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Research Progress of the Pediatric Cardiology: 2nd Edition
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Research Progress of the Pediatric Cardiology: 2nd Edition

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

Deadline for manuscript submissions: closed (1 April 2024) | Viewed by 16670

Special Issue Editor

Dr. Massimo Mapelli

E-Mail Website
Guest Editor
1. Cardiology Center Monzino, Scientific Institute for Research, Hospitalization and Healthcare (IRCCS), 20138 Milano, Italy
2. Department of Clinical Sciences and Community Health, Cardiovascular Section, University of Milan, 20122 Milan, Italy
Interests: cardiologist; heart failure; exercise
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Considering the success and popularity of the Special Issue "Research Progress of the Pediatric Cardiology" previously published in the journal Children (https://www.mdpi.com/journal/children/special_issues/Research_Progress_Pediatric_Cardiology), we now release a second issue aiming to gather original research papers and review articles focused on pediatric cardiology. I am honored to have been asked again to serve as a Guest Editor for this valuable project.

The world of cardiology has undergone a heavy and favorable revolution in recent years. Heart disease that previously seemed incurable can now be addressed with far better diagnostic and therapeutic ability than in the recent past. Opportunities for the percutaneous or surgical treatment of structural heart disease now permit much less invasive but still effective interventions. In parallel, multimodal imaging techniques (advanced echocardiogram, magnetic resonance imaging, cardiac CT) allow the visualization of the cardiovascular system as has never been possible before, helping our understanding of cardiomyopathies and structural alterations that underlie many diseases of the heart.

The aim of this Special Issue is to produce a rich collection of research, articles, and opinions for clinicians, academics, and policy makers in the field of pediatric cardiology. Contributions related to all aspects of this topic and, in particular, the understanding, management, and improvement of cardiovascular diseases in children, are welcome.

Our work will be based on an interdisciplinary approach to disseminate advanced knowledge and evidence with the aim of improving pediatric cardiovascular care. High-quality papers in all areas of pediatric cardiology will be handled through a fair and rigorous peer review process.

Innovative papers on new cardiovascular treatments, prognostic indicators, and imaging techniques, as well as studies focused on the impact of disruptive events (i.e., the COVID-19 pandemic), are particularly welcome.

I look forward to receiving your contributions

Dr. Massimo Mapelli
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pediatric cardiology
  • congenital heart disease
  • heart failure
  • myocarditis
  • COVID-19 and cardiovascular system
  • heart transplantation
  • cardiac imaging in children
  • pediatric cardiology in low-income countries
  • rheumatic heart disease
  • child/children
  • adolescent

Published Papers (11 papers)

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Editorial

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5 pages, 1497 KiB  
Editorial
Editorial: Celebrating Diversity and Advancements in Pediatric Cardiology—A Journey through Specialized Research
by Massimo Mapelli, Paola Zagni and Irene Picciolli
Children 2024, 11(4), 455; https://doi.org/10.3390/children11040455 - 10 Apr 2024
Viewed by 387
Abstract
The field of pediatric cardiology is as vast and diverse as the young patients it serves (Figure 1) [...] Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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Research

