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Longitudinal Assessment of the Doppler-Estimated Maximum Gradient in Patients With Congenital Valvar Aortic Stenosis Pre- and Post-Balloon Valvuloplasty.

Circ Cardiovasc Imaging. 2018 03;11(3):e006708

Authors: Kuebler JD, Shivapour J, Yaroglu Kazanci S, Gauvreau K, Colan SD, McElhinney DB, Brown DW

BACKGROUND: Aortic stenosis has been reported to manifest a slow rate of progression in mild disease, with a greater likelihood of progression in patients with moderate-severe disease. The natural history of the Doppler-estimated maximum gradient (DEMG) in patients after balloon aortic valvuloplasty (BAVP) has not previously been studied on a large scale.
METHODS AND RESULTS: A retrospective review was performed of 360 patients from 1984 to 2012 with aortic stenosis, providing a total of 2059 echocardiograms both before and after BAVP. Patients were excluded if they had an intervention within the first 30 days of life. The relationships between the aortic stenosis DEMG and several predictors (age at initial study, body surface area, valve morphology, and initial DEMG) were explored using linear mixed effect models. Patients with a unicommissural aortic valve had a significantly higher rate of progression compared with those with a bicommissural aortic valve (0.81 and 0.45 mm Hg/year; P<0.001). The median rate of progression in the post-BAVP group was significantly lower than the median pre-BAVP rate of progression (n=34; pre-BAVP 3.97 [1.69-8.7] mm Hg/year; post-BAVP 0.40 [-1.80 to 3.88] mm Hg/year; P<0.008). When adjusted for body surface area, there was no significant increase in the DEMG (-0.03 mm Hg/m2 per year; P<0.001).
CONCLUSIONS: There is a statistically significant increase in the DEMG over time in patients with aortic stenosis. After balloon dilation, the DEMG rate of change is reduced compared with that pre-dilation. Given the effect of body surface area on DEMG progression, more frequent observation should be made during periods of rapid somatic growth.

PMID: 29555832 [PubMed - indexed for MEDLINE]

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Long-Term Risk of Cardiovascular Disease in Women Who Have Had Infants With Heart Defects.

Circulation. 2018 05 29;137(22):2321-2331

Authors: Auger N, Potter BJ, Bilodeau-Bertrand M, Paradis G

BACKGROUND: The possibility that congenital heart defects signal a familial predisposition to cardiovascular disease has not been investigated. We aimed to determine whether the risk of cardiovascular disorders later in life was higher for women who have had newborns with congenital heart defects.
METHODS: We studied a cohort of 1 084 251 women who had delivered infants between 1989 and 2013 in Quebec, Canada. We identified women whose infants had critical, noncritical, or no heart defects, and tracked the women over time for future hospitalizations for cardiovascular disease, with follow-up extending up to 25 years past pregnancy. We calculated the incidence of cardiovascular hospitalization per 1000 person-years, and used Cox proportional hazards regression to estimate hazard ratios and 95% confidence intervals (CIs) for the association between infant heart defects and risk of maternal cardiovascular hospitalization. Models were adjusted for age, parity, preeclampsia, comorbidity, material deprivation, and time period.
RESULTS: Women whose infants had heart defects had a higher overall incidence of cardiovascular hospitalization. There were 3.38 cardiovascular hospitalizations per 1000 person-years for those with critical defects (95% CI, 2.67-4.27), 3.19 for noncritical defects (95% CI, 2.96-3.45), and 2.42 for no heart defects (95% CI, 2.39-2.44). In comparison with no heart defects, women whose infants had critical defects had a hazard ratio of 1.43 (95% CI, 1.13-1.82) for any cardiovascular hospitalization, and women whose infants had noncritical defects had a hazard ratio of 1.24 (95% CI, 1.15-1.34), in adjusted models. Risks of specific causes of cardiovascular hospitalization, including myocardial infarction, heart failure, and other atherosclerotic disorders, were also greater for mothers of infants with congenital heart defects than with no defects.
CONCLUSIONS: Women whose infants have congenital heart defects have a greater risk of cardiovascular hospitalization later in life. Congenital heart defects in offspring may be an early marker of predisposition to cardiovascular disease.

PMID: 29610262 [PubMed - indexed for MEDLINE]

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Temporal trends in secondary prevention in myocardial infarction patients discharged with left ventricular systolic dysfunction in Poland.

