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Feasibility assessment for successfully visualizing the fetal heart utilizing spatiotemporal image correlation.

J Med Ultrason (2001). 2018 Apr;45(2):269-279

Authors: Inubashiri E, Tatedo S, Nishiyama N, Minami H, Watanabe Y, Akutagawa N, Kuroki K, Sugawara M, Maeda N, Haseyama K

PURPOSE: Spatiotemporal image correlation (STIC) is an excellent imaging modality for observing the fetal heart. High-quality STIC volume data are needed for an antenatal anatomic survey to diagnose congenital heart disease. We aimed to clarify the causes of unsuccessful STIC volume data acquisition and describe a more accurate, efficient STIC examination.
METHODS: This cross-sectional study of 1124 women with fetuses assessed risk factors for unsuccessful acquisition of STIC volume data. Logistic regression analysis quantified the relation between unsuccessful acquisition and clinical variables, including maternal body mass index (BMI), shadowing artifacts due to unexpected fetal limb movement (SAU), estimated fetal weight (EFW), gestational age (GA), use of volume rendering images in four-dimensional ultrasonography (4D-US), fetal heart rate (FHR), maternal age, anterior placenta, and prior lower abdominal surgery.
RESULTS: STIC volume data acquisition was unsuccessful in 210 of 1124 (18.6%) cases. SAU, BMI ≥ 28 kg/m2, not using volume rendering images in 4D-US, EFW ≥ 1300 g, and anterior placenta were independent risk factors for unsuccessful STIC data acquisition.
CONCLUSIONS: Avoiding SAU was the most important factor for accurate, efficient STIC evaluations for diagnosing congenital heart disease antenatally. The risk was not explained by lack of sonographer proficiency. Volume rendering images in 4D-US is a promising approach to successful acquisition of STIC volume data.

PMID: 28836088 [PubMed - indexed for MEDLINE]

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Reduced aggrecan expression affects cardiac outflow tract development in zebrafish and is associated with bicuspid aortic valve disease in humans.

Int J Cardiol. 2017 Dec 15;249:340-343

Authors: Rambeau P, Faure E, Théron A, Avierinos JF, Jopling C, Zaffran S, Faucherre A

Hemodynamic forces have been known for a long time to regulate cardiogenic processes such as cardiac valve development. During embryonic development in vertebrates, the outflow tract (OFT) adjacent to the ventricle comes under increasing hemodynamic load as cardiogenesis proceeds. Consequently, extracellular matrix components are produced in this region as the cardiac cushions form which will eventually give rise to the aortic valves. The proteoglycan AGGRECAN is a key component of the aortic valves and is frequently found to be deregulated in a variety of aortic valve diseases. Here we demonstrate that aggrecan expression in the OFT of developing zebrafish embryos is hemodynamically dependent, a process presumably mediated by mechanosensitive channels. Furthermore, knockdown or knockout of aggrecan leads to failure of the OFT to develop resulting in stenosis. Based on these findings we analysed the expression of AGGRECAN in human bicuspid aortic valves (BAV). We found that in type 0 BAV there was a significant reduction in the expression of AGGRECAN. Our data indicate that aggrecan is required for OFT development and when its expression is reduced this is associated with BAV in humans.

PMID: 28986054 [PubMed - indexed for MEDLINE]

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Ubiquitin C-Terminal Hydrolase 1 and Phosphorylated Axonal Neurofilament Heavy Chain in Infants Undergoing Cardiac Surgery: Preliminary Assessment as Potential Biomarkers of Brain Injury.

World J Pediatr Congenit Heart Surg. 2018 Jul;9(4):412-418

Authors: Lee T, Chikkabyrappa SM, Reformina D, Mastrippolito A, Chakravarti SB, Mosca RS, Shaw G, Malhotra SP

BACKGROUND: There are no reliable markers to assess brain injury in neonates following cardiac surgery. We examine ubiquitin C-terminal hydrolase 1 (UCHL1) and phosphorylated axonal neurofilament heavy chain (pNF-H), neuronal-specific biomarkers released following axonal and cortical injury, in neonates undergoing cardiac surgery involving cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA).
METHODS: Twenty-six patients younger than three months were prospectively enrolled (CPB only, n = 12 and DHCA, n = 14). Healthy newborns (n = 22) served as the control. Blood samples were collected preoperatively and postoperatively upon intensive care unit admission (hour 0) and subsequently at 12, 24, 36, and 48 hours. Serum was tested for UCHL1 and pNF-H using enzyme-linked immunosorbent assay. Concomitant arterial blood gas, lactate, and cerebral near-infrared spectroscopy (NIRS) monitoring were performed.
RESULTS: Ubiquitin C-terminal hydrolase 1 showed a significant rise at 0 hours in the DHCA group compared to baseline (74.9 ± 13.7 pg/mL vs 33.9 ± 37.3 pg/mL, P < .0001). Levels returned to baseline at 12 hours. There was an early rise in UCHL1 at 0 hours in the CPB group, P = .09. Phosphorylated axonal neurofilament heavy chain was decreased at 0 hours in both the CPB and DHCA groups compared to baseline, P = .06. There was no difference between control and baseline levels of UCHL1 ( P = .9) or pNF-H ( P = .77). Decreased NIRS was observed in the DHCA group at 0 hours (57.3 ± 10.5) versus baseline (64.2 ± 12.3), but not significant ( P = .21). There was no correlation between biomarkers and NIRS at 0 hours.
CONCLUSION: A rapid rise in UCHL1 levels was observed in the DHCA group, suggesting that it may be a marker for acute brain injury. Follow-up with neurodevelopmental studies is ongoing.

