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Percutaneous patent foramen ovale (PFO) closure by traditional, double disc occluder devices was shown to be safe for patients with PFO, and more effective than prolonged medical therapy in preventing recurrent thromboembolic events. The novel suture-mediated “deviceless” PFO closure system overcomes most of the risks and limitations associated with the traditional PFO occluders, appearing to be feasible in most interatrial septum anatomies, even if data about its long-term effectiveness and safety are still lacking. The aim of the present review was to provide to the reader the state of the art about the traditional and newer techniques of PFO closure, focusing both on the procedural aspects and on the pivotal role of transesophageal echocardiography (TEE) in patient’s selection, peri-procedural guidance, and post-interventional follow-up.

The levoatriocardinal vein is a rare vascular anomalous connection between the left atrium and the superior vena cava (or left innominate vein). This defect is usually associated with left heart obstructive lesions, while it is rarely found in an isolated form. In the former case, the anomalous connection causes a pre-tricuspid left-to-right shunt with right-heart volume overload. We describe the first case of “double” homolateral levoatriocardinal vein in a child with signs and symptoms of right-heart failure and pulmonary blood-flow overload. A trans-catheter closure of both vascular connections was performed with two Amplatzer Vascular Plug type II (Abbott, Plymouth, MN, USA). The percutaneous approach proved to be safe and effective, with early improvement in the signs and symptoms of heart failure.

In the recent years, the pregnancy trend among women with Congenital Heart Disease (CHD) has increased; this has leaded to a growing demand for specialized care both in mother and in children. Although pregnancy is often well tolerated, maternal CHD may affect in some cases a maladaptive hemodynamic response carrying additional risks of cardiovascular events like arrhythmias, heart failure and, in rare cases, death. The impaired utero-placental perfusion due to maternal cardiac status may result in placental dysfunction, which may be associated with fetal growth restriction, preeclampsia, premature birth and perinatal morbidity. Systemic Right Ventricle (SRV) is one of the main conditions under which pregnancy is challenging. The sub-aortic position of morphological Right Ventricle (RV) is "physiologically" predisposed to fail at the adult age and may be potentially inadequate to support the hemodynamic stress of the pregnancy. Current literature about pregnancy in women with SRV consists of small retrospective series not providing conclusive evidence about the feasibility of a successful pregnancy outcomes. In addition, the long-term effects of pregnancy on SVR are not still adequately investigated and it remains unclear if maternal complications reported are due to pregnancy or to natural history of SVR. The aim of this paper is to offer a critical review of the knowledges at regard and to provide a practice update on the risk assessment and the pregnancy management in women with SRV in order to support the decision making and to optimize outcomes in these patients.

Background To date, extensive experience in transcatheter closure of fenestrated atrial septal aneurysm (ASA) in the pediatric population is limited. Methods To report on procedural feasibility, efficacy, and long-term outcome, we enrolled all children submitted to an attempt of transcatheter closure of fenestrated ASA at two, large volume, pediatric cardiology units (Naples and Massa, Italy) between April 2000 to May 2020. Results This retrospective study included 139 patients (median age 9 years [range 2-18] and weight 36 kg [range 10-102]); 19 (13.7%) children were = 20 mm. The procedural success rate was 99% (95% confidence interval [CI]: 94.9-99.8) using a single device in 75 (69%), two devices in 31 (28%), and three devices in 3 (3%) cases. Early minor adverse events (AEs) occurred in four patients (2.8%). Late minor AEs were recorded in one patient (0.7%) over a median follow-up of 5 years ([range 0-18 years; total 890.2 person-years, and with 30 patients (22%) followed >= 10 years). Neither mortality nor major AEs were recorded. Freedom from AEs was 99.1% at 10-15 years (95% CI: 93.5-99.8%), without any difference according to atrial septum anatomy or patient age and weight. Conclusion Transcatheter closure of fenestrated ASA is technically feasible and effective in children with excellent long-term outcomes.