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13 pages, 1725 KiB  
Article
Correlation of Speckle-Tracking Echocardiography with Traditional Biomarkers in Predicting Cardiotoxicity among Pediatric Hemato-Oncology Patients: A Comprehensive Evaluation of Anthracycline Dosages and Treatment Protocols
by Andrada Mara Ardelean, Ioana Cristina Olariu, Raluca Isac, Ruxandra Jurac, Cristiana Stolojanu, Mircea Murariu, Ana-Olivia Toma, Laurentiu Braescu, Adelina Mavrea and Gabriela Doros
Children 2023, 10(9), 1479; https://doi.org/10.3390/children10091479 - 30 Aug 2023
Cited by 1 | Viewed by 824
Abstract
Speckle tracking-echocardiography (STE) is a novel non-invasive imaging tool capable of quantifying myocardial deformation, and thus holds promise in detecting early subclinical myocardial injury. This study aimed to evaluate the correlation of STE with traditional biomarkers in predicting anthracycline-induced cardiotoxicity in the context [...] Read more.
Speckle tracking-echocardiography (STE) is a novel non-invasive imaging tool capable of quantifying myocardial deformation, and thus holds promise in detecting early subclinical myocardial injury. This study aimed to evaluate the correlation of STE with traditional biomarkers in predicting anthracycline-induced cardiotoxicity in the context of varying dosages and treatment protocols in pediatric hemato-oncology patients. We conducted a retrospective study involving pediatric hemato-oncology patients undergoing anthracycline-based chemotherapy. A total of 99 patients were included in the final analysis, with 82 receiving Doxorubicin, of which 58.5% were males, and 17 receiving Epirubicin, of which 70.6% were males, with a median of 10 years old. Traditional biomarkers, such as Troponin I (cTnI) and B-type natriuretic peptide (BNP), were compared with STE parameters, including the global longitudinal strain (GLS), Simpson method of discs (SMOD), and myocardial performance index (MPI). A comprehensive evaluation was conducted based on different dosages of anthracyclines and different treatment protocols, with a follow-up period of one year post-chemotherapy. It was observed that the cTnI levels in the Doxorubicin group were significantly higher (3.2 ng/mL, p = 0.002) than in the Epirubicin group (2.7 ng/mL). However, BNP and NT-proBNP levels were not significantly different between the two groups (p = 0.096 and p = 0.172, respectively). Regarding STE parameters, a significant negative correlation was observed between the anthracycline dose and GLS (Rho = −0.411, p = 0.001), indicating increased cardiotoxicity with dose elevation. The SMOD and MPI gave significantly better values in the Epirubicin group (59.2 and 0.41 vs. 54.4 and 0.36, respectively). However, the ROC analysis did not find GLS, SMOD, or MPI to be significant independent predictors of cardiotoxicity (p > 0.05). There was also considerable variation in cardiotoxicity between the Doxorubicin and Epirubicin study groups, suggesting that the risk of cardiotoxicity is not solely determined by dose. Our study underlines the potential of STE as a sensitive tool for the early detection and prediction of anthracycline-induced cardiotoxicity in pediatric hemato-oncology patients, but only in association with the clinical findings and cardiac biomarkers. While traditional biomarkers still play a role, STE can offer a more accurate prediction of cardiac risk, potentially leading to better management and outcomes for these patients. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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13 pages, 1385 KiB  
Article
Elevated Copeptin Levels Are Associated with Heart Failure Severity and Adverse Outcomes in Children with Cardiomyopathy
by Karan B. Karki, Jeffrey A. Towbin, Samir H. Shah, Ranjit R. Philip, Alina N. West, Sachin D. Tadphale and Arun Saini
Children 2023, 10(7), 1138; https://doi.org/10.3390/children10071138 - 30 Jun 2023
Cited by 1 | Viewed by 973
Abstract
In children with cardiomyopathy, the severity of heart failure (HF) varies. However, copeptin, which is a biomarker of neurohormonal adaptation in heart failure, has not been studied in these patients. In this study, we evaluated the correlation of copeptin level with functional HF [...] Read more.
