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The role of preoperative locoregional therapy (LRT) for hepatocellular carcinoma (HCC) before liver transplantation (LT) remains unclear. Moreover, LRT in the setting of living donor liver transplantation (LDLT) merits further exploration. The objective of the current study was to determine risk factors for poor outcomes after LDLT in patients who received locoregional therapy (LRT). We reviewed patients (n = 46) who underwent LDLT after LRT. Multivariate analysis was performed to determine independent predictors of recurrence-free survival (RFS). Risk scores were developed to define prognostic groups. Median tumor size was 3.7 (1.2–12) cm and tumor number was 1 (1–6). Macrovascular invasion was seen in 10/46 (21.7%) patients. There was a significant difference in 5-year RFS with > 3 tumor nodules (P = 0.005), tumors outside University of California San Francisco criteria (P = 0.03), bilobar disease (P = 0.002), AFP > 600 ng/ml (P = 0.006), and poor response to LRT (P = 0.007). On multivariate analysis, bilobar disease (HR = 2.9, P = 0.01), AFP > 600 ng/ml (HR = 2.3 P = 0.008), and poor response to LRT (HR = 2, P = 0.02) were predictors of 5-year RFS. The 5-year RFS in low risk (score = 0), intermediate risk (score = 1–3), and high risk (score = 4–7) groups was 86%, 76%, and 9% (P

Graphical abstract, Highlights • TAVR has become a prevalent procedure. • PVL is a complication of TAVR with increased risk for mortality. • TEE, TTE, and angiography may misrepresent TAVR eccentric regurgitation for PVL. • ICE may be used to distinguish TAVR regurgitation.

Fenestrated grafts form an alternative to open surgery, and are already increasingly being offered as part of the standard spectrum of treatment in developed countries. The past two decades have seen rapid developments in fenestrated endovascular aneurysm repair (fEVAR)/branched (bEVAR) expertise, to the point where the results are striking and can compete with those of conventional surgery. We here report the case of an 82-year male, who was diagnosed with an abdominal aortic aneurysm 10 years ago. Computed tomography showed a large fusiform juxta-renal abdominal aortic aneurysm extending into bilateral common iliac arteries to their bifurcation. Bilateral femoral access was acquired surgically. Fenestrated part of the stent grafted was deployed successfully. Right and left renal arteries and superior mesenteric artery were stented. Celiac artery showed good flow and no stenting was required. Distal stent graft and bilateral iliac grafts were deployed successfully. Follow up computed tomography scan showed 99% patency and no endoleak. Patient remains stable and asymptomatic at 6-month follow-up. To the best of our knowledge, it is the first reported case of successful fEVAR from Pakistan. Key Words: Fenestrated grafts, Abdominal aortic aneurysm, Endovascular.

Graphical abstract, Highlights • Hemodynamic instability occurs during advancement of delivery sheath. • Tricuspid valve tethering creates tricuspid valve obstruction. • Right atrial distension and leftward deviation of the atrial septum occurs. • Hemodynamic instability reverses with loosening of the arteriovenous loop.

Background The indications for liver transplantation (LT) in patients with hepatocellular carcinoma (HCC) continue to evolve. The aim of this study was to report outcomes in patients who underwent living donor liver transplantation (LDLT) for HCC outside traditional criteria including macrovascular invasion (MVI). Methods We reviewed outcomes in patients who met the University of California San Francisco (UCSF) criteria (n = 159) and our center-specific criteria (UCSF+) (largest tumor diameter ≤ 10 cm, any tumor number, AFP ≤ 1000 ng/ml) (n = 58). We also assessed outcomes in patients with MVI (n = 27). Results The median follow was 28 (10.6–42.7) months. The 5 year overall survival and risk of recurrence (RR) in the UCSF and UCSF + group was 71% vs 69% (P = 0.7) and 13% vs 36% (P = 0.1) respectively. When patients with AFP > 600 ng/ml were excluded from the UCSF + group, RR was 27% (P = 0.3). Among patients with MVI who had downstaging (DS), 4/5(80%) in low-risk group (good response and AFP ≤ 100 ng/ml) and 2/10 (20%) in the high-risk group (poor response or AFP > 100 ng/ml) were alive at the last follow-up. When DS was not feasible, 3/3 (100%) in the low-risk group (AFP ≤ 100 ng/ml + Vp1-2 MVI) and 1/9 (9.1%) in the high-risk group (AFP > 100 or Vp3 MVI) were alive. The 5 year OS in the low-risk MVI group was 85% (P = 0.003). Conclusion With inclusion of AFP, response to downstaging and degree of MVI, acceptable survival can be achieved with LDLT for HCC outside traditional criteria.

Extracorporeal membrane oxygenation complications involving vascular injury or intracardiac thrombosis in neonates are rare. We present a series of unique complications and describe their variable treatments.

