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Are Blood Products Routinely Required in Pediatric Heart Surgery?

Pediatr Cardiol. 2020 Mar 13;:

Authors: Kato H, Chasovskyi K, Gandhi SK

Abstract
A restrictive blood transfusion strategy has emerged in adult cardiac surgery. However, the feasibility in children is poorly investigated. 352 consecutive patients undergoing open-heart surgery were retrospectively reviewed, excluding patients requiring extracorporeal membrane oxygenation. Patient demographics, perioperative blood product usage, and clinical outcome parameters were investigated. Variables predicting the need for blood products were delineated. Of the 352 study patients, 148 patients (42%) underwent bloodless surgery and 204 (58%) were transfused. Of the 204 transfused patients, 170 (83.4%) patients received one blood transfusion and 34 (16.6%) received two or more blood transfusions. Patient's weight and preoperative hematocrit (Hct) were statistically significant in predicting the need for blood priming the CPB circuit (AUC 0.99, p < 0.001, sensitivity 96.6%, specificity 95.2%). A body weight of 8.5 kg carried a sensitivity of 100% and specificity of 94.5% (p < 0.001) for a blood prime. Among patients with a weight less than 8.5 kg (n = 171), only 27 patients (15.8%, p < 0.001) required additional transfusion of PRBCs. Factors impacting the need for a blood transfusion during CPB included redo surgery [odds ratio (OR) 4.61, p = 0.001] and the highest lactate level on CPB (OR 1.65, p = 0.006). Redo surgery had the highest impact (OR 7.27, p = 0.012) for requiring a postoperative PRBC transfusion. A restrictive transfusion strategy can be safely implemented in pediatric cardiac surgery. The majority of children with a BW > 8.5 kg required no blood products and those with a BW ≤ 8.5 kg required only 1 unit of blood, to prime the cardiopulmonary bypass circuit.

PMID: 32170329 [PubMed - as supplied by publisher]

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Spontaneous coronary artery dissection in systemic lupus erythematosus: case-based review.

Rheumatol Int. 2019 Oct;39(10):1821-1827

Authors: Álvarez-Lario B, Álvarez-Roy L, Mayordomo-Gómez S, García-García JM

Abstract
Patients with systemic lupus erythematosus (SLE) present an increased prevalence of coronary heart disease. The majority of cases of acute coronary syndrome (ACS) in patients with SLE are due to atherosclerosis. Less common causes include thrombosis of an angiographically normal coronary artery and coronary vasculitis. Spontaneous coronary artery dissection (SCAD) is a rare cause of ACS in these patients. We report the case of a 53-year-old female diagnosed of SLE presenting with an ACS caused by SCAD. She was treated medically and her clinical course was favorable. A literature search identified seven additional cases of SCAD associated with SLE. The main clinical features found in these reports are revised. ACS caused by SCAD in SLE patients is a condition likely under-reported in literature. SCAD should be suspected in patients with SLE and ACS, especially in younger women without evident cardiovascular risk factors. An early accurate diagnosis of SCAD is key to provide specific treatment, which differs from that of usual atherosclerotic ACS.

PMID: 31227856 [PubMed - indexed for MEDLINE]

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Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study.

Clin Respir J. 2019 Nov;13(11):693-699

Authors: Deng X, Jin B, Li S, Li Y, Zhou H, Wu Y, Yan M, Hu Y, Qiu Q, Zhang G, Zheng X

Abstract
INTRODUCTION: Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD).
OBJECTIVES: The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up.
METHODS: We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed.
RESULTS: There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO2 were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001).
CONCLUSIONS: Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients.

PMID: 31419027 [PubMed - indexed for MEDLINE]

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Coronary-cameral fistula with double-chambered right ventricle: appearance on cardiac magnetic resonance imaging and 3D printed anatomic modeling.

Clin Imaging. 2020 Jan;59(1):84-87

Authors: Mokkarala M, Ballard DH, Wesley RA, Gutierrez FR, Javidan-Nejad C, Singh GK, Woodard PK, Lindley KJ

Abstract
The present case illustrates cardiac magnetic resonance imaging (MRI) and three-dimensional (3D) printed anatomic model findings of a coronary-cameral fistula (CCF) and double-chambered right ventricle (DCRV). A pregnant woman presented with palpitations and near syncope. A non-contrast cardiac MRI showed CCF connecting to a DCRV. Post-delivery, the patient had a contrast-enhanced MRI and 3D printed anatomic model to better evaluate her aberrant anatomy.

PMID: 31760282 [PubMed - indexed for MEDLINE]

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Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis.

