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Single-Center Experience Using Selexipag in a Pediatric Population.

Pediatr Cardiol. 2017 Oct;38(7):1405-1409

Authors: Gallotti R, Drogalis-Kim DE, Satou G, Alejos J

Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi®), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH.

PMID: 28702718 [PubMed - indexed for MEDLINE]

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Prevalence of Coronary Artery Disease Risk Factors and Metabolic Syndrome in Children with Heart Disease.

Pediatr Cardiol. 2018 Feb;39(2):261-267

Authors: Ware AL, Young PC, Weng C, Presson AP, Minich LL, Menon SC

Children with acquired and congenital heart disease (CHD) are increasingly surviving to adulthood. Our aim was to determine the prevalence of coronary artery disease (CAD) risk factors in children at known risk for early CAD or with severe CHD. We recruited children (8-19 years) at risk for early CAD-Kawasaki disease (KD, N = 36) and coarctation (69) or severe CHD: > 1 cardiopulmonary bypass surgery (60), single ventricle (15), prosthetic valves (13). Anthropometric measurements, blood pressure, and fasting lipid data were compared with summaries from National Health and Nutrition Examination Survey (NHANES) publications (1999-2012). Relative risk (RR) ratios were calculated based on age classification and pooled to obtain overall RR. Of 174 subjects, 106 were male (61%) and 138 (79%) had CHD. Compared to NHANES data, hypertension and low HDL were higher in the study cohort [RR 11.7 (CI 6.34-21.6), p < 0.001; and 1.79 (CI 1.36-2.35). p < 0.001] and obesity and elevated total cholesterol were lower [RR 0.59 (CI 0.37-0.96), p = 0.03; and 0.42 (CI 0.19-0.95), p = 0.04]. Elevated non-HDL was similar between groups. Age category had similar RR for all CAD risk factors. Eight subjects had metabolic syndrome. Risk factors were similar between KD versus CHD subgroups. Both coarctation and non-coarctation subjects had increased RR for hypertension. Hypertension is the most common risk factor for children at risk of early CAD and severe CHD. Metabolic syndrome is rare. These patients should be screened and treated for hypertension and current recommendations for universal lipid screening are adequate for follow-up.

PMID: 29058030 [PubMed - indexed for MEDLINE]

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Magnetic Resonance Myocardial Perfusion Imaging: Safety and Indications in Pediatrics and Young Adults.

Pediatr Cardiol. 2018 Feb;39(2):275-282

Authors: Biko DM, Collins RT, Partington SL, Harris M, Whitehead KK, Keller MS, Fogel MA

The purpose of this study was to assess the safety and indications for cardiac magnetic resonance (CMR) with myocardial perfusion imaging (MPI) in a cohort of children and young adults. A retrospective review of 178 children and young adults who underwent CMR with MPI was performed. Studies were categorized based on study protocols as MPI with resting perfusion only, adenosine stress MPI, exercise-induced stress MPI, and MPI for cardiac mass diagnosis. Relevant clinical history, exam indications, and adverse reactions following gadolinium-based contrast agent and adenosine administration were recorded. Studies were reviewed for the presence of myocardial perfusion defects, wall motion abnormalities, and delayed myocardial enhancement. The most common indications from MPI were congenital heart disease (CHD), Kawasaki disease, anomalous coronary artery, or myocardial mass characterization. Of these, 51% were protocoled with adenosine stress, 23% without stress, 6% with exercise stress, and 20% for cardiac mass evaluation. Excluding patients for myocardial mass evaluation, MPI defects were present in 16% (14 with adenosine stress, 1 with exercise stress, 8 on resting studies only). For cardiac mass evaluation, a mass was confirmed in 58%. No adverse reactions occurred with intravenous administration of a gadolinium-based contrast agent. Three self-limited adverse reactions, 2 patients with chest pain, and 1 patient with bradycardia, occurred following adenosine administration. MPI is a safe modality for the evaluation of pediatric and young adults with minimal adverse events. The most common indications for MPI were for the evaluation of CHD, Kawasaki disease, anomalous coronary artery, or myocardial mass characterization.

PMID: 29063953 [PubMed - indexed for MEDLINE]

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Usefulness of Routine Coronary CT Angiography in Patients with Transposition of the Great Arteries After an Arterial Switch Operation.