Objectives To assess disease-related knowledge of rheumatoid arthritis (RA) patients Patients and methods Consecutive RA patients were invited from the rheumatology departments of BSMM University, Dhaka, Bangladesh. The Bangla version of the Patient Knowledge Questionnaire (B-PKQ) was used. Correlations between the B-PKQ scores and clinical-demographic data were measured using Pearson’s correlation coefficient. Impact of independent variables on the level of knowledge about RA was analyzed through multiple regression analysis. Possible explanatory variables included the following: age, disease duration, formal education level, and Bangla Health Assessment Questionnaire (B-HAQ) score. Analysis of variance (ANOVA) was used to test the difference between demographical, clinical, and socioeconomic variables. For statistical analysis, SPSS statistics version 20 was used. Results A total of 168 RA patients could be included. The mean B-PKQ score was 9.84 (range 1–20) from a possible maximum of 30. The mean time for answering the questionnaire was 24.3 min (range 15–34). Low scores were observed in all domains but the lowest were in medications and joint protection/energy conservation. Knowledge level was higher (15.5) in 6 patients who had RA education before enrollment. B-PKQ showed positive correlation with education level (r = 0.338) and negative correlation with HAQ (r = −0.169). The B-PKQ showed no correlation with age, disease duration, having first degree family member with RA, education from other sources (neighbor, RA patient, nurses), or information from mass media. Conclusions Disease-related knowledge of Bangladeshi RA patients was poor in all domains. Using these findings, improved education and knowledge will result in better disease control.Key Points• Little is known about the knowledge of RA patients regarding their disease and its treatment in Bangladesh and in developing countries in general.• We found that the knowledge of Bangladeshi RA patients regarding their disease was poor in all domains; it correlated positive with education level and negative with function (HAQ), but showed no correlation with age or disease duration.• The findings of this study can be used for improving current patient education programs by health professionals and through mass media.• Better disease control of RA may be achieved by improving patient knowledge in a developing country like Bangladesh, but also in other parts of the world.

Background Variable patent ductus arteriosus (PDA) morphology and the need to close PDAs in small size patients has led physicians to use Amplatzer Vascular Plugs (AVP) and recently available Amplatzer Duct Occluder II - Additional Sizes (ADO II AS). The purpose of this study was to analyze the safety, efficacy, and complication rates of the ADO II AS and AVPs, specifically AVP II. Methods All patients undergoing PDA closure with an AVP or ADO II AS from 2011 to 2016 were included. Clinical, echocardiographic, and angiographic data were collected and reviewed. Results Four hundred and sixty-nine patients were included. Median age was 27 months (0.75-852) and the median weight was 11.4 kg (1-92). There were 51 patients ≤5 kg. Type A PDA was most common in 48% (n = 225), followed by type E (27.5%, n = 129), type D (13%, n = 61), type C (10.2%, n = 48), and type B (1.3%, n = 6). Devices included AVP II (n = 421), ADO II AS (n = 30), and AVP IV (n = 18), left pulmonary artery stenosis occurred in 4.3% (n = 20). One patient required surgery for severe stenosis; the remaining cases were mild and required no intervention. Device embolization occurred in 3 patients (10%) with the ADO II AS. Successful device closure was achieved in 98.9% of cases. Conclusions The AVP II was highly effective for closing PDAs in smaller babies with varying morphologies and is safe when used in small sized patients with relatively low risk of complications. More studies are warranted to clarify the risks of ADO II AS.

Purpose: High-dose chemotherapy (HDCT) and autologous stem cell transplantation (ASCT) is used to treat patients with relapsed Hodgkin’s lymphoma. In this retrospective study we report our experience with patients who underwent HDCT and ASCT. Methods: All patients ≥15 years old with relapsed/refractory Hodgkin’s lymphoma who underwent HDCT and ASCT between June 2001 and December 2013 were included. Results: Fifty-four patients were identified. Median age at transplant was 22 years (range 15-49 years); 26 were men and 28 were women. Forty-eight patients (89%) underwent HDCT and ASCT after achieving a radiological response to salvage chemotherapy. The rate of radiological complete response to salvage chemotherapy was 13% and reached 50% within 3 months of ASCT in assessable patients. After a median follow-up of 25 months, 31 patients (57%) were still alive with no evidence of relapse or progression. Median event-free survival (EFS) was 24 months (95% CI 8.7-39.3) and 3-year EFS was 56%. Median overall survival (OS) was not reached and 3-year OS was 82.5%. Bulky mediastinal disease at relapse, hemoglobin level, and number of salvage regimens did not significantly impact EFS in univariate and multivariate analyses. After transplantation there was a trend towards longer EFS (30 vs. 24 months; p = 0.36) in patients with a longer time from the end of first-line treatment until relapse (≥12 vs. Conclusions: HDCT and ASCT for relapsed/refractory Hodgkin’s lymphoma is safe. Our findings are consistent with published phase III results. Longer follow-up is warranted.

Advances in surgical techniques in tetralogy of Fallot (TOF) patients have improved survival of these patients into adulthood. The procedure requires right ventricular outflow tract or trans-annular patch with resultant pulmonary stenosis and/or regurgitation. As such, adult patients seen with this condition may have increasing right ventricular hypertrophy and/or right ventricular dilation. Recently, the Sapien XT valve (Edwards Lifesciences, CA) was approved by the FDA for pulmonary implantation. In some cases, advancing the valve in right ventricular outflow tract is difficult. This is a case series of delivering Sapien XT valves in TOF patients with severe pulmonary regurgitation and/or stenosis, using the anchor balloon, buddy wire, and the novel, wire and sheath techniques. © 2017 Wiley Periodicals, Inc.

