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Response by Heng et al to Letter Regarding Article, "Immediate and Midterm Cardiac Remodeling After Surgical Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study".

Circulation. 2018 May 15;137(20):2186-2187

Authors: Heng EL, Diller GP, Gatzoulis MA, Babu-Narayan SV

PMID: 29760230 [PubMed - in process]

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Analysis of Serum Metabolites to Diagnose Bicuspid Aortic Valve.

Sci Rep. 2016 11 15;6:37023

Authors: Wang W, Maimaiti A, Zhao Y, Zhang L, Tao H, Nian H, Xia L, Kong B, Wang C, Liu M, Wei L

Bicuspid aortic valve (BAV) is the most common congenital heart disease. The current study aims to construct a diagnostic model based on metabolic profiling as a non-invasive tool for BAV screening. Blood serum samples were prepared from an estimation group and a validation group, each consisting of 30 BAV patients and 20 healthy individuals, and analyzed by liquid chromatography-mass spectrometry (LC-MS). In total, 2213 metabolites were detected and 41 were considered different. A model for predicting BAV in the estimation group was constructed using the concentration levels of monoglyceride (MG) (18:2) and glycerophospho-N-oleoyl ethanolamine (GNOE). A novel model named Zhongshan (ZS) was developed to amplify the association between BAV and the two metabolites. The area under curve (AUC) of ZS for BAV prediction was 0.900 (0.782-0.967) and was superior to all single-metabolite models when applied to the estimation group. Using optimized cutoff (-0.1634), ZS model had a sensitivity score of 76.7%, specificity score of 90.0%, positive predictive value of 80% and negative predictive value of 85.0% for the validation group. These results support the use of serum-based metabolomics profiling method as a complementary tool for BAV screening in large populations.

PMID: 27845433 [PubMed - indexed for MEDLINE]

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[Percutaneous coronary intervention and minimally invasive aortic valve replacement for patients with aortic valve disease and coronary artery disease].

Przegl Lek. 2017;74(3):96-100

Authors: Stoliński J, Plicner D, Mędrzyński M, Kapelak B

Objectives: To report the results of hybrid approach combining percutaneous coronary intervention (PCI) and minimally invasive aortic valve replacement through right anterior minithoracotomy (RT-AVR) for patients with aortic valve disease and coronary artery disease.
Materials and Methods: Retrospective analysis of 53 hybrid RT-AVR/ PCI procedures where RT-AVR was performed first in the operating room and followed immediately by PCI performed in the catheterization laboratory.
Results: Predicted with Euro- SCORE II and observed hospital mortality was 8.7±2.9% and 1.9% respectively. Hospital and intensive care unit length of stay were 5.6±2.4 and 1.8±1.4 days respectively. Biological aortic valve prosthesis was implanted in 40 (75.5%) patients. PCI of LAD was performed in 5 patients (9.4%), of Dg in 10 (18.9%) patients, of Mg or Cx in 21 (39.6%) patients, of PDA or RCA in 25 (47.2%) patients. Two vessels and three vessels PCI were performed in 5 (9.4%) and 3 (5.7%) patients respectively. DES were used during PCI in 42 (79.2%) patients. Dual antiplatelet therapy with 75 mg of Aspirin and 75 mg of Clopidogrel started after RTAVR/ PCI. Complications occurred in 16 (30.2%) patients after hybrid RT-AVR/PCI procedure. Prolonged above 24 hours mechanical ventilation time was necessary in 3 patients (5.7%). Renal insufficiency occurred in 4 (7.5%), stroke in 1 (1.9%) patient. Pacemaker was implanted in 2 (3.8%) patients after surgery. Conversion to conventional surgery through median sternotomy was performed in 1 patient (1.9%), surgical revision due to postoperative bleeding in 2 patients (3.8%). No perioperative myocardial infarction and no mediastinitis was diagnosed after RT-AVR/PCI procedure. Postoperative chest blood drainage was 245.0±181.0 ml. Red blood cells transfusion was required in 10 (18.9%) patients.
Conclusions: The hybrid RT-AVR/PCI procedure for these high risk patients with aortic valve disease and coronary artery disease presented in our series favourable mortality results compared to predicted with EuroSCORE II mortality for conventional cardiac surgery.