PMID: 29945509 [PubMed - in process]

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History of the World Society for Pediatric and Congenital Heart Surgery: The First Decade.

World J Pediatr Congenit Heart Surg. 2018 Jul;9(4):392-406

Authors: Jacobs JP, Tchervenkov CI, Stellin G, Kurosawa H, Mavroudis C, Jatene MB, Al-Halees Z, Cicek SM, Sandoval NF, Backer CL, Cervantes J, Dearani JA, Ebels T, Edwin F, Finucane K, Fragata J, Iyer KS, Kinsley RH, Kirklin JK, Kreutzer C, Liu J, Maruszewski B, St Louis JD, Sarris GE, Jonas RA

The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) is the largest professional organization in the world dedicated to pediatric and congenital heart surgery. The purpose of this article is to document the first decade of the history of WSPCHS from its formation in 2006, to summarize the current status of WSPCHS, and to consider the future of WSPCHS. The WSPCHS was incorporated in Canada on April 7, 2011, with a head office in Montreal, Canada. The vision of the WSPCHS is that every child born anywhere in the world with a congenital heart defect should have access to appropriate medical and surgical care. The mission of the WSPCHS is to promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service.

PMID: 29945512 [PubMed - in process]

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Minimally Invasive Implantation of a Micropacemaker Into the Pericardial Space.

Circ Arrhythm Electrophysiol. 2018 Jul;11(7):e006307

Authors: Bar-Cohen Y, Silka MJ, Hill AC, Pruetz JD, Chmait RH, Zhou L, Rabin SM, Norekyan V, Loeb GE

BACKGROUND: Permanent cardiac pacemakers require invasive procedures with complications often related to long pacemaker leads. We are developing a percutaneous pacemaker for implantation of an entire pacing system into the pericardial space.
METHODS: Percutaneous micropacemaker implantations were performed in 6 pigs (27.4-34.1 kg) using subxyphoid access to the pericardial space. Modifications in the implantation methods and hardware were made after each experiment as the insertion method was optimized. In the first 5 animals, nonfunctional pacemaker devices were studied. In the final animal, a functional pacemaker was implanted.
RESULTS: Successful placement of the entire nonfunctional pacing system into the pericardial space was demonstrated in 2 of the first 5 animals, and successful implantation and capture was achieved using a functional system in the last animal. A sheath was developed that allows retractable features to secure positioning within the pericardial space. In addition, a miniaturized camera with fiberoptic illumination allowed visualization of the implantation site before electrode insertion into myocardium. All animals studied during follow-up survived without symptoms after the initial postoperative period.
CONCLUSIONS: A novel micropacemaker system allows cardiac pacing without entering the vascular space or surgical exposure of the heart. This pericardial pacemaker system may be an option for a large number of patients currently requiring transvenous pacemakers but is particularly relevant for patients with restricted vascular access, young children, or those with congenital heart disease who require epicardial access.

PMID: 29945929 [PubMed - in process]

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Medical Management of the Systemic Right Ventricle.

Heart. 2018 Jul;104(14):1226-1227

Authors: Kutty S, Tutarel O, Diller GP, Danford DA

PMID: 29945949 [PubMed - in process]

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Long-term Management of the Arterial Switch Patient.

Curr Cardiol Rep. 2018 Jun 26;20(8):68

Authors: Kirzner J, Pirmohamed A, Ginns J, Singh HS

PURPOSE OF REVIEW: This review paper describes the management of patients with dextro-transposition of the great arteries (D-TGA) with a focus on the complications seen and the appropriate care required to identify and prevent adverse events.
RECENT FINDINGS: D-TGA is a form of cyanotic congenital heart disease (CHD) representing ~ 3% of all CHD and almost 20% of all cyanotic CHD. Since the late 1980s, standard of care is to repair these patients with an arterial switch operation (ASO) as opposed to a Mustard/Senning operation. The long-term survival and complication rates are superior in the ASO. Long-term follow-up is recommended for all D-TGA patients and includes management with adult congenital heart disease specialists and the use of echocardiography and advanced imaging with CT or MRI. The most common complications seen are pulmonary stenosis, coronary artery stenosis, and neo-aortic regurgitation. Careful evaluation of new symptoms or declining function is essential in preventing and treating these long-term sequelae.

