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Transcatheter closure of large patent ductus arteriosus using custom made devices.

Catheter Cardiovasc Interv. 2017 May;89(6):E194-E199

Authors: Rohit MK, Gupta A

There has been a paradigm shift in the transcatheter closure of patent ductus arteriosus (PDA) over the last 45 years. With the availability of various coils, plugs and occluders, PDA of almost all shapes and sizes are amenable to transcatheter closure. However, very large PDA diagnosed late in life are being referred for surgical closure in the absence of availability of large size devices, especially in developing countries. In this case series, we have described four patients with large PDA, three of which were closed by transcatheter custom made PDA occluders. © 2014 Wiley Periodicals, Inc.

PMID: 24323820 [PubMed - indexed for MEDLINE]

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Single-center experience in percutaneous closure of arterial duct with Amplatzer duct Occluder II additional sizes.

Catheter Cardiovasc Interv. 2017 May;89(6):1045-1050

Authors: Mahmoud HT, Santoro G, Gaio G, D'Aiello FA, Capogrosso C, Palladino MT, Russo MG

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

PMID: 27862909 [PubMed - indexed for MEDLINE]

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

Catheter Cardiovasc Interv. 2017 May;89(6):1035-1043

Authors: Dalvi B, Sheth K, Jain S, Pinto R

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.

PMID: 27862916 [PubMed - indexed for MEDLINE]

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The Congenital Heart Disease Genetic Network Study: Cohort description.

PLoS One. 2018;13(1):e0191319

Authors: Hoang TT, Goldmuntz E, Roberts AE, Chung WK, Kline JK, Deanfield JE, Giardini A, Aleman A, Gelb BD, Mac Neal M, Porter GA, Kim R, Brueckner M, Lifton RP, Edman S, Woyciechowski S, Mitchell LE, Agopian AJ

The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010-2014. Information on cases (N = 9,727) and their parents was collected through interviews and medical record abstraction. Four case characteristics, eleven parental characteristics, and thirteen parent-reported neurodevelopment outcomes were summarized using counts and frequencies and compared across CHD types and subtypes. Eleven percent of cases had a genetic diagnosis. Among cases without a genetic diagnosis, the majority had conotruncal heart defects (40%) or left ventricular outflow tract obstruction (21%). Across CHD types, there were significant differences (p<0.05) in the distribution of all four case characteristics (e.g., sex), four parental characteristics (e.g., maternal pregestational diabetes), and five neurodevelopmental outcomes (e.g., learning disabilities). Several characteristics (e.g., sex) were also significantly different across CHD subtypes. The PCGC cohort is one of the largest CHD cohorts available for the study of genetic determinants of risk and outcomes. The majority of cases do not have a genetic diagnosis. This description of the PCGC cohort, including differences across CHD types and subtypes, provides a reference work for investigators who are interested in collaborating with or using publically available resources from the PCGC.

PMID: 29351346 [PubMed - indexed for MEDLINE]

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The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome.

BMC Psychiatry. 2017 Jul 17;17(1):253

Authors: Benke K, Ágg B, Pólos M, Sayour AA, Radovits T, Bartha E, Nagy P, Rákóczi B, Koller Á, Szokolai V, Hedberg J, Merkely B, Nagy ZB, Szabolcs Z

BACKGROUND: Marfan syndrome is a genetic disease, presenting with dysfunction of connective tissues leading to lesions in the cardiovascular and skeletal muscle system. Within these symptoms, the most typical is weakness of the connective tissue in the aorta, manifesting as aortic dilatation (aneurysm). This could, in turn, become annuloaortic ectasia, or life-threatening dissection. As a result, life-saving and preventative cardiac surgical interventions are frequent among Marfan syndrome patients. Aortic aneurysm could turn into annuloaortic ectasia or life-threatening dissection, thus life-saving and preventive cardiac surgical interventions are frequent among patients with Marfan syndrome. We hypothesized that patients with Marfan syndrome have different level of anxiety, depression and satisfaction with life compared to that of the non-clinical patient population.
METHODS: Patients diagnosed with Marfan syndrome were divided into 3 groups: those scheduled for prophylactic surgery, those needing acute surgery, and those without need for surgery (n = 9, 19, 17, respectively). To examine the psychological features of the patients, Spielberger's anxiety (STAI) test, Beck's Depression questionnaire (BDI), the Berne Questionnaire of Subjective Well-being, and the Satisfaction with Life scale were applied.
RESULTS: A significant difference was found in trait anxiety between healthy individuals and patients with Marfan syndrome after acute life-saving surgery (p < 0.01). The mean score of Marfan syndrome patients was 48.56 (standard deviation (SD): 5.8) as compared to the STAI population mean score of 43.72 (SD: 8.53). No difference was found between groups on the BDI (p > 0.1). Finally, a significant, medium size effect was found between patient groups on the Joy in Living scale (F (2.39) = 3.51, p = 0.040, η2 = 0.15).
CONCLUSIONS: Involving psychiatric and mental-health care, in addition to existing surgical treatment interventions, is essential for more successful recovery of patients with Marfan syndrome.

