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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

Abstract
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

Abstract
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

Abstract
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

Abstract
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]

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Fetal and Postnatal Echocardiographic Diagnosis of Ebstein Anomaly of the Mitral Valve.

Pediatr Cardiol. 2018 Aug;39(6):1276-1279

Authors: Aly S, Bokowski J, Diab K, Muller BA

Abstract
Ebstein anomaly of mitral valve (MV) is an extremely rare congenital heart disease. In the current report, we present a case of Ebstein of MV that was diagnosed prenatally. Fetal echocardiogram showed that the posterior leaflet of MV was tethered to the lateral wall of left ventricle (LV) with downward displacement into LV cavity. Postnatal transthoracic and transesophageal echocardiograms confirmed the diagnosis with apical displacement of the level of coaptation MV into the LV cavity. To the best of our knowledge, fetal diagnosis of Ebstein anomaly of MV has not yet been reported in the medical literature.

PMID: 29756160 [PubMed - indexed for MEDLINE]

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Successful simultaneous transcatheter treatment for a secundum atrial septal defect complicated by valvular pulmonary stenosis in an infant.

Cardiol Young. 2018 Oct;28(10):1162-1164

Authors: Asada D, Tomita H, Fujii T

Abstract
Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully. Simultaneous transcatheter correction is an effective therapeutic option even in infants.

PMID: 29991370 [PubMed - indexed for MEDLINE]

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Advanced care planning in adult congenital heart disease: Transitioning from repair to palliation and end-of-life care.

Int J Cardiol. 2018 Oct 26;:

Authors: Troost E, Roggen L, Goossens E, Moons P, De Meester P, Van De Bruaene A, Budts W

Abstract
As a result of advances in pediatric care, the majority of patients born with congenital heart disease (CHD) survive into adulthood [1]. Effective transfer and transition programs assure that patients with CHD remain in follow-up and receive continuous holistic care. Unfortunately, adult patients with CHD carry residual lesions and sequelae putting them at risk for premature death related to re-interventions or complications; most commonly heart failure and arrhythmia [2]. The scientific adult CHD (ACHD) community has been working hard to identify variables related to worse outcomes, modifying those where possible in order to improve survival. Indeed, survival in adults with CHD has increased, but consequently, on top of CHD-related complications, patients are increasingly exposed to the standard cardiovascular risk factors. Therefore, a program for lifelong coaching on health behavior and life style management becomes indispensable. More emerging is that a substantial number of patients, in particular those with complex heart defects, will eventually end up in a stage with hardly any medical or interventional options left. Our healthcare provision has to be prepared to organize care for this specific group of patients who will die prematurely and require the timely development and establishment of advanced care planning. Advanced care planning should preferentially be set-up in expert CHD centers. The long-lasting relationship in ACHD care with healthcare providers offers an excellent basis with regards to prognosis, advanced care planning and end-of-life issues.

PMID: 30413300 [PubMed - as supplied by publisher]

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Assessment of a novel radiation reduction protocol for pediatric and adult congenital device implantation.

J Interv Card Electrophysiol. 2018 Nov 09;:

Authors: Clark BC, Janson CM, Ceresnak SR, Osei FA, Bonney WJ, Nappo L, Pass RH

Abstract
PURPOSE: Device implantation requires fluoroscopic guidance, which carries inherent risks of ionizing radiation. We evaluated the impact of a low-dose fluoroscopic protocol on radiation exposure during device implantation.
METHODS: All patients who underwent pacemaker or ICD implantation with new transvenous leads from July 2011 to January 2018 were included. A novel ALARA protocol consisting of ultra-low frame rates (2-3 frames/s), low dose/frame (6-18 mGy/frame), and use of the "air-gap" technique in patients < 20 kg was employed. Demographics, procedural data, and radiation exposure levels were collected and analyzed.
RESULTS: Thirty patients underwent device implantation without additional catheterization, electrophysiology study, or ablation procedure (median age 15 years; range 5-50) with a total of 43 leads placed. Forty-seven percent of patients had a primary rhythm disturbance, 33% had cardiomyopathy, and 20% had congenital heart disease. Fifty percent were pacemakers (53% dual-chamber, 27% ventricle, 20% atrial) and 50% of devices implanted were ICDs (87% single-chamber). All implants were acutely successful with acceptable atrial and ventricular sensing and capture thresholds. The median fluoroscopy time was 11.5 min (inter-quartile range (IQR) 8.0-18.2), median air kerma dose 4.0 mGy (IQR 2.5-19.5), and median dose-area product 27.8 μGy/m2 (IQR 17.1-106.5). Median implant procedure time was 133 min. One patient required revision secondary to device migration without lead derangement 2 days post-procedure.
CONCLUSIONS: Use of a novel fluoroscopic protocol may help decrease radiation exposure to patients and staff without affecting efficacy or risk. These data may represent benchmarks against which future device implantation procedures can be compared.