In children with cardiomyopathy, the severity of heart failure (HF) varies. However, copeptin, which is a biomarker of neurohormonal adaptation in heart failure, has not been studied in these patients. In this study, we evaluated the correlation of copeptin level with functional HF grading, B-type natriuretic peptide (BNP), and echocardiography variables in children with cardiomyopathy. Furthermore, we determined if copeptin levels are associated with adverse outcomes, including cardiac arrest, mechanical circulatory support, heart transplant, or death. In forty-two children with cardiomyopathy with a median (IQR) age of 13.1 years (2.5–17.2) and a median follow-up of 2.5 years (2.2–2.7), seven (16.7%) children had at least one adverse outcome. Copeptin levels were highest in the patients with adverse outcomes, followed by the patients without adverse outcomes, and then the healthy children. The copeptin levels in patients showed a strong correlation with their functional HF grading, BNP level, and left ventricular ejection fraction (LVEF). Patients with copeptin levels higher than the median value of 25 pg/mL had a higher likelihood of experiencing adverse outcomes, as revealed by Kaplan–Meier survival analysis (p = 0.024). Copeptin level was an excellent predictor of outcomes, with an area under the curve of 0.861 (95% CI, 0.634–1.089), a sensitivity of 86%, and a specificity of 60% for copeptin level of 25 pg/mL. This predictive value was superior in patients with dilated and restrictive cardiomyopathies (0.97 (CI 0.927–1.036), p < 0.0001, n = 21) than in those with hypertrophic and LV non-compaction cardiomyopathies (0.60 (CI 0.04–1.16), p = 0.7, n = 21). Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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13 pages, 2950 KiB  
Article
Evaluation of Right Ventricular Function in Patients with Propionic Acidemia—A Cross-Sectional Study
by Alexander Kovacevic, Sven F. Garbade, Friederike Hörster, Georg F. Hoffmann, Matthias Gorenflo, Derliz Mereles, Stefan Kölker and Christian Staufner
Children 2023, 10(1), 113; https://doi.org/10.3390/children10010113 - 05 Jan 2023
Viewed by 1469
Abstract
(1) Background: In propionic acidemia (PA), myocardial involvement often leads to progressive cardiac dysfunction of the left ventricle (LV). Cardiomyopathy (CM) is an important contributor to mortality. Although known to be of prognostic value in CM, there are no published data on right [...] Read more.
(1) Background: In propionic acidemia (PA), myocardial involvement often leads to progressive cardiac dysfunction of the left ventricle (LV). Cardiomyopathy (CM) is an important contributor to mortality. Although known to be of prognostic value in CM, there are no published data on right ventricular (RV) function in PA patients. (2) Methods: In this cross-sectional single-center study, systolic and diastolic RV function of PA patients was assessed by echocardiography, including frequency, onset, and combinations of echocardiographic parameters, as well as correlations to LV size and function. (3) Results: N = 18 patients were enrolled. Tricuspid annulus S’ was abnormal in 16.7%, RV-longitudinal strain in 11.1%, tricuspid annular plane systolic excursion (TAPSE) in 11.1%, Tricuspid valve (TV) E/e’ in 33.3%, and TV E/A in 16.7%. The most prevalent combinations of pathological parameters were TV E/A + TV E/e’ and TAPSE + TV S’. With age, the probability of developing abnormal RV function increases according to age-dependent normative data. There is a significant correlation between TAPSE and mitral annular plane systolic excursion (MAPSE), and RV/LV-longitudinal strain (p ≤ 0.05). N = 5 individuals died 1.94 years (mean) after cardiac evaluation for this study, and all had abnormal RV functional parameters. (4) Conclusions: Signs of diastolic RV dysfunction can be found in up to one third of individuals, and systolic RV dysfunction in 16.7% of individuals in our cohort. RV function is impaired in PA patients with a poor outcome. RV functional parameters should be used to complement clinical and left ventricular echocardiographic findings. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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Review