We report a case of Figulla-II Occlutech septal occluder malposition with residual shunt at posteriosuperior margin of an atrial septal defect. Improvising its bioptome type delivery cable, same system was used to recapture the device and redeploy it successfully. This report highlights a potential malfunction of Figulla-II Occlutech disc and the advantage of its delivery system for retrieval of the device.

Importance Recent epidemiologic and therapeutic advances have transformed understanding of the role of and therapeutic approach to patent foramen ovale (PFO) in ischemic stroke. Patent foramen ovale is likely responsible for approximately 5% of all ischemic strokes and 10% of those occurring in young and middle-aged adults. Observations Randomized clinical trials have demonstrated that, to prevent recurrent ischemic stroke in patients with PFO and an otherwise-cryptogenic index ischemic stroke, PFO closure is superior to antiplatelet medical therapy alone; these trials have provided some evidence that, among medical therapy options, anticoagulants may be more effective than antiplatelet agents. Conclusions and Relevance These new data indicate a need to update classification schemes of causative mechanisms in stroke, developed in an era in which an association between PFO and stroke was viewed as uncertain. We propose a revised general nomenclature and classification framework for PFO-associated stroke and detailed revisions for the 3 major stroke subtyping algorithms in wide use.

Traumatic aortic injury (TAI) or aortic transection is the second most common cause of death after blunt trauma. The conventional management approach towards a thoracic aortic injury is open thoracotomy and surgical repair; however, there is a recent increase in the trend towards the use of endovascular approach in this regard, particularly in the developed countries. We here report the cases of two young healthy male patients presenting in emergency department with thoracic aortic injury, following a road traffic accident (RTA). Thoracic endovascular aortic repair (TEVAR) was planned for both patients, as open repair was risky owing to other systemic injuries. Aortic stent graft was deployed just below the origin of the left subclavian artery in one patient, while it partially covered the left subclavian in the other. End runs showed good position of the graft with perfusion of aortic branches and exclusion of flow to the transection. Both patients had an uneventful recovery with no episodes of cerebrovascular accident (CVA) or paraplegia. Our study gives an insight into the recent trends of endovascular aortic repair (EVAR) becoming the mainstay treatment for TAIs. As far as we know this is the first reported successful case series of thoracic endovascular stent graft deployment for blunt thoracic aortic trauma in Pakistan. We recommend education and further teaching of surgeons to acquire expertise in this novel approach.

Until recently, evidence to support Patent Foramen Ovale (PFO) closure for secondary prevention of recurrent stroke has been controversial. Publication of high-quality evidence from randomized clinical trials and the subsequent FDA approval of two devices for percutaneous PFO closure is expected to increase the volume of PFO closure procedures not only in the United States but worldwide. As this technology is disseminated broadly to the public, ensuring the safe and efficacious performance of PFO closure is essential to mitigate risk and avoid unnecessary procedures. This document, prepared by a multi-disciplinary writing group convened by the Society for Cardiovascular Angiography and Interventions and including representatives from the American Academy of Neurology, makes recommendations for institutional infrastructure and individual skills necessary to initiate and maintain an active PFO/stroke program, with emphasis on shared decision making and patient-centered care.

Percutaneous patent foramen ovale (PFO) closure is an effective treatment for preventing stroke recurrence in patients with a history of cryptogenic stroke. However, comparative data between the two FDA-approved PFO closure devices is limited. This study aims to compare peri-procedural

Coarctation of the aorta (COA) is one of the more common congenital anomalies, occurring in four in ten thousand live births and accounting for five to eight percent of all congenital heart defects. COA lesions can be challenging to treat percutaneously, especially if complex vascular anatomy is a barrier to crossing the lesion. We present two cases of COA that utilized a trans-septal approach to cross the lesion in anterograde fashion, subsequently facilitating retrograde stenting of the lesions after snaring and externalizing the wire. In both cases, the trans-septal approach was employed because traditional femoral and radial approaches failed due to complex anatomy, and the trans-septal approach allowed for effective intervention without the need for surgery.

Objectives To investigate technical approaches for transcatheter closure of coronary artery fistula based on anatomic type of the fistula. Background The variability in coronary artery fistulae (CAF) anatomy that necessitates different transcatheter closure (TCC) approaches has not been well documented. Methods Records of patients with CAF who underwent TCC at 2 centers were reviewed for technical details and procedural outcome. CAF were classified as proximal and distal. TCC approaches employed were arterio-venous or arterio-arterial loop, retrograde arterial, and antegrade venous. Results Eighteen patients with CAF, mean age 12.6 years (0.07–60), 11 male (61%), underwent TCC. All CAF drained predominantly into the right side of the heart. Types of CAF were proximal in 15 and distal in 3 patients. CAF calibers were large in 7, medium in 9, and small in 2 patients. The arterio-venous loop approach was used in the majority of the cases (11 patients) and the CAF size were medium to large. The retrograde arterial approach was used in 4; of these, 3 patients had small to medium sized CAF. In 2 patients with long tortuous CAF an antegrade venous approach was employed. TCC was successful in 17 of the 18 patients (94.4%). There were no peri-procedural deaths or vascular complications. Conclusions This study documents transcatheter closure approaches for CAF and device selection based on fistula origin. The choices of TCC technique and device selection vary, and are primarily determined by the heterogeneous anatomic characteristics of the fistulae.

