European position paper on the management of patients with patent foramen ovale. Part II - Decompression sickness, migraine, arterial deoxygenation syndromes and select high-risk clinical conditions.

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.

Novel arterioplasty of severe left pulmonary artery stenosis using native main pulmonary artery.

There are multiple approaches described for the repair of stenosed branch pulmonary arteries. Regardless of the technique used, restenosis is common. We describe a case of severe left pulmonary artery stenosis repaired with a novel technique using the native main pulmonary artery which was transected and turned down to create a direct anastomosis with the left pulmonary artery. This tension-free tissue to tissue anastomosis resulted in hemodynamically gratifying results with the potential for growth.

One stage combined repair of intracardiac defects with vascular ring employing sternotomy approach.

We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.

Transcatheter closure of elongated and pulmonary hypertensive patent arterial duct in infants using Amplatzer vascular plug II.

OBJECTIVE: To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants. INTRODUCTION: Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems. METHOD: This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II. RESULTS: Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome. CONCLUSIONS: Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

Device closure of atrial septal defect with severe pulmonary hypertension in adults: Patient selection with early and intermediate term results.

OBJECTIVE: To describe a subset of atrial septal defect (ASD) with severe pulmonary hypertension (PHT) that is suitable for closure. BACKGROUND: As per American Heart Association/American College of Cardiology guidelines, ASD with elevated pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >2/3rd systemic is considered as a contraindication for closure. METHODS: Patients with anatomically large ASD measuring >25 mm and a high probability of reversible pulmonary vascular disease were subjected to fenestrated device closure, despite severe PHT and elevated PVR. They were discharged on pulmonary vasodilators and were followed for 39.5 ± 8.5 months. Four patients had repeat cardiac catheterization. RESULTS: Six patients underwent successful device closure of large ASD with severe PHT. Their basal Qp:Qs was 2.8 ± 0.3:1 while the systolic PAP and the PVR index (PVRI) were 102.6 ± 11.5 mm Hg and 9.6 ± 1.6 Wu m2 , respectively. Post 100% oxygen inhalation, the Qp:Qs increased to 3.5 ± 0.3:1, systolic PAP remained 103.5 ± 7.6 mm Hg while the PVRI dropped to 5.4 ± 1.1 Wu m2 . The postballoon occlusion systolic PAP decreased to 86.6 ± 8.8 mm Hg. At the last follow-up, their pulmonary artery systolic pressure by tricuspid regurgitation (TR) jet decreased from 105.6 ± 12.6 mm Hg to 45 ± 7.0 mm Hg. During follow-up cardiac catheterization (n = 4), the systolic PAP and PVRI were 55.7 ± 9.2 mm Hg and 3.2 ± 0.4, respectively. CONCLUSIONS: Patients with anatomically big defect and a large left to right shunt at baseline with a high probability of reversible PVR benefit with ASD closure and pulmonary vasodilators, despite significantly elevated PAP and PVRI.

Rheumatic Mitral Valve Stenosis: Diagnosis and Treatment Options.

PURPOSE OF REVIEW: This review provides an update on rheumatic mitral stenosis. Acute rheumatic fever (RF), the sequela of group A ß-hemolytic streptococcal infection, is the major etiology for mitral stenosis (MS). RECENT FINDINGS: While the incidence of acute RF in the Western world had substantially declined over the past five decades, this trend is reversing due to immigration from non-industrialized countries where rheumatic heart disease (RHD) is higher. Pre-procedural evaluation for treatment of MS using a multimodality approach with 2D and 3D transthoracic and transesophageal echo, stress echo, cardiac CT scanning, and cardiac MRI as well as hemodynamic assessment by cardiac catheterization is discussed. The current methods of percutaneous mitral balloon commissurotomy (PMBC) and surgery are also discussed. New data on long-term follow-up after PMBC is also presented. For severe rheumatic MS, medical therapy is ineffective and definitive therapy entails PMBC in patients with suitable morphological mitral valve (MV) characteristics, or surgery. As procedural outcomes depend heavily on appropriate case selection, definitive imaging and interpretation are crucial. It is also important to understand the indications as well as morphological MV characteristics to identify the appropriate treatment with PMBC or surgery.

