usually resulted in progressive mitral regurgitation, which can be treated by surgical closure of the cleft. In the cases with abnormal conus, the morphology of the ventricular septal defect and the mitral cleft did not resemble atrioventricular canal defects. The attachment of the cleft usually produced obstruction of the left ventricular pulmonary outflow tract. Surgical repair of the cleft cannot eliminate this obstruction. CONCLUSIONS There are two different anatomic types of isolated cleft mitral valve: the canal type, and abnormal conus type. Diagnosis of the associated ventriculoarterial relationships helps to guide their surgical treatment.

Role of endovascular repair in the management of late pseudo-aneurysms following open surgery for aortic coarctation

BACKGROUND Coarctation of the aorta accounts for almost 5% of all congenital cardiac malformations, and it is usually treated by open surgical procedures. Despite the excellent primary results, many patients may develop anastomotic pseudo-aneurysms, associated with considerable morbidity and mortality rates. We investigated the role of endovascular repair as an alternative to open re-do surgery on the descending aorta. METHODS We retrospectively collected data of 11 consecutive patients who presented with pseudo-aneurysms of the descending aorta following previous surgical repair of congenital aortic coarctation. Nine patients developed a pseudo-aneurysm after patch aortoplasty, while an interposition graft repair was performed in two cases. Seven patients underwent an endovascular repair (26.9 years after the previous open surgery). Four patients with a pseudo-aneurysm after coarctation repair associated with arch hypoplasia underwent conventional open re-operation. RESULTS No complications or death were observed in the conventional surgical group. Technical success was obtained in all patients who received a stent-graft procedure. More than one stent graft was necessary only in one case. A left carotid-left subclavian artery bypass was performed in three patients. Type II endoleak was detected by angiography in one patient who had previously undergone subclavian re-vascularisation. The median follow-up (FU) was 44.5 months and was 100% complete. Neither re-operations nor complications or deaths occurred during FU. CONCLUSIONS Endovascular repair of pseudo-aneurysms following open surgery for aortic coarctation is feasible and safe. However, more patients and longer follow-up are necessary to assess the efficacy of this promising less-invasive alternative to open re-do surgery.

Results of the modified Fontan procedure are not related to age at operation.

OBJECTIVE The modified Fontan procedure represents the final stage for the palliation of hearts with single-ventricle physiology. Different opinions exist regarding the optimal timing of the operation, with most centres advocating early intervention. By contrast, over the past decade, we have progressively increased the age at Fontan operation with the aim to potentially delay the onset of late Fontan failure, and to possibly use larger extracardiac conduits. We retrospectively reviewed our surgical experience with Fontan operation, to understand the impact of this strategy on morbidity and mortality. METHODS Between 1990 and 2008, 65 patients underwent a modified Fontan operation at our institution (extracardiac conduit in 52 and lateral tunnel in 13). The median age at operation in our series was 7.3 years (range: 2.2-15.8 years) and this value was used to divide the study cohort into two groups. Group A (n=28) included patients with an age at Fontan operation <or=7 years, whereas group B (n=37) included patients who had a Fontan operation at >7 years of age. Preoperative characteristics, intra-operative data and short- and medium-term results were assessed. RESULTS No differences in baseline characteristics, morbidity and mortality were evident between groups. Hospital mortality was 0% in group A and 5.4% (2/37) in group B (p=0.5). Prolonged pleural effusions were present in eight patients in group A (29%) and seven in group B (19%, p=0.39). After a mean follow-up of 5.7+/-5.4 years (range: 0.3-18 years), the overall mortality of group A (1/28) was similar to that of group B (2/37) (3.6% vs 5.4%, p=0.999). The incidence of arrhythmias, protein-losing enteropathy, Fontan take down and re-operation were not different between the two groups. CONCLUSIONS The modified Fontan operation can be performed safely in older patients without affecting operative and medium-term follow-up results. Postponing the extracardiac Fontan operation may have the advantage of the use of a larger conduit.

