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Multiperforated atrial septal "aneurysm in aneurysm": Percutaneous closure guided by real-time three-dimensional transesophageal echocardiography.

J Clin Ultrasound. 2018 Jul;46(6):421-423

Authors: Sun F, Fan M, Li Y, Ren W, Chen Y

Atrial septal aneurysm (ASA) is a localized deformity of the interatrial septum, which protrudes into the right and/or left atrium. ASA with defects is a source of cardiac embolism. Although transcatheter closure is now a common procedure, it is still challenging in patients with complicated ASA. We describe a case of giant atypical multiperforated ASA with irregular mobility, which showed a rare "aneurysm in aneurysm" structure. Real-time three-dimensional transesophageal echocardiography showed vivid images, which clearly demonstrated these irregular shaped defects mimicking the atriotomy view, and played a crucial role in guiding the transcatheter closure.

PMID: 29080307 [PubMed - indexed for MEDLINE]

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Prenatal diagnosis of Berry syndrome by fetal echocardiography: A report of four cases.

Echocardiography. 2018 04;35(4):563-565

Authors: Zhang X, Liu XW, Gu XY, Han JC, Hao XY, Fu YW, He YH

Berry syndrome is a rare congenital cardiac malformation. We describe 4 cases of Berry syndrome diagnosed by fetal echocardiography. Based on our experience, the three-vessel view is important for diagnosing the aortopulmonary window and aortic origin of the right pulmonary artery. Furthermore, the true cross-sectional and sagittal views obtained by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch are required to image the interruption or coarctation of the aortic arch. An early and accurate prenatal diagnosis of Berry syndrome is feasible and helps to improve patient outcomes.

PMID: 29430703 [PubMed - indexed for MEDLINE]

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Risk Factors for Survival After Heart Transplantation in Children and Young Adults: A 22-Year Study of 179 Transplants.

World J Pediatr Congenit Heart Surg. 2018 09;9(5):557-564

Authors: Shah S, Asante-Korang A, Ghazarian SR, Stapleton G, Herbert C, Decker J, Almodovar MC, Karl TR, Do NL, Quintessenza JA, Mavroudis C, Vricella LA, van Gelder HM, Kartha V, Alexander P, Carapellucci J, Krasnopero D, Hanson J, Amankwah E, Roth J, Jacobs JP

BACKGROUND: This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality.
METHODS: Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis.
RESULTS: Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups ( P = .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P = .422).
CONCLUSIONS: Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.

PMID: 30157732 [PubMed - indexed for MEDLINE]

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[2017 ESC/EACTS Guidelines for the management of valvular heart disease].

Kardiol Pol. 2018;76(1):1-62

Authors: Baumgartner H, Falk V, Bax JJ, Bonis M, Hamm C, Holm PJ, Iung B, Lancellotti P, Lansac E, Munoz DR, Rosenhek R, Sjögren J, Mas PT, Vahanian A, Walther T, Wendler O, Windecker S, Zamorano JL

PMID: 29399765 [PubMed - indexed for MEDLINE]

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Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: Varied clinical presentation, therapeutic approach and outcome.

Int J Cardiol. 2018 06 15;261:49-53

Authors: Boutsikou M, Shore D, Li W, Rubens M, Pijuan A, Gatzoulis MA, Babu-Narayan SV

INTRODUCTION: The diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death.
CASES: We present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome.
CONCLUSION: ALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy.

PMID: 29548537 [PubMed - indexed for MEDLINE]

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Is the new Occlutech duct occluder an appropriate device for transcatheter closure of patent ductus arteriosus?

Int J Cardiol. 2018 06 15;261:54-57

Authors: Godart F, Houeijeh A, Domanski O, Guillaume MP, Brard M, Lucron H

AIM: To describe our initial experience with the Occlutech Duct Occluder (ODO) for percutaneous closure of patent ductus arteriosus (PDA).
METHODS: Retrospective review of patients undergoing transcatheter PDA closure with the ODO in 2 academic centers.
RESULTS: From April 2013 to September 2017, 42 patients underwent PDA closure. Median age at implantation was 34 months (range 4 months-68 years) and median weight was 12 kg (range 4.1-57 kg). Ducts were Krichenko type A duct (n = 34), type E (n = 6), and type C (n = 2). The mean duct diameter was 3.76 mm (range 1.69 to 9.95 mm, median 3.1 mm). Implantation succeeded in all. There was neither device embolization nor hemolysis. At device release, immediate angiogram showed a small residual shunt in 54.7%. During follow-up, Doppler echocardiography demonstrated 71% of full occlusion at day one, rising to 95% at one month and 100% at one year and half after implantation. The mean maximal systolic pressure gradient in left pulmonary artery was 4.2 ± 4.3 mm and across the distal aortic arch 5.4 ± 4.7 mm Hg. No patient had any significant stenosis with clinical relevance.
CONCLUSIONS: ODO is safe and effective in transcatheter closure of PDA including relatively large sized ducts. The results are satisfactory with a high level of full occlusion and a low rate of complications. Further evaluation with larger studies and longer follow-up will be required to confirm these preliminary good results.

