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Congenital anomalies of the left brachiocephalic vein detected in adults on computed tomography.

Jpn J Radiol. 2017 Oct;35(10):597-605

Authors: Yamamuro H, Ichikawa T, Hashimoto J, Ono S, Nagata Y, Kawada S, Kobayashi M, Koizumi J, Shibata T, Imai Y

PURPOSE: Anomalous left brachiocephalic vein (BCV) is a rare and less known systemic venous anomaly. We evaluated congenital anomalies of the left BCV in adults detected during computed tomography (CT) examinations.
MATERIALS AND METHODS: This retrospective study included 81,425 patients without congenital heart disease who underwent chest CT. We reviewed the recorded reports and CT images for congenital anomalies of the left BCV including aberrant and supernumerary BCVs. The associated congenital aortic anomalies were assessed.
RESULTS: Among 73,407 cases at a university hospital, 22 (16 males, 6 females; mean age, 59 years) with aberrant left BCVs were found using keyword research on recorded reports (0.03%). Among 8018 cases at the branch hospital, 5 (4 males, 1 female; mean age, 67 years) with aberrant left BCVs were found using CT image review (0.062%). There were no significant differences in incidences of aberrant left BCV between the two groups. Two cases had double left BCVs. Eleven cases showed high aortic arches. Two cases had the right aortic arch, one case had an incomplete double aortic arch, and one case was associated with coarctation.
CONCLUSION: Aberrant left BCV on CT examination in adults was extremely rare. Some cases were associated with aortic arch anomalies.

PMID: 28849388 [PubMed - indexed for MEDLINE]

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Outcome of antibody-mediated rejection compared to acute cellular rejection after pediatric heart transplantation.

Pediatr Transplant. 2018 02;22(1):

Authors: Vaughn GR, Jorgensen NW, Law YM, Albers EL, Hong BJ, Friedland-Little JM, Kemna MS

Outcomes of ACR after pediatric HTx have been well described, but less has been reported on outcomes of AMR. We compared the clinical characteristics and cardiovascular outcomes (composite end-point of death, retransplantation, or allograft vasculopathy) of pediatric HTx recipients with AMR, ACR, and no rejection in a retrospective single-center study of 104 recipients. Twenty were treated for AMR; 15 were treated for ACR. Recipients with AMR had an increased frequency of congenital heart disease (90% vs ACR 67% vs no rejection 59%, P = .03), homograft (68% vs 7% vs 18%, P < .001), HLA sensitization (45% vs 13% vs 13%, P = .008), and positive cross-match (30% vs 7% vs 9%, P = .046). AMR caused hemodynamic compromise more often than ACR (39% vs 4%, P = .02). AMR recipients had worse cardiovascular outcome than recipients with ACR or no rejection (40% vs 20% vs 8.6%, P = .003). In bivariate Cox analysis, AMR (HR 4.1, CI 1.4-12.0, P = .009) and ischemic time (HR 1.6, CI 1.1-2.3, P = .02) were associated with worse cardiovascular outcome; ACR was not. In summary, pediatric HTx recipients who develop AMR have worse cardiovascular outcome than recipients who develop only ACR or experience no rejection at all.

PMID: 29222866 [PubMed - indexed for MEDLINE]

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A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease.

Cardiol Young. 2018 Apr;28(4):561-570

Authors: Hancock HS, Pituch K, Uzark K, Bhat P, Fifer C, Silveira M, Yu S, Welch S, Donohue J, Lowery R, Aiyagari R

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.

PMID: 29316996 [PubMed - indexed for MEDLINE]

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Noteworthy Literature published in 2017 for Congenital Cardiac Anesthesiologists.

Semin Cardiothorac Vasc Anesth. 2018 Mar;22(1):35-48

Authors: Ing RJ, Twite MD

This review focuses on the literature published during the 13 months from December 2016 to December 2017 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Five themes are addressed during this time period and 100 peer-reviewed articles are discussed.

PMID: 29400257 [PubMed - indexed for MEDLINE]

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Surgical Correction of Supravalvar Aortic Stenosis: 52 Years' Experience.

World J Pediatr Congenit Heart Surg. 2018 03;9(2):131-138

Authors: Roemers R, Kluin J, de Heer F, Arrigoni S, Bökenkamp R, van Melle J, Ebels T, Hazekamp M

OBJECTIVES: Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The "single-patch technique," "'two sinus augmentation with an inverted Y-patch" (both nonsymmetrical corrections), "three-patch technique," and the "slide aortoplasty" (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands.
METHODS: We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality.
RESULTS: A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313).
CONCLUSION: In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

PMID: 29544407 [PubMed - indexed for MEDLINE]

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Brom Aortoplasty for Supravalvular Aortic Stenosis.

