Cardiovascular Disease in Children

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Cardiology".

Deadline for manuscript submissions: closed (20 March 2023) | Viewed by 23158

Special Issue Editor


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Guest Editor
1. Pediatric Cardiology Unit, Department of Pediatrics, Lausanne University Hospital and University of Lausanne, 1011 Lausanne, Switzerland
2. Heart failure and Transplant, Pediatric Cardiology, Great Ormond Street Hospital, London WC1N 3JH, UK
3. Division of Clinical Pharmacology and Toxicology, Institute of Pharmacological Sciences of Southern Switzerland, Ente Ospedaliero Cantonale, 6900 Lugano, Switzerland
Interests: cardiovascular function; heart failure; heart transplant; arterial hypertension; cardiovascular pharmacology; endothelial function; cardiac disease in chronic kidney disease

Special Issue Information

Dear Colleagues,

Cardiovascular disease is the leading cause of mortality worldwide [1]. An increasing body of evidence underscores the importance of fetal and paediatric programming of cardiovascular health and disease [2-3]. On the other side, over the last years, Paediatric Cardiology has assisted an impressive increase in diagnostic and therapeutic possibilities. After the big progresses and achievements of paediatric cardiac surgery in the second half of the last century [4-5], we are now in a golden era for Paediatric Cardiology. If during that period, thanks to improved imaging techniques [6], a main task of the cardiologist was to decide indication as well as timing of surgery and delivering the best accurate images to the surgeon for operative planning, nowadays the cardiologist has a much broader role.

First, a plethora of interventional techniques are meanwhile established and allow to efficiently address several structural problems without the need of open cardiac surgery and extracardiac circulation [7-8].

Second, the role of the paediatric cardiologist in the perioperative intensive care management continuously increases in importance and potentiality.

Third, perioperative survival is nowadays a quality criterium and not more an “achievement”. We meanwhile must focus on long-term survival and morbidity, aiming at bringing this “congenital heart disease population” into their eighties in excellent physical and psychological conditions.

Fourth, we nowadays can positively influence the course of several disease processes such as myocarditis, pericarditis, cardiomyophaties, pulmonary hypertension, cardiac involvement in oncological, haematological, neuromuscular, respiratory and metabolic (including among others arterial hypertension, diabetes mellitus and chronic kidney disease) diseases.

Fifth, we are continuously increasing our attention to cardiovascular health, from fetal life into adulthood. Perinatal conditioning, paediatric arterial hypertension, hypercholesterolemias, endothelial function, vascular health of solid-organ transplant including heart-transplant recipients represent main challenges for the future generation of paediatric cardiologists [3,9].

In conclusion, Paediatric Cardiology is no more a small alcove branch of Pediatrics. In fact, the modern cardiologist is not a specialized technician, but a full-blown Paediatrician, as recently demonstrated in the context of the SARS-CoV-2 pandemics and the Paediatric Inflammatory Multisystem Syndrome [10-11]. Paediatric Cardiologists nowadays face a series of challenging but inspiring and exciting tasks. In this special issue, we would like to cover some aspects of this amazing, growing field of Paediatrics. We will particularly appreciate and prioritize concise and clear presentations.