Aortic translocation, Senning procedure and right ventricular outflow tract augmentation for congenitally corrected transposition, ventricular septal defect and pulmonary stenosis

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Significant pulmonary stenosis is a contraindication to the conventional double-switch. Instead repair may be accomplished by the Rastelli-Senning procedure, using an extracardiac conduit to achieve continuity between the morphological left ventricle and the pulmonary arteries. This however can be accompanied by conduit and intra-ventricular baffle-related problems that can necessitate surgical re-intervention and lead to late mortality. We describe the use of aortic translocation, Senning procedure and reconstruction of the right ventricular outflow tract using autologous tissue and valved homograft to facilitate anatomical correction in congenitally corrected transposition. The advantages of this technique in this group of patients and the implications for conduction tissue are discussed.

Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery.

OBJECTIVES To identify determinants of adverse outcomes in patients with atrial isomerism. METHODS Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism. RESULTS There were 78 cases with right and 104 with left atrial isomerism. Nineteen patients were not offered surgery; 49 (47%) of those with left atrial isomerism and 60 (77%) with right atrial isomerism were directed to single ventricle palliation. A total of 67 patients died. Survival to 25 years was 62% (95% confidence interval [CI], 53%-69%). Independent predictors of mortality were obstructed total anomalous pulmonary venous drainage (P < .001; hazard ratio [HR], 5.2; 95% CI, 2.7-9.7) and asplenia (P = .008; HR, 2; 95% CI, 1.2-3.3). There was no evidence that patients born in the recent era had improved survival (P = .47; HR, 1.1; 95% CI, 0.8-1.5). Survival was better for patients with left than right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%). Independent predictors of mortality for patients with left atrial isomerism were dextrocardia (P = .009; HR, 3.0; 95% CI, 1.3-6.7) and pulmonary stenosis (P = .042; HR, 0.3; 95% CI, 0.1-0.9) and patients with right atrial isomerism, biventricular repair (P < .001; HR, 6.0; 95% CI, 2.8-13), and obstructed total anomalous pulmonary venous drainage (P < .001; HR, 4.2; 95% CI, 2.0-8.6). CONCLUSIONS A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism.

Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis

underline the relative greater frequency of this complication in heterotaxy syndrome when associated with anomalous pulmonary and systemic venous connections, which can make surgical repair complicated with the need to create a tortuous and potentially obstructive pathway. Despite this concern, a recent report from our institution 5 has shown excellent outcome with cavopulmonary connection in patients with heterotaxy syndrome and anomalous pulmonary venous return. This is likely due to improved surgical technique and use of extracardiac conduit in cases with complex atrial and venous anatomy (18%). None of the 3 patients had evidence of obstruction at the pulmonary venous pathway early after the Fontan procedure, as determined by 2-dimensional echocardiography. Pulmonary venous obstruction developed slowly through a period of several months to years. In patient 2, pulmonary venous obstruction developed 4 months after device closure of the fenestration, suggesting that the additional scarring from device implantation contributed to the obstruction. The obstruction was due to fibrotic tissue at either the interatrial septum or in the interatrial course of the pulmonary veins (patient 2). To prevent this complication, and to ensure a wide open pulmonary venous outflow, particularly in patients with mitral atresia or stenosis, we currently enlarge the communication between the pulmonary veins and the right atrium by unroofing the coronary sinus posteriorly into the left atrium, in addition to resection of the interatrial septum. In conclusion, late onset obstruction of pulmonary venous pathway after lateral tunnel cavopulmonary connection is a rare but serious complication that may be prevented by unroofing the coronary sinus at the time of Fontan procedure.

Dilated cardiomyopathy presenting in childhood: aetiology, diagnostic approach, and clinical course

OBJECTIVE To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification. METHODS Retrospective study of 41 consecutive children affected by dilated cardiomyopathy - aged 0-14 years; median 33.4 plus or minus 49.25 - between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan-Meier method. RESULTS Follow-up ranged from 10 days to 162 months - median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%. CONCLUSIONS In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.