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Atrial Septal Defect Revealed by Repeat Echocardiography in a Brain-Dead Donor: A Case Report.

Transplant Proc. 2019 Oct;51(8):2848-2850

Authors: Lee HW, Kim MS, Song S, Kim SP

BACKGROUND: The number of patients undergoing heart transplantation is limited because of the shortage of donor hearts. Expanding the donor pool with precise evaluation of donor heart is the most practical way to increase the number of patients undergoing heart transplantation. However, echocardiographic evaluation of especially right ventricle (RV) in brain-dead donors is challenging. Repeated echocardiography with volume overload may reveal unrecognized congenital heart disease such as atrial septal defect (ASD).
MATERIALS AND METHODS: A case of repeated echocardiography revealed ASD secundum in a donor with significant RV enlargement and dysfunction from previously unknown origin. An almost-discarded donor heart was used in heart transplantation after simple patch closure.
RESULTS: The patient was discharged after a month. One year later, he has been doing well without significant adverse events.
CONCLUSIONS: RV dysfunction prior to heart transplantation increases the risk of post-transplant graft failure. Secundum-type ASD and ventricular septal defect, which are relatively common adult congenital heart diseases that cause RV enlargement and dysfunction, are not contraindications for heart transplantation. Our case showed that repeat echocardiography revealed congenital heart disease and rescued the almost-discarded heart. This simple maneuver is essential in donor heart evaluation to maximize the utility of marginal donors.

PMID: 31563249 [PubMed - in process]

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Parents' Experiences of Having a Baby With a Congenital Heart Defect and the Child's Heart Surgery.

Compr Child Adolesc Nurs. 2019 Mar;42(1):10-23

Authors: Sjostrom-Strand A, Terp K

The incidence of children born with congenital heart disease is 1%. Congenital heart disease is among the birth defects that lead to the longest hospital stays, and children with congenital heart disease often require frequent hospitalization and several heart operations, along with lifelong follow-up visits. This study aims to describe parents' experiences when their child has a heart defect and undergoes open heart surgery. A total of 10 parents were interviewed: 8 mothers and 2 fathers. The interviews took place 2 years after the heart surgery. The interviews were analyzed using a content analysis method, which resulted in 4 categories: maintaining belief, experiencing the surgery as a turning point, experiencing the pediatric intensive care unit with anxiety and fear, and perception of support. When parents face their child having a congenital heart defect and plan heart surgery, the whole family is living through a stressful time and has to handle many difficult situations. Parents need support from the health care team.

PMID: 28786702 [PubMed - indexed for MEDLINE]

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Epidemiology, Clinical Features, and Outcome of Infective Endocarditis due to Abiotrophia Species and Granulicatella Species: Report of 76 Cases, 2000-2015.

Clin Infect Dis. 2018 01 06;66(1):104-111

Authors: Téllez A, Ambrosioni J, Llopis J, Pericàs JM, Falces C, Almela M, Garcia de la Mària C, Hernandez-Meneses M, Vidal B, Sandoval E, Quintana E, Fuster D, Tolosana JM, Marco F, Moreno A, Miro JM, Hospital Clínic Infective Endocarditis Investigators

Background: Infective endocarditis (IE) caused by Abiotrophia (ABI) and Granulicatella (GRA) species is poorly studied. This work aims to describe and compare the main features of ABI and GRA IE.
Methods: We performed a retrospective study of 12 IE institutional cases of GRA or ABI and of 64 cases published in the literature (overall, 38 ABI and 38 GRA IE cases).
Results: ABI/GRA IE represented 1.51% of IE cases in our institution between 2000 and 2015, compared to 0.88% of HACEK (Haemophilus, Aggregatibacter, Cardiobacterium, Eikenella, Kingella)-related IE and 16.62% of Viridans group streptococci (VGS) IE. Institutional ABI/GRA IE case characteristics were comparable to that of VGS, but periannular complications were more frequent (P = .008). Congenital heart disease was reported in 4 (10.5%) ABI and in 11 (28.9%) GRA cases (P = .04). Mitral valve was more frequently involved in ABI than in GRA (P < .001). Patient sex, prosthetic IE, aortic involvement, penicillin susceptibility, and surgical treatment were comparable between the genera. New-onset heart failure was the most frequent complication without genera differences (P = .21). Five (13.2%) ABI patients and 2 (5.3%) GRA patients died (P = .23). Factors associated with higher mortality were age (P = .02) and new-onset heart failure (P = .02). The genus (GRA vs ABI) was not associated with higher mortality (P = .23).
Conclusions: GRA/ABI IE was more prevalent than HACEK IE and approximately one-tenth as prevalent as VGS; periannular complications were more frequent. GRA and ABI genera IE presented similar clinical features and outcomes. Overall mortality was low, and related to age and development of heart failure.