Embolisation of ceramic-coated PDA devices into the descending thoracic aorta: probable mechanisms and retrieval strategies.

We describe two cases of spontaneous embolisation and successful retrieval of ceramic-coated patent arterial duct devices. In both, the device embolised to the descending aorta in the absence of pulmonary hypertension and despite optimum placement. We have discussed possible mechanisms for embolisation in these patients and suggested alternative methods for device retrieval. Based on this limited experience, we conclude that for tubular ducts, ceramic-coated devices should be oversized to form a tighter waist or alternate devices may be considered.

Use of Amplatzer vascular plugs and Amplatzer duct occluder II additional sizes for occlusion of patent ductus arteriosus: A multi-institutional study.

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.

Transcatheter closure of large atrial septal defects using 40 mm amplatzer septal occluder: Single group experience with short and intermediate term follow-up.

OBJECTIVES: To assess feasibility, safety, and efficacy of the use of 40 mm Amplatzer septal occluder (ASO 40) for the closure of large atrial septal defects (ASD). BACKGROUND: There is very little data available on closure of large ASDs with ASO 40. MATERIALS AND METHODS: Case records of patients who underwent ASD closure with ASO 40 between 2002 and 2014 were retrospectively analyzed. All patients had clinical, transthoracic, and transesophageal echocardiographic (TEE) evaluation prior to device closure. Postclosure follow-up was done at 6 weeks, 6 months, and annually thereafter. RESULTS: 87 patients underwent ASD closure using ASO 40 during the study period. Mean age and weight of the group was 32.4 ± 11.6 years and 59.5 ± 11.3 kg respectively. Mean ASD diameter on TEE was 32 ± 2.8 mm. The balloon stretched diameter (N = 40) was 37.8 ± 1.3 mm. The balloon assisted technique was used in 80/87 patients for device deployment. The procedure was successful in 84/87 patients. Follow-up was available in 77 patients over a period of 44 ± 15.7 months. 3/77 patients had a small residual shunt. The severity of tricuspid regurgitation decreased in 40/77 patients. The pulmonary artery systolic pressure decreased from 49.7 ± 9.2 to 41.2 ± 6.2 mm Hg (N = 61; P < 0.05). The right ventricular diameter decreased from 35.1 ± 2.8 to 26.1 ± 3 mm (N = 77; P < 0.05). CONCLUSION: ASO 40 can be used safely and effectively with promising short and intermediate term results.© 2016 Wiley Periodicals, Inc.

Spontaneous closure of right pulmonary artery-to-left atrium fistula: a case report.

Direct communication between the right pulmonary artery and the left atrium is rare. We report a case that was first detected in utero and confirmed by a two-dimensional echocardiographic study performed postnatally. The patient remained asymptomatic, and hence was managed conservatively. The fistula gradually decreased in size and closed spontaneously at 15 months of age. The rarity of the case is discussed.

Transcatheter closure of large atrial septal defects in children using the left atrial disc engagement-disengagement technique (LADEDT)-technical considerations and short term results.

OBJECTIVE: To report our experience of transcatheter closure (TCC) of large atrial septal defects (ASDs) in children using the left atrial (LA) disc engagement-disengagement technique (LADEDT). METHODS: This is a retrospective study comprising 21 children diagnosed with large secundum ASDs. All of them had an attempt at TCC with the Amplatzer septal occluder (ASO) using LADEDT. They were assessed by clinical examination, ECG and echocardiography before the procedure, at 24 hours, 6 weeks, 6 months, and every 1 year after the device closure. RESULTS: The median age of the cohort was 3 years (range 1-12 years). The weight ranged from 5 to 40 kg (median = 12 kg). The mean ASD diameter was 16.7 ± 4.3 mm. The median device size used was 22 mm (range 15-40 mm). The device was successfully placed from the superior pulmonary vein (left: 12; right: 4) and the LA appendage (LAA) in 2. In three patients the technique failed and we had to resort to the balloon assisted technique (BAT) for a successful deployment. The number of attempts for deployment ranged from 1 to 4 (median 2). Despite appropriate placement, the device was not released in one patient because of deficient and floppy inferior margin. One patient had worsening of mitral regurgitation from mild to moderate grade without further progression till last follow up. There were no other major complications during the follow up (6.2 ± 2.4 months) period. CONCLUSION: TCC of large ASDs in small children using the LADEDT is predictable, reproducible, relatively simple, safe, and effective.