The aim of the study was to evaluate the safety, feasibility, and economic benefit of a high-volume operator performing common interventional procedures in a rural region of the United States (U.S.). Rural areas of the U.S., even with well-equipped cardiac catheterization laboratories, may not have a full-time pediatric interventional cardiologist (PIC); this requires patients to travel out of state even for simple interventions. Since 2005, we have adopted a policy of performing cardiac catheterizations and common interventional procedures with a visiting PIC. We reviewed data of all patients who underwent cardiac catheterizations from May 2005 through March 2015 at our center. Variables analyzed were type of procedure, results, and follow-up six months after procedure. 197 catheterizations were performed, of which 80 were for single atrial septal defect (ASD), 29 for fenestrated ASD, 49 for patent ductus arteriosus (PDA), and 39 for other procedures. Device closure of single ASD was successful in 75 of 79 attempts and in 27 of 28 attempts for fenestrated ASD. PDA closure was successful in 45 of 46 attempts. Follow-up data of 6 months or more were available for 127 patients. All but two patients had no cardiovascular symptoms at 6 months. There were four minor complications and no major complications. With a technical success rate of 94.9% for single ASD closure, 97.8% for PDA closure, and results comparable to those of multi-institutional registries, cardiac catheterization and interventions can be performed safely with excellent results. Performing procedures in such an arrangement is safe, feasible, and economically beneficial.

As surgical and catheter interventions advance, patients with congenital heart disease are now offered alternative treatment options that cater to their individual needs. Furthermore, collaboration between interventional cardiologists and cardiac surgeons have led to the development of hybrid procedures, using the best techniques of each respective field to treat these complex cardiac entities from initial treatment in the pediatric patient, to repeat intervention in the adult. We present a review of the increased popularity and trend in hybrid procedures in congenital heart disease without the use of cardiopulmonary bypass.

Percutaneous pulmonary valve placement in patients with an anomalous coronary artery is rare and can be complicated by coronary artery compression. We report successful implantation of a percutaneous pulmonary valve in a patient with an anomalous left anterior descending artery.

Although catheter-based intervention is generally accepted as the treatment of choice for branch pulmonary artery (PA) stenosis, there are no data directly comparing both the need for reintervention and time to reintervention in patients undergoing transcatheter stenting versus surgical arterioplasty. We compared children who underwent surgical branch pulmonary arterioplasty and branch PA stent placement between January 2008 and May 2012 at a single tertiary center. Need for reintervention and mean time to reintervention were assessed using chi-square and independent sample Student t test. Thirty-seven patients were included (surgery n = 18, stent n = 19). Mean weight at initial intervention was 11.3 ± 8.8 kg for surgical and 20.1 ± 15.5 kg for stent (p = 0.041). Intervention was performed on the left PA in 17 patients, the right PA in 12 patients, and both PAs in 8 patients. Five patients had undergone previous intervention. On mean follow-up of 807 ± 415 days, 50 % (9 of 18) of the surgery cohort and 5.3 % (1 of 19) of the stent cohort required reintervention (p = 0.002). In all but one case reintervention was catheter-based. Mean time to reintervention for the surgery cohort was 272 ± 162 days and for the single stent cohort it was 150 days. When comparable age and weight groups were analyzed, reintervention was still more common in the surgery cohort (p = 0.007). Children undergoing surgical branch pulmonary arterioplasty are more likely to require reintervention than those undergoing stent placement.

The risk of erosion after Amplatzer septal occluder (ASO) device placement in atrial septal defects is well described. Aortic rim deficiency and use of over-sized device increase the risk of erosion. This study attempts to describe device characteristics, anatomical features and echocardiographic predictors that increase the risk of erosion. Methods: From 2005 through 2012, 12 new cases, with nine confirmed and three suspected device erosions where pre-procedural, intra-procedural, and/or post-procedural echocardiograms were available and, were reviewed. Following parameters were evaluated: ASD location (high or low), rims deficiency and consistency, septal mal-alignment, dynamic nature of the defect; device edge relationship toward the transverse sinus (TS), atrial free wall tenting and the size of the defect compared with the size of the device used for closure. Results: We found poor posterior rim consistency, aortic rim absence (in multiple views) and absent aortic rim at O degree in 100% of the patients. Septal mal-alignment and dynamic ASD was present in nearly 50% of the cases. The device was over-sized in three patients only. A 26-mm device was the most common device that resulted in erosion. In cases, where patient had experienced bloody pericardial effusion and the device was in place, device tenting in the TS was observed. Surgical explantation of the device confirmed presence of erosion in all cases. Conclusion: Aortic rim absence in multiple views, poor posterior rim consistency, septal mal-alignment, and dynamic ASD appear to be factors where erosion risk increases significantly. A thorough assessment of the device edge by echocardiography in short-axis may show device tenting of the atrial free wall into the TS. This finding should be a strong indictor to recommend surgical removal of device after occurrence of pericardial effusion. © 2013 Wiley Periodicals, Inc.

Transcatheter pulmonic valve implantation has emerged as a safe and effective alternative to valve surgery in patients with surgically corrected congenital heart disease. In cases where the transcatheter approach has failed, or was thought to be technically challenging, a hybrid approach to pulmonic valve implantation has been described. This approach involves a small subxyphoid incision made by the cardiac surgeon and implantation of the pulmonic valve through the appropriate delivery sheath through this incision. Here we describe a case of a successful implantation of a Melody valve in a 12 kg child with tetralogy of Fallot. To our knowledge this is the smallest child to have undergone this procedure reported. © 2013 Wiley Periodicals, Inc.

