A Valved Expanded Polytetrafluoroethylene Conduit can Acquire Endothelium After Implantation.

We demonstrate histopathology, neointimal proliferation, and neo-endothelialization in an explanted valved expanded polytetrafluoroethylene (ePTFE) conduit 40 months postimplantation that was void of calcification and inflammation, confirmed by CD-31 positivity on immunohistochemistry. Grossly, there was no distortion with preserved leaflets and lack of calcification. Good biocompatibility, nonreactivity, and low antigenicity, combined with neointimal and endothelial layer generation within the conduit might explain the low infection rates and minimal thrombogenicity. These findings support the use of handmade, valved ePTFE conduits as an economically viable option as a right ventricle to pulmonary artery conduit.

Iatrogenic Anomalous Origin of the Right Pulmonary Artery From the Aorta: A Rare Complication of Ligation of Aortopulmonary Window.

An aortopulmonary window (APW) is a communication between the main pulmonary artery (MPA) and the ascending aorta in the presence of two separate semilunar valves. Surgical strategies described for the management of APW include ligation, division and suturing, patch closure of APW (transaortic or transpulmonary or transwindow), and device closure. Ligation of a type-2 APW can result in stenosis or complete closure of the right pulmonary artery (RPA) off the MPA, while leaving the aorta still opening into the RPA. We describe a delayed presentation of iatrogenic anomalous origin of the RPA from the aorta, post APW ligation, and its management.

Right ventricular outflow tract histology post-stenting and in-stent stenosis.

The right ventricular outflow tract (RVOT) stenting is used for the treatment of Fallot-type lesions when not amenable to complete correction or when surgical palliation carries a substantial risk. Despite the increasing clinical application, there is a lack of data that describe the RVOT morphology after stenting. This article elucidates post-RVOT stenting and in-stent stenosis, which is thought to be a zonal phenomenon, in this case, predominantly occurring proximally, in the portion of the stent apposing the RVOT infundibulum.

Branch Pulmonary Artery Jailing During Patent Ductus Arteriosus Stenting: Recruitment and Immediate Outcomes.

BACKGROUND: Branch pulmonary artery (PA) occlusion during patent ductus arteriosus (PDA) stenting procedure is the main reason why branch PA origin stenosis was considered as a contraindication for PDA stenting. This study was designed to assess the incidence of branch PA jailing during PDA stenting for cyanotics with duct-dependent pulmonary circulation and its immediate outcome. METHODS: All the completed PDA stenting patients in our hospital between April 2017 and June 2019 were retrospectively analyzed for branch PA jailing and its outcome. RESULTS: Of 63 completed PDA stenting, there was branch PA jailing in 13 (20.6%) patients, all successfully recruited either by strut dilatation or by surgery. The median duration of ventilation was 16 (interquartile range [IQR]: 8-22) hours for jailed patients and 17.5 (IQR: 5.25-34.25) hours for nonjailed patients (P = .978). Median intensive care unit [ICU] stay was 69 (IQR: 47.75-96) hours for jailed patients and 79.5 (IQR: 66.75-135.25) hours for nonjailed patients (P = .394). Procedural mortality was 1 (7.6%) for jailed patients and 3 (6%) for nonjailed patients. Since all the jailed pulmonary arteries were recruited, there was proportionate growth of branch PA till the most recent follow-up. CONCLUSION: Jailing of branch PA does not increase the ventilation duration, ICU stay, or mortality risk if recruited immediately. Proportionate growth of branch PAs can be achieved in spite of jailing, if addressed aggressively. Branch PA stenosis should not be considered as a contraindication for PDA stenting.

Clinically relevant variants in a large cohort of Indian patients with Marfan syndrome and related disorders identified by next-generation sequencing.