Transcatheter closure of a large ASD from the femoral vein in a patient with Budd-Chiari syndrome.

Anomalies of the inferior vena cava are rare in patients with isolated atrial septal defect (ASD). When present, they usually preclude successful transcatheter closure of the ASD using the femoral route. We report a case of a large secundum ASD in a 3-year-old child, who had a chronic Budd-Chiari syndrome incidentally detected on the cardiac catheterization table. We were able to successfully deploy the device from the femoral route itself using a collateral channel to reach the heart and then using the pulmonary vein deployment technique to successfully close the defect.

Balloon Interrogation of Intervening Tissue: A Novel Method to Decide Strategy for Closing Multiple Atrial Septal Defects.

BACKGROUND: Two separate ostium secundum atrial septal defects are a challenging substrate for device closure due to lack of a well-described strategy or an adequately evaluated protocol. METHODS: This is a prospective study comprising 20 patients with 2 atrial septal defects who underwent device closure. All of them underwent balloon interrogation (BI) of the intervening tissue to decide 1- versus 2-device strategy. During BI, if the flow through both the defects could be stopped completely implying adequate mobility of the separating tissue, a single device strategy was used. The size of the device in this subset was determined by BI diameter. In case the flow persisted, 2 devices were used to close the defects separately. RESULTS: The mean age was 24±17 years. The main defect size was (mean 14.5 mm±SD 2.69 mm), whereas the second defect measured (mean 8.5±SD 3.02 mm). The tissue separating the 2 defects was measured (mean 6.1±SD 2.6 mm). In 15 of them, based on the BI results, a single device was used successfully to close both the defects without a residual shunt. In the remaining 5 patients, 2 devices were used. There were no complications during the procedure or at follow-up period of 41.9±16.9 months. CONCLUSIONS: BI in patients with 2 atrial septal defects is helpful in defining 1- versus 2-device strategies and in choosing the size of the device to be used. Nearly 3/4 of the patients may get away with a single device for closing both the defects successfully thereby decreasing the cost and complexity of the procedure.

Use of cribriform amplatzer septal occluder in the pediatric population: Feasibility, safety, and technical considerations.

PURPOSE: Fenestrated atrial septal defects (F-ASDs) in the pediatric population pose a challenge for transcatheter device closure since multiple devices are not preferred in small hearts. Oversizing the Amplatzer Septal Occluder (ASO) to cover the surrounding fenestrations usually distorts the central waist as well as the disc profile and often defeats the purpose. This is a retrospective observational study with an aim to assess the feasibility and safety of cribriform ASO in closing F-ASDs in small children. METHODS: Sixteen children with F-ASD who underwent device closure with cribriform ASO were included in the study. The fenestrated septal length (FSL) and the total septal length (TSL) were measured on transesophageal echocardiogram. A device size which was 1.5-2 times the FSL but smaller than the TSL was selected. The defect was closed with a device passed through a relatively centrally placed smaller fenestration. RESULTS: The median age of the cohort was 5 years (2.5-10.5). Majority (14/16) required 25 or 30 mm cribriform ASO. Aneurysmal interatrial septum was seen in most of our patients (11/15). All the patients had successful device implantation. Complete closure of the defect was seen in 11 patients while 5 patients had insignificant residual shunt at a median follow-up of 40 months (1-60 months). There were no other complications. CONCLUSIONS: Cribriform ASO can be used safely and effectively in closing F-ASDs in children. Deployment of the device through a small central hole allows covering maximum fenestrations and gives more stability to the device. Residual shunts, although not infrequent, are insignificant.

