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Pulmonary Artery Stenting.

Interv Cardiol Clin. 2019 Jan;8(1):33-46

Authors: Zablah JE, Morgan GJ

Stent implantation for pulmonary artery stenosis has advanced. Newer generations of stents allow confidence in long-term results, even in pediatric interventions. Primary intravascular stent implantation is recommended in significant branch pulmonary artery stenosis when the vessel or patient is large enough to accommodate a stent that can be dilated to an adult diameter. A variety of specialized stents are now available, improving applicability despite complex vessel size characteristics. Developments in bioresorbable stents and patient-specific rapid prototyping are anticipated.

PMID: 30449420 [PubMed - in process]

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Transcatheter Pulmonary Valve Replacement in Congenital Heart Disease.

Interv Cardiol Clin. 2019 Jan;8(1):59-71

Authors: Sinha S, Aboulhosn J, Levi DS

Patients with dysfunctional right ventricular outflow tracks comprise a large portion of patients with severe congenital heart disease. Transcatheter pulmonary valve replacement in patients with dysfunctional right ventricular outflow tracks is feasible, safe, and efficacious. This article reviews current transcatheter valve replacement technology for dysfunctional right ventricular outflow tract and pulmonary valvular disease and its applications to patients with congenital heart disease. Discussed are the approach and preprocedural planning, current options, and applications of transcatheter pulmonary valve therapy. Also considered are future directions in this field as the technologies begin to develop further.

PMID: 30449422 [PubMed - in process]

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Biodegradable Stents for Congenital Heart Disease.

Interv Cardiol Clin. 2019 Jan;8(1):81-94

Authors: Welch TR, Nugent AW, Veeram Reddy SR

The quest for an ideal biodegradable stent for both adult coronary and pediatric congenital heart disease applications continues. Over the past few years, a lot of progress has been made toward development of a dedicated pediatric biodegradable stent that can be used for congenital heart disease applications. At present, there are no biodegradable stents available for use in congenital heart disease. In this article, the authors review the different biodegradable materials and their limitations and provide an overview of the current biodegradable stents being evaluated for congenital heart disease applications.

PMID: 30449424 [PubMed - in process]

Predictors and rates of recurrence of atrial arrhythmias following catheter ablation in adults with congenital heart disease.

Congenit Heart Dis. 2018 Nov 19;:

Authors: Lewis M, Whang W, Biviano A, Hickey K, Garan H, Rosenbaum M

BACKGROUND: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined.
OBJECTIVE: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias.
METHODS: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review.
RESULTS: Among 124 patients (mean age: 45 years) who underwent catheter ablation, 96 (77%) were treated for macro-reentrant atrial tachycardia, 10 (7%) for focal atrial tachycardia, 9 (7%) for atrial fibrillation, 7 (6%) for atrioventricular nodal reentrant tachycardia, and 2 (2%) for atrioventricular reentrant tachycardia. 15 (12%) required transseptal/transbaffle puncture. Fifty-one percent of patients recurred with a median time to recurrence of 1639 days. By univariate and multivariable analysis, body mass index (BMI) and Fontan status were the only variables associated with recurrence. Dose-dependent effect was observed with overweight (HR = 2.37, P = .012), obese (HR = 2.67, P = .009), and morbidly obese (HR = 4.23, P = .003) patients demonstrating an increasing risk for arrhythmia recurrence postablation. There was no significant different in recurrence rates by gender, age, non-Fontan diagnosis, or need for transseptal puncture.
CONCLUSIONS: In our cohort of ACHD patients, BMI was a significant risk factor for arrhythmia recurrence postablation, independent of Fontan status. These findings may help guide treatment decisions for persistent arrhythmias in the ACHD population.

PMID: 30451375 [PubMed - as supplied by publisher]

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery.