AIM To report on the feasibility, efficacy and long-term outcome of transcatheter closure of fenestrated Atrial Septal Aneurysm (ASA). METHODS Between April 2000 and May 2020, 568 patients with fenestrated ASA underwent transcatheter closure at two large-volume Pediatric Cardiology and GUCH Units (Naples and Massa, Italy). Procedural indications were cardiac volume overload in 223 patients (39%) or prophylaxis of paradoxical embolization in 345 patients (61%). Patients' median age and weight were 42 years (range 19-52) and 66 kg (range 54-79), respectively. One-hundred and thirty-nine patients (25%) were younger than 18 years. RESULTS Single fenestration was found in 311 patients (55%) (Group 1), whereas 257 patients (45%) had multifenestrated ASA (Group 2). The procedure was successful in all but seven patients (1.2%). In Group 1, closure was achieved with a single device. In Group 2, one device was used in 168 patients (67%), two devices in 74 patients (28%), three devices in 12 patients (5%), and four devices in 1 patient (0.3%). The early complication rate was 3%, without any difference according to anatomy or patient's age. At discharge, complete shunt closure was higher in Group 1 (92% vs. 72%, P

Background: Patent foramen ovale (PFO) closure after a cryptogenic cerebral ischemic event is a routinely procedure. The most used device is Amplatzer™ PFO Occluder 25 mm, but PFOs with complex anatomy require larger device for closure. We compared Amplatzer™ Septal Occluder (ASO) device versus Amplatzer™ PFO Occluder 30 or 35 mm (A-PFO 30/35) about the safety of procedure and the presence of residual shunt during the follow-up. Methods: From June 2002 to July 2016, 355 patients (pts) with PFO undergone closure at our institution. Among these ones, 70 pts (19.7%) had a PFO with complex anatomy and a single device with greater diameter was implanted. In these cases, the following devices were used: Gore® Septal Occluder (GSO) in 4 pts; ASO device in 33 pts (group I) and A-PFO 30/35 in 33 pts (group II). Patients treated with GSO device were excluded by our analysis. Results: Comparing group I and group II, there weren't complications during the procedures. Two patients of group II were lost at follow-up. At last follow-up, 1 pt of group I (3%) and 10 pts of group II (32.3%) had a residual shunt (p < 0.01). 7 of 10 pts of group II and the only 1 of group I with residual shunt underwent a complete closure by Amplatzer™ Vascular Plug (AVP) devices. Conclusions: ASO devices and A-PFO 30/35 devices are both safe to close complex PFO; but A-PFO 30/35 is associated with a more incidence of residual shunt.

IntroductionCareful, stepwise assessment is required in all patients with atrial septal defect (ASD) to exclude pulmonary vascular or left ventricular (LV) disease. Fluid challenge and balloon occlusion may unmask LV disease and post-capillary pulmonary hypertension, but their role in the evaluation of patients with ‘operable’ ASDs is not well established.MethodsWe conducted a prospective study in three Italian specialist centres between 2018 and 2020. Patients selected for percutaneous ASD closure underwent assessment at baseline and after fluid challenge, balloon occlusion and both.ResultsFifty patients (46 (38.2, 57.8) years, 72% female) were included. All had a shunt fraction >1.5, pulmonary vascular resistance (PVR) ConclusionsIn adults with ASD without overt LV disease, even small rises in PVR may have significant implications on cardiovascular haemodynamics. Fluid challenge may provide additional information to balloon occlusion in this setting.

Background A fluid challenge test (FCT) with a rapid infusion of saline allows for discrimination between pre- and post-capillary pulmonary hypertension (PH) and may unmask hidden post-capillary PH. Patients with atrial septal defect (ASD) may develop pre- or post-capillary PH after shunt closure respectively in case of pulmonary vascular disease or left ventricular disease. Aim To evaluate the haemodynamic changes of the pulmonary circulation in ASD patients undergoing percutaneous closure with indicated according to the current ESC guidelines. Methods Twenty-three patients (mean age 42.9±12.4 years; 15 female) underwent right heart catheterization in basal conditions and after FCT (volume loading with rapid saline infusion of 7 ml/kg in 10 min) before percutaneous closure of the ASD. Right atrial pressure (RAP), systolic, mean and diastolic pulmonary arterial pressure (sPAP, mPAP and dPAP), pulmonary arterial wedge pressure (PAWP), cardiac output (CO), pulmonary vascular resistance (PVR), systemic vascular resistance (SVR) and the ratio between pulmonary and systemic flow (QP/QS) were calculated four times: before and after inflating the sizing balloon both at baseline and immediately after FCT (Fig. 1). Results The patients had an increase in pressures and flows after FCT with open ASD: mPAP (18.7±4.4 vs 16.7±4.6 mmHg, p During a temporary ASD closure by sizing balloon, the patients had increases of RAP (9.0±2.6 vs 7.6±2.6 mmHg, p Conclusions None of the reported ASD patients presented with FCT criteria of post-capillary PH (that is a PAWP >18 mmHg). The FCT was associated with an increase in QP/QS suggesting that the patients still had a distensible pulmonary circulation. Further studies are needed to explore the relevance of a FCT in ASD patients, particularly those with higher PVR values. Figure 1 Funding Acknowledgement Type of funding source: None