Pediatr Cardiol. 2018 Feb;39(2):335-346

Authors: Szymczyk K, Moll M, Sobczak-Budlewska K, Moll JA, Stefańczyk L, Grzelak P, Moll JJ, Michalak KW

Coronary complications in patients with transposition of the great arteries (TGA) after an arterial switch operation (ASO) are relatively rare, but of all the possible postoperative adverse events, they are potentially the most dangerous. The fate of the coronary arteries, which are transplanted during the neonatal ASO, remains uncertain. There is also no consensus regarding their postoperative evaluation, especially in asymptomatic patients. The aim of this study was to present the early results of routinely performed coronary computed tomography angiography (CCTA) in asymptomatic adolescents and young adults with TGA after an ASO. An initial series of 50 CCTAs performed in asymptomatic patients with TGA after an ASO were evaluated. In each case, a detailed examination of the coronary anatomy, its relationship to the surrounding structures, its exact position in the neoaortic sinus, and the presence of significant coronary abnormalities was performed. The CT scans revealed significant coronary abnormalities in 12 asymptomatic patients: three had acute proximal angulation and stenosis, four had an intra-arterial course, seven had a muscular bridge, one had a left anterior descending artery with an intramuscular course, and one had coronary fistulas to the pulmonary arteries. Additionally, in 25 patients, proximal acute angulation of at least one coronary artery was detected, and four of them had a high ellipticity index. Most of the potentially severe anatomical features were related to the left coronary artery or the left anterior descending artery. CCTA routinely performed on asymptomatic patients with TGA after an ASO provides accurate and useful information for postoperative management. The frequency of coronary anomalies and potentially dangerous anatomical features in this group of patients is high, and their impact on postoperative follow-up remains unknown.

PMID: 29090351 [PubMed - indexed for MEDLINE]

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Pulmonary Valve Morphology in Patients with Bicuspid Aortic Valves.

Pediatr Cardiol. 2018 Apr;39(4):690-694

Authors: Koenraadt WMC, Bartelings MM, Gittenberger-de Groot AC, Bökenkamp R, DeRuiter MC, Schalij MJ, Jongbloed MRM

The aortic and pulmonary valve share a common developmental origin from the embryonic arterial trunk. Bicuspid aortic valve is the most common congenital anomaly and can occur isolated as well as in association with other congenital heart disease (CHD). Data on pulmonary valve morphology in these cases are scarce. In this study, we aimed to determine pulmonary valve morphology in hearts with BAV associated with CHD. In 83 post-mortem heart specimens with BAV and associated CHD, pulmonary valve morphology was studied and related to BAV morphology. In 14/83 (17%) hearts, the pulmonary valve was affected, bicuspid in 8/83 (10%), dome-shaped in 3/83 (4%) and atretic in 3/83 (4%). In specimens with a bicuspid pulmonary valve, 5/8 (63%) had a strictly bicuspid aortic valve (without raphe), 2/3 hearts (67%) with dome-shaped pulmonary valves and 2/3 hearts (67%) with atretic pulmonary valves had BAV without raphe. Six out of eight (75%) specimens with a bicuspid pulmonary valve had a perimembranous ventricular septal defect (VSD). 4/8 (50%) specimens with a bicuspid pulmonary valve were associated with chromosomal abnormalities: 3 (38%) had trisomy 18 and 1 (13%) had trisomy 13. In BAV with associated CHD, abnormal pulmonary valve morphology was observed in 17% of the hearts. The majority of hearts with abnormal pulmonary valve morphology had a Type B bicuspid aortic valve (without raphe). Bilateral semilunar valvular disease is associated with Type B BAVs and in many cases related to chromosomal abnormalities. As this study was performed in post-mortem specimens with high frequency of associated CHD, caution is warranted with application of these results to the general BAV population.

PMID: 29340729 [PubMed - indexed for MEDLINE]

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Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance.

Pediatr Cardiol. 2018 Apr;39(4):695-704

Authors: Ait Ali L, Pingitore A, Piaggi P, Brucini F, Passera M, Marotta M, Cadoni A, Passino C, Catapano G, Festa P

Fontan palliation allows patients with "single ventricle" circulation to reach adulthood with an acceptable quality of life, although exercise tolerance is significantly reduced. To assess whether controlled respiratory training (CRT) increases cardiorespiratory performance. 16 Adolescent Fontan patients (age 17. 5 ± 3.8 years) were enrolled. Patients were divided into CRT group (n = 10) and control group (C group, n = 6). Maximal cardiopulmonary test (CPT) was repeated at the end of CRT in the CRT group and after an average time of 3 months in the C group. In the CRT group a CPT endurance was also performed before and after CRT. In the CRT group there was a significant improvement in cardiovascular and respiratory response to exercise after CRT. Actually, after accounting for baseline values, the CRT group had decreased breathing respiratory reserve (- 15, 95% CI -22.3 to - 8.0, p = 0.001) and increased RR peak (+ 4.8, 95% CI 0.7-8.9, p = 0.03), VE peak (+ 13.7, 95% CI 5.6-21.7, p = 0.004), VO2 of predicted (+ 8.5, 95% CI 0.1-17.0, p = 0.05), VO2 peak (+ 4.3, 95% CI 0.3 to 8.2, p = 0.04), and VO2 workslope (+ 1.7, 95% CI 0.3-3.1, p = 0.02) as compared to the control group. Moreover, exercise endurance time increased from 8.45 to 17.7 min (p = 0.01). CRT improves cardiorespiratory performance in post-Fontan patients leading to a better aerobic capacity.

