Carola Iacono

Stenting of the arterial duct in newborns with duct-dependent pulmonary circulation

Objective: To evaluate the feasibility and results of stenting of the arterial duct in newborns with duct-dependent pulmonary circulation using low-profile, high-flexibility premounted coronary stents. Design: Prospective interventional and clinical follow-up study. Setting: Tertiary referral centre. Patient population: Between April 2003 and December 2006, 26 neonates (mean (SD) age 15.2 (19.9) days, mean (SD) weight 3.3 (0.8) kg) underwent attempts at stenting of the arterial duct. Main outcome measures: Procedural success and complication rates. Early and mid-term follow-up results. Results: The procedure was successfully completed in 24/26 (92.3%) cases. Minor complications occurred in 2/26 (7.7%) cases. No mortality occurred. After stenting, the ductal diameter increased from 1.2 (1.0) mm to 3.1 (0.4) mm (p<0.001) and the percutaneous O2 saturation increased from 70 (14)% to 86 (10)% (p<0.001), respectively. Over a mid-term follow-up, 2/24 patients (8.3%) needed a systemic-to-pulmonary artery shunt because of inadequate ductal flow and 4/24 patients (16.7%) underwent stent redilatation after 6.0 (4.4) months, but before corrective surgery. Cardiac catheterisation before corrective surgery in 9 patients showed an increase of the Nakata index from 112 (49) mm/mm2 to 226 (108) mm/mm2 (p<0.001), without any left-to-right imbalance of the pulmonary artery size. In the subset of 11 patients who improved without needing an additional source of pulmonary blood supply, the stented arterial duct closed uneventfully in 45.5% of cases after 4.0 (2.2) months. Conclusions: Stenting of the arterial duct is a feasible, safe and effective palliation in newborns with duct-dependent pulmonary circulation, supporting the spontaneous improvement process or promoting significant and balanced pulmonary artery growth for subsequent corrective surgery.

Exercise Capacity in Young Patients After Total Repair of Tetralogy of Fallot

Abstract. Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. Measurement of physical activity is usually performed as a routine part of the patients cardiac evaluation. The aim of this study was to examine the exercise performance of young patients operated on for tetralogy of Fallot, assessing the possible influence of known negative prognostic factors related to the surgical repair. The study group comprised 41 consecutive patients (29 male and 12 female, ages 11.2 ± 3.9 years, range 6–16 years) operated on for tetralogy of Fallot. Patients in the study group were divided in subgroups in relation to the age of surgical intervention (before or after 2 years of life), the surgical approach (combined transatrial/transpulmonary approach or right ventriculotomy), and the presence of aortopulmonary shunts prior to performing total correction. Their data were compared with those of 33 aged-matched asymptomatic control subjects (19 male and 14 female, ages 11.9 ± 1.3 years, range 11–16 years). Blood pressure and heart rate measured at rest were similar between control and Fallot groups. A normal increase in systolic blood pressure was observed in response to exercise intensity for all subgroups. No significant difference between control and Fallot groups was found under conditions of mild or moderate exercise or for diastolic blood pressure at rest and in response to exercise. Lower maximal heart rate and systolic blood pressure values were recorded in all patients when compared with the control subjects. Significant differences in peak workload were detected between control and Fallot groups and between the control and each subgroup; however, no difference was found between subgroups. In conclusion, despite their very satisfactory clinical status, all patients showed a reduced peak workload, irrespective of the surgical approach, age at surgery, and aortopulmonary shunts prior to performing total correction.

Congenital heart disease in live-born children: incidence, distribution, and yearly changes in the Campania Region.

Objectives Congenital heart disease (CHD), the most common cardiac malformation in the fetal and neonatal period, represents a heterogeneous group of defects with little known cause. The aim of this study was to investigate the incidence rate (IR) of CHD without spontaneous resolution in the first year of life, the age at diagnosis, the most common type of CHD, and the IR changes in severe and nonsevere CHD during the study period. Methods From January 1997 to December 2002, 4559 live births with CHD (diagnosed until December 2004) were enrolled in the Campania Region. Number of live-born children by month was derived from national registries. Results About 70% of CHD patients were diagnosed within the first year of life. The overall mean regional IR of CHD was 11.1 (confidence interval 10.8–11.4) patients per 1000 live-born children. The overall mean IR of CHD was significantly different in the five provinces of the Campania Region, but the annual IR remained stable in each of them throughout the study period. The relative risk in live-born children and residents in the areas with an elevated IR of CHD was higher for nonsevere CHD, but of low significance for severe CHD. At least one-third of CHD patients had atrial septal defects. Conclusions We described the highest IR of CHD from one of the largest samples used up to now for this purpose (410 181 live-born children), but we think that this value could be higher (poor surveillance in some areas). Moreover, this study increases the awareness that CHD patients with a few symptoms are frequently underdiagnosed in areas with inadequate health services and that by an improved availability of pediatric echocardiographic assesments seem warranted. Eight years of surveillance and an increasing attention of both pediatricians and cardiologists to mild CHD in some provinces allowed us to demonstrate that, despite previous reports, the most common CHD in our region was atrial septal defect.