Congenit Heart Dis. 2018 Nov 19;:

Authors: Poterucha JT, Vallabhajosyula S, Egbe AC, Krien JS, Aganga DO, Holst K, Golden AW, Dearani JA, Crow SS

BACKGROUND: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
METHODS: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admission were examined to determine the relationship between VIS score and poor outcome of early mortality, early morbidity, or complication related morbidity.
RESULTS: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty-two patients (25%), experienced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty-eight patients (15%) endured complication related early morbidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86-0.98) for in-hospital mortality; and 0.82 (95% CI: 0.76-0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of composite adverse outcome (OR: 14.2, 95% CI: 7.2-28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7-42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4-41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4-15.5; P < 0.0001).
CONCLUSIONS: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.

PMID: 30451381 [PubMed - as supplied by publisher]

Historical investigation of medical treatment for adult congenital heart disease: A Canadian perspective.

Congenit Heart Dis. 2018 Nov 19;:

Authors: Lapum JL, Fredericks S, Bailey B, Yau TM, Graham J, Marelli AJ

OBJECTIVE: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?
DESIGN/METHODS: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order to articulate a contextualized understanding of the evolution of adult CHD treatment in Canada. The informants recruited were currently or previously located in and/or involved in treatment in seven provinces and two territories across Canada including patients, families, advocates, researchers, and practitioners located in disciplines that included cardiology, cardiovascular surgery, nursing, psychology, dietetics, and kinesiology.
RESULTS: Alongside findings that highlighted the significant demographic shift, the findings highlight key themes related to temporal shifts in treatment, emergence of organizational structures and use of evidence, comprehensive approaches to care, and future directions. A critical finding that requires immediate attention is the significantly disproportionate resources to the number of adults living with CHD, and as a result, the real risk of premature death for this population.
CONCLUSIONS: The insights provided behoove the community of stakeholders to think creatively on how to draw attention to the inadequacy of resources and the unique and diverse needs of this population.

PMID: 30451387 [PubMed - as supplied by publisher]

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Psychosocial functioning of parents of children with heart disease-describing the landscape.

Eur J Pediatr. 2018 Dec;177(12):1811-1821

Authors: Wray J, Cassedy A, Ernst MM, Franklin RC, Brown K, Marino BS

The aim was to describe the psychological functioning of parents of school-age children with heart disease (HD) in a large-scale, transnational evaluation of parent dyads across the spectrum of cardiac diagnoses and a range of psychosocial domains. Parents of children with HD attending routine out-patient cardiology follow-up visits completed questionnaires assessing their mental health, coping, and family functioning. Parents (1197 mothers and 1053 fathers) of 1214 children (mean age: 12.6 years; S.D. 3.0 years; median time since last surgery: 8.9 years) with congenital or acquired HD from three centers each in the UK and the USA participated (80% response rate). Parents of children with milder HD demonstrated few differences from healthy norms and had significantly lower scores on measures of illness-related stress and post-traumatic stress than parents of children with single ventricle conditions or cardiomyopathy. Parents in these latter two diagnostic sub-groups had significantly higher levels of anxiety and depression than healthy norms but did not differ on other measures of family functioning and coping skills. There were few differences between parents from the UK and the USA. Agreement between mothers and fathers within a dyad was highest for the measure of frequency of illness-related stressors (ICC = 0.67) and lowest for anxiety (ICC = 0.12).Conclusion: Our results suggest two different pathways for the long-term psychological well-being of parents of children with HD: on the one hand, more complex HD is associated with poorer long-term psychosocial outcomes; in contrast, there are also grounds for optimism, particularly for parents of children with less complex conditions, with better psychological outcomes noted for some groups of parents compared to previously reported early psychosocial outcomes. Future work needs to identify factors other than disease severity which might explain poorer (or better) functioning in some parents of children with more complex HD. What is Known: • Parents of children with congenital heart disease report elevated levels of anxiety, depression, and stress after cardiac surgery in infancy. • Maternal mental health problems can have an adverse impact on the psychological adjustment of the child with congenital heart disease. What is New: • Parents of children with milder forms of heart disease do not differ from healthy norms in the longer term and psychological outcomes are better than might be expected from early findings. • More complex diagnoses, particularly functional single ventricle conditions and cardiomyopathy, are associated with poorer long-term psychosocial outcomes for parents.