PMID: 29694767 [PubMed - indexed for MEDLINE]

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Trans-septal approach for percutaneous closure of infra-diaphragmatic veno-venous collateral in a patient after Fontan palliation.

Cardiol Young. 2017 Sep;27(7):1413-1415

Authors: Chatterjee A, Cribbs MG, Law MA

In this article, we present a case of a desaturated Fontan patient with an infra-diaphragmatic venous collateral to the pulmonary vein, which was too tortuous to attempt closure at the source. A trans-septal approach was successfully used to close the collateral in a retrograde manner.

PMID: 28782497 [PubMed - indexed for MEDLINE]

Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein's anomaly.

Acta Obstet Gynecol Scand. 2018 May 16;:

Authors: Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T

INTRODUCTION: We examined the perinatal outcomes and right ventricular function before pregnancy, during pregnancy, and after delivery in women with Ebstein's anomaly.
MATERIAL AND METHODS: We retrospectively investigated the clinical course and mode of delivery and monitored hemodynamic parameters throughout pregnancy in 17 women with Ebstein's anomaly, who delivered at our institution during the period of 1995-2015.
RESULTS: Eight women, including nine pregnancies, underwent elective cesarean section, and nine women, including 14 pregnancies, underwent vaginal delivery. Elective cesarean section was performed in cases with significant heart failure or arrhythmias and in the presence of more than 2 of the following: cardiothoracic ratio ≥60%, moderate or severe tricuspid valve regurgitation, or tricuspid valve regurgitation pressure gradient ≥35 mmHg during pregnancy. The cardiothoracic ratio and tricuspid valve regurgitation pressure gradient significantly increased during pregnancy compared to pre-pregnancy values. New York Heart Association classification deteriorated from class I to class II or III in five cases during pregnancy.
CONCLUSIONS: Although pregnancy was relatively safe among women with Ebstein's anomaly, some women developed cyanosis, arrhythmia, and heart failure, leading to elective cesarean section. Monitoring clinical and hemodynamic changes throughout pregnancy is advised in order to minimize maternal cardiac risk and select the appropriate mode of delivery. This article is protected by copyright. All rights reserved.

PMID: 29768659 [PubMed - as supplied by publisher]

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Recent advances in managing vascular occlusions in the cardiac catheterization laboratory.

F1000Res. 2018;7:

Authors: Qureshi AM, Mullins CE, Latson LA

Vascular occlusions continue to be a significant cause of morbidity and mortality. The management of vascular occlusions in patients is complex, requiring specialized expertise in the cardiac catheterization laboratory and from other disciplines. Knowledge of currently available tools at the operator's disposal is important to optimize the success of these procedures. In this review, we discuss some of the recent advances in recanalization procedures of vascular occlusions and thrombotic lesions in the cardiac catheterization laboratory.

PMID: 29770200 [PubMed]

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Regenerative medicine therapy for single ventricle congenital heart disease.

Transl Pediatr. 2018 Apr;7(2):176-187

Authors: Ambastha C, Bittle GJ, Morales D, Parchment N, Saha P, Mishra R, Sharma S, Vasilenko A, Gunasekaran M, Al-Suqi MT, Li D, Yang P, Kaushal S

