Emanuela Angeli

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.

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.

Early and mid-term clinical experience with extracellular matrix scaffold for congenital cardiac and vascular reconstructive surgery: a multicentric Italian study

OBJECTIVES The aim of this multicentric study was to outline surgical indications and evaluate mid-term outcomes of porcine extracellular matrix (ECM) in surgery for congenital heart disease (CHD). METHODS The use of ECM was categorized into four major groups: A, valve repair; B, septal reconstruction; C, arterial plasty; D, other use. Primary endpoints of analysis were reintervention (either surgical or interventional) when related to ECM, and functional ECM failure. Secondary endpoints were evidence of calcification and of persistent inflammation at follow-up. RESULTS One hundred and three patients (M/F = 61/42, median age 19.7 months, 1 day-62 years) underwent surgical repair for CHD. Among ECM use categories, 38 patients were in Group A, 16 in Group B, 71 in Group C and 7 in Group D. There were neither complications nor deaths related to ECM. At a median follow-up of 23.3 months (0.3-55.2), 19 patients underwent reoperation (ECM-related in 6); 11 patients underwent interventional cardiology procedures (ECM-related in 8). Reinterventions were significantly more frequent on the aortic valve (surgical, P = 0.0056) and pulmonary arteries (interventional, P = 0.0159). In addition, interventional procedures on pulmonary arteries were significantly more frequent in infants <12 months (P = 0.0474). No calcifications were detected. CONCLUSIONS Surgical use of ECM in CHD repair is characterized by a suboptimal functional late performance on reconstruction of valve leaflet or pulmonary artery wall. Longer follow-up and larger clinical experience may support these preliminary results on mid-term outcomes, so as to assess the optimal indication for an ECM graft.

Single coronary artery and neonatal arterial switch operation: early and long-term outcomes†

OBJECTIVES The presence of single coronary artery (CA) in the arterial switch operation (ASO) for neonatal treatment with transposition of the great arteries (TGA) has been reported to be an independent risk factor for early death after surgical repair and late reintervention. The study objective was to evaluate the mortality and the CA stenosis risk at early and long term in neonatal ASO for TGA and single CA. METHODS Between January 1987 and January 2010, 979 neonates underwent an ASO, of which 73 had a single CA (7.5% of all cohort): right ostium with posterior left CA loop was the most frequent pattern (63%), followed by left ostium with an anterior right CA loop (26%). Mean age at operation was 9.3 ± 5.7 days. Mean follow-up was 9.8 years (range: 1-20 years). RESULTS Eight patients (11%) died, 6 of coronary-related death. Overall, survival was 90.1 ± 1.9% at 1 year and 88.6% ± 3.8% at 2, 5, 10 and 15 years, respectively. Independent risk factor for mortality was associated surgery before 2001. Freedom from coronary events was 91.6 ± 3.3% at 1 year and 88.7 ± 3.8% at 5, 10 and 15 years respectively. No patients required late reintervention for CA surgery or angioplasty. CONCLUSIONS All coronary-related death occurred within the first 6 months after ASO, and all patients but 1 were operated before 2001. In our experience, it appears that a single CA is not any more a risk factor for early and late mortality after ASO for TGA. Mortality has drastically reduced since 2001 and is now close to that found in TGA with standard coronary patterns. The acquired experience shared between the surgeons and the institution offsets the undeniable surgical difficulty.

Aortopulmonary Window and Anomalous Coronary Artery: An Exceptional Association

Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative course.

Neonatal coarctation repair using extended end-to-end anastomosis

Aortic coarctation without associated intracardiac lesions, with or without posterior arch hypoplasia referred as simple coarctation, is safely and effectively repaired via left posterolateral thoracotomy, whereas median sternotomy and cardiopulmonary bypass are necessary for aortic arch reconstruction in case of complete aortic arch hypoplasia, or coarctation with associated cardiac lesions. There is a wide variety of techniques currently proposed to repair neonatal coarctation, all reported associated with very low operative mortality, nevertheless which one would be the best technique in terms of minimal rate of residual or recurrent obstruction or late complications is still under debate.

Initial clinical experience with Dideco Kids D100 neonatal oxygenator.

We describe our initial clinical experience with the new hollow fibre membrane oxygenator Dideco Kids D100, used in five patients with a mean age of 21 ± 28 days. The priming volume is 31 ml with a membrane surface area of 0.22 m2. Data were compared with those deriving from five patients treated with Dideco D901 Lilliput 1 oxygenator. Among patients treated by the new Dideco Kids D100 oxygenator, priming volume was significantly reduced and an easier blood gas delivery was evident.

Pulmonary artery banding

Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation in some congenital heart disease. In the beginning, when the use of cardiopulmonary bypass was affected by many deleterious effects, this technique played a fundamental role in the treatment of patients with congenital heart defects and an intracardiac left-to-right shunt. The use of PAB has decreased during the last two decades, due to the increasing popularity of early complete intracardiac repair, which results have shown to be superior to staged repair, even in low body weight patients. Moreover, several authors have emphasized the negative effects of PAB such as pulmonary arterial branch distortion, abnormal right ventricular hypertrophy, pulmonary valve insufficiency, sub-aortic obstruction and decreased ventricular compliance in patients with univentricular heart. For all these reasons, this procedure has been placed in the dark corner of surgery, representing, between 2002 and 2005, ∼2% of the total amount of cardiac surgery procedures. In a more recent era, PAB has been performed in instances other than classic univentricular heart, as palliation in small infants with cardiac defects with a left-to-right shunt and pulmonary overcirculation, thus gaining some time prior to a planned staged repair. Recently, the role of PAB is becoming more important in selected subsets of congenital cardiac defects: L-transposition of the great arteries, D-transposition of the great arteries, hypoplastic left heart syndrome, moderately hypoplastic left ventricle (congenitally corrected transposition of the great arteries). This renewed interest in the banding procedure is spurring all surgeons and cardiologists to find new solutions for an easier banding procedure while making debanding less traumatic.

Anomalous origin of additional coronary artery arising from the pulmonary artery in complex congenital heart disease.

Anomalous origin of coronary arteries is a rare congenital defect and usually presents as an isolated defect. In only 5% of cases, it may be associated with other cardiac anomalies. In this article, we present a case of a newborn with a very complex congenital cardiac disease accompanied with an anomalous origin of an additional coronary artery.

Diagnostic Accuracy of Aortic Root Cross-sectional Area/Height Ratio in Children and Young Adults with Marfan and Loeys-Dietz Syndrome

BACKGROUND Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation. We evaluated the diagnostic accuracy of an height-based aortic root-indexing method, aortic root cross-sectional area/height ratio (AHr), in children with Marfan and Loeys-Dietz syndromes. METHODS A cohort of 54 children with Marfan or Loeys-Dietz syndromes, aged 3 months to 17 years, were evaluated with a transthoracic echocardiogram. AHr was measured in diastole at sinuses of Valsalva (SoV) and proximal ascending aorta (pAA) in a group of normal subjects matched for age and body surface area and normal values were provided. AHr values were recorded for patients and compared with z-scores results obtained with Gautiers and Campenss nomograms. RESULTS AHr values in the group of normal subjects were 2.6 ± 0.6 at SoV and 2 ± 0.5 at pAA. Categorization of z-scores and AHr showed good correspondence between AHr and Gautiers method (P = .341 at SoV and .185 at pAA) and AHr and Campens method (P =.465 at SoV and 0.110 at pAA). CONCLUSIONS There was a good correspondence of AHr results with two different z-scores. AHr is a simple to use and valid option to quantify aortic root dilatation in pediatric patients.