PMID: 28716062 [PubMed - indexed for MEDLINE]

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Impact of sample collection participation on the validity of estimated measures of association in the National Birth Defects Prevention Study when assessing gene-environment interactions.

Genet Epidemiol. 2017 Dec;41(8):834-843

Authors: Jenkins MM, Reefhuis J, Herring AH, Honein MA

To better understand the impact that nonresponse for specimen collection has on the validity of estimates of association, we examined associations between self-reported maternal periconceptional smoking, folic acid use, or pregestational diabetes mellitus and six birth defects among families who did and did not submit buccal cell samples for DNA following a telephone interview as part of the National Birth Defects Prevention Study (NBDPS). Analyses included control families with live born infants who had no birth defects (N = 9,465), families of infants with anorectal atresia or stenosis (N = 873), limb reduction defects (N = 1,037), gastroschisis (N = 1,090), neural tube defects (N = 1,764), orofacial clefts (N = 3,836), or septal heart defects (N = 4,157). Estimated dates of delivery were between 1997 and 2009. For each exposure and birth defect, odds ratios and 95% confidence intervals were calculated using logistic regression stratified by race-ethnicity and sample collection status. Tests for interaction were applied to identify potential differences between estimated measures of association based on sample collection status. Significant differences in estimated measures of association were observed in only four of 48 analyses with sufficient sample sizes. Despite lower than desired participation rates in buccal cell sample collection, this validation provides some reassurance that the estimates obtained for sample collectors and noncollectors are comparable. These findings support the validity of observed associations in gene-environment interaction studies for the selected exposures and birth defects among NBDPS participants who submitted DNA samples.

PMID: 29071735 [PubMed - indexed for MEDLINE]

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Accessory mitral valve mimicking aortic valve endocarditis as a cause of cerebrovascular accident.

J Card Surg. 2017 Nov;32(11):691-693

Authors: Degner BC, Gaudino M, Iannacone E, Lau C, Girardi LN

Accessory mitral valve (AMV) is a rare congenital cardiac malformation associated with left ventricular outflow tract obstruction and endocarditis. We report a patient with an AMV who presented with a cerebellar infarct and discuss the management of this anomaly.

PMID: 29169202 [PubMed - indexed for MEDLINE]

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Outcomes of Patients With Hypoplastic Left Heart Syndrome Reaching Adulthood After Fontan Palliation: Multicenter Study.

Circulation. 2018 Feb 27;137(9):978-981

Authors: Wilson WM, Valente AM, Hickey EJ, Clift P, Burchill L, Emmanuel Y, Gibson P, Greutmann M, Grewal J, Grigg LE, Gurvitz M, Hickey K, Khairy P, Mayer JE, Teo E, Vonder Muhll I, Roche SL, Silversides CK, Wald RM

PMID: 29483175 [PubMed - in process]

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Outcomes of Totally Endoscopic Atrial Septal Defect Closure Using a Glutaraldehyde-Treated Autologous Pericardial Patch.

Circ J. 2017 Apr 25;81(5):689-693

Authors: Nishida H, Nakatsuka D, Kawano Y, Hiraiwa N, Takanashi S, Tabata M

BACKGROUND: We evaluated the outcomes of totally endoscopic minimally invasive surgery for atrial septal defect (ASD) using a glutaraldehyde-treated autologous pericardial patch in the transcatheter interventional era.Methods and Results:We retrospectively reviewed 37 consecutive patients who underwent totally endoscopic ASD closure with a glutaraldehyde-treated autologous pericardial patch between June 2011 and April 2015. All patients had been deferred from catheter-based intervention for clinical or anatomical reasons. We analyzed operative outcomes and postoperative echocardiographic data. The mean age was 45.7±16.5 years, and 25 patients (67.6%) were women. The mean ratio of pulmonary to systemic flow was 2.4±0.7. Six patients (16.2%) underwent concomitant tricuspid valve repair, and 3 patients (8.1%) underwent concomitant atrial fibrillation surgery. There were no operative deaths, and the median length of hospital stay was 5 days. Postoperative echocardiography revealed trivial residual shunt in 1 patient. During the follow-up period, there were no re-interventions for ASD or readmission for heart failure. Follow-up echocardiography revealed no recurrent shunt or calcification of the autologous pericardial patch.
CONCLUSIONS: Totally endoscopic ASD closure with a glutaraldehyde-treated autologous pericardial patch demonstrated excellent outcomes. It is a useful option for patients with unfavorable anatomy or other reasons excluding transcatheter intervention.

PMID: 28179595 [PubMed - indexed for MEDLINE]

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Atrial arrhythmias in adults with congenital heart disease. Listening to your heart sound can save your life.

Int J Cardiol. 2017 12 01;248:159-160

Authors: Limongelli G, Sarubbi B

PMID: 28942871 [PubMed - indexed for MEDLINE]