J Am Heart Assoc. 2020 Mar 17;9(6):e014172

Authors: Marshall KH, D'Udekem Y, Sholler GF, Opotowsky AR, Costa DSJ, Sharpe L, Celermajer DS, Winlaw DS, Newburger JW, Kasparian NA

Abstract
Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019. Standardized mean differences (SMD) were calculated using fixed and random-effects models. Fifty articles reporting on 29 unique studies capturing HRQOL outcomes for 2793 people with a Fontan circulation and 1437 parent-proxies were analyzed. HRQOL was lower in individuals with a Fontan circulation compared with healthy referents or normative samples (SMD, -0.92; 95% CI, -1.36 to -0.48; P<0.001). Lower scores were reported across all HRQOL domains, with the largest differences found for physical (SMD, -0.90; 95% CI, -1.13 to -0.67; P<0.001) and school/work functioning (SMD, -0.71; 95% CI, -0.90 to -0.52; P<0.001). Meta-regression analyses found no significant predictors of self-reported physical functioning, but older age at Fontan operation was associated with poorer emotional functioning (β=-0.124; P=0.004), and diagnosis of hypoplastic left heart was associated with poorer social functioning (β=-0.007; P=0.048). Sensitivity analyses showed use of the PedsQL Core Module was associated with lower HRQOL scores compared with the Short-Form Health Survey-36. Conclusions HRQOL outcomes for people with a Fontan circulation are lower than the general population. Optimal care acknowledges the lifelong impact of the Fontan circulation on HRQOL and offers targeted strategies to improve outcomes for this growing population.

PMID: 32172648 [PubMed - in process]

Related Articles

Paediatric haemodynamic modelling: development and experimental validation using quantitative flow MRI.

Eur Radiol Exp. 2020 Mar 16;4(1):16

Authors: Mohammadyari P, Gadda G, Taibi A, Munuera Del Cerro J

Abstract
BACKGROUND: Congenital vascular disease is one of the leading causes of death in paediatric age. Despite the importance of paediatric haemodynamics, large investigations have been devoted to the evaluation of circulation in adults. The novelty of this study consists in the development of a well calibrated mathematical model of cardiovascular circulation in paediatric subjects. To reach the purpose, a model for adult circulation was modified and recalibrated with experimental data and literature from children to be able to calculate the flow rates and pressures in the brain and neck.
METHODS: The haemodynamic model simulates the 76 main arteries, together with the main veins in brain and neck. A proper magnetic resonance imaging (MRI) dataset of 29 volunteers aged 12 ± 5 years (mean ± standard deviation) was used to extract age-dependent physiological and clinical parameters such as heart rate, flow rate, vessel cross section area, and blood pressure. The computational model was calibrated using such experimental data. The paediatric and adult model results were compared.
RESULTS: Increase of the vessels stiffness due to aging contributes to a flow rate decrease while blood pressure increases. In accordance, our simulation results show about 16% decrease in mean pressure of internal jugular vein in paediatric rather than adult subjects. The model outcomes indicated about 88% correlation with MRI data.
CONCLUSIONS: The mathematical model simulates the paediatric head and neck blood circulation. The model provides detailed information of human haemodynamics including arterial and venous network to study both paediatric and adult blood circulation.

PMID: 32173785 [PubMed - in process]

Related Articles

Determinants of Sudden Cardiac Death in Adult Patients With Eisenmenger Syndrome.

J Am Heart Assoc. 2020 Mar 17;9(6):e014554

Authors: Chiriac A, Riley DC, Russell M, Moore JP, Padmanabhan D, Hodge DO, Spiegel MR, Vargas ER, Phillips SD, Ammash NM, Madhavan M, Asirvatham SJ, McLeod CJ

Abstract
Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We sought to define the predictors of SCD in this population. Methods and Results A retrospective analysis of all patients with Eisenmenger syndrome from 2 large tertiary referral centers was performed. ECGs, prolonged ambulatory recordings, echocardiograms, and clinical histories were reviewed; and the cause of death was identified. A total of 246 patients (85 [34.6%] men) with a mean age of 37.3 (±14.2) years were followed up for a median of 7 years. Over the study period, 136 patients died, with 40 experiencing SCD and 74 experiencing cardiac death (sudden and nonsudden). Age, atrial fibrillation, prolonged QRS duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased risk of SCD, whereas advanced pulmonary hypertension therapies were protective. Atrial fibrillation (11.45-fold increased risk; P<0.001) and QRS duration ≥120 ms (2.06-fold increased risk; P=0.034) remained significant predictors of SCD in the multivariate analysis, whereas advanced pulmonary hypertension therapies were strongly protective against SCD (P<0.001). Conclusions Atrial arrhythmias, impaired ventricular function, and conduction system disease were associated with increased risk of SCD in this cohort of patients with Eisenmenger syndrome, providing an opportunity for early risk stratification and potential intervention. Clinical heart failure symptoms (New York Heart Association class ≥II) were predictive of increased mortality but not of SCD, suggesting a potential arrhythmic cause behind SCD.

PMID: 32174228 [PubMed - in process]

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Modified delivery of SAPIEN 3 valve in the pulmonary position: Evolution of technique or dire need?