PMID: 29349618 [PubMed - indexed for MEDLINE]

Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI.

Eur Heart J Cardiovasc Imaging. 2018 Aug 06;:

Authors: Di Salvo G, Miller O, Babu Narayan S, Li W, Budts W, Valsangiacomo Buechel ER, Frigiola A, van den Bosch AE, Bonello B, Mertens L, Hussain T, Parish V, Habib G, Edvardsen T, Geva T, Baumgartner H, Gatzoulis MA, 2016–2018 EACVI Scientific Documents Committee

Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.

PMID: 30084968 [PubMed - as supplied by publisher]

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Hemodynamic Evaluation of a Biological and Mechanical Aortic Valve Prosthesis Using Patient-Specific MRI-Based CFD.

Artif Organs. 2018 Jan;42(1):49-57

Authors: Hellmeier F, Nordmeyer S, Yevtushenko P, Bruening J, Berger F, Kuehne T, Goubergrits L, Kelm M

Modeling different treatment options before a procedure is performed is a promising approach for surgical decision making and patient care in heart valve disease. This study investigated the hemodynamic impact of different prostheses through patient-specific MRI-based CFD simulations. Ten time-resolved MRI data sets with and without velocity encoding were obtained to reconstruct the aorta and set hemodynamic boundary conditions for simulations. Aortic hemodynamics after virtual valve replacement with a biological and mechanical valve prosthesis were investigated. Wall shear stress (WSS), secondary flow degree (SFD), transvalvular pressure drop (TPD), turbulent kinetic energy (TKE), and normalized flow displacement (NFD) were evaluated to characterize valve-induced hemodynamics. The biological prostheses induced significantly higher WSS (medians: 9.3 vs. 8.6 Pa, P = 0.027) and SFD (means: 0.78 vs. 0.49, P = 0.002) in the ascending aorta, TPD (medians: 11.4 vs. 2.7 mm Hg, P = 0.002), TKE (means: 400 vs. 283 cm2 /s2 , P = 0.037), and NFD (means: 0.0994 vs. 0.0607, P = 0.020) than the mechanical prostheses. The differences between the prosthesis types showed great inter-patient variability, however. Given this variability, a patient-specific evaluation is warranted. In conclusion, MRI-based CFD offers an opportunity to assess the interactions between prosthesis and patient-specific boundary conditions, which may help in optimizing surgical decision making and providing additional guidance to clinicians.

PMID: 28853220 [PubMed - indexed for MEDLINE]

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Embolic stroke in a postpartum patient with Fontan physiology.

BMJ Case Rep. 2018 Jan 05;2018:

Authors: VanHise K, Li L, Smith E, Condon N

In cardiac physiology, single ventricle typically refers to the presence of a dominant systemic ventricle along with a hypoplastic ventricle. The Fontan operation is used to repair the single ventricle by directing deoxygenated systemic venous flow to the pulmonary arterial circulation. Normal pregnancy physiology, particularly increased intravascular volume, increased heart rate, increased cardiac output, hypercoagulability and decreased systemic vascular resistance, can exacerbate cardiac disease in patients with Fontan circulation, leading to pregnancy complications. Despite the known risks, there are limited data addressing the question of anticoagulation in pregnant patients with Fontan physiology. Herein we present the case of a Fontan patient who had a successful pregnancy and delivery, and developed an embolic stroke in the postpartum period.

PMID: 29305367 [PubMed - indexed for MEDLINE]

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In silico thrombin and factor Xa generation profiles in adult patients after Fontan operation.

Blood Coagul Fibrinolysis. 2018 Mar;29(2):236-240

Authors: Gissel M, Tomkiewicz-Pajak L, Podolec P, Hoffman P, Trojnarska O, Lipczyńska M, Undas A, Brummel-Ziedins KE

: Single-ventricle defects are associated with increased risk of thromboembolic events. To analyze the prothrombotic potential in a long-term follow-up on Fontan patients via plasma contribution to thrombin and factor (F)Xa generation profiles. Thrombin and FXa generation was simulated from plasma concentrations of FII, FV, FVII, FVIII, FIX, FX, antithrombin and tissue factor (TF) pathway inhibitor from Fontan patients (n = 48) and healthy controls (n = 34). TF and thrombin-antithrombin complex (TAT) were measured by ELISA. Fontan patients had significantly reduced procoagulant protein concentrations and increased anticoagulant protein concentrations over controls, resulting in a lowered procoagulant potential. However, Fontan patients showed increased hemostatic activation as evidenced by increased TF and TAT. Modeling this increased TF showed a more prothrombotic profile. Observed changes in procoagulant and anticoagulant proteins may be a compensatory mechanism aimed at mitigating the underlying disease effects characterized by elevated TF and TAT.

PMID: 29406387 [PubMed - indexed for MEDLINE]