Eur J Prev Cardiol. 2018 06;25(9):960-969

Authors: Gasior M, Gierlotka M, Pyka Ł, Zdrojewski T, Wojtyniak B, Chlebus K, Rozentryt P, Niedziela J, Jankowski P, Nessler J, Opolski G, Hoffman P, Jankowska E, Polonski L, Ponikowski P

Background The proportion of patients discharged after myocardial infarction with left ventricular systolic dysfunction remains high and the prognosis is unfavourable. The aim of this study was to analyse the temporal trends in the treatment and outcomes of a nationwide cohort of patients. Methods and results Data from the Polish Registry of Acute Coronary Syndromes and Acute Myocardial Infarction in Poland Registry were combined to achieve complete information on inhospital course, treatment and outcomes. An all-comer population of patients discharged with left ventricular ejection fraction of 40% or less formed the sample population ( n = 28,080). The patients were analysed for the incidence of significant temporal trends and their possible consequences. The implementation of guideline-based treatment at discharge was high. In the post-discharge course a trend towards a higher frequency of percutaneous coronary intervention and a lower prevalence of planned coronary artery bypass grafting procedures was observed. The number of implantable cardioverter defibrillator/cardiac resynchronisation therapy defibrillator implantations was increasing. Cardiac rehabilitation was performed in 19-23% cases. The post-discharge outpatient care was based on general practitioner visits, with only 47.9-48.1% of patients attending an ambulatory cardiology specialist visit. In 12 months of observation the frequency of heart failure rehospitalisations was 17.5-19.1%, while the prevalence of rehospitalisations due to myocardial infarction decreased (8.3% in 2009 to 6.7% in 2013, P < 0.001). A trend towards lower all-cause mortality was observed. Assessment of composite outcomes (death, myocardial infarction, stroke or heart failure rehospitalisation) adjusted for sex and age at 12 months revealed a significant decreasing trend. Conclusion The overall prognosis in this population is improving slowly. This may be due to the increasing prevalence of guideline-based forms of secondary prevention. Efforts aimed at maintaining these trends are essential, as overall compliance with these guideline remains suboptimal.

PMID: 29692221 [PubMed - indexed for MEDLINE]

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Congenital heart disease: New challenges.

Rev Port Cardiol. 2018 11;37(11):933-934

Authors: Santana I

PMID: 30458974 [PubMed - indexed for MEDLINE]

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Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry.

Ter Arkh. 2019 Mar 10;91(1):24-31

Authors: Chazova IE, Arkhipova OA, Martynyuk TV

AIM: Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry.
MATERIALS AND METHODS: In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug- and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017.
RESULTS: Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug- and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years.
CONCLUSION: The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.

PMID: 31090367 [PubMed - indexed for MEDLINE]

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Very long-term outcomes of transcatheter secundum atrial septal defect closure using intracardiac echocardiography without balloon sizing.

Clin Radiol. 2019 Jun 20;:

Authors: Rigatelli G, Nghia NT, Zuin M, Conte L, D'Elia K, Nanjundappa A

AIM: To assess the long-term outcomes of device-based closure of atrial septal defects (ASDs) with no sizing balloon.
MATERIAL AND METHODS: Two hundred and eighty-one consecutive patients (mean age 34±13 years, 178 women) underwent intracardiac echocardiography (ICE)-aided transcatheter closure of secundum ASDs over a 15-year period (September 2002 to March 2017). Sizing of the ASDs was calculated under ICE guidance (UltraICE, EP Technologies, Boston Scientific Corporation, San Jose, CA, USA) using the concept of "supportive rim" for ASDs without the aid of a sizing balloon. Follow-up was conducted by transoesophageal and transthoracic echocardiography.
RESULTS: The procedure was carried out successfully in all patients with 0% related mortality and 5.7% procedural complications. The Amplatzer ASD Occluder was implanted in 251 patients (89.3%, mean size 26.4±10.2 mm) whereas the Gore Cardioform was used in 30 patients (10.6%). Over 10.3±3 years of follow-up (range 1-15) 100% of patients were alive. The complete occlusion rate was 97.8%. No aortic or atrial free wall erosions, device thrombosis, or device frame fractures were detected during the follow-up period.
CONCLUSION: The present study suggested that ICE-guided closure of ASDs with current devices without sizing balloons is safe and effective with very low procedural and late complications even in the very long-term follow-up.