Perventricular Melody valve placement has been described as a bailout approach or alternative RVOT approach in patients >30 kg. We present three patients

The Amplatzer septal occluder is commonly used to close secundum atrial septal defects (ASDs). Recently, the manufacturer's recommendations have been modified with regard to contraindications for its use. These changes primarily focus on evaluation of the aortic rim (anterio-superior rim) with transesophageal echocardiography (TEE) and intracardiac echocardiography (ICE). In this editorial, we will review systematically all of the sequential views in TEE and ICE that are required for thorough assessment of the aortic rim and provide explanation of what constitutes deficiency of the aortic rim as seen on ICE.

Objectives The purpose of this study was to compare the safety and efficacy of surgical, stent, and balloon angioplasty (BA) treatment of native coarctation acutely and at follow-up. Background Controversy surrounds the optimal treatment for native coarctation of the aorta. This is the first multicenter study evaluating acute and follow-up outcomes of these 3 treatment options in children weighing >10 kg. Methods This is a multicenter observational study. Baseline, acute, short-term (3 to 18 months), and intermediate (>18 months) follow-up hemodynamic, imaging data, and complications were recorded. Results Between June 2002 and July 2009, 350 patients from 36 institutions were enrolled: 217 underwent stent, 61 underwent BA, and 72 underwent surgery. All 3 arms showed significant improvement acutely and at follow-up in resting systolic blood pressure and upper to lower extremity systolic blood pressure gradient (ULG). Stent was superior to BA in achieving lower ULG acutely. Surgery and stent were superior to BA at short-term follow-up in achieving lower ULG. Stent patients had shorter hospitalization than surgical patients (2.4 vs. 6.4 days; p Conclusions Stent patients had significantly lower acute complications compared with surgery patients or BA patients, although they were more likely to require a planned reintervention. At short-term and intermediate follow-up, stent and surgical patients achieved superior hemodynamic and integrated aortic arch imaging outcomes compared with BA patients. Because of the nonrandomized nature of this study, these results should be interpreted with caution.

Objective: We describe our medium-term outcomes for peripheral pulmonary artery stenting in adults with congenital heart disease. Background: Improved outcomes in congenital heart disease have lead to the evolution of transcatheter therapies in adults aimed at maintaining normal hemodynamics. Stenting for pulmonary artery narrowing is effective in children, however little is known about outcomes in adults. Methods: Retrospective data analysis and follow-up review of our complete experience with peripheral pulmonary artery stenting in adults. Results: Over a 9-year period we carried out 15 procedures, implanting 23 stents (11—left pulmonary artery) in the pulmonary arteries of 12 adult patients (7 female). Eleven patients had previous cardiac surgery. Median age at implantation was 32.5 years (range, 18.7–56.7 years) with median weight of 71.3 kg (range, 44.5–95 kg). Six patients underwent bilateral pulmonary artery stenting. Median procedure time was 140 minutes (range, 76–263 minutes) and 4 patients had other interventions performed during the same procedure. Median systolic pressure gradient across the narrowing's of 24 mmHg (range, 11–61 mmHg) was reduced to 3 mmHg (range, 0–17 mmHg) postprocedure (P < 0.001). Three patients had acute stent embolization, one of whom required surgical removal. No aneurysm formation or significant stent fractures have been noted on median follow-up of 27.4 months (range, 1–97 months). Two patients required reintervention with further stent implantation. Conclusions: Pulmonary artery stenting provides effective relief of narrowing in adults with congenital heart disease. Bilateral and/or multiple stenting are often required. Stent embolization may occur particularly in patients with associated significant pulmonary regurgitation. (J Interven Cardiol 2011;24:373–377)

Blind pouch formation of the pulmonary artery (PA) in patients having undergone a Fontan operation can present a serious risk for thromboembolic events. Either primary or secondary closure of this stump is necessary to reduce this risk. Unfortunately, secondary closure is oftentimes difficult due to the size and anatomy of the presenting patient. We describe the insertion of a muscular ventricular septal defect (VSD) device via a perventricular approach for successful closure of a pulmonary stump in a 3-year old, 16-kg child. To our knowledge, this is the first report of a perventricular approach for successful closure of a PA stump. © 2014 Wiley Periodicals, Inc.

Variable patent ductus arteriosus (PDA) morphology and the desire to close PDA in small size patients (pts) has led physicians to use Amplatzer Vascular Plug (AVP) I - IV and newly available Amplatzer Duct Occluder II - Additional Sizes (ADO II-AS). The purpose of this study was to analyze data

Multiple congenital cardiac defects are usually addressed by cardiac surgery. We present our experience with simultaneous transcatheter treatment of multiple defects in children. Ten children, six females and four males, with multiple defects underwent treatment with interventional technique. The mean age was 4.4 +/- 2.6 years (range, 7 months to 8 years). The cardiac diagnosis was patent ductus arteriosus (PDA) and valvular pulmonary stenosis (PS) in two, atrial septal defect (ASD) and PDA in two, ASD and PS in two, PDA and aortic stenosis (AS) in three (severe left ventricular dysfunction in two), and perimembranous ventricular septal defect (VSD) and valvular PS in one. The ASDs were closed with an Amplatzer septal occluder (mean size, 16 +/- 4 mm), four PDAs were closed with an Amplatzer duct occluder, and three with a Cook's detectable coil. Mean balloon size used to dilate the pulmonary valve was 18 +/- 4 mm, and for the aortic valve this was 12 +/- 2 mm. There was a 70% (+/-15%) postprocedure reduction of gradients across the stenotic valves. The closure rate was 75% for PDAs in the catheterization laboratory, 80% for ASDs, and there was a mild residual intradevice leak in the VSD. In conclusion, interventional technology addressing multiple congenital cardiac defects as a combined procedure in the catheterization laboratory is safe and effective.