Looking forward for your contributions,

Dr. Sebastiano A.G. Lava

Guest Editor

References

  1. GBD 2016 Causes of Death Collaborators. Global, regional, and national age-sex specific mortality for 264 causes of death, 1980-2016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet. 2017; 390: 1151-1210
  2. Barker DJ et al. Growth in utero, blood pressure in childhood and adult life, and mortality from cardiovascular disease. Br Med J 1989;298:564-7.
  3. Pool LR, Aguayo L, Brzezinski M, Perak AM, Davis MM, Greenland P, Hou L, Marino BS, Van Horn L, Wakschlag L, Labarthe D, Lloyd-Jones D, Allen NB. Childhood Risk Factors and Adulthood Cardiovascular Disease: A Systematic Review. J Pediatr. 2021; 232:118-126.e23.
  4. Kirklin JW, DuShane JW, Patrick RT, et al. Intracardiac surgery with the aid of a mechanical pumpoxygenator system (gibbon type): report of eight cases. Mayo Clin Proc 1955; 30: 201–206.
  5. Freedom RM, Lock J, Bricker JT. Pediatric cardiology and cardiovascular surgery: 1950-2000. Circulation. 2000;102(20 Suppl 4):IV58-6
  6. Gursu, H., & Cetin, I. The history of paediatric cardiology on stamps. Cardiology in the Young. 2018; 28(1): 1-8.
  7. Kim SH. Recent advances in pediatric interventional cardiology. Korean J Pediatr. 2017;60(8):237-244.
  8. Schranz D, Esmaeili A, Akintuerk H. Hypoplastic Left Heart: Stage-I Will be Performed Interventionally, Soon. Pediatr Cardiol. 2021;42(4):727-735.
  9. de Ferranti SD, Steinberger J, Ameduri R, Baker A, Gooding H, Kelly AS, Mietus-Snyder M, Mitsnefes MM, Peterson AL, St-Pierre J, Urbina EM, Zachariah JP, Zaidi AN. Cardiovascular Risk Reduction in High-Risk Pediatric Patients: A Scientific Statement From the American Heart Association. Circulation. 2019;139(13):e603-e634.
  10. Alsaied T, Tremoulet AH, Burns JC, Saidi A, Dionne A, Lang SM, Newburger JW, de Ferranti S, Friedman KG. Review of Cardiac Involvement in Multisystem Inflammatory Syndrome in Children. Circulation. 2021;143(1):78-88.
  11. Strah DD, Kowalek KA, Weinberger K, Mendelson J, Hoyer AW, Klewer SE, Seckeler MD. Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease. Pediatr Cardiol. 2021 Oct 11:1–6 (Epub ahead of print)

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Keywords

  • paediatric cardiology
  • congenital heart disease
  • endothelial function
  • arterial hypertension
  • echocardiography
  • interventional cardiology
  • arrhythmias
  • fontan circulation
  • heart failure
  • paediatric inflammatory multisystem syndrome, SARS-CoV-2, COVID-19
  • smartwatch
  • cardiovascular disease

Published Papers (12 papers)

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Editorial

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3 pages, 192 KiB  
Editorial
Cardiovascular Disease in Children: The Future Is Now
by Sebastiano A. G. Lava
Children 2023, 10(5), 886; https://doi.org/10.3390/children10050886 - 15 May 2023
Viewed by 902
Abstract
Cardiovascular disease is a leading cause of morbidity and mortality worldwide [...] Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)