World J Pediatr Congenit Heart Surg. 2018 03;9(2):139-146

Authors: Mongé MC, Eltayeb OM, Costello JM, Johnson JT, Popescu AR, Rigsby CK, Backer CL

BACKGROUND: Controversy remains regarding the optimal surgical approach for children with supravalvular aortic stenosis (SVAS).
METHODS: Since 1997 we have used Brom three-patch aortoplasty for patients with SVAS. We prefer computed tomography (CT) imaging for preoperative evaluation rather than cardiac catheterization as it avoids the well-known morbidity of general anesthesia. The purpose of this study was to present our intermediate-term results of this strategy.
RESULTS: Twenty consecutive patients with SVAS were treated with Brom aortoplasty. Mean age was 3.7 ± 5.9 years (median, 1.5 years). Twelve patients had Williams syndrome. Ten patients had preoperative advanced medical imaging (seven CT, three magnetic resonance imaging) and did not have cardiac catheterization. Mean times for cardiopulmonary bypass and cross-clamp were 172 ± 29 minutes and 110 ± 21 minutes, respectively. Ten patients had simultaneous pulmonary artery stenosis patching. Median length of stay was seven days. There was no operative or late mortality. Mean follow-up was 6 ± 5 years. There were no reoperations on the aortic root. Fifteen patients had mild or less aortic insufficiency (AI) and two had moderate AI. One patient who had infant balloon dilation of the aortic valve and postoperative subacute bacterial endocarditis had moderate-to-severe AI and aortic stenosis (AS). One patient had moderate residual SVAS; all others had no AS. No patients had late coronary insufficiency.
CONCLUSION: Brom aortoplasty promotes restoration of normal aortic root geometry and relief of coronary ostial stenosis, which is important in preventing myocardial ischemia. Computed tomography imaging is our preferred diagnostic modality. Intermediate-term outcomes are excellent with no recurrent SVAS, coronary events, or reoperations on the aortic valve.

PMID: 29544418 [PubMed - indexed for MEDLINE]

Development and validation of a risk prediction model in patients with adult congenital heart disease.

Int J Cardiol. 2018 Aug 22;:

Authors: Baggen VJM, Venema E, Živná R, van den Bosch AE, Eindhoven JA, Witsenburg M, Cuypers JAAE, Boersma E, Lingsma H, Popelová JR, Roos-Hesselink JW

AIMS: To develop and validate a clinically useful risk prediction tool for patients with adult congenital heart disease (ACHD).
METHODS AND RESULTS: A risk model was developed in a prospective cohort of 602 patients with moderate/complex ACHD who routinely visited the outpatient clinic of a tertiary care centre in the Netherlands (2011-2013). This model was externally validated in a retrospective cohort of 402 ACHD patients (Czech Republic, 2004-2013). The primary endpoint was the 4-year risk of death, heart failure, or arrhythmia, which occurred in 135 of 602 patients (22%). Model development was performed using multivariable logistic regression. Model performance was assessed with C-statistics and calibration plots. Of the 14 variables that were selected by an expert panel, the final prediction model included age (OR 1.02, 95%CI 1.00-1.03, p = 0.031), congenital diagnosis (OR 1.52, 95%CI 1.03-2.23, p = 0.034), NYHA class (OR 1.74, 95%CI 1.07-2.84, p = 0.026), cardiac medication (OR 2.27, 95%CI 1.56-3.31, p < 0.001), re-intervention (OR 1.41, 95%CI 0.99-2.01, p = 0.060), BMI (OR 1.03, 95%CI 0.99-1.07, p = 0.123), and NT-proBNP (OR 1.63, 95%CI 1.45-1.84, p < 0.001). Calibration-in-the-large was suboptimal, reflected by a lower observed event rate in the validation cohort (17%) than predicted (36%), likely explained by heterogeneity and different treatment strategies. The externally validated C-statistic was 0.78 (95%CI 0.72-0.83), indicating good discriminative ability.
CONCLUSION: The proposed ACHD risk score combines six readily available clinical characteristics and NT-proBNP. This tool is easy to use and can aid in distinguishing high- and low-risk patients, which could further streamline counselling, location of care, and treatment in ACHD.