Marfan syndrome and related disorders are a group of heritable connective tissue disorders and share many clinical features that involve cardiovascular, skeletal, craniofacial, ocular, and cutaneous abnormalities. The majority of affected individuals have aortopathies associated with early mortality and morbidity. Implementation of targeted gene panel next-generation sequencing in these individuals is a powerful tool to obtain a genetic diagnosis. Here, we report on clinical and genetic spectrum of 53 families from India with a total of 83 patients who had a clinical diagnosis suggestive of Marfan syndrome or related disorders. We obtained a molecular diagnosis in 45/53 (85%) index patients, in which 36/53 (68%) had rare variants in FBN1 (Marfan syndrome; 63 patients in total), seven (13.3%) in TGFBR1/TGFBR2 (Loeys-Dietz syndrome; nine patients in total) and two patients (3.7%) in SKI (Shprintzen-Goldberg syndrome). 21 of 41 rare variants (51.2%) were novel. We did not detect a disease-associated variant in 8 (15%) index patients, and none of them met the Ghent Marfan diagnostic criteria. We found the homozygous FBN1 variant p.(Arg954His) in a boy with typical features of Marfan syndrome. Our study is the first reporting on the spectrum of variants in FBN1, TGFBR1, TGFBR2, and SKI in Indian individuals.

Staged Hybrid Approach for Ductal Origin of the Pulmonary Artery in a Low Birth Weight Neonate.

Early recruitment of a discontinuous branch pulmonary artery will give the best long-term outcome for congenitally disconnected pulmonary arteries. This may be achieved using a staged approach. Even in low birth weight neonates, a hybrid approach with the first stage consisting of transcervical (via the common carotid artery) patent ductus arteriosus stenting can be performed to recruit the pulmonary artery where conventional treatment strategies may not be feasible or may be associated with higher risk.

Embryological basis of coarctation in cervical aortic arch: Ductus caroticus anomaly

Coarctation of the cervical aortic arch (CAA), though rare, is a clinical entity of embryological significance. Based on Rathke's aortic arch diagrams and Edward's concept of functioning double aortic arch, we are postulating that the coarctation in a CAA occurs due to partial involution of the dorsal aorta developed from the "ductus caroticus". Hence coarctation in CAA should be classified as a "clinically significant aortic arch anomaly of ductus caroticus origin". Conversely, location of coarctation in CAA substantiates the theory that embryogenesis of CAA is due to aortic arch development from the third or second branchial arch vessel, rather than the usual fourth arch vessel

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Hybrid approach for aortic embolization of Amplatzer duct occluder.

Embolization of the patent ductus arteriosus (PDA) device is a known adverse event of percutaneous PDA closure, which can lead to complications. Embolization can occur into the pulmonary artery or into the aorta. Device embolization can be moderate adverse event (when retrieved percutaneously) or major adverse event (when retrieved surgically). We are describing a hybrid approach for aortic embolization of PDA device when the percutaneous retrieval fails, where device retrieval and PDA ligation can be done through thoracotomy incision, thus decreasing the complications.

Hybrid Approach for Embolized Right Ventricular Outflow Tract Stent.

Displacement and embolization of the stent is one of the major complications of right ventricular outflow tract (RVOT) stenting. Since embolized stents cannot be retrieved percutaneously as they cannot be crimped into pre-stenting state (unlike duct occluders and septal devices, which can be pulled back into the sheath), surgery remains the gold standard treatment. We describe a hybrid approach for the retrieval of the embolized RVOT stent, which will decrease morbidity when compared to the standard surgical approach that is otherwise required.

Improved Technique for Interventional Extracardiac Fontan.

We have previously reported a simple technique for preparatory staging and subsequent interventional completion of an extracardiac Fontan procedure that anatomically and hemodynamically closely mimics a standard extracardiac Fontan. We describe herein modifications that simplify the original procedural sequence and that may allow wider application. Percutaneous completion can be achieved even without a radiofrequency probe, using electrocautery. Fenestration is also easy to perform if there is a clinical indication.

Late Complication of Amplatzer Duct Occluder: Erosion and Pseudoaneurysm.

We are presenting a delayed complication of an Amplatzer duct occluder (ADO) in which a patient presented with increasing hemoptysis owing to ADO eroding the vessel wall and forming a pseudoaneurysm that communicated with the left main bronchus. Although ADO is considered as the safest for catheter-based patent ductus arteriosus closures, isolated complication as reported can occur. Just like atrial septal defect device closures, which were considered safe initially and found to be having an erosion incidence of 0.1% to 0.3%, this case could represent an isolated event (the index case) or a long-term complication occurring at a very low incidence.

Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children.

OBJECTIVE: We aimed to assess the change in global and regional myocardial function before and after surgical revascularization and their added value when compared with conventional measures in children with anomalous left coronary artery from the pulmonary artery (ALCAPA). METHODS: Advanced echocardiographic assessment was performed pre- and postoperatively in 22 children with ALCAPA (eight male; median surgery age, 0.4 years; interquartile range, 0.21-1.05) and 22 healthy controls. Measurements included global and segmental longitudinal, radial, and circumferential two-dimensional speckle-tracking strain and postsystolic index. RESULTS: Global strains were lower in preoperative patients than in controls (longitudinal: -9% vs -21%; P < .001; circumferential: -11% vs -21%; P < .001; radial: 18% vs 60%; P < .001) and improved postoperatively when compared with preoperative findings (longitudinal: -9% pre vs -16% post; P = .002, circumferential:-11% pre vs -17% post; P = .012, radial: 18% pre vs 53% post; P = .001). Preoperatively, patients with normal global systolic function on conventional echocardiography had significantly impaired global longitudinal and radial strain compared with healthy controls. Global mechanical dyssynchrony improved significantly postoperatively (longitudinal postsystolic index 43 pre vs 6 post, P < .001; circumferential 15 pre vs 2 post, P = .001; radial 48 pre vs 5 post, P = .003). Despite overall improvement in most segments, global longitudinal and circumferential and segmental peak strain in some of the segments supplied by the ALCAPA remained postoperatively abnormal. CONCLUSIONS: This study shows that myocardial deformation indices were a more sensitive measure of LV dysfunction in patients before and after ALCAPA repair than conventional echocardiographic measures. We believe, therefore, they should be added to routine preoperative and serial postoperative follow-up assessment.

Percutaneous Device Closure of Congenital Isolated Ventricular Septal Defects: A Single-Center Retrospective Database Study Amongst 412 Cases.

To identify suitable cases and reduce failure/complication rates for percutaneous ventricular septal defect (VSD) closure, we aimed to (1) study causes of device failure and (2) compare outcomes with different VSD types and devices in a high-volume single center with limited resources. Retrospective data of 412 elective percutaneous VSD closure of isolated congenital VSDs between 2003 and 2017 were analyzed. Out of 412, 363 were successfully implanted, in 30 device implantation failed, and in 19 the procedure was abandoned. Outcome was assessed using echocardiography, electrocardiography, and catheterization data (before procedure, immediately after and during follow-up). Logistic regression analyses were performed to assess effects of age, VSD type, and device type and size on procedural outcome. Median [interquartile range] age and body surface area were 6.6 [4.1-10.9] years and 0.7 [0.5-1.0] m2, respectively. Device failure was not associated with age (p = 0.08), type of VSD (p = 0.5), device type (p = 0.2), or device size (p = 0.1). Device failure occurred in 7.6% of patients. As device type is not related to failure rate and device failure and complication risk was not associated with age, it is justifiable to use financially beneficial ductal devices in VSD position and to consider closure of VSD with device in clinically indicated children.

Does heart-type fatty acid-binding protein predict clinical outcomes after pediatric cardiac surgery?

INTRODUCTION: The early identification of vulnerable pediatric cardiac surgery patients can help clinicians provide them with timely support. Heart-type fatty acid-binding protein. (H-FABP) is an early biomarker of myocardial injury in acute myocardial infarction in adults. In this study, we evaluated the correlations between postoperative H-FABP, creatine kinase-myocardial band (CK-MB), troponin-I, total bypass time, and clinical outcomes. METHODS: In 32 pediatric patients that underwent ventricular septal defect. closure we measured H-FABP, troponin-I and CK-MB preoperatively and 1, 3, and 6 h after aortic declamping. Spearman's Rho correlations were calculated between laboratory and clinical parameters including inotropic support duration, aortic cross-clamp time, total bypass time, ventilation-weaning-time, and total Intensive Care Unit stay. RESULTS: H-FABP, CK-MB, troponin-I, and total bypass time have a similarly weak to moderate correlation with clinical outcome measures. CONCLUSIONS: The predictive value of H-FABP for clinical outcome is not stronger than that of CK-MB, Troponin-I, or bypass times.