PMID: 29020360 [PubMed - indexed for MEDLINE]

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[The effect of the transition care model on health perception among adolescents with congenital heart disease: a quasi-experimental study].

G Ital Cardiol (Rome). 2018 Jun;19(6):386-393

Authors: Flocco SF, Caruso R, Dellafiore F, Pittella F, Giamberti A, Micheletti A, Negura DG, Piazza L, Chessa M

BACKGROUND: The multidisciplinary standardized interventions to educate and support patients with congenital heart disease (CHD) are described as "Transition Clinic" (TC). TC represents a key element to deliver care for patients during the transition from childhood to adulthood. So far, there is a lack of empirical evidence regarding the impact of TC models on the improvement of health perception in adolescent patients with CHD (CHD-specific TC model). For this reason, the aim of this study is assess the impact of the TC model on CHD adolescent patients' health perception outcomes.
METHODS: This study has a quasi-experimental design. Quality of life, satisfaction and health perception were assessed in T0 and after 1 year from enrollment (T1). During the follow-up period, the patients enrolled (aged 14 to 21 years) were involved in the CHD-specific TC model.
RESULTS: The results are referred to the first 100 patients enrolled (mean age 14.79 ± 1.85 years; 60% male), as they have already completed the follow-up. The overall study is currently ongoing. According to Warnes' classification, 29% of patients had simple heart defects, 46% showed moderate complexity, and 25% showed severe complexity. The comparison between T0 and T1 showed statistically significant improvement in T1 regarding pain/discomfort, anxiety/depression and perception of health status (EQ-5D), general satisfaction and quality of life (LAS QoL).
CONCLUSIONS: The preliminary results showed in this study are encouraging, and confirm the need to create a multidisciplinary standardized intervention of education and support to deliver care for adolescent patients with CHD.

PMID: 29912228 [PubMed - indexed for MEDLINE]

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Burden of Diseases and Injuries in Afghanistan, 1990-2016: Findings From the Global Burden of Disease 2016 Study.

Arch Iran Med. 2018 08 01;21(8):324-334

Authors: Massahikhaleghi P, Tehrani-Banihashemi A, Saeedzai SA, Hossaini SM, Hamedi SA, Moradi-Lakeh M, Naghavi M, Murray CJL, Mokdad AH

BACKGROUND: Afghanistan is one of the low-income countries in the Eastern Mediterranean Region with young population and myriad of healthcare needs. We aim to report the burden of diseases and injuries in Afghanistan between 1990 and 2016.
METHODS: We used the Global Burden of Disease (GBD) 2016 study for estimates of deaths, disability-adjusted life years (DALYs), years of life lost, years of life lived with disability, maternal mortality ratio (MMR), neonatal mortality rates (NMRs) and under 5 mortality rates (U5MR) in Afghanistan.
RESULTS: Total mortality rate, NMR and U5MR have progressively decreased between 1990 and 2016. Mortality rate was 909.6 per 100000 (95% UI: 800.9-1023.3) and MMR was 442.8 (95% UI: 328.3-595.8) per 100000 live births in 2016. Conflict and terrorism, ischemic heart disease (IHD) and road injuries were the leading causes of DALY among males of all ages in 2016 with 10.9%, 7.8% and 7.6% of total DALYs respectively, whereas among females of all ages lower respiratory infections (LRIs), IHD and congenital birth defects were the leading causes of DALY with 8.7%, 7.0% and 6.5% of total DALYs respectively.
CONCLUSION: Despite improvements in certain health indicators, our study suggests an urgent intervention to improve health status of the country. Peace and safety by means of stopping the conflict and terrorism are the mainstay of all other health interventions. Improving health infrastructures, boosting maternal and child health (MCH), battling infectious diseases as well as chronic disease risk factor modification programs can help to decrease burden of diseases.

PMID: 30113853 [PubMed - indexed for MEDLINE]

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Comparing Serum Level of Vitamin D3 in Patients With Isolated Coronary Artery Ectasia and Normal Coronary Artery Individuals.