Background: Despite recent technical advances, interventional cardiac catheterization is still challenging in neonatal age and no specific data concerning early outcome are so far published in literature. Methods: Neonatal trans-catheter cardiac interventions performed in high-volume Italian referral centers were retrospectively analyzed. Primary outcomes were procedural major adverse events, in-hospital mortality and procedural failure. Secondary outcomes were minor adverse events and need for blood transfusion. Results: From January 2000 to December 2017, 1423 newborns (mean weight 3.0 ± 0.6 kg, range 1.0–5.8; median age 2.0 days) underwent interventional cardiac catheterization. Overall, global procedure adverse event rate and in-hospital mortality were 10.2% and 5.2%, respectively. At multi-variable analysis, primary composite outcome was significantly related to low-weight (

Percutaneous stenting of the pulmonary artery is a diffuse procedure, above all in patients with congenital heart disease. The effectiveness of the procedure is associated with potential and feared complications. This case describes the embolization of a premounted stent into the left lower lobe pulmonary artery in a 3-years old patient with univentricular heart and left-side superior vena cava undergone to Norwood procedure modified according to Sano (stage I) and then bilateral cavo-pulmonary anastomosis according to Glenn (stage II). The retrieval procedures were complicated by the severe hypoplasia of the left pulmonary artery and by the complex anatomy of the patient. This case shows how to pull-back an embolized stent in a child with a complex congenital heart disease despite the limitations related to the low weight and the complex vascular anatomy.

Objectives This study aimed to report a large, single-center experience of percutaneous arterial duct (AD) closure using Amplatzer Duct Occluder II Additional Sizes device (ADO II-AS)(St. Jude Medical Corp, St. Paul, MN, USA). Background Transcatheter closure of AD remains challenging in low body weight patients and those who have a persisting shunt following a previous attempt at interventional closure. Recent technical advances in device design may address these issues. Methods From May 2011 to April 2016, 109 patients underwent attempted percutaneous closure of AD with ADO II-AS at our Institution. Mean age and weight were 4.8 ± 8.1 years (range 0-48) and 21.4 ± 20.6 kg (range 3-93), respectively. Fifteen patients (13.8%) were ≤6 kg (age 3.5 ± 2.0 months; weight 4.7 ± 1.1 kg). Arterial duct morphology was type A in 62 (57%), type B in 1 (1%), type C in 32 (29%), type D in 7 (6%) and type E in 6 patients (6%), respectively. Arterial approach was used to negotiate and deploy the occluding device in 103 patients (94.5%). Results AD diameter was 2.2 ± 0.6 (range 1.5–4.5) resulting in QP/QS of 1.9 ± 0.7 (range 1-3.3). Mean pulmonary artery pressure and PA/aortic pressure ratio were 19.3 ± 5.0 mm Hg (range 12-38) and 0.34 ± 0.14 (range 0.14-0.95), respectively. Successful device deployment was achieved in 107 patients (98.2%). Neither procedural morbidity nor mortality was recorded. Immediate, 24h and mid-term (30 ± 17 months) complete occlusion was recorded in 71%, 98.1%, and 100% of patients, respectively. Conclusion In our experience, trans-catheter closure of AD of different sizes and morphologies using ADO II-AS is highly feasible, safe and effective also in challenging anatomic/clinical settings. © 2016 Wiley Periodicals, Inc.