Transcatheter treatment of an unusual coronary arteriovenous malformation with a fistulous sac in the interventricular septum.

Coronary artery fistula (CAF) is a rare congenital anomaly with a reported incidence of 0.2%-0.6%. There is a wide variation in the clinical presentation depending on the size and the site of the fistula. Transcatheter closure is currently the treatment of choice in these patients. We report a case of CAF with an unusually large fistulous sac within the interventricular septum. The fistula had connections with all the three major coronary arteries, namely, left anterior descending (LAD), left circumflex, and right coronary arteries but did not have any exit resulting in to and fro movement of blood within the sac and the feeding vessels. The patient was managed successfully by transcatheter coil embolization.

Persistent atypical atrial flutter after device closure of the atrial septal defect in a young man.

Atrial flutter is uncommon in young patients with uncorrected atrial septal defect (ASD). Although rare, it has been reported in the younger population following device closure of ASD/patent foramen ovale. We describe a case of persistent atypical atrial flutter following device closure of ASD in a young man and discuss the management strategy given the various underlying dilemmas.

Early multicenter experience of Melody valve implantation in India.

BACKGROUND: Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). METHODS: Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. RESULTS: Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure (n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1-39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. CONCLUSIONS: Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients.

Anomalous origin of the left coronary artery from the pulmonary artery with patent ductus arteriosus: a must to recognize entity.

Anomalous left coronary artery from the pulmonary trunk (ALCAPA) presents in early infancy with a clinical picture of congestive heart failure with left ventricular (LV) dysfunction and mitral insufficiency. These manifestations of myocardial ischaemia may be masked in the presence of an associated patent ductus arteriosus (PDA) or ventricular septal defect (VSD) which prevents the fall of pulmonary artery pressures and allows perfusion of the anomalous coronary artery. We present a case of a patient with large PDA-associated ALCAPA and preserved LV function. The importance of such a finding lies in the fact that VSD closure or PDA ligation in such cases would unmask the ALCAPA.

Transcatheter closure of atrial septal defect in symptomatic children weighing ≤10 kg: Addressing unanswered issues from a decade of experience.

BACKGROUND AND OBJECTIVES: Device closure of secundum atrial septal defect is shown to be feasible and effective in children weighing ≤10 kg. Issues such as how large is too large, how to choose device size, does the length of the interatrial septum (IAS) matter, and need for technical modifications for successful device delivery have not been systematically addressed. MATERIALS AND METHODS: This is a retrospective study, comprising 45 patients weighing ≤10 kg, who were chosen for device closure between January 2010 and June 2018. Patient selection was done on basis of transthoracic echocardiography. Device closure was done using Amplatzer septal occluder. The device size was selected primarily based on transesophageal echocardiography (TEE)-measured defect diameter. Although IAS length was taken into consideration, adequate rim size was the key factor in deciding device closure of the defect. RESULTS: Forty-three out of 45 patients had successful device closure. The mean age and weight were 25.71 ± 8.62 months and 8.99 ± 1.24 kg, respectively. The defect measuring as large as 27 mm (14.89 ± 3.89) on TEE was closed and device as big as 28 mm was successfully deployed (16.7 ± 4.31). Regular technique of device deployment was successful in only 15 cases. In the remaining 28, one of the modified techniques was used. There was no mortality, failure of the procedure, device embolization, thromboembolism, or pericardial effusion. One patient developed moderate mitral regurgitation and two patients had transient atrioventricular block. At follow-up, all patients showed significant improvement in symptoms and growth without any complications. CONCLUSIONS: Defect size as large as three times the weight in kg can be closed in small children. Devices as large as 28 mm can be deployed in these hearts provided the surrounding rims are adequate. In majority of cases, one of the modified techniques is essential for successful deployment. IAS length is not a limiting factor for deciding the size of the device used.