Arch Iran Med. 2018 09 01;21(9):393-398

Authors: Hosseinsabet A, Faal M, Shafiee A, Aghajani H, Sotoudeh Anvari M, Jalali A, Nozari Y, Pourhosseini H, Salarifar M, Amirzadegan A, Kassaian SE, Alidoosti M, Hajizeinali A, Nematipour E

BACKGROUND: Coronary artery ectasia (CAE) is identified as dilation of one or more segments of coronary arteries that reaches 1.5 times or more, compared with near segments that are normal. Several etiologies like atherosclerosis, autoimmune diseases and congenital anomalies have been proposed for this condition. Vitamin D deficiency activates the renin-angiotensin-aldosterone system, which affects the cardiovascular system. For these reasons, we investigated the serum level of vitamin D in patients with CAE compared with individuals with normal coronary arteries.
METHODS: The study group included 30 patients (20 males and 10 females, mean age: 57 ± 9 years) with isolated CAE without any stenotic lesions, and the control group consisted of 60 age/gender matched subjects who had normal coronary angiograms (CAG) (40 males and 20 females, mean age: 57 ± 8 years). All participants underwent CAG at Tehran Heart Center between December 2015 and March 2016. Along with routine lab tests, vitamin D, serum albumin, calcium, phosphorus and alkaline phosphatase levels were analyzed and the unadjusted and adjusted effects of vitamin D on CAE were evaluated using logistic regression model.
RESULTS: The median vitamin D level of the patients with CAE was lower than that of the control group (6.5 [3.0, 18.8] ng/mL vs. 17.7 [8.9, 27.1] ng/mL; P = 0.002). The logistic regression model showed that vitamin D deficiency was a predictor for the presence of CEA (P = 0.013). After adjustment for confounding variables, this association remained significant (P = 0.025).
CONCLUSION: An association between CAE and vitamin D deficiency was found in our study.

PMID: 30221529 [PubMed - indexed for MEDLINE]

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Alcohol Consumption and Aortic Root Dilatation: Insights from the Corinthia Study.

Angiology. 2019 Nov;70(10):969-977

Authors: Oikonomou E, Lazaros G, Tsalamandris S, Vogiatzi G, Christoforatou E, Papakonstantinou M, Goliopoulou A, Tousouli M, Chasikidis C, Tousoulis D

Aortic diameter and progression to thoracic aortic aneurysm are influenced by several factors. In this study, we investigated the association of alcohol consumption with aortic root and ascending aorta dilatation. In the context of the Corinthia study, we examined 1751 patients with echocardiography. Several demographic and clinical characteristics were recorded. Alcohol consumption was assessed based on a questionnaire of frequency, type, and quantity. Accordingly, patients were categorized as everyday alcohol consumers (EDACs) and as social drinkers (SoD). Everyday alcohol consumers were further categorized to group 1: 0 to 1 drink/d; group 2: 1 to 2 drinks/d; and group 3: ≥3 drinks/d. From the study population, 40% were categorized as EDAC and had an increased aortic root diameter (AoRD) and an elevated AoRD index compared with SoD. Interestingly, there was a stepwise increase in aortic root and ascending aorta diameter according to daily alcohol consumption. Specifically, patients consuming ≥3 drinks of alcohol/d had increased indexed aortic by 1.4 mm/m2 compared with SoD even after adjustment for possible confounders. Daily alcohol consumption is associated with increased aortic root diameter. These findings may have important clinical implications, especially in patients with borderline or dilated aortic root, and merit further investigation.

PMID: 31064194 [PubMed - indexed for MEDLINE]

End-Stage Liver Disease Models and Outcomes in Pediatric Patients Supported With Short-Term Continuous-Flow Ventricular Assist Devices.

ASAIO J. 2019 Sep 25;:

Authors: Malik G, Pidborochynski T, Buchholz H, Freed DH, Al-Aklabi M, Bozso SJ, Choudhry S, Anand V, Holinski P, Conway J

Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being utilized in pediatrics. End-stage liver disease (ELD) models have been associated with outcomes in adult patients on mechanical circulatory support. We sought to determine the relationship between outcomes in children on STCF-VADs and three ELD models: model for end-stage liver disease-excluding international normalized ratio (MELD-XI; all) and MELD-XI (> 1 year), PELD, and a novel score, PedMELD-XI. All patients (< 19 years) supported with STCF-VADs, between June 2009 and December 2016 were included. The MELD-XI, PELD, and PedMELD-XI scores were calculated and their association with adverse events and a composite measure of death, major bleeding, and neurologic dysfunction was analyzed. Of 32 patients, median age was 0.57 years (interquartile range [IQR], 0.10-4.43), median weight was 7.15 kg (IQR, 3.68-16.53), 53.1% had congenital heart disease, and 53.1% were male. In total, 78.1% patients experienced an adverse event (78.1% a major bleed, 25.0% neurologic dysfunction, and 15.6% death). The median MELD-XI score was 11.17 (IQR, 9.44-30.01), MELD-XI (>1 year) 9.44 (IQR, 9.44-24.33), PELD 6.00 (IQR, 4.00-13.75), and PedMELD-XI -14.91 (IQR, -18.85 to -12.25). A higher MELD-XI for all ages (13.80 vs. 9.44, p = 0.037) and less negative PedMELD-XI (-14.16 vs. -19.34, p = 0.028) scores were significantly associated with bleeding and the composite outcome. PedMELD-XI was significantly associated with death (-12.87 vs. -16.84, p = 0.041) while a trend was seen for increased MELD-XI in all ages being associated with death (31.52 vs. 10.11, p = 0.051). Last, there was no association with the models and neurologic events. MELD-XI and PedMELD-XI were significantly associated with major bleeding and the composite endpoints with PedMELD-XI also being associated with death. These results suggest that ELD models can be used to predict outcomes in this specific patient population, however, further analysis in a larger population is required.

PMID: 31567409 [PubMed - as supplied by publisher]

Targeted panel sequencing in pediatric primary cardiomyopathy supports a critical role of TNNI3.

Clin Genet. 2019 Sep 30;:

Authors: Kühnisch J, Herbst C, Al-Wakeel-Marquard N, Dartsch J, Holtgrewe M, Baban A, Mearini G, Hardt J, Kolokotronis K, Gerull B, Carrier L, Beule D, Schubert S, Messroghli D, Degener F, Berger F, Klaassen S

The underlying genetic mechanisms and early pathological events of children with primary cardiomyopathy (CMP) are insufficiently characterized. In this study we aimed to characterize the mutational spectrum of primary CMP in a large cohort of patients ≤18 years referred to a tertiary center. Eighty unrelated index patients with pediatric primary CMP underwent genetic testing with a panel-based NGS approach of 89 genes. At least one pathogenic or likely pathogenic variant was identified in 30/80 (38%) index patients. In all CMP subgroups, patients carried most frequently variants of interest in sarcomere genes suggesting them as a major contributor in pediatric primary CMP. In MYH7, MYBPC3 and TNNI3 we identified 18 pathogenic/likely pathogenic variants (MYH7 n=7, MYBPC3 n=6, TNNI3 n=5, including one homozygous (TNNI3 c.24+2T>A) truncating variant. Protein and transcript level analysis on heart biopsies from individuals with homozygous mutation of TNNI3 revealed that the TNNI3 protein is absent and associated with upregulation of the fetal isoform TNNI1. The present study further supports the clinical importance of sarcomeric mutation - not only in adult - but also in pediatric primary CMP. TNNI3 is the third most important disease gene in this cohort and complete loss of TNNI3 leads to severe pediatric CMP. This article is protected by copyright. All rights reserved.

PMID: 31568572 [PubMed - as supplied by publisher]

Neurocognition in Adult Congenital Heart Disease: How to Monitor and Prevent Progressive Decline.

Can J Cardiol. 2019 Jun 26;:

Authors: Keir M, Ebert P, Kovacs AH, Smith JMC, Kwan E, Field TS, Brossard-Racine M, Marelli A

Children born with congenital heart disease (CHD) are now living to adulthood in unprecedented numbers and many will eventually live to become senior citizens. As care goals shift from surviving to thriving, a new focus on quality of life has emerged. Neurocognition and the ability to participate fully in society, form meaningful relationships, and collaborate effectively with the health care system are important considerations. As adults with CHD age, research regarding their cognitive function becomes prescient. The focus is now shifting from defining neurocognitive deficits in children with CHD to preventing neurocognitive decline in adults living with CHD. In this review, we describe the possible etiologies and predictors of neurocognitive decline in adults with CHD. We performed a comprehensive literature review to identify all of the current data available on neurocognitive function in adults with CHD. We summarize the available evidence by describing common deficits in this patient population and the potential effects of these deficits on adult functioning, health care decision-making, and long-term relationships with care providers. We review potential modifiable etiologies for progressive neurocognitive decline and suggest strategies for surveillance and prevention of the potential decline. We conclude that the current information available regarding the aging brain of adults with CHD and the effect of neurocognitive decline on morbidity and mortality is woefully insufficient. This review, therefore, provides a roadmap for future research endeavours to study neurocognition in older adults with CHD.

PMID: 31570238 [PubMed - as supplied by publisher]