Congenital ventricular septal defects (VSDs) can exist as isolated defects or may be associated with other cardiac anomalies, such as tetralogy of Fallot, double outlet right ventricle (DORV), D-transposition of great arteries (D-TGA), truncus arteriosus, or interrupted aortic arch. To date, there is no consensus on the best way to classify such defects, nor even on the curved surface that is taken to represent the defect [1]. Muscular VSDs (mVSDs) are the second most common type of VSD after perimembranous defects based on most series and account for 10–15 % of all VSDs [2, 3]. These have exclusively muscular borders and can have gross malalignment between their caudal and cranial borders. In addition to this malalignment, they can open into different parts of the right ventricle. They can be further subclassified based on their geographical location whether they open centrally, apically, anteriorly, or to the right ventricular inlet or outlet components [1]. The left ventricular view shows fewer overlying trabeculae, and multiple defects frequently coalesce to form a single defect on the left side. Occasionally such apical defects are quite large [4, 5].

Patients and methods: Atrial septostomy was carried out in three children with end-stage pulmonary arterial hypertension and recurrent syncope. Continuous intravenous epoprostenol infusion was started concomitantly, in association with oral bosentan therapy.Results: Within a few weeks, the functional New York Heart Association (NYHA) class improved from III to II in all three patients. No recurrent syncope occurred and the children remained in NYHA functional class II during follow-up. The atrial septostomy remained patent and there was no need to increase the dose of intravenous epoprostenol.Conclusion: This small case series demonstrates that children with end-stage pulmonary arterial hypertension may benefit from an aggressive management, including fenestration of the atrial septum associated with the combination of bosentan and epoprostenol.

Background: We report a multiinstitutional study on intermediate-term outcome of intravascular stenting for treatment of coarctation of the aorta using integrated arch imaging (IAI) techniques. Methods and Results: Medical records of 578 patients from 17 institutions were reviewed. A total of 588 procedures were performed between May 1989 and Aug 2005. About 27% (160/588) procedures were followed up by further IAI of their aorta (MRI/CT/repeat cardiac catheterization) after initial stent procedures. Abnormal imaging studies included: the presence of dissection or aneurysm formation, stent fracture, or the presence of reobstruction within the stent (instent restenosis or significant intimal build-up within the stent). Forty-one abnormal imaging studies were reported in the intermediate follow-up at median 12 months (0.5–92 months). Smaller postintervention of the aorta (CoA) diameter and an increased persistent systolic pressure gradient were associated with encountering abnormal follow-up imaging studies. Aortic wall abnormalities included dissections (n = 5) and aneurysm (n = 13). The risk of encountering aortic wall abnormalities increased with larger percent increase in CoA diameter poststent implant, increasing balloon/coarc ratio, and performing prestent angioplasty. Stent restenosis was observed in 5/6 parts encountering stent fracture and neointimal buildup (n = 16). Small CoA diameter poststent implant and increased poststent residual pressure gradient increased the likelihood of encountering instent restenosis at intermediate follow-up. Conclusions: Abnormalities were observed at intermediate follow-up following IS placement for treatment of native and recurrent coarctation of the aorta. Not exceeding a balloon:coarctation ratio of 3.5 and avoidance of prestent angioplasty decreased the likelihood of encountering an abnormal follow-up imaging study in patients undergoing intravascular stent placement for the treatment of coarctation of the aorta. We recommend IAI for all patients undergoing IS placement for treatment of CoA. © 2007 Wiley-Liss, Inc.

Background: We report a multi-institutional experience with intravascular stenting (IS) for treatment of coarctation of the aorta. Methods and Results: Data was collected retrospectively by review of medical records from 17 institutions. The data was broken down to prior to 2002 and after 2002 for further analysis. A total of 565 procedures were performed with a median age of 15 years (mean = 18.1 years). Successful reduction in the post stent gradient ( 0.8 was achieved in 97.9% of procedures. There was significant improvement (P < 0.01) in pre versus post stent coarctation dimensions (7.4 mm ± 3.0 mm vs. 14.3 ± 3.2mm), systolic gradient (31.6 mm Hg ± 16.0 mm Hg vs. 2.7 mm Hg ± 4.2 mm Hg) and ratio of the coarctation segment to the DAo (0.43 ± 0.17 vs. 0.85 ± 0.15). Acute complications were encountered in 81/565 (14.3%) procedures. There were two procedure related deaths. Aortic wall complications included: aneurysm formation (n = 6), intimal tears (n = 8), and dissections (n = 9). The risk of aortic dissection increased significantly in patients over the age of 40 years. Technical complications included stent migration (n = 28), and balloon rupture (n = 13). Peripheral vascular complications included cerebral vascular accidents (CVA) (n = 4), peripheral emboli (n = 1), and significant access arterial injury (n = 13). Older age was significantly associated with occurrence of CVAs. A significant decrease in the technical complication rate from 16.3% to 6.1% (P < 0.001) was observed in procedures performed after January 2002. Conclusions: Stent placement for coarctation of aorta is an effective treatment option, though it remains a technically challenging procedure. Technical and aortic complications have decreased over the past 3 years due to, in part, improvement in balloon and stent design. Improvement in our ability to assess aortic wall compliance is essential prior to placement of ISs in older patients with coarctation of the aorta. © 2007 Wiley-Liss, Inc.