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8 pages, 230 KiB  
Review
Global Cardiac Surgery—Accessibility to Cardiac Surgery in Developing Countries: Objectives, Challenges, and Solutions
by Salvatore Agati and Ermanno Bellanti
Children 2023, 10(11), 1789; https://doi.org/10.3390/children10111789 - 06 Nov 2023
Viewed by 924
Abstract
Cardiac surgery is a modern science in the history of medicine. The impact of cardiac disease, in terms of treatment and prognosis, has made this discipline indispensable to global health. In recent decades, the greatest investment has been dispensed to technological and material [...] Read more.
Cardiac surgery is a modern science in the history of medicine. The impact of cardiac disease, in terms of treatment and prognosis, has made this discipline indispensable to global health. In recent decades, the greatest investment has been dispensed to technological and material improvements to increase life expectancy. This surgery must address different epidemiological aspects dictated by the geography and economic–social conditions of the global populations. For this reason, it is progressively important to address the cardiac surgery accessibility disparity. Many scientific papers and international meetings have studied how cardiac surgery can be more accessible in various countries around the world. In this review, we analyze all the challenges, solutions, and suggestions that can make this surgery accessible to the entire global population, with the purpose of reducing its disparity across all seven continents. For a long time, high-income countries have invested in technological capabilities and experimental advancements without caring about unequal access in the rest of the world. We believe that it is time to reverse this growth trajectory, placing the accessibility and distribution of surgical science as a priority, which is significant for the right to health of all people worldwide. This is the real new challenge in cardiosurgery. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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Graphical abstract