Objective Significant and balanced pulmonary artery (PA) growth following arterial duct (AD) stenting has already been reported in literature. However, no data are so far available about the role of this percutaneous approach in promoting PA growth in the case of congenital heart disease (CHD) with completely ductdependent pulmonary blood flow (CDD-PBF). Aim of this study was to evaluate the effect of AD stenting in this pathophysiological setting. Methods PA growth was evaluated as Nakata index and McGoon ratio as well as individual PA z-score changes in 49 patients submitted to neonatal AD stenting according to their pathophysiology (CDD-PBF (n=15) versus multiple PBF sources (n=34)). Results Control angiography was performed 7.2 ±6.4 months (range 1-8, median 6) after AD stenting. In the whole population, significant and balanced PA growth was recorded (Nakata index+122±117%; left pulmonary artery (LPA) z-score +84±52%; right pulmonary artery (RPA) z-score +92±53% versus preprocedure, p

Background: Elderly patients (≥60 years) with an atrial septal defect (ASD) are a complex subgroup with several haemodynamic limitations. The aim of this study is to describe the clinical and haemodynamic features of these patients and the effectiveness of percutaneous ASD closure in this setting. Methods: This is an observational retrospective study. From 2000 to 2018, sixty-eight elderly patients (65.1 ± 3.8 years, range 60–75) underwent ASD assessment at our institution. Right heart catheterization was always performed before closing the ASD. At last follow-up, clinical and echocardiographic data were recorded. Results: Among these 68 elderly patients, in 62 cases (91.2%) with a favourable haemodynamic profile, ASD closure was performed immediately. In 2 patients the ASD was closed after 3 months of medical therapy. A fenestrated device was used in 1 case. The remaining 3 patients with pulmonary arterial hypertension (PAH) and high pulmonary vascular resistance (>4.6 WU) were treated with PAH specific therapy. At a mean follow-up of 4.3 ± 1.5 years, a significant improvement of NYHA class (pre-interventional NYHA III-IV 21.5% vs. post-interventional NYHA III-IV 4.6%; p

Transcatheter closure of arterial duct (AD) remains challenging in low-weight patients and using Amplatzer Duct Occluder devices is still considered off-label in infants < 6kg. This study aimed to report a large, single-center experience of percutaneous AD closure in infants < 6kg as well as to compare the most frequently used devices, Amplatzer Duct Occluder type I (ADO I) and Amplatzer Duct Occluder type II Additional Sizes devices (ADO II-AS) (St. Jude Medical Corp, St. Paul, MN, USA). From March 2000 to March 2017, among the 762 patients submitted to percutaneous closure of AD at our Institution, 33 were infants < 6kg (age 4.8 ± 2.1months; weight 5.0 ± 0.9kg). Fourteen patients (45%) underwent ADO I (Group I) and 19 patients ADO II-AS (Group II) device implantation. AD diameter was 2.6 ± 0.8 (range 1.5–4.0) mm resulting in QP/QS of 2.6 ± 0.0.9 (range 1.1–4.5). Successful device deployment was achieved in all patients without procedural morbidity or mortality. Procedural and fluoroscopy times were not significantly different between the groups. However, total X-ray absorbed dose was significantly higher in Group I (121 ± 69 vs 29 ± 16mGy/cm2, p < 0.01). Immediate, 24h, and mid-term (46 ± 37 months, median 39) complete occlusion were recorded in 72.7, 90.9, and 97% of patients, respectively without significant difference between the groups. In conclusion, transcatheter closure of AD with Amplatzer Duct Occluder devices is feasible, safe, and effective also in infants less than 6kg, without significant difference between the most commonly used devices, namely, ADO I and ADO II-AS.

Objectives 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. Background In spite of wide acceptance as cost-effective alternative to surgical palliation, AD stenting indications, results and mid-term outcome still largely depend on small series, experiences in particular subsets of patients or multicenter series with different approaches and interventional philosophy. Methods Between April 2003 and December 2013, 119 patients underwent AD stenting as lower-risk palliation of CHD-DPC at our Institution. Procedural and mid-term follow-up data of these patients are reported. Results The procedure was successfully completed in 93.3% of cases, with a complication rate and in-hospital mortality of 17.6% and 3.6%, respectively. No patient underwent rescue surgical shunt but elective Blalock-Taussig shunt was needed in 15 patients (13.5%). Over time, favorable trends toward higher feasibility and efficacy in complex ductal anatomy as well as lower procedural risk were recorded. Presurgical cardiac catheterization (n = 36) showed significant and balanced pulmonary artery (PA) growth (Nakata Index +113 ± 101%; left PA z-score +87 ± 52%; right PA z-score +97 ± 53%, P

Post-myocardial infarction (MI) ventricular septal defects (VSD) are a rare but life-threatening complication of acute MI, with very high mortality rates even if timely approached by surgical repair. Transcatheter closure is an attractive alternative to surgery. However, this option is currently deemed challenging and often unsuitable in complex VSD. We report the case of a young woman in poor hemodynamic conditions due to a complex post-MI VSD. A two-step percutaneous VSD closure was successfully performed adopting a patient-tailored approach based on a throughout knowledge of the anatomic and functional picture using both commercially available dedicated and off-label devices.