PMID: 29551254 [PubMed - indexed for MEDLINE]

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Ventricular efficiency in pregnant women with congenital heart disease.

Int J Cardiol. 2018 06 15;261:58-61

Authors: Muneuchi J, Yamasaki K, Watanabe M, Fukumitsu A, Kawakami T, Nakahara H, Joo K

BACKGROUNDS: Pregnant women with congenital heart disease (CHD) are at risk of cardiovascular events during pregnancy as well as postpartum. The aim of our study is to address the feasibility of echocardiography-derived ventricular-arterial coupling during pregnancy and postpartum among women with CHD.
METHODS: In 31 pregnant women with CHD, we performed serial echocardiography at the first and third trimesters, early and late postpartum. The indices of contractility (single-beat determined end-systolic elastance, Eesab) and afterload (effective arterial elastance, Ea) were approximated on the basis of the systemic blood pressure and systemic ventricular volume. The ratio of stroke work and pressure-volume area (SW/PVA) representing ventricular efficiency was also calculated.
RESULTS: Age at the delivery was 28 (24-31) years. ZAHARA score was 0.75 (0.75-1.50). Gestational age and birth weight of newborns were 38 (37-39) weeks and 2.73 (2.42-2.92) kg, respectively. Heart rate, systemic ventricular end-diastolic volume and stroke volume significantly increased from the first trimester to the third trimester and reversed postpartum to the values of the first trimester. Eesab and Ea significantly decreased from the first trimester to the third trimester (Eesab; 4.90 [2.86-7.14] vs 3.41 [2.53-4.61] mm Hg/ml, p = 0.0001, Ea; 2.83 [1.74-3.30] vs 2.18 [1.67-2.68] mm Hg/ml, p = 0.0012), and reversed early postpartum parallelly. Ejection fraction and SW/PVA remained unchanged throughout pregnancy and postpartum.
CONCLUSIONS: Echocardiography-derived ventricular-arterial coupling is feasible to understand ventricular function in pregnant women with CHD.

PMID: 29657057 [PubMed - indexed for MEDLINE]

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Long noncoding RNA TUG1 promotes cardiac fibroblast transformation to myofibroblasts via miR‑29c in chronic hypoxia.

Mol Med Rep. 2018 Sep;18(3):3451-3460

Authors: Zhu Y, Feng Z, Jian Z, Xiao Y

Cardiac fibroblast‑myofibroblast transformation (FMT) contributes to the fibrotic deterioration evoked by chronic hypoxia. Growing evidence implicates long noncoding RNAs (lncRNAs) in various types of cardiac physiological and pathological processes, especially in cardiac fibrosis. In the present study, the lncRNA Taurine Upregulated Gene 1 (TUG1), reported as a regulator of hypoxia fibrosis in the lungs, was found to also be an important regulator of cardiac FMT. Specifically, the possible role of TUG1 in cardiac FMT and fibrosis under chronic hypoxia was investigated. It was revealed that the degree of fibrosis in heart tissues collected from congenital heart surgery patients with low pulse oxygen saturation and mice housed under chronic hypoxic and atmospheric pressure conditions was negatively correlated with pulse oxygen saturation. Moreover, TUG1 expression was positively correlated with the degree of fibrosis but negatively correlated with pulse oxygen saturation. Cardiac fibroblasts showed increased myofibroblast marker, collagen I and α‑SMA expression levels as the hypoxia time increased. TUG1 knockdown ameliorated the hypoxia‑induced FMT. A bioinformatics analysis predicted that TUG1 had miR‑29c binding sites in its 3'‑UTR and miR‑29c is a key regulator of cardiac fibrosis. The present study demonstrated that TUG1, along with miR‑29c, may contribute to cardiac FMT activation and promote fibrosis in chronic hypoxia.

PMID: 30066872 [PubMed - indexed for MEDLINE]

Insights on Atrial Fibrillation in Congenital Heart Disease.

Can J Cardiol. 2018 Nov;34(11):1531-1533

Authors: Ebrahim MA, Escudero CA, Kantoch MJ, Vondermuhll IF, Atallah J

Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias being the most common long-term complication. In recent reports, atrial fibrillation (AF) tended to be the most common form of arrhythmias among groups of patients with adult CHD (ACHD) older than 50 years of age. When compared with their adult counterparts without CHD, AF in patients with ACHD has been characterized by a higher incidence and prevalence, younger age of onset, and a greater risk of progression to persistent AF. Risk factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension, and multiple cardiac surgeries. Data on management options such as optimal antiarrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation are limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology, and management options for AF in this complex patient population.

PMID: 30404756 [PubMed - in process]

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Additional superior vena cava combined with abnormal inflow of the hepatic vein.

Asian Cardiovasc Thorac Ann. 2018 Sep;26(7):566-569

Authors: Bockeria LA, Bockeria OL, Sanakoev MK, Shvartz VA, Le TG

We represent a case of successful surgical treatment of a rare congenital heart disease: abnormal inflow of an additional superior vena cava into the left atrium, combined with atrial septal defect, mitral and tricuspid valve regurgitation, and abnormal inflow of the left hepatic vein into a roofless coronary sinus.

PMID: 27206782 [PubMed - indexed for MEDLINE]