PMID: 30172474 [PubMed - as supplied by publisher]

Echocardiography in adults with congenital heart disease: Combining the best of both worlds.

Int J Cardiol. 2018 Aug 29;:

Authors: Tretter JT, Oechslin EN, Veldtman GR

PMID: 30172476 [PubMed - as supplied by publisher]

The role of GPCR signaling in cardiac Epithelial to Mesenchymal Transformation (EMT).

Trends Cardiovasc Med. 2018 Aug 22;:

Authors: Nebigil CG, Désaubry L

Congenital heart disease is the most common birth defect, affecting 1.35 million newborns every year. Heart failure is a primary cause of late morbidity and mortality after myocardial infarction. Heart development is involved in several rounds of epithelial-to-mesenchymal transition (EMT) and mesenchymal-to-epithelial transition (MET). Errors in these processes contribute to congenital heart disease, and exert deleterious effects on the heart and circulation after myocardial infarction. The identification of factors that are involved in heart development and disease, and the development of new approaches for the treatment of these disorders are of great interest. G protein coupled receptors (GPCRs) comprise 40% of clinically used drug targets, and their signaling are vital components of the heart during development, cardiac repair and in cardiac disease pathogenesis. This review focuses on the importance of EMT program in the heart, and outlines the newly identified GPCRs as potential therapeutic targets of reprogramming EMT to support cardiac cell fate during heart development and after myocardial infarction. More specifically we discuss prokineticin, serotonin, sphingosine-1-phosphate and apelin receptors in heart development and diseases. Further understanding of the regulation of EMT/MET by GPCRs during development and in the adult hearts can provide the following clinical exploitation of these pathways.

PMID: 30172578 [PubMed - as supplied by publisher]

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Center volume and post-transplant survival among adults with congenital heart disease.

J Heart Lung Transplant. 2018 Aug 30;:

Authors: Menachem JN, Lindenfeld J, Schlendorf K, Shah AS, Bichell DP, Book W, Brinkley DM, Danter M, Frischhertz B, Keebler M, Kogon B, Mettler B, Rossano J, Sacks SB, Young T, Wigger M, Zalawadiya S

BACKGROUND: The number of adult congenital heart disease (ACHD) patients requiring heart transplantation (HT) continues to grow, and if they survive the first year after transplant, their long-term survival is at least equivalent to non-ACHD patients. The 1-year survival of ACHD patients with HT remains lower than non-ACHD patients. We evaluated the affect of transplant center volume on 1-year survival of ACHD patients. We analyzed United Network of Organ Sharing patients (age ≥18 years) who underwent their first orthotopic HT between January 1, 2000, and December 31, 2015, to assess the association between transplant center volume and 1-year survival of ACHD patients.
RESULTS: We identified 827 ACHD patients at 113 centers who underwent HT during the study period. The average age of the recipients and donors was 36 ± 13 years (60% men and 84% Caucasian) and 28 ± 11 (63% men and 66% Caucasian), respectively. Of the ACHD patients undergoing HT, 27% (n = 60) were done at low-volume centers, 30% (n = 10) were reported at high-volume centers, and the remaining (n = 43) were at medium-volume centers. A total of 96 patients died within 30 days, including 37 (16.7%) at low-volume, 37 (10.2%) at medium-volume, and 22 (9.0%) at high-volume centers (p = 0.019). The average unadjusted Kaplan-Meier 30-day survival at low-volume centers was 83% ± 2%, which was significantly lower than medium-volume (90% ± 1%) and high-volume (91% ± 2%) centers (log-rank p < 0.05). Within 1 year, 154 patients had died, including 56 (36.4%) at low-volume, 60 (38.9%) at medium-volume, and 38 (24.7%) at high-volume centers (p = 0.011). Average unadjusted Kaplan-Meier 1-year survival at low-volume centers was 75% ± 3%, which was significantly lower than medium-volume (83% ± 2%) and high-volume (84% ± 2%) centers (log-rank p < 0.05).
CONCLUSIONS: The 30-day and 1-year survival of ACHD patients undergoing HT is partly influenced by overall transplant center volume and, potentially, volume of ACHD HTs, with low-volume centers performing poorly relative to medium-volume and high-volume centers. The role of peri-operative care and multidisciplinary management in improving survival at low-volume centers required further investigations.

PMID: 30174166 [PubMed - as supplied by publisher]