Transcatheter closure of complex atrial septal defects: feasibility and mid-term results.

Objective Transcatheter closure of atrial septal defects (ASDs) is currently a reliable alternative to surgery, even though challenging in the case of complex septal anatomy. The aim of this study was to evaluate the feasibility and mid-term results of percutaneous closure of complex ASDs in a tertiary referral centre compared with simple ASD closure. Methods Between April 2000 and November 2004, 209 patients were submitted to transcatheter ASD closure; 83 patients (39.7%) presented with a complex defect (large ASDs with a deficient rim or a multifenestrated/aneurysmal septum) and were treated using different devices tailored to the atrial septal anatomy. Results The transcatheter procedure was successful in 72 patients (86.8%), using a single device in 69 patients and two devices in the remaining three patients. Overall, 71 Amplatzer septal occluders, two multifenestrated Amplatzer septal occluders and two Cardioseal/Starflex devices were used. Procedural and fluoroscopy times were 141 ± 45 min and 28 ± 22 min, respectively (P < 0.0001 vs. simple ASD closure for both comparisons). Procedure-related complications were recorded in nine patients (12.5%) (P < 0.01 vs. simple ASD closure). One patient required surgical repair of a femoral arteriovenous fistula and another developed mitral valve dysfunction. Immediate ASD occlusion was recorded in 59.7% of patients, reaching 95.9% at the last follow-up control (P = NS vs. simple ASD closure for both comparisons). Conclusions Percutaneous closure of complex ASDs may be considered technically feasible, relatively safe and highly effective, although the procedure is still significantly more demanding than transcatheter closure of simple ASDs.

Ten‐years, single‐center experience with arterial duct stenting in duct‐dependent pulmonary circulation: Early results, learning‐curve changes, and mid‐term outcome

To evaluate early results, learning‐curve changes, and mid‐term outcome of arterial duct (AD) stenting in congenital heart disease with duct‐dependent pulmonary circulation (CHD‐DPC) in a high‐volume, tertiary referral center.

Transcatheter ductal stenting in critical neonatal Ebsteinʼs anomaly

Background Symptomatic neonatal Ebsteins anomaly shows a very poor outcome, most frequently due to duct-dependent pulmonary circulation caused by functional pulmonary valve atresia. In this setting, percutaneous ductal stenting using high-flexibility coronary stents might be a highly cost-effective alternative to systemic-to-pulmonary shunt. Methods Three newborns (age 4.7 ± 2.9 days; weight 3.0 ± 0.3 kg) with critical, duct-dependent tricuspid valve Ebsteins anomaly and functional pulmonary atresia unresponsive to prostaglandin infusion and multidrug vasodilator therapy, underwent duct stabilization with high-flexibility, open-cell stents as an alternative to surgical palliation. Their echocardiographic picture predicted a very high mortality rate. In two cases, the ductus arteriosus was recanalized using a combined pharmacological (local prostaglandin infusion) and mechanical (hydrophilic coronary guide-wire manipulation) approach. Results Stent implantation increased duct diameter from 0.5 ± 0.7 to 3.2 ± 0.2 mm (P < 0.0001), resulting in a significant rise in oxygen saturation from 67 ± 9 to 92 ± 4% (P < 0.00001). After the procedure, the patients were quickly weaned from mechanical ventilation and discharged after 16 ± 7 days. Uneventful spontaneous ductal closure ensued in two patients in a few months, and percutaneous oxygen saturation was 87 ± 3% at the last follow-up session (12 ± 10 months). Conclusions In critical newborns with severe, duct-dependent Ebsteins anomaly unresponsive to medical therapy, ductal stenting could be a reliable, safe and cost-effective alternative to either long-term prostaglandin treatment or surgical palliation, in view of lower-risk corrective surgery at older age. This option may also be attempted successfully within a few days of spontaneous ductal closure using a combined pharmacological and mechanical approach.