Significant and balanced PA growth following arterial duct (AD) stenting has already been consistently reported in literature. However, to date, no data are available about the role of this approach as palliation of congenital heart disease with a duct-dependent discontinuous pulmonary artery (dPA). The aim of this study was to evaluate the fate of a dPA of ductal origin following trans-catheter AD stabilization. Angiographic PA evaluation was performed in seven patients submitted to neonatal AD stenting as palliative recruitment of dPA. Five patients showed discontinuity of one PA, while two patients had both PAs served by bilateral ducts. PA growth was evaluated as per the Nakata index, McGoon ratio as well as dPA (n=9) versus heart-dependent PA (hPA; n=5) size and z-score changes. AD stabilization was performed using coronary stents dilated to 3.2±0.3mm (median 3.4), with significant increase of O2 saturation (from 83±11 to 95±5%, p&nbsp

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.

Unroofed coronary sinus (coronary sinus-type atrial septal defect) is a rare congenital anomaly resulting from imperfect development of left atrio-venous fold. This malformation is not considered suitable for percutaneous treatment due to complex anatomy and risk of coronary sinus obstruction. This article reports on the first successful treatment of partially unroofed coronary sinus by implantation of an Amplatzer(®) Septal Occluder device (AGA Medical Co., Golden Valley, MN). In this patient, the malformation was deemed favorable for device closure due to the local anatomy and the association with persistent left superior vena cava draining in coronary sinus.

ObjectivesThis study sought to evaluate the impact of arterial duct (AD) stenting in promoting catch-up growth of hypoplastic pulmonary artery (PA) tree in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC).BackgroundSignificant and balanced PA growth following AD stenting has already been consistently reported in the literature. However, no data are so far available about the role of this approach in severe PA hypoplasia, which significantly impacts the risk of surgical repair.MethodsPre-surgical angiographic PA evaluation was performed in 45 patients with confluent PAs submitted to neonatal AD stenting as palliation of CHD-DPC. PA growth was evaluated as Nakata Index and McGoon ratio as well as individual PA z-score changes, both in the whole population and according to the original vessel size (Nakata Index 100 mm2/m2, Group II [n = 30]).ResultsControl angiography was performed 7.5 ± 6.5 months (median 6 months) after duct stenting, showing significant and balanced PA growth. The Nakata Index increased from 143 ± 73 mm2/m2 to 270 ± 88 mm2/m2 (124 ± 118%, p < 0.0001); left PA z-score from −0.7 ± 1.7 to 1.0 ± 1.4; right PA z-score from −0.6 ± 1.3 to 1.2 ± 1.3 (p < 0.0001 for both comparisons). Group I showed a greater increase of global PA growth (Nakata Index increase 227 ± 141% vs. 72 ± 57%, p < 0.001) as compared with Group II. Final PA size did not significantly differ between the groups (246 ± 105 mm2/m2 vs. 282 ± 78 mm2/m2, p = NS).ConclusionsPercutaneous AD stenting is highly effective in promoting a significant and balanced catch-up growth of diminutive PAs, being therefore advisable in this subset of patients as a reliable alternative to surgical palliation.

An 8-year-old asymptomatic child was referred for surgical repair of coronary sinus atrial septal defect resulting in significant left-to-right shunt and right chamber volume overload. The septal fenestration was located near to its drainage site into the right atrium. Due to this seemingly favourable anatomy, transcatheter closure of the septal defect was performed using an Amplatzer Septal Occluder device. The echocardiographic postprocedural evaluation imaged the occluding device almost perpendicular to the atrial septum, seemingly floating above the mitral valve orifice, like an alien spaceship inside the heart.

Transcatheter closure is nowadays considered as the first-choice treatment of atrial septal defect (ASD). However, indication, technique, and results of this approach are still challenging and under debate in particular settings, as in elderly or in patients with pulmonary atresia with intact ventricular septum (PA-IVS) submitted to right ventricular decompression.