Background— Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors. Methods and Results— All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size–ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion. Conclusions— In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1-29 days) and median weight of 3.4 kg (range 2.4-4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1-74 days). Median hospital stay post-procedure was 47 days (range 15-270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1-4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.

A 7.5-lb female neonate was delivered at term, by means of a caesarian section, to a 26-year-old mother (her 2nd pregnancy). The infant had good Apgar scores but was transferred to the neonatal intensive care unit because of respiratory distress. She was treated for transient tachypnea and for bilateral spontaneous pneumothoraces. She improved with minimal respiratory support. Transthoracic echocardiography (TTE) revealed an aneurysmal ductus arteriosus (DA) with a large thrombus (Fig. 1). The patient was observed clinically and was started on 40 mg/d of aspirin. When the infant was 3 weeks old, TTE showed that the DA lumen was almost completely obliterated by the thrombus (Fig. 2). Two weeks later, TTE revealed complete resolution of the DA aneurysm and no thrombus (Fig. 3). The aspirin therapy was discontinued. Fig. 1. Transthoracic echocardiograms (parasternal short-axis views) show A) the pulmonary branch arteries and an aneurysmal ductus arteriosus with an evident thrombus (arrows); B) in color-flow Doppler mode, continuous flow through the ductus arteriosus into ... Fig. 2. Transthoracic echocardiograms. The parasternal short-axis views reveal A) the organization of the thrombus and almost complete obliteration of the ductus arteriosus lumen, and B) the absence of ductal flow, in color-flow Doppler mode. C) The suprasternal ... Fig. 3. Transthoracic echocardiograms. A) The parasternal short-axis view reveals confluent pulmonary branch arteries with unobstructed flow and complete resolution of the ductus arteriosus aneurysm and thrombus. B) The suprasternal view shows a patent aortic ...

In a 4.5-year-old child with refractory pulmonary arterial hypertension, we performed atrial septostomy with the application of an Amplatzer fenestrated device designed to maintain patency. Continuous intravenous epoprostenol infusion was started concomitantly. Forty-two months after the procedure, the patient had no recurrent syncope and remained in New York Heart Association functional class II. Fenestration of the atrial septum is feasible in children with pulmonary artery hypertension. No conclusion regarding the patient's need for an atrial septal defect can be drawn since concomitant prostanoid therapy was administered. The long-term patency of the atrial communication needs further confirmation and the optimal timing for its application has to be determined.

Objective Robotic systems allow surgeons to perform minimally invasive cardiac surgery in adults. Experience in the pediatric population, however, is limited. Perventricular closure of muscular ventricular septal defects has been reported in humans but requires a median sternotomy. The objective of this study was to assess the feasibility of robotically assisted closure of perimembranous ventricular septal defects by using the perventricular approach. Methods The procedure was attempted in 7 pigs with naturally occurring perimembranous ventricular septal defects. Echocardiography was performed to confirm the presence and assess the size of the defect. A 3-armed da Vinci system consisting of two 8-mm instrument ports and a 12-mm endoscopy port was used. A pericardiotomy was performed, and the right ventricular free wall was visualized. A spinal needle was advanced into the right ventricular cavity. By using echocardiographic guidance, a glide wire was advanced through the angiocatheter and manipulated through the defect into the left ventricle or the ascending aorta. A delivery sheath was advanced over the wire. An appropriately sized Amplatzer device was deployed through the sheath. Results The procedure was successful in 5 pigs. One device was removed because it was smaller than the defect and an appropriately sized device was not available. The placement failed in the second pig in the series. Four pigs were followed up for 1 to 4 months. Angiograms performed before the pigs were killed documented complete occlusion in 3 and mild-to-moderate shunt in 1. Conclusions Robotically assisted perventricular closure with the Amplatzer Membranous VSD Occluder is feasible. This approach avoids the associated morbidities of cardiopulmonary bypass and median sternotomy. Further investigation and refinements are needed, however, before application of this approach in humans.