Catheter Cardiovasc Interv. 2019 02 15;93(3):484-487

Authors: Shahanavaz S, Rockefeller T, Nicolas R, Balzer D

Abstract
Transcatheter pulmonary valve replacement (TPVR) is now common practice in patients with significant pulmonary regurgitation (PR), stenosis (PS), or mixed pulmonary valve disease. While the Melody valve (Medtronic, Inc.) and its delivery system were specifically designed for use in the right ventricular outflow tract (RVOT), Sapien valves (XT and S3, Edwards Lifesciences, Inc.) and their delivery systems (Novaflex and Commander) were not. As a result, placement of Sapien valves in the RVOT can be challenging secondary to the stiffness of the current delivery system. Manipulating a stiff delivery system within the RV also introduces risk to the tricuspid valve and its chordal attachments. We report a novel delivery technique for the Sapien S3 valve which has greatly simplified valve delivery into the RVOT and may potentially reduce the risk of tricuspid valve injury.

PMID: 30408317 [PubMed - indexed for MEDLINE]

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Initial results from the off-label use of the SAPIEN S3 valve for percutaneous transcatheter pulmonary valve replacement: A multi-institutional experience.

Catheter Cardiovasc Interv. 2019 02 15;93(3):455-463

Authors: Sinha S, Aboulhosn J, Asnes J, Bocks M, Zahn E, Goldstein BH, Zampi J, Hellenbrand W, Salem M, Levi D

Abstract
OBJECTIVES: To describe a multi-center experience of percutaneous transcatheter pulmonary valve replacement (TPVR) using the Edwards Sapien S3 Valve.
BACKGROUND: Off-label use of the Sapien S3 valve can allow for TPVR in patients with congenital heart disease (CHD) and large diameter dysfunctional right ventricular outflow tract (RVOT). The initial experience at five centers with the SAPIEN S3 valve for TPVR is presented with a focus on procedural techniques, success, complications, and efficacy.
METHODS: A retrospective review was performed of all patients with CHD and dysfunctional RVOT who underwent TPVR using Sapien S3 valve. Imaging data, procedural elements, and clinical follow-up data were collected to evaluate acute and short-term results.
RESULTS: A total of 50 patients underwent percutaneous placement of the Sapien S3 in the pulmonary position. Of these, 38 were placed into "native RVOTs", measuring 24-32 mm in diameter, as assessed by compliant balloon sizing. In all cases, the valve was implanted after introduction and there were no cases of valve embolization. On follow up (range 1-9 months, median 3 months), no patients had significant obstruction or regurgitation through or around the valve requiring intervention. There were no procedural deaths. Major complications included severe aortic compression (n = 1) and tricuspid valve (TV) injury related to valve placement (n = 2) and prestenting (n = 1).
CONCLUSIONS: TPVR in patients with large diameter dysfunctional RVOTs can be effectively performed with the Sapien S3. All procedures were technically successful with no embolizations, no perivalvular leaks, and excellent short-term valve function. Tricuspid valve injury from implantation of an uncovered valve was a serious procedural complication.

PMID: 30537348 [PubMed - indexed for MEDLINE]

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A comparison of oral flecainide and amiodarone for the treatment of recurrent supraventricular tachycardia in children.

Pacing Clin Electrophysiol. 2019 06;42(6):670-677

Authors: Hill AC, Silka MJ, Bar-Cohen Y

Abstract
BACKGROUND: Supraventricular tachycardia (SVT) in children can be difficult to treat when first-line therapies (beta-blockade or digoxin) are not effective. Both flecainide and amiodarone are used as second-line therapies. We sought to compare the efficacy and safety of flecainide and amiodarone in pediatric patients with recurrent SVT.
METHODS: Pediatric patients treated with oral flecainide or oral amiodarone for SVT between 2006 and 2015 were studied. Tachycardia mechanisms included orthodromic reciprocating tachycardia (ORT), intra-atrial reentrant tachycardia (IART), and ectopic atrial tachycardia (EAT). Outcomes were classified as full success, partial success (requiring additional intervention), or failure.
RESULTS: Seventy-four patients were included (median age 46 days, range 1 day to 19 years). Flecainide was used in 47 patients and amiodarone in 27 patients. Full success was achieved in 68% and 59%, respectively (P = 0.28). Partial success was achieved in 13% and 19%, respectively (P = 0.12). Treatment failed in 19% and 22%, respectively (P = 0.97). Ten crossover patients received the second medication after the first failed. Of five amiodarone-to-flecainide crossovers, four achieved success on flecainide alone. Of five flecainide-to-amiodarone crossovers, two achieved success. Minor adverse events occurred in 9% of flecainide and 22% of amiodarone patients (P = 0.16). No significant differences were seen by arrhythmia subtype (36 EAT, 28 ORT, 10 IART), congenital heart disease (n = 38), or age group (56 infants).
CONCLUSIONS: Oral flecainide and amiodarone achieved meaningful arrhythmia control in 81% and 78% of pediatric patients with recurrent SVT, respectively. Those who failed amiodarone had encouraging outcomes when changed to flecainide.

PMID: 30875081 [PubMed - indexed for MEDLINE]

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