Percutaneous closure of multiple ventricular septal defects: simultaneous use of muscular ventricular septal defect device and Multi-Fenestrated Septal Occluder - "Cribriform" to close residual ventricular septal defects after complex cardiac surgery in a child.

A male child, with d-transposition of great arteries, a large perimembranous ventricular septal defect, multiple additional ventricular septal defects, small muscle-bound right ventricle, and severe pulmonary stenosis with confluent, moderate-sized branch pulmonary arteries, underwent an emergency right modified Blalock-Taussig shunt on day 15 of life and réparation à l'étageventriculaire procedure with ventricular septal defect closure with takedown of the Blalock-Taussig shunt at 2.5 years of age. On follow-up, he showed a moderate residual upper ventricular septal defect and multiple apical ventricular septal defects, mild mid-right pulmonary artery stenosis, free pulmonary regurgitation, and right ventricular dysfunction. Surgical re-intervention was deemed extremely risky, the upper muscular ventricular septal defect was closed using an 8-mm Amplatzer Muscular Ventricular Septal Defect Occluder Device, and an 18 mm Amplatzer Multi-Fenestrated Septal Occluder - Cribriform was used for the multiple apical muscular ventricular septal defects. After 1 year, his right pulmonary artery stenosis worsened, for which right pulmonary artery angioplasty was carried out using an 8×20 mm cutting balloon followed by a 10×20 mm Tyshak II balloon. This is the only case reported for the paediatric age group using a cribriform septal occluder device for percutaneous closure of multiple apical ventricular septal defects.

Preoperative Sildenafil administration in children undergoing cardiac surgery: a randomized controlled preconditioning study.

OBJECTIVES: Sildenafil has strong cardiac preconditioning properties in animal studies and has a safe side-effect profile in children. Therefore, we evaluated the application of Sildenafil preconditioning to reduce myocardial ischaemia/reperfusion injury in children undergoing surgical ventricular septal defect (VSD) closure. METHODS: This is a randomized, double-blind study. Children (1-17 years) undergoing VSD closure were randomized into three groups: placebo (Control group), preconditioning with 0.06 mg/kg (Sild-L group) and 0.6 mg/kg Sildenafil (Sild-H group). PRIMARY ENDPOINT: troponin release. CK-MB, Troponin I, inflammatory response (IL-6 and TNF-α), bypass and ventilation weaning times, inotropy score and echocardiographic function were assessed. Data expressed as median (range), and a value of P < 0.05 was considered significant. RESULTS: Thirty-nine patients were studied (13/group). Aortic cross-clamp time was similar [27 (18-85) and 27 (12-39) min] in the Control and Sild-L groups, respectively, but significantly longer [39 (20-96) min] in the Sild-H group when compared with the Control group. Area under the curve of CK-MB release was 1105 (620-1855) h ng/ml in the Control group, 1672 (564-2767) h ng/ml in the Sild-L group and was significantly higher in the Sild-H group [1695 (1252-3377) h ng/ml] when compared with the Control group. There were no significant differences in inflammatory response markers, cardiopulmonary bypass and ventilation weaning times, inotropy scores and echocardiographic function between the groups. CONCLUSIONS: In this small study, Sildenafil failed to reduce myocardial injury in children undergoing cardiac surgery, nor does it alter cardiac function, inotropic needs or postoperative course. A subclinical increase in cardiac enzyme release after Sildenafil preconditioning cannot be excluded. CLINICAL TRIALS REGISTRY: CTRI/2014/03/004468.

Renal sympathetic denervation using an externally irrigated radiofrequency ablation catheter for treatment of resistant hypertension - Acute safety and short term efficacy.