PMID: 29946937 [PubMed - in process]

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Heart and heart-liver transplantation in adults with failing Fontan physiology.

Clin Transplant. 2018 Jun 26;:e13329

Authors: Reardon LC, DePasquale EC, Tarabay J, Cruz D, Laks H, Biniwale RM, Busuttil RW, Kaldas FM, Saab S, Venick RS, Lin JP, Nsair A, Deng MC, Ardehali A, Caderias M, Iygengar A, Aboulhosn JA

BACKGROUND: As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart-liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients have improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology.
METHODS AND RESULTS: Retrospective analysis of patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center who underwent OHT or CHLT for failing Fontan physiology from January 1, 2002 to May 31, 2017. We identified 20 patients with single ventricle physiology and Fontan palliation who underwent OHT or CHLT. The median age was 29.5 years (range 19-44). Five patients underwent CHLT because of biopsy-proven hepatic cirrhosis. The median length of hospital stay was 23 days (range 8-76) post OHT and 51 (range 26-77) days post CHLT. During a median follow-up of 56 months (range 2-178) there was one mortality occurring at 34 months post OHT due to coronary vasculopathy. Most frequent early post-operative complications included bleeding and infection (55% and 20%, respectively) and surgical re-intervention for bleeding complications (n=8, 40%). One CHLT patient experienced clinically significant hepatic rejection requiring admission and steroid treatment.
CONCLUSIONS: Despite inherent risks and complexities of OHT or CHLT in patients with a failed Fontan, transplant is a reasonable therapy. Peri- and post-operative complications are common and may require surgical re-intervention. Continued observation of practices and unifying themes may help improve patient selection, pre- and post-operative treatment and ultimately outcomes. This article is protected by copyright. All rights reserved.

PMID: 29947122 [PubMed - as supplied by publisher]

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Longitudinal Outcomes of Epicardial and Endocardial Pacemaker Leads in the Adult Fontan Patient.

Pediatr Cardiol. 2018 Jun 14;:

Authors: Huntley GD, Deshmukh AJ, Warnes CA, Kapa S, Egbe AC

Placement of an epicardial pacemaker system is often preferred over an endocardial system in patients who have undergone a Fontan operation, but data are limited on how these two systems perform over time in patients with Fontan palliation. We performed a retrospective review of adults with Fontan palliation who had pacemaker implantation and interrogation data at Mayo Clinic from 1994 to 2014. Lead parameters, pacing mode, and polarity were collected at the earliest device interrogation report. Clinic notes and device interrogation reports were reviewed at implantation, 6 months, and yearly after implantation to determine impedance, capture threshold (CT), and energy threshold (ET). There were 87 patients with 168 leads in the study cohort. The mean follow-up time was 7.7 years (6 months-19 years). There were 143 epicardial leads (57 atrial and 86 ventricular) and 25 endocardial leads (20 atrial and 5 ventricular). There was no difference in the baseline lead parameters between epicardial and endocardial leads for impedance (610 ± 259 versus 583 ± 156 Ω, p = 0.93), CT (2.0 ± 1.3 versus 1.8 ± 1.3 V, p = 0.28), or ET (7.1 ± 12.5 versus 6.8 ± 18.1 µJ, p = 0.29). Compared to endocardial leads, ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Regarding clinical outcomes, epicardial leads had higher rates of failure but similar generator longevity in comparison to endocardial leads. Ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Epicardial leads had a higher rate of failure but similar generator longevity compared to endocardial leads.

PMID: 29948032 [PubMed - as supplied by publisher]

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The conversion to Rastelli's type operation from Patrick-McGoon's procedure of an adult with Taussig-Bing heart: a case report.

Gen Thorac Cardiovasc Surg. 2018 Jun 11;:

Authors: Fujiwara K, Yoshizawa K, Ohno N, Sakazaki H

A 23-year-old female of Taussig-Bing heart with antero-posterior relation of the great arteries was underwent Patrick-McGoon's intraventricular rerouting at 6 years old of age. The left ventricular outflow obstruction (peak pressure gradient of 100 mmHg) developed, and severe aortic valve regurgitation following bacterial endocarditis was noted. The conversion to Rastelli's type operation and aortic valve replacement were performed successfully at 23 years old of age. She is doing well without any significant left or right ventricular outflow obstruction at 7 years postoperatively.

PMID: 29948796 [PubMed - as supplied by publisher]