Research

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13 pages, 431 KiB  
Article
Change in Prevalence of Hypertension among Korean Children and Adolescents during the Coronavirus Disease 2019 (COVID-19) Outbreak: A Population-Based Study
by Kyungchul Song, Se Yong Jung, Juyeon Yang, Hye Sun Lee, Ho-Seong Kim and Hyun Wook Chae
Children 2023, 10(1), 159; https://doi.org/10.3390/children10010159 - 14 Jan 2023
Cited by 5 | Viewed by 1762
Abstract
During the coronavirus disease 2019 (COVID-19) outbreak, the prevalence of obesity increased globally; this may be associated with hypertension incidence. However, investigations on the changes in the prevalence of hypertension among children and adolescents are limited. This cross-sectional study investigated the prevalence of [...] Read more.
During the coronavirus disease 2019 (COVID-19) outbreak, the prevalence of obesity increased globally; this may be associated with hypertension incidence. However, investigations on the changes in the prevalence of hypertension among children and adolescents are limited. This cross-sectional study investigated the prevalence of hypertension among 1428 youths aged 10–18 years using data from the Korea National Health and Nutrition Examination Survey 2018–2020. We assessed the prevalence of hypertension according to sex, age, body mass index (BMI), and residential district. The prevalence of hypertension increased from 7.1% to 12.5% in all participants. In the sex-specific analysis, the prevalence was found to be increased in boys. In the age-specific analysis, the prevalence was found to be increased in youths aged 13–15 years. In the BMI-specific analysis, an increase in the prevalence was prominent in the normal BMI group. In the residential district-specific analysis, the prevalence of hypertension among youth increased in urban areas. Our results show that the prevalence of hypertension increased among Korean children and adolescents during the COVID-19 outbreak. These findings suggest the importance of close monitoring of hypertension among youth during the COVID-19 pandemic. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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10 pages, 243 KiB  
Article
Cardiovascular Outcomes in Children with Multisystem Inflammatory Syndrome Treated with Therapeutic Plasma Exchange
by Tunç Tunçer and Fatih Varol
Children 2022, 9(11), 1640; https://doi.org/10.3390/children9111640 - 27 Oct 2022
Cited by 2 | Viewed by 1298
Abstract
Background: Multisystem inflammatory syndrome in children is a new, rare, post-infectious complication of SARS-CoV-2 infection in children. The aim of this study was to display the role of therapeutic plasma exchange on cardiovascular outcomes in children with multisystem inflammatory syndrome. Methods: This study [...] Read more.
Background: Multisystem inflammatory syndrome in children is a new, rare, post-infectious complication of SARS-CoV-2 infection in children. The aim of this study was to display the role of therapeutic plasma exchange on cardiovascular outcomes in children with multisystem inflammatory syndrome. Methods: This study included children who met the diagnostic criteria for multisystem inflammatory syndrome and who were admitted to the intensive care unit. This is a prospective single-center study conducted between August 2020 and September 2021. Subjects had cardiac involvement which was defined as elevated troponin I, abnormal electrocardiographic and echocardiographic findings. Patients were followed by a pediatric cardiologist throughout the intensive care unit stay and after discharge for 12 months. Patients were divided into two groups which received therapeutic plasma exchange and which did not. Results: 38 children were included in this study. There were 16 patients in the no plasma exchange group and 22 patients in the plasma exchange group. The two groups were similar in age, sex, leucocyte, thrombocyte count, neutrophil percentage, hemoglobin, C-reactive protein, erythrocyte sedimentation rate, alanine aminotransferase, albumin, ferritin, fibrinogen, D-dimer, IL-6, troponin I, number of electrocardiographic abnormalities and patients with mitral valve regurgitation detected at admission (p > 0.05). There was no significant difference between the two groups in terms of duration of normalization of electrocardiographic abnormalities and disappearance of mitral valve regurgitation (p > 0.05). Duration of normalization of troponin I (2, IQR 1–4, versus 5, IQR 3–9 days; p = 0.044) and length of hospital stay (7, IQR 6–10, versus 13, 8–20 days; p = 0.001) was longer in the plasma exchange group. Conclusions: We did not observe any significant improvement in children having undergone plasma exchange as compared to children who did not. On the opposite, their length of hospital stay and time to troponin I normalisation were even longer. Some baseline differences in cardiac attainment severity may partly explain this finding. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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6 pages, 459 KiB  
Article
Exploring the Possible Impact of Echocardiographic Diastolic Function Parameters on Outcome in Paediatric Dilated Cardiomyopathy
by Sabrina Bressieux-Degueldre, Matthew Fenton, Troy Dominguez and Michael Burch
Children 2022, 9(10), 1500; https://doi.org/10.3390/children9101500 - 30 Sep 2022
Cited by 2 | Viewed by 1094
Abstract
Diastolic dysfunction is an important determinant for prognosis and survival in several paediatric heart diseases. We aimed to explore its possible impact on outcome in children with dilated cardiomyopathy. From 2006 to 2016, children less than 18 years old with dilated cardiomyopathy were [...] Read more.
Diastolic dysfunction is an important determinant for prognosis and survival in several paediatric heart diseases. We aimed to explore its possible impact on outcome in children with dilated cardiomyopathy. From 2006 to 2016, children less than 18 years old with dilated cardiomyopathy were retrospectively enrolled. Echocardiographic diastolic function parameters and child outcomes were analysed. Of 43 children aged 0.2 to 16.1 years old referred with dilated cardiomyopathy, 8 patients required cardiac transplant or mechanical assist devices (18%), 24 had persistently abnormal left ventricular function and/or dilatation (56%) and 11 patients recovered (26%). There was no significant difference in mitral velocities on Tissue Doppler Imaging, mitral valve inflow velocities, isovolumic relaxation time, left atrial area z-score and mitral lateral E/e’ ratios between patients with recovery and patients with disease progression or persistently abnormal ventricular function and/or dilation. This is the first study on childhood dilated cardiomyopathy to address individual echocardiographic diastolic function parameters and their association to recovery. In this study, echocardiographic parameters for diastolic function did not predict recovery. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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11 pages, 725 KiB  
Article
Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study
by Ji Hee Kwak, Seung Won Lee, Hye Ryeong Cha, June Huh, I-Seok Kang, Tae-Gook Jun, Ji-Hyuk Yang, Man Yong Han and Jinyoung Song
Children 2022, 9(9), 1329; https://doi.org/10.3390/children9091329 - 31 Aug 2022