OBJECTIVES This phase I study attempted to report the initial safety and efficacy results of transcatheter closure of perimembranous ventricular septal defects (PmVSDs) using the new Amplatzer Membranous VSD Occluder (AGA Medical Corp., Golden Valley, Minnesota) in the U.S. BACKGROUND The most common congenital heart disease is PmVSD. Surgical repair is widely accepted, but still carries a small but definite risk of morbidity and mortality. METHODS Between October 2003 and August 2004, a total of 35 patients with PmVSD underwent an attempt of transcatheter closure under transesophageal and/or intracardiac echocardiographic guidance. The median age was 7.7 years (range, 1.2 to 54.4 years) and median weight was 25 kg (range, 8.3 to 110 kg). The median Qp/Qs ratio was 1.8 (range, 1 to 4), and the median VSD size as assessed by echocardiography was 7 mm (range, 4 to 15 mm). RESULTS The attempt to place a device was successful in 32 patients (91%). The median device size used was 10 mm (range, 6 to 16 mm). The complete closure rates by echocardiography at 10 min (transesophageal/intracardiac), 24 h, 1 month, and 6 months (transthoracic) were 47% (15/32), 63% (20/32), 78% (25/32), and 96% (27/28), respectively. The median fluoroscopy time was 36 min (range, 14 to 191 min), and the median total procedure time was 121 min (range, 67 to 276 min). Three patients (8.6%) had serious adverse events of complete heart block, peri-hepatic bleeding, and rupture of tricuspid valve chordae tendineae. No other patient encountered serious adverse events during the follow-up. CONCLUSIONS Transcatheter closure of a PmVSD is technically feasible and seems safe enough in children over 8 kg in weight to warrant continuation of clinical trials to assess the long-term safety and efficacy.

Objective In infants undergoing closure of perimembranous ventricular septal defects, cardiopulmonary bypass remains one of the factors that prolongs hospital stay and morbidity. A new technique was used to close the defects under echocardiographic guidance without cardiopulmonary bypass to prevent the deleterious effects of bypass. Methods Recently, the Amplatzer membranous ventricular septal defect device (AGA Medical Corp, Golden Valley, Minn) was introduced. The device has a double-disc design with a short connecting waist. The left ventricular disc has an eccentric design to prevent encroachment on the aortic valve leaflets. Eight Yucatan miniature pigs with naturally occurring perimembranous ventricular septal defects underwent closure of the defect in the operating room by using the perventricular technique. After median sternotomy, a purse-string suture was placed on the free wall of the right ventricle. An angiocatheter was advanced in the right ventricle, and through the catheter, a wire was advanced from the right ventricle through the ventricular septal defect into the left ventricle. A delivery sheath and the dilator were advanced over the wire. The wire and catheter were removed, and an appropriately sized Amplatzer membranous device was advanced through the sheath. The device was deployed under echocardiographic guidance with the heart beating. Results The procedure was successful in all animals. There was no incidence of device embolization, heart block, or aortic insufficiency. Angiograms at 3 and 6 months revealed no residual defects and no aortic insufficiency. Pathologically, the devices were completely endothelialized when examined grossly. Conclusions The perventricular technique appears to be excellent for closure of perimembranous ventricular septal defects in the operating room. The technique might be feasible in smaller babies, who are high-risk candidates for closure in the catheterization laboratory. Cardiopulmonary bypass and prolonged hospital stay are avoided.

Noonan Syndrome is an autosomal dominant disorder characterized by short stature, typical facial features and congenital heart defects. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, bleeding tendency and lymphatic dysplasia. We are reporting a 13 year old boy of Noonan syndrome presenting with lymphoedema of lower limb and genitalia. DOI: http://dx.doi.org/10.3329/jom.v13i2.12771 J Medicine 2012; 13 : 243-245

This study evaluated use of Amplatzer fenestrated device to maintain patency of the Fontan fenestration and atrial septal defect. Fenestrations are routinely created in patients with lateral tunnel or extracardiac Fontan. Spontaneous closure of the fenestration can lead to Fontan circulation failure. Other patients without single-ventricle physiology may benefit from a small communication between the left and right atria for decompression if closure of the atrial septal defect leads to failure of a dysfunctional ventricle. Amplatzer septal occluder device was modified to create a fenestration through the disks. Three patients with modified Fontan and one patient with a large atrial septal defect underwent placement of the device by transcatheter technique. The device deployment was guided by transesophageal echocardiography. The procedure was successful in all patients. Contrast injection after placement revealed patent fenestration with free flow. Follow-up ranged from 3 months to 1 year. All devices were patent by transthoracic echocardiography. These preliminary results suggest that the Amplatzer fenestrated device can serve as a valuable tool in failing Fontan circulation and may help to avoid surgical intervention. More studies are needed to assess long-term efficacy of the device.

The patent foramen ovale (PFO) is a normal interatrial communication during fetal life that remains after birth in nearly 20 % of adults. Percutaneous transcatheter PFO closure has been performed for various compelling reasons for more than two decades. The complication rate related to the use of these percutaneous devices has decreased remarkably with the use of new devices and the availability of tertiary care centers. Nevertheless complications do occur and rarely can be fatal. The purpose of this review is to summarize the major complications that happen during and after percutaneous PFO closure, their incidence among different devices, clinical implications, diagnosis and treatment.

Failure of the atrial septum primum and secundum to fuse after birth results in the persistence of foramen ovale (Patent Foramen Ovale). Variations in the atrial septal morphology of the PFO are frequent and have important technical implications on the success of transcatheter PFO closure. Patients should be carefully selected on the basis of morphology and location of the interatrial defect to minimize the rate of complications of percutaneous closure of PFO and to avoid residual shunts that can result in recurrence of symptoms. To achieve a successful, complete closure of the PFO, a detailed understanding of PFO anatomy is required. In this chapter we will review the different anatomical variations of PFO and their clinical implications.