OBJECTIVES: This study was conducted to assess the acute safety and short term efficacy of renal sympathetic denervation (RSDN) using solid tip radiofrequency ablation (RFA) catheter and saline irrigation through the renal guiding catheter to achieve effective denervation. BACKGROUND: RSDN using a specialized solid-tip RFA catheter has recently been demonstrated to safely reduce systemic blood pressure in patients with refractory hypertension, the limitation being inadequate power delivery in renal arteries. So, we used solid-tip RFA catheter along with saline irrigation for RSDN. METHODS: Nine patients with resistant hypertension underwent CT and conventional renal angiography, followed by bilateral or unilateral RSDN using 5F RFA catheter with saline irrigation through renal guiding catheter. Repeat renal angiography was performed at the end of the procedure. In all patients, pre- and post-procedure serum creatinine was measured. RESULTS: Over 1-month period: 1) the systolic/diastolic blood pressure decreased by -57 ± 20/-25 ± 7.5 mm Hg; 2) all patients experienced a decrease in systolic blood pressure of at least -36 mm Hg (range 36-98 mm Hg); 3) there was no evidence of renal artery injury immediate post-procedure. There was no significant change in serum creatinine level. CONCLUSIONS: This data shows the acute procedural safety and short term efficacy of RSDN using modified externally irrigated solid tip RFA catheter.

Early postoperative outcomes following surgical repair of complete atrioventricular septal defects: is down syndrome a risk factor?

OBJECTIVE: To evaluate the impact of Down syndrome on the early postoperative outcomes of children undergoing complete atrioventricular septal defect repair. DESIGN: Retrospective cohort study. SETTING: Single tertiary pediatric cardiac center. PATIENTS: All children admitted to PICU following biventricular surgical repair of complete atrioventricular septal defect from January 2004 to December 2009. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 107 children, 67 with Down syndrome, were included. Children with Down syndrome were operated earlier: 4 months (interquartile range, 3.5-6.6) versus 5.7 months (3-8.4) for Down syndrome and non-Down syndrome groups, respectively (p < 0.01). There was no early postoperative mortality. There was no significant difference in the prevalence of dysplastic atrioventricular valve between the two groups. Two children (2.9%) from Down syndrome and three children (7.5%) from non-Down syndrome group required early reoperation (p = 0.3). Junctional ectopic tachycardia was the most common arrhythmia, and the prevalence of junctional ectopic tachycardia was similar between the two groups (9% and 10% in Down syndrome and non-Down syndrome, respectively, p = 1). One patient from each group required insertion of permanent pacemaker for complete heart block. Children with Down syndrome had significantly higher prevalence of noncardiac complications, that is, pneumothorax, pleural effusions, and infections (p < 0.01), than children without Down syndrome. There was a trend for longer duration of mechanical ventilation in children with Down syndrome (41 hr [20-61 hr] vs 27.5 hr [15-62 hr], p = 0.2). However, there was no difference in duration of PICU stay between the two groups (2 d [1.3-3 d] vs 2 d [1-3 d], p = 0.9, respectively). CONCLUSIONS: In our study, we found no difference in the prevalence of atrioventricular valve dysplasia between children with and without Down syndrome undergoing complete atrioventricular septal defect repair. This finding contrasts with previously published data, and further confirmatory studies are required. Although clinical outcomes were similar, children with Down syndrome had a significantly higher prevalence of noncardiac complications in the early postoperative period than children without Down syndrome.

Endovascular exclusion of complex postsurgical aortic arch pseudoaneurysm using vascular plug devices and a review of vascular plugs.

We report the management of a patient presenting with haemoptysis due to aortobronchial fistula. He had previously undergone emergency exclusion bypass of a ruptured pseudoaneurysm developing post-aortic coarctation repair. Computed tomography scan showed persistent filling of pseudoaneurysm sac from proximal and distal aortic ligature sites tied during previous exclusion bypass surgery. Successful exclusion of aneurysm was achieved by using 3 vascular plug devices (1 Amplatzer plug II and 2 Amender patent ductus arteriosus occluder devices). We also review types of Amplatzer vascular plugs and their use in peripheral vascular interventions.