PMID: 30232593 [PubMed - indexed for MEDLINE]

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Two case reports of neonatal autoantibody-associated congenital heart block.

Medicine (Baltimore). 2018 Nov;97(45):e13185

Authors: Li X, Huang X, Lu H

RATIONALE: Neonatal lupus erythematosus (NLE) is an infrequent disease caused by transplacental maternal autoantibodies. The most common effects of NLE include cutaneous involvement and congenital heart block (CHB), although it might involve multiple organs, such as the liver, lungs, blood, and nervous or digestive systems. Izmirly PM1 and Tonello et al recently reported cutaneous manifestations of neonatal lupus and risk of subsequent CHB. The most serious complication of NLE is complete atrioventricular (AV) block.
PATIENT CONCERNS: We experienced 2 cases of NLE that were diagnosed in the past year in our Neonatal Intensive Care Unit. These cases showed 2 different clinical spectrums (CHB, multisystemic effects). One case was a 32-week pregnant woman with combined liver damage and fever, and her fetus was premature due to bradycardia and pericardial effusion. The second case was a young pregnant woman who had systemic lupus erythematosus for 2 years and had been taking methylprednisolone and hydroxychloroquine for a long time since her illness. When prenatal testing at 28 weeks of pregnancy showed that the fetus had CHB, the mother began taking dexamethasone.
DIAGNOSIS: The first case was diagnosed as NLE with CHB after birth, while the second was diagnosed as NLE with CHB, ductus arteriosus, and atrial septal defect when she was born at 34 weeks.
INTERVENTIONS: Both of 2 cases were treated with steroids, intravenous immunoglobulin, and a diuretic. But the second case was treated with isoprenaline in addition to the above.
OUTCOMES: Both of the infants was followed up and found to be clinically normal. During the clinic follow-up of the first case, the 8-month-old infant was still asymptomatic with normal growth and development. Her heart rate fluctuated from 40 to 90 beats/minute.
LESSONS: Autoimmune CHB is a severe, potentially life-threatening disorder associated with passive transfer of maternal anti-Sjogren's syndrome A/Ro and anti-Sjogren's syndrome B/La autoantibodies. Mothers who are positive for these autoantibodies are recommended to have serial echocardiography and obstetric ultrasonography from the early second trimester. Newborns should be delivered at an early stage of gestation if there is evidence of pericardial effusion, ascites, increasing ventricular ectopy, reduced ventricular shortening fraction, or AV valve regurgitation. Aggressive medical management after birth should be coupled with pacemaker implantation in infants who do not respond to medical therapies alone.

PMID: 30407356 [PubMed - indexed for MEDLINE]

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Computed Tomography in Adult Congenital Heart Disease.

Radiol Clin North Am. 2019 Jan;57(1):85-111

Authors: Ranganath P, Singh S, Abbara S, Agarwal PP, Rajiah P

The prevalence of adult congenital heart disease (ACHD) is increasing due to advances in surgical techniques, anesthesia, and perioperative care. Imaging plays an important role, not only in the surveillance of ACHD, but also in the initial evaluation of cases that have escaped detection early in life and present with symptoms later in adulthood. In this article, we review the role of computed tomography in the comprehensive evaluation of ACHD.

PMID: 30454820 [PubMed - in process]

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Adult Congenital Heart Disease Care Provision: the Need for a Jack/Jackie of All Trades.

Prog Cardiovasc Dis. 2018 Sep - Oct;61(3-4):273-274

Authors: Krasuski RA

PMID: 30454840 [PubMed - in process]