Short-term electrogeometric atrial remodelling after percutaneous atrial septal defect closure.

Background Atrial arrhythmias are well known long-term complications of atrial septal defect (ASD), presumably due to chronic atrial enlargement and stretch. Surgical repair often fails to revert the arrhythmic risk despite atrial volumetric unloading, maybe as a consequence of atriotomic scar. Avoiding atrial incision, percutaneous ASD closure should result in atrial unloading and arrhythmic risk decrease. This study evaluated the atrial short-term electrogeometric effects of percutaneous ASD closure. Methods Fifteen asymptomatic patients (age 23 ± 5 years) submitted to percutaneous closure of large ASD (QP/QS 2.4 ± 0.3) using the Amplatzer Septal Occluder device (24 ± 2 mm) underwent atrial echocardiographic (indexed right atrial size and left atrial size as well as right atrial/left atrial volume ratio) and electrocardiographic (P-wave duration and dispersion, PR conduction time and dispersion) evaluation at 1 and 6 months after ASD closure. Results After 6 months, the indexed right atrial volume and the right atrial/left atrial volume ratio had significantly decreased (from 39 ± 5 to 20 ± 2 ml/m2, P < 0.001 and from 2.0 ± 0.2 to 1.0 ± 0.1, P < 0.0001, respectively) as did the P-wave dispersion (from 32 ± 2 to 28 ± 1 ms, P = 0.03) despite a significant increase of both P-wave maximal (from 75 ± 3 to 81 ± 3 ms, P < 0.01) and minimal (from 42 ± 3 to 53 ± 3 ms, P < 0.005) duration. Conclusion Percutaneous ASD closure causes a short-term positive atrial electrogeometric remodelling. The electrocardiographic predictors of atrial arrhythmias, however, tend to worsen early after device implantation despite a marked volumetric unloading, possibly due to a ‘foreign body’ effect of the occluding device. This might warrant a closer follow-up during the first few weeks after device implantation.

Arterial duct stenting: Do we still need surgical shunt in congenital heart malformations with duct-dependent pulmonary circulation?

Background Despite current trends toward primary repair, surgical systemic-to-pulmonary shunt is still an invaluable palliative option in some patients with congenital heart defects and duct-dependent pulmonary circulation. However, arterial duct stabilization with a high-flexibility coronary stent could be an effective alternative in high-risk surgical candidates or whenever short-term pulmonary blood flow support is anticipated. Methods and results On the basis of ductal origin and morphology, the stenting procedure can be perfomed from an arterial or venous route. Following arterial duct angiographic imaging, the stabilizing stent is chosen to completely cover the entire ductal length and dilated slightly less than the proposed surgical shunt. Procedural failure depends mainly on ductal tortuosity and ranges around 10% of cases. Morbidity and mortality are 8–11% and less than 1%, respectively. Mid-term fate of the stented duct is spontaneous, slow and progressive closure within a few months. Compared with a Blalock–Taussig shunt, stented ducts result in similar but more uniform pulmonary artery growth over a mid-term follow-up. Conclusion Arterial duct stenting is a technically feasible, well tolerated and effective palliation in congenital heart disease with duct-dependent pulmonary circulation. It is advisable either in high-risk neonates or whenever a short-term pulmonary blood flow support is anticipated. The stented duct appears less durable than a conventional surgical shunt although it is highly effective in promoting a global and uniform pulmonary artery growth.

Transcatheter treatment of Starr-Edwards paravalvular leaks.

: A 56-year-old patient was referred because of refractory heart failure and mild haemolysis caused by multiple, severe paravalvular leaks of a Starr-Edwards valve implanted in mitral position 23 years before. Owing to perceived high risk of surgical re-valving, percutaneous paravalvular leak occlusion was performed by implantation of multiple, simultaneously deployed Amplatzer Vascular Plugs.

Cardioembolic stroke: who is the guilty?

A young woman was referred following a transient ischemic attack due to suspect patent foramen ovale. At the diagnostic workup a spontaneous mild right-to-left atrial shunt owing to fenestrated aneurysmal septum was found. However, also a large arteriovenous malformation of the left lung was also imaged. At interventional cardiac catheterization, both malformations were closed using an Amplatzer Cribriform occluding device and a Amplatzer Vascular Plug II, respectively. In conclusion, patent foramen ovale is just one of the potential causes of cardioembolic stroke and a thorough diagnostic workup is mandatory after a cryptogenic stroke to rule out any additional source of paradoxical embolism.