Cited by 3 | Viewed by 1632
Abstract
Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between [...] Read more.
Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10–25 vs. 6–19; p < 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3–8 vs. 4–9; p < 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2–7 vs. 1–4; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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8 pages, 929 KiB  
Article
Twenty-Four-Hour Urinary Sodium Excretion Predicts Therapeutic Effectiveness of Oral Rehydration Saline in Pediatric Vasovagal Syncope
by Xiaojuan Du, Chunyan Tao, Yaru Wang, Yan Sun, Qingyou Zhang, Chunyu Zhang, Ping Liu, Yuli Wang, Ying Liao, Junbao Du and Hongfang Jin
Children 2022, 9(7), 992; https://doi.org/10.3390/children9070992 - 01 Jul 2022
Cited by 4 | Viewed by 1416
Abstract
The study was designed to explore whether 24-hour urinary sodium excretion could predict the therapeutic effectiveness of oral rehydration saline in pediatric cases of vasovagal syncope. Eighty children suffering from vasovagal syncope with oral rehydration saline treatment in Department of Pediatrics, Peking University [...] Read more.
The study was designed to explore whether 24-hour urinary sodium excretion could predict the therapeutic effectiveness of oral rehydration saline in pediatric cases of vasovagal syncope. Eighty children suffering from vasovagal syncope with oral rehydration saline treatment in Department of Pediatrics, Peking University First Hospital, China, were recruited into the study. They were followed up for 3 (2, 3) months after treatment. Pre-treatment demographic, clinical, head-up tilt test-based hemodynamic and laboratory variables were compared between responders and non-responders. After univariate analysis, variables with p value < 0.05 in the comparison between responders and non-responders were further analyzed by binary logistic regression analysis. Receiver operating characteristic (ROC) curve was conducted to assess the value in predicting effectiveness of oral rehydration saline treatment. The results showed that 33 cases were responders, and 47 were non-responders. Blood sodium (138 ± 2 mmol/L vs. 139 ± 2 mmol/L, p < 0.05) and pre-treatment 24-hour urinary sodium excretion (74 ± 29 mmol/24 h vs. 109 (93, 141) mmol/24 h, p < 0.001) were lower in responders than in non-responders. The baseline 24-hour urinary sodium excretion was positively correlated to the duration from tilting to the positive response appearance in head-up tilt test (r = 0.289, p < 0.01). The cut-off value of baseline 24-hour urinary sodium excretion of the therapeutic effectiveness of oral rehydration saline on vasovagal syncope cases was 83 mmol/24 h, yielding a sensitivity of 87% and a specificity of 73% with AUC of 0.842 (p < 0.001). In conclusion, 24-hour urinary sodium excretion could be a useful biomarker to predict the therapeutic response to oral rehydration saline in pediatric cases of vasovagal syncope. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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8 pages, 668 KiB  
Article
Bacille Calmette-Guérin Site Reactivation of Kawasaki Disease in Infants under 3 Months of Age: Relation with Diagnosis and Prognosis
by Da Eun Roh, Jung Eun Kwon and Yeo Hyang Kim
Children 2022, 9(6), 857; https://doi.org/10.3390/children9060857 - 08 Jun 2022
Cited by 3 | Viewed by 1932
Abstract
Diagnosis of Kawasaki disease in infants under 3 months of age is challenging. This study aimed to confirm the diagnostic efficacy of BCGitis in patients with Kawasaki disease aged <3 months. Overall, 473 children were enrolled; they were grouped by age into group [...] Read more.
Diagnosis of Kawasaki disease in infants under 3 months of age is challenging. This study aimed to confirm the diagnostic efficacy of BCGitis in patients with Kawasaki disease aged <3 months. Overall, 473 children were enrolled; they were grouped by age into group 1 (≤3 months, n = 19) and group 2 (>3 months, n = 454). Data, including clinical features and laboratory results, were analyzed and compared between the groups. In group 1, 89% of patients showed Bacille Calmette-Guérin site reactivation. In group 1, total duration of fever and fever duration before initial treatment were significantly shorter than in group 2 (p = 0.001). The incidences of conjunctival injection, changes in extremities (erythema and edema), and cervical lymphadenopathy were significantly lower (p = 0.006, p = 0.040, and p < 0.001, respectively), and desquamation was higher in group 1 (p = 0.004). The incidences of incomplete Kawasaki disease, coronary artery complications, and resistance to intravenous immunoglobulin did not differ between the groups. Kawasaki disease should be suspected in infants aged <3 months with unexplained fever and BCGitis, even if the principal clinical symptoms are not fully presented. BCGitis in infantile Kawasaki disease is a useful sign and can help in the diagnosis of Kawasaki disease. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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8 pages, 481 KiB  
Article
QTc Intervals Are Prolonged in Late Preterm and Term Neonates during Therapeutic Hypothermia but Normalize Afterwards
by Karel Allegaert, Thomas Salaets, Robert M. Ward, Pieter Annaert and Anne Smits
Children 2021, 8(12), 1153; https://doi.org/10.3390/children8121153 - 08 Dec 2021
Cited by 4 | Viewed by 2187
Abstract
Background: There are anecdotal reports on reversible QTc prolongation during therapeutic hypothermia (TH) for moderate to severe neonatal encephalopathy after asphyxia. As the QTc interval is a relevant biomarker for pharmacovigilance during medication development, a structured search and review on published neonatal QTc [...] Read more.
Background: There are anecdotal reports on reversible QTc prolongation during therapeutic hypothermia (TH) for moderate to severe neonatal encephalopathy after asphyxia. As the QTc interval is a relevant biomarker for pharmacovigilance during medication development, a structured search and review on published neonatal QTc values to generate reference values is warranted to facilate medication development in this specific population. Methods: A structured search and literature assessment (PubMed, Embase, and Google Scholar) with ‘Newborn/Infant, QT and hypothermia’ was conducted (October 2021). Retrieved individual values were converted to QTc (Bazett) over postnatal age (day 1–7). Results: We retrieved 94 QTc intervals (during TH (n = 50, until day 3) or subsequent normothermia (n = 44, day 4–7)) in 33 neonates from 6 publications. The median (range) of QTc intervals during TH was 508 (430–678), and 410 (317–540) ms afterwards (difference 98 ms, or +28 ms/°C decrease). Four additional cohorts (without individual QTc intervals) confirmed the pattern and magnitude of the effect of body temperature on the QTc interval. Conclusions: We highlighted a relevant non-maturational covariate (°C dependent TH) and generated reference values for the QTc interval in this specific neonatal subpopulation. This knowledge on QTc during TH should be considered and integrated in neonatal medication development. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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Review