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 tortuosity syndrome is a connective tissue disorder characterized by elongation and tortuosity of large elastic arteries, potentially resulting in multiple vascular stenoses. This paper reports a novel hybrid strategy to treat 'complex' bilateral peripheral pulmonary artery stenoses consisting of transcatheter stent implantation via midline sternotomy and reductive angioplasty of the proximal pulmonary arteries.

A critical 1-day-old male neonate was referred to cardiac evaluation because of deep cyanosis due to a severe tricuspid valve Ebstein's anomaly with large atrial right-to-left shunt and duct-dependent pulmonary circulation. Ductus arteriosus re-opening by prostaglandin infusion resulted in significant clinical improvement but, after a few hours, it irreversibly closed, and pulmonary vasodilator treatment with inhaled nitric oxide and oral sildenafil did not significantly increase the oxygen saturation. Therefore, it was decided to proceed to ductal recanalization and stenting as an alternative to the surgical shunt. After the procedure, oxygen saturation was raised to over 90%, allowing the baby to be weaned from mechanical ventilation. At 9-month follow-up, he was asymptomatic and showed a systemic saturation over 90% despite complete closure of the stented ductus. In conclusion, ductus arteriosus stenting might be considered to be a reliable and life-saving therapeutic option in severe forms of Ebstein's anomaly as a temporary support to a multidrug vasoactive therapy.

Double right aortic arch with mirror-image branching is a very uncommon vascular anomaly as a cause of symptomatic dysphagia in neonatal age [1,2]. It results from persistence of the right fourth embryonic aortic arch and involution of the left fourth arch between the subclavian artery and the dorsal descending aorta. When the ductus arteriosus or its “remnant” (ligamentum arteriosus) originates from the descending aorta, a complete vascular ring is formed. A 10 day-old neonate underwent cardiac evaluation due to the suspicion of vascular ring causing swallowing difficulty and habitual regurgitation. Clinical examination and electrocardiography did not show any significant finding. Echocardiography imaged just a right aortic arch with mirror-image branching without any intra-cardiac abnormality. However, the unusual location of a small patent ductus arteriosus, arising very distal from the midthoracic aorta, made us to suspect a vascular compression of the oesophagus. Simultaneous oesophagography and aortic angiography confirmed the oesophageal compression due to the Kommerell's diverticulum that gave rise to a left-sided ductus (Figs. 1 and 2). Surgical exploration found that the

Background Pulmonary arterial hypertension (PAH) after shunt closure is associated with a poor prognosis. The aim of this study was to assess retrospectively the hemodynamics of patients developing PAH after shunt closure. Methods Hemodynamic data obtained by right heart catheterization (RHC) performed at baseline and after shunt closure were analyzed. Results Twenty-two patients, 13 with atrial septal defect (ASD), 6 with ventricular septal defect (VSD), 1 with patent ductus arteriosus, 1 with both ASD and VSD, and 1 with complete atrio-ventricular canal have been considered. The mean age at closure was 25.3±20.1years (range of 3months to 56.7years), and the mean age at PAH diagnosis was 37.0±20.8years (range of 5 to 61.2years). The time delay between shunt closure and PAH diagnosis was 140.2±100.2months. At baseline RHC, hemodynamic data were as follows: pulmonary vascular resistance (PVR) of 8.6±2.6Woodunits, PVR index (PVRi) of 10.1±2.7Woodunits∗m 2 , mean pulmonary arterial pressure of 43.7±9.7mmHg, PVR to systemic vascular resistance ratio (PVR/SVR) of 0.70±0.23, and Qp/Qs of 1.6±0.4. In particular, 18/22 (81%) had PVR≥5Woodunits, 21/22 (95%) PVRi≥6Woodunits∗m 2 , 21/22 (95%) PVR/SVR≥0.33, and 11/22 (50%) Qp/Qs≤1.5. During the follow-up, 5/22 (22%) patients died and one patient underwent successful double lung transplantation. Conclusions High baseline values of PVR (≥5Woodunits), PVRi (≥6Woodunits∗m 2 ) and PVR/SVR (≥0.33) are common findings in patients who develop PAH late after shunt closure. Large prospective clinical trials are needed to establish the safe limits for shunt closure.