PMID: 31230756 [PubMed - as supplied by publisher]

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Sarcopenia is common in adults with complex congenital heart disease.

Int J Cardiol. 2019 Jun 10;:

Authors: Sandberg C, Johansson K, Christersson C, Hlebowicz J, Thilén U, Johansson B

BACKGROUND: Adults with complex congenital heart disease (CHD) have reduced aerobic capacity and impaired muscle function. We therefore hypothesized that patients have a lower skeletal muscle mass and higher fat mass than controls.
METHODS: Body composition was examined with full body Dual-Energy x-ray Absorptiometry (DXA) in 73 patients with complex CHD (mean age 35.8 ± 14.3, women n = 22) and 73 age and sex matched controls. Patients fulfilling criteria for low skeletal muscle mass in relation to their height and fat mass were defined as sarcopenic.
RESULTS: Male patients (n = 51) were shorter (177.4 ± 6.6 cm vs. 180.9 ± 6.7 cm, p = 0.009) and weighed less (76.0 ± 10.8 kg vs. 82.0 ± 12.4 kg, p = 0.01) than controls. Also, patients had a lower appendicular lean mass-index (ALM-index) (7.57 ± 0.97 kg/m2vs. 8.46 ± 0.90 kg/m2, p < 0.001). Patients' relative tissue fat mass (27.9 ± 7.0% vs. 25.4 ± 8.6%, p = 0.1) did not differ. Forty-seven percent of the men (n = 24) were classified as sarcopenic. Female patients (n = 22) were also shorter (163.5 ± 8.7 cm vs. 166.7 ± 5.9 cm, p = 0.05) but had a higher BMI (25.7 ± 4.2 vs. 23.0 ± 2.5, p = 0.02) than controls. Patients also had a lower ALM-index (6.30 ± 0.75 vs. 6.67 ± 0.55, p = 0.05), but their relative body fat mass (40.8 ± 7.6% vs. 32.0 ± 7.0%, p < 0.001) were higher. Fifty-nine percent of the women (n = 13) were classified as sarcopenic.
CONCLUSIONS: The body composition was altered toward lower skeletal muscle mass in patients with complex CHD. Approximately half of the patients were classified as sarcopenic. Contrary to men, the women had increased body fat and a higher BMI. Further research is required to assess the cause, possible adverse long-term effects and whether sarcopenia is preventable or treatable.

PMID: 31230936 [PubMed - as supplied by publisher]

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Clinical outcomes after Descemet's stripping endothelial keratoplasty using donor corneas from children younger than 3 years.

Clin Exp Ophthalmol. 2018 09;46(7):721-729

Authors: Peng RM, Guo YX, Qiu Y, Hong J, Xiao GG, Qu HQ

IMPORTANCE: There is limited literature on paediatric donors in endothelial keratoplasty.
BACKGROUND: This study investigated the efficacy of and appropriate paediatric donor age for Descemet's stripping endothelial keratoplasty (DSEK).
DESIGN: Retrospective and observational case series.
PARTICIPANTS: Thirty-eight consecutive patients underwent DSEK with paediatric donor corneas.
METHODS: The age of the donors ranged from 32 weeks gestation (premature neonate) to 3 years old. All donor consents were obtained from the parents. The causes of donor death included traffic accident, congenital heart disease and neonatal respiratory distress syndrome.
MAIN OUTCOME MEASURES: The outcome measures included best-corrected visual acuity, endothelial cell loss and complications.
RESULTS: Best-corrected visual acuity at last follow-up was >20/40 in 28 of 38 eyes (73.7%). The mean preoperative endothelial cell density of donor corneas was 4682 ± 520 cells/mm2 . The mean endothelial cell density of grafts was 3977 ± 556 cells/mm2 at 18 months postoperatively. Three lenticules from premature neonate donors exhibited severe contraction postoperatively. The edge of six lenticules from donors <1-year-old exhibited contraction in the early postoperative period and gradually flattened spontaneously. Graft detachment occurred in one patient.
CONCLUSIONS AND RELEVANCE: DSEK with paediatric donor corneas can achieve good clinical outcomes. The corneal lenticules from 1- to 3-year- old donors are suitable for DSEK while those from donors <1-year-old are less suitable due to the possibility of severe postoperative graft contraction.