PMID: 30413993 [PubMed - as supplied by publisher]

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Current understanding and perioperative management of pediatric pulmonary hypertension.

Paediatr Anaesth. 2018 Nov 10;:

Authors: Latham GJ, Yung D

Abstract
Pediatric pulmonary hypertension (PH) is a complex disease with multiple, diverse etiologies affecting the premature neonate to the young adult. Pediatric pulmonary arterial hypertension (PAH), whether idiopathic or associated with congenital heart disease, is the most commonly discussed form of pediatric PH, as it is progressive and lethal. However, neonatal forms of PH are vastly more frequent, and while most cases are transient, the risk of morbidity and mortality in this group deserves recognition. PH due to left heart disease is another subset increasingly recognized as an important cause of pediatric PH. One aspect of pediatric PH is very clear: anesthetizing the child with PH is associated with a significantly heightened risk of morbidity and mortality. It is therefore imperative that anesthesiologists who care for children with PH have a firm understanding of the pathophysiology of the various forms of pediatric PH, the impact of anesthesia and sedation in the setting of PH, and anesthesiologists' role as perioperative experts from preoperative planning to postoperative disposition. This review summarizes the current understanding of pediatric PH physiology, preoperative risk stratification, anesthetic risk, and intraoperative considerations relevant to the underlying pathophysiology of various forms of pediatric PH. This article is protected by copyright. All rights reserved.

PMID: 30414333 [PubMed - as supplied by publisher]

Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease.

Int J Cardiol. 2018 Oct 27;:

Authors: Vehmeijer JT, Hulleman M, Kuijpers JM, Blom MT, Tan HL, Mulder BJM, de Groot JR, Koster RW

Abstract
AIMS: Adult congenital heart disease (ACHD) patients are at increased risk of sudden cardiac death and out-of-hospital cardiac arrest (OHCA). Currently, insufficient data exist on outcome, causes and circumstances of OHCA of ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population.
METHODS AND RESULTS: We identified ACHD patients with OHCA by linking data from a Dutch nationwide registry of ACHD patients (CONCOR, n = 15,727), and ARREST, a cohort of OHCA cases (n = 17,868). 62 ACHD patients with OHCA were identified. Ventricular septal defect (n = 11), bicuspid aortic valve (n = 10) and atrial septal defect (n = 8) were the most common diagnoses. We included OHCA cases from the general population as controls. ACHD patients were younger than controls (n = 11,624) at the time of OHCA (47 (SD ± 17) years vs. 66 (SD ± 15) years, respectively, p < 0.001), and more often had a shockable initial rhythm (67% vs 40%, respectively, p < 0.001). A cardiac cause of OHCA was identified in 76% of ACHD patients, with only 7% due to myocardial infarction or ischemia. Survival was better in ACHD patients than in controls (44% vs. 19%, p < 0.001), but this difference disappeared after correction for age, gender, witnessed arrest, bystander resuscitation, public location and shockable rhythm.
CONCLUSIONS: OHCA in ACHD patients occurs at young age, is rarely caused by ischemia and occurs mainly in patients with simple congenital defects. Risk stratification efforts should therefore not be restricted to ACHD patients with severe congenital defects.

PMID: 30414749 [PubMed - as supplied by publisher]

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