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14 pages, 780 KiB  
Review
Novel Biomarkers of Heart Failure in Pediatrics
by Teja Senekovič Kojc and Nataša Marčun Varda
Children 2022, 9(5), 740; https://doi.org/10.3390/children9050740 - 18 May 2022
Cited by 3 | Viewed by 2988
Abstract
Novel biomarkers of heart failure are the subject of numerous studies. Biomarkers of heart failure can be determined in the blood and in the urine. Seven groups of biomarkers of heart failure based on pathophysiological mechanisms are presented in this review, namely biomarkers [...] Read more.
Novel biomarkers of heart failure are the subject of numerous studies. Biomarkers of heart failure can be determined in the blood and in the urine. Seven groups of biomarkers of heart failure based on pathophysiological mechanisms are presented in this review, namely biomarkers of myocardial stretch, myocyte injury, myocardial remodeling, biomarkers of inflammation, renal dysfunction, neurohumoral activation, and oxidative stress. Studies of biomarkers in the pediatric population are scarce, therefore, further investigation is needed for reliable prognostic and therapeutic implications. The future of biomarker use is in multimarker panels that include a combination of biomarkers with different pathophysiological mechanisms in order to improve their diagnostic and prognostic predictive value. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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13 pages, 1003 KiB  
Review
Current Knowledge of Selected Cardiovascular Biomarkers in Pediatrics: Kidney Injury Molecule-1, Salusin-α and -β, Uromodulin, and Adropin
by Mirjam Močnik and Nataša Marčun Varda
Children 2022, 9(1), 102; https://doi.org/10.3390/children9010102 - 13 Jan 2022
Cited by 7 | Viewed by 2288
Abstract
Cardiovascular diseases are the leading cause of morbidity and mortality in the modern world. Their common denominator is atherosclerosis, a process beginning in childhood. In pediatrics, the aim of preventive measures is to recognize children and adolescents at risk for accelerated atherosclerosis and [...] Read more.
Cardiovascular diseases are the leading cause of morbidity and mortality in the modern world. Their common denominator is atherosclerosis, a process beginning in childhood. In pediatrics, the aim of preventive measures is to recognize children and adolescents at risk for accelerated atherosclerosis and possible premature cardiovascular events in adulthood. Several diagnostic procedures and biomarkers are available for cardiovascular risk assessment in adults. However, reliable markers in pediatrics are still insufficiently studied. In this contribution, we discuss five potential biomarkers of particular interest: kidney injury molecule-1, salusin-α and -β, uromodulin, and adropin. Studies regarding the pediatric population are scarce, but they support the evidence from studies in the adult population. These markers might entail both a prognostic and a therapeutic interest. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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Other