PMID: 29498188 [PubMed - indexed for MEDLINE]

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Comorbidities, Sociodemographic Factors, and Hospitalizations in Outpatients With Heart Failure and Preserved Ejection Fraction.

Am J Cardiol. 2018 05 15;121(10):1207-1213

Authors: Georgiopoulou VV, Velayati A, Burkman G, Li S, Farooq K, Samman-Tahhan A, Papadimitriou L, Butler J, Kalogeropoulos AP

Patients with heart failure and preserved ejection fraction (HFpEF) tend to be older and have a high co-morbidity burden. The impact of co-morbid conditions and sociodemographic risk factors on outcomes in these patients has not been quantified. We evaluated 445 consecutive outpatients with HFpEF, defined as established diagnosis of heart failure (HF) with left ventricular ejection fraction at presentation >40% and no previous left ventricular ejection fraction ≤40%. Patients with specific cardiomyopathies, congenital heart disease, primary right-sided disease, valvular disease, or previous advanced HF therapies were excluded. After 2 years, there were 44 deaths and 609 all-cause hospitalizations; of these, 260 (42.7%) were cardiovascular hospitalizations, including HF, and 173 (28.4%) were specifically for HF. The highest attributable risk for hospitalizations was associated with marital status (single, divorced, and widowed had higher hospitalization rates compared with married patients), hypoalbuminemia, diabetes, atrial fibrillation, and renal dysfunction. The proportion of hospitalizations potentially attributable to these factors was 66.6% (95% confidence interval [CI] 56.4 to 74.4) for all-cause hospitalizations, 76.9% (95% CI 65.2 to 84.6) for cardiovascular hospitalizations, and 83.0% (95% CI 70.3 to 90.3) for HF hospitalizations. For composite end points, the proportion was 46.9% (95% CI 34.0% to 57.3%) for death or all-cause hospitalization, 45.7% (95% CI 29.3% to 58.2%) for death or cardiovascular hospitalization, and 43.7% (95% CI 24.2% to 58.2%) for death or HF-related hospitalization. In conclusion, among outpatients with HFpEF, most hospitalizations could be attributed to co-morbidities and sociodemographic factors. Effects of HF therapies on hospitalizations and related end points may be difficult to demonstrate in these patients. Multidisciplinary approaches are more likely to impact hospitalizations in HFpEF.

PMID: 29525061 [PubMed - indexed for MEDLINE]

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Cost analysis of combining congenital cardiac catheterization and electrophysiology procedures in an outpatient setting.

Pacing Clin Electrophysiol. 2018 11;41(11):1428-1434

Authors: Bansal M, Molian VA, Maldonado JR, Aldoss O, Ochoa LA, Law IH

BACKGROUND: Patients with congenital heart disease require multiple procedures over their lifetime. These procedures increase cost and time commitment. Previous studies in the field of medicine have shown that combining procedures is an effective method to reduce cost and time. There has been no such study to evaluate the cost and efficiency of combining pediatric cardiac procedures.
OBJECTIVE: The objective of this study was to compare the cost and time commitment of combined cardiac catheterization (cath) and electrophysiology (EP) outpatient procedures against separate cath and EP procedure.
METHODS: Outpatient combination procedures performed in the pediatric cardiac cath lab from 2013 to 2016 were matched to a control population of two or three similar single outpatient procedures from 2009 to 2016 for patients of similar age and cardiac anatomy. Procedure duration, recovery duration, length of stay, equipment charges, physician charges, all other hospital charges, and total admission charges were analyzed. The two groups were compared using an unpaired t-test.
RESULTS: A total of 92 subjects, 32 study subjects and 60 control subjects, were included in this study. Study group procedures had a significantly shorter recovery duration (P = 0.04) and length of stay (P = 0.01). Study group procedure duration trended shorter on average but statistically insignificant (P = 0.20). The total median savings for patients undergoing combined procedures in the study group was $13,181 (interquartile range $423.8-$26710).
CONCLUSIONS: Combining cath and EP outpatient procedures reduces the time commitment and provides some economic advantage.

PMID: 30151836 [PubMed - indexed for MEDLINE]