One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years. At the conclusion of the third surgery, systemic perfusion is provided by the right ventricle (RV), and deoxygenated blood flows passively to the pulmonary vasculature. Despite these palliative interventions, the RV, which is ill suited to provide long-term systemic perfusion, is prone to eventual failure. In the absence of satisfying curative treatments, stem cell therapy may represent one innovative approach to the management of RV dysfunction in HLHS patients. Several stem cell populations from different tissues (cardiac and non-cardiac), different age groups (adult- vs. neonate-derived), and different donors (autologous vs. allogeneic), are under active investigation. Preclinical trials in small and large animal models have elucidated several mechanisms by which these stem cells affect the injured myocardium, and are driving the shift from a paradigm based upon cellular engraftment and differentiation to one based primarily on paracrine effects. Recent studies have comprehensively evaluated the individual components of the stem cells' secretomes, shedding new light on the intracellular and extracellular pathways at the center of their therapeutic effects. This research has laid the groundwork for clinical application, and there are now several trials of stem cell therapies in pediatric populations that will provide important insights into the value of this therapeutic strategy in the management of HLHS and other forms of CHD. This article reviews the many stem cell types applied to CHD, their preclinical investigation and the mechanisms by which they might affect RV dysfunction in HLHS patients, and finally, the completed and ongoing clinical trials of stem cell therapy in patients with CHD.

PMID: 29770299 [PubMed]

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A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava.

J Clin Ultrasound. 2017 Sep;45(7):430-433

Authors: Moradi B, Moosavi NS, Kazemi MA, Tahmasebpour AR, Fattahi Masrour F

Heterotaxy syndromes are defined as the disorders that involve abnormal arrangement of viscera. We present a case of prenatally diagnosed left isomerism in a 30-year-old primigravida woman referred to our hospital for complex cardiac abnormality. Sonographic findings included heart block, unbalanced atrioventricular septal defect, interruption of the inferior vena cava with hemiazygos continuation, double superior vena cava, a right-sided stomach, and biliary atresia. The hemiazygos vein drained into the right atrium by the persistent left superior vena cava. This type of inferior vena cava interruption and continuation is rarely reported in prenatally detected cases of left isomerism. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:430-433, 2017.

PMID: 28256002 [PubMed - indexed for MEDLINE]

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Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

Eur J Cardiothorac Surg. 2017 Sep 01;52(3):581-587

Authors: Vergnat M, Asfour B, Arenz C, Suchowerskyj P, Bierbach B, Schindler E, Schneider M, Hraska V

OBJECTIVES: Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease.
METHODS: From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% <1 year); 86 (45%) had a preceding balloon valvuloplasty. The indications for the procedure were stenotic (n = 123; 64%), regurgitant (n = 63; 33%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients.
RESULTS: The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%).
CONCLUSIONS: Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes.

PMID: 28874025 [PubMed - indexed for MEDLINE]

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Extracorporeal Cardiopulmonary Resuscitation Among Patients with Structurally Normal Hearts.

ASAIO J. 2017 Nov/Dec;63(6):781-786

Authors: Conrad SJ, Bridges BC, Kalra Y, Pietsch JB, Smith AH

Extracorporeal cardiopulmonary resuscitation (eCPR) has been well described as a rescue therapy in refractory cardiac arrest among patients with congenital heart disease. The purpose of this retrospective analysis of data from the Extracorporeal Life Support Organization was to evaluate outcomes of eCPR in patients with structurally normal hearts and to identify risk factors that may contribute to mortality. During the study period, 1,431 patients met inclusion criteria. Median age was 16 years. Overall survival to hospital discharge was 32%. Conditional logistic regression demonstrated an independent survival benefit among smaller patients, patients with a lower partial pressure of carbon dioxide (PaCO2) on cannulation, and those with a shorter duration from intubation to eCPR cannulation. A diagnosis of sepsis was independently associated with a nearly threefold increase in odds of mortality, whereas the diagnosis of myocarditis portended a more favorable outcome. Neurologic complications, pulmonary hemorrhage, disseminated intravascular coagulation, CPR, pH less than 7.20, and hyperbilirubinemia after eCPR cannulation were independently associated with an increase in odds of mortality. When utilizing eCPR in patients with structurally normal hearts, a diagnosis of sepsis is independently associated with mortality, whereas a diagnosis of myocarditis is protective. Neurologic complications and pulmonary hemorrhage while on extracorporeal membrane oxygenation (ECMO) are independently associated with mortality.

PMID: 29084037 [PubMed - indexed for MEDLINE]