9 pages, 2420 KiB  
Case Report
Acute Coronary Syndrome Treated with Percutaneous Coronary Intervention in Hutchinson–Gilford Progeria
by Luciano De Simone, Serena Chiellino, Gaia Spaziani, Giulio Porcedda, Giovan Battista Calabri, Sergio Berti, Silvia Favilli, Laura Stefani and Giuseppe Santoro
Children 2023, 10(3), 526; https://doi.org/10.3390/children10030526 - 08 Mar 2023
Cited by 1 | Viewed by 1557
Abstract
Hutchinson–Gilford progeria syndrome is an extremely rare genetic disease caused by a de novo mutation in the LMNA gene, leading to an accumulation of a form of Lamin A, called Progerin, which results in a typical phenotype and a marked decrease in life [...] Read more.
Hutchinson–Gilford progeria syndrome is an extremely rare genetic disease caused by a de novo mutation in the LMNA gene, leading to an accumulation of a form of Lamin A, called Progerin, which results in a typical phenotype and a marked decrease in life expectancy, due to early atherosclerosis and cardiovascular disease. We report the case of a fourteen-year-old Chinese boy with Hutchinson–Gilford progeria syndrome admitted to the emergency room because of precordial pain. Physical examination showed tachycardia 130 beats/min and arterial hypertension: 170/120 mmHg, normal respiratory rate, no neurological impairment; ECG evidenced sinus tachycardia, left ventricular hypertrophy, horizontal ST-segment depression in I, aVL, II, III, aVF leads, and V4–V6 and ST-segment elevation in aVR and V1 leads. Echocardiography highlighted preserved global left ventricular function with concentric hypertrophy, altered diastolic flow pattern, mitral valve insufficiency, and minimal aortic regurgitation. Blood tests evidenced an increase in high-sensitivity troponin T level (335 pg/mL). NSTEMI diagnosis was performed, and the patient was admitted to the intensive care unit. A coronary CT angiography showed a severe obstruction of the common trunk of the left coronary artery, for which an urgent percutaneous coronary intervention (PCI) was proposed. A selective coronary angiography imaged complete chronic occlusion of the left main coronary artery as well as severe stenosis at the origin of a very enlarged right coronary artery that vascularized the left coronary artery through collaterals. Afterwards, the right coronary artery was probed using an Amplatz right (AR1) guiding catheter, through which a large 3.5 mm drug-eluting coronary stent (Xience Sierra, Abbott, Abbott Park, IL, USA) was implanted. At the end of the procedure, no residual stenosis was imaged and improved vascularization of the left coronary artery distribution segments was observed. Dual antiplatelet therapy (DAPT) consisting of aspirin (75 mg daily) and clopidogrel (37.5 mg daily) and anti-hypertensive therapy were started. At the one-year follow-up, the patient had not reported any occurrence of anginal chest pain. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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5 pages, 1147 KiB  
Opinion
What Is New on Paediatric Echocardiography for the Diagnosis, Management and Follow-Up of the Multisystem Inflammatory Syndrome Associated with COVID-19?
by Matteo Di Nardo, Alessio Franceschini, Pierre Tissieres and Marcello Chinali
Children 2022, 9(2), 146; https://doi.org/10.3390/children9020146 - 24 Jan 2022
Cited by 4 | Viewed by 2397
Abstract
Conventional echocardiography is an essential tool for the diagnosis, bedside management and follow-up evaluations of children with multisystem inflammatory syndrome associated with COVID-19. However, a more comprehensive echocardiographic exam, including myocardial deformation parameters, may allow early identification of subtle changes in ventricular function, [...] Read more.
Conventional echocardiography is an essential tool for the diagnosis, bedside management and follow-up evaluations of children with multisystem inflammatory syndrome associated with COVID-19. However, a more comprehensive echocardiographic exam, including myocardial deformation parameters, may allow early identification of subtle changes in ventricular function, provide risk stratification and, identify sub-clinical cardiac dysfunction at follow-up. Thus, myocardial deformation analysis should be routinely integrated to conventional echocardiography assessment in these patients. Full article
(This article belongs to the Special Issue Cardiovascular Disease in Children)
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