Unroofed coronary sinus (coronary sinus-type atrial septal defect) is a rare congenital anomaly resulting from imperfect development of left atrio-venous fold. This malformation is not considered suitable for percutaneous treatment due to complex anatomy and risk of coronary sinus obstruction. This article reports on the first successful treatment of partially unroofed coronary sinus by implantation of an Amplatzer(®) Septal Occluder device (AGA Medical Co., Golden Valley, MN). In this patient, the malformation was deemed favorable for device closure due to the local anatomy and the association with persistent left superior vena cava draining in coronary sinus.

Objectives: To evaluate feasibility and results of arterial duct (AD) stenting in low-weight newborns with congenital heart disease and duct-dependent pulmonary circulation (CHD-DPC). Background: AD stenting is nowadays considered a cost-effective alternative to surgical shunt in CHD-DPC. This option might be even more advisable in low-weight neonates (

A 37-year-old woman, nonsmoker, with a history of chronic migraines with aura, was referred to our emergency room due to dysarthria and left faciobrachial paresis. Her family history was unremarkable. Physical examination was normal except for a mild oxygen desaturation (88% on room air). Electrocardiogram, echocardiography, peripheral venous and arterial Doppler ultrasound, and brain magnetic resonance imaging were normal. However, transcranial Doppler analysis showed a significant (shower pattern) right-to-left shunt late after isotonic saline. This pattern was present at rest and did not significantly change after Valsalva maneuver. At chest radiograph, a lobular smoothmargined opacity in the right lower lobe made us suspect the diagnosis of pulmonary arteriovenous malformation (PAVM). Chest computed tomography (CT) showed a large complex PAVM fed by two large and tortuous descending pulmonary arteries and draining through a straight vessel directed posterosuperiorly to the right lower pulmonary vein (Fig. 1). Thus, cardiac catheterization was planned to occlude the vascular malformation. The patient’s informed consent to the procedure and agreement with the thoracic surgeons were obtained. The procedure was performed with local anesthesia through the femoral vein entry. Pulmonary angiography confirmed the presence of multiple large PAVMs into the right lower lobe fed by a single 1.1-cm-large vessel originating from the right inferior pulmonary artery (Fig. 2a). Transcatheter embolization of the feeding vessel was achieved with a 14-mm-large Amplatzer Vascular Plug type II (AGA Medical, Golden Valley, Minnesota, USA) device (Fig. 2b) and resulted in a sudden increase in the oxygen saturation, from 88 to 96% on room air. Clinical status and

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 performed 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.

A critical infant with unrestrictive ventricular septal defect and absence of the left pulmonary artery underwent transcatheter re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. After this hybrid approach, the baby was discharged under mild anticongestive therapy in view of a later surgical repair.

A previously healthy 12-year-old boy was admitted to the hospital with acute pneumonitis. On admission, a heart murmur was detected. The transthoracic echocardiogram showed multiple secundum atrial septal defects with left-to-right shunt and signs of right ventricular volume overload. He underwent right cardiac catheterization and transesophageal echocardiogram (transesophageal echocardiography): 3 sizeable atrial septal defects (anterosuperior, measuring 10 mm; posterior, 9 mm; anteroinferior, 6 mm) were closed with …

Objectives: To evaluate the possibility to redilate covered Cheatham-Platinum stents during follow-up, in particular in growing children with aortic coarctation. Background: There are no data in the literature about the redilation of ePTFE covered CP stents. Methods: Sixty covered CP stents were implanted in patients with aortic coarctation or recoarctation between January 2004 and October 2007. Seven patients (mean age 14.2 ± 3.7 years) needed to repeat the hemodynamic study due to somatic growth and increase of aortic gradient with the occurrence of systemic hypertension. Two had near-atretic aortic coarctation, three had postsurgical recoarctation and aneurysm formation, one had native aortic coarctation associated with aneurysm of the arterial wall, and one had severe native aortic coarctation. Results: Procedures were performed a mean of 20 ± 5 months (range, 12–24 months) after the primary stent implantation. Fluoroscopy time ranged between 7 and 15 min (median, 10 min) whereas procedure time ranged between 60 and 75 min (median, 65 min). After redilation the gradient across the stenosis decreased from a median value of 35 mm Hg to a median value of 5 mm Hg. The stent diameter increased of 20–50% the predilation value. No complications occurred and angiographic controls showed that the stenoses have been relieved. Follow-up: During a median follow-up of 12 months (6–30 months) the results were stable without complications. Conclusion: Covered Cheatham-Platinum stents can be easily redilated © 2008 Wiley-Liss, Inc.

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