19 pages, 1927 KiB  
Review
Multimodality Imaging Assessment of Tetralogy of Fallot: From Diagnosis to Long-Term Follow-Up
by Sara Moscatelli, Valeria Pergola, Raffaella Motta, Federico Fortuni, Nunzia Borrelli, Jolanda Sabatino, Isabella Leo, Martina Avesani, Claudia Montanaro, Elena Surkova, Massimo Mapelli, Marco Alfonso Perrone and Giovanni di Salvo
Children 2023, 10(11), 1747; https://doi.org/10.3390/children10111747 - 27 Oct 2023
Viewed by 1584
Abstract
Tetralogy of Fallot (TOF) is the most common complex congenital heart disease with long-term survivors, demanding serial monitoring of the possible complications that can be encountered from the diagnosis to long-term follow-up. Cardiovascular imaging is key in the diagnosis and serial assessment of [...] Read more.
Tetralogy of Fallot (TOF) is the most common complex congenital heart disease with long-term survivors, demanding serial monitoring of the possible complications that can be encountered from the diagnosis to long-term follow-up. Cardiovascular imaging is key in the diagnosis and serial assessment of TOF patients, guiding patients’ management and providing prognostic information. Thorough knowledge of the pathophysiology and expected sequalae in TOF, as well as the advantages and limitations of different non-invasive imaging modalities that can be used for diagnosis and follow-up, is the key to ensuring optimal management of patients with TOF. The aim of this manuscript is to provide a comprehensive overview of the role of each modality and common protocols used in clinical practice in the assessment of TOF patients. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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14 pages, 301 KiB  
Review
Predicting Therapeutic Efficacy of Pharmacological Treatments in Children with Postural Orthostatic Tachycardia Syndrome: A Mini-Review
by Siying Fan, Yaxi Cui, Ying Liao and Hongfang Jin
Children 2023, 10(7), 1093; https://doi.org/10.3390/children10071093 - 21 Jun 2023
Viewed by 1977
Abstract
Postural orthostatic tachycardia syndrome (POTS) is common in children, with an excessive increment in heart rate when moving from the supine to upright position. It has significant negative impacts on the daily life of pediatric patients. The pathogenesis of POTS includes peripheral vascular [...] Read more.
Postural orthostatic tachycardia syndrome (POTS) is common in children, with an excessive increment in heart rate when moving from the supine to upright position. It has significant negative impacts on the daily life of pediatric patients. The pathogenesis of POTS includes peripheral vascular dysfunction, central hypovolemia, abnormal autonomic function, a high-adrenergic state, impaired skeletal-muscle pump function, the abnormal release of vasoactive factors, and autoimmune abnormalities. Therefore, the empirical use of pharmacological treatments has limited therapeutic efficacy due to the diversity of its mechanisms. A crucial aspect of managing POTS is the selection of appropriate treatment targeting the specific pathogenesis. This review summarizes the commonly used pharmacological interventions, with a focus on their predictive indicators for treatment response. Factors such as heart rate variability, plasma biomarkers, and cardiac-function parameters are discussed as potential predictors of therapeutic efficacy, enabling the implementation of individualized treatment to improve therapeutic effectiveness. This review consolidates the current knowledge on POTS, encompassing its clinical characteristics, epidemiological patterns, underlying pathogenic mechanisms, and predictive indicators for treatment response. Further research is warranted to enhance the understanding of POTS and facilitate the development of more effective therapeutic approaches for this challenging syndrome. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
19 pages, 3076 KiB  
Review
Cardiovascular Magnetic Resonance from Fetal to Adult Life—Indications and Challenges: A State-of-the-Art Review
by Sara Moscatelli, Isabella Leo, Veronica Lisignoli, Siobhan Boyle, Chiara Bucciarelli-Ducci, Aurelio Secinaro and Claudia Montanaro
Children 2023, 10(5), 763; https://doi.org/10.3390/children10050763 - 23 Apr 2023
Cited by 2 | Viewed by 2120
Abstract
Cardiovascular magnetic resonance (CMR) imaging offers a comprehensive, non-invasive, and radiation-free imaging modality, which provides a highly accurate and reproducible assessment of cardiac morphology and functions across a wide spectrum of cardiac conditions spanning from fetal to adult life. It minimises risks to [...] Read more.
Cardiovascular magnetic resonance (CMR) imaging offers a comprehensive, non-invasive, and radiation-free imaging modality, which provides a highly accurate and reproducible assessment of cardiac morphology and functions across a wide spectrum of cardiac conditions spanning from fetal to adult life. It minimises risks to the patient, particularly the risks associated with exposure to ionising radiation and the risk of complications from more invasive haemodynamic assessments. CMR utilises high spatial resolution and provides a detailed assessment of intracardiac and extracardiac anatomy, ventricular and valvular function, and flow haemodynamic and tissue characterisation, which aid in the diagnosis, and, hence, with the management of patients with cardiac disease. This article aims to discuss the role of CMR and the indications for its use throughout the different stages of life, from fetal to adult life. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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15 pages, 1232 KiB  
Review
Strategies to Aid Successful Transition of Adolescents with Congenital Heart Disease: A Systematic Review
by Pier Paolo Bassareo, Massimo Chessa, Giovanni Di Salvo, Kevin Patrick Walsh and Colin Joseph Mcmahon
Children 2023, 10(3), 423; https://doi.org/10.3390/children10030423 - 22 Feb 2023
Cited by 4 | Viewed by 1705
Abstract
The majority of patients born with congenital heart disease (CHD) need lifelong surveillance with serial clinical attendance and examinations. However, loss of follow-up (namely no documented follow-up for 3 years or more) is a recognised common problem since it is often related to [...] Read more.
The majority of patients born with congenital heart disease (CHD) need lifelong surveillance with serial clinical attendance and examinations. However, loss of follow-up (namely no documented follow-up for 3 years or more) is a recognised common problem since it is often related to remarkable worsening in the health of CHD patients with increased morbidity and mortality. Transitioning from paediatric to adult care has proven to be the most vulnerable point in the care of these subjects. As such, a systematic review was carried out to ask the following questions: What is the percentage of loss of follow-up worldwide? Are there regional fluctuations in the percentage? Is there a link between loss of follow-up and the complexity of CHD? What strategies should be employed to lower the risk of discontinuity in care? The most recent worldwide averaged loss of follow-up is 26.1%, with significant fluctuations across continents and countries. This percentage is even higher (31.9%) when one includes all untraceable patients, presuming that they are not having any cardiac follow-up. The highest discontinuity of care was reported in the USA and in patients with simple CHD. Planning the rules of transition seems to be one of the most reliable tools to minimise the number of CHD patients who are lost in transition. Recalling patients, with general practitioners who are crucial in readdressing half of the lost to follow-up CHD patients to adult CHD specialists, and a good relationship between paediatric cardiologists and the adult CHD team are two other valuable strategies in aiding successful transition. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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Graphical abstract

18 pages, 3712 KiB  
Review
The Use of Stress Cardiovascular Imaging in Pediatric Population
by Sara Moscatelli, Francesco Bianco, Andrea Cimini, Mario Panebianco, Isabella Leo, Chiara Bucciarelli-Ducci and Marco Alfonso Perrone
Children 2023, 10(2), 218; https://doi.org/10.3390/children10020218 - 26 Jan 2023
Cited by 8 | Viewed by 2225
Abstract
Although not frequent in the pediatric population, ischemia could occur in children due to several congenital and acquired disease. Stress imaging is key for the non-invasive evaluation of myocardial abnormalities and perfusion defect in this clinical setting. Moreover, beyond ischemia assessment, it can [...] Read more.
Although not frequent in the pediatric population, ischemia could occur in children due to several congenital and acquired disease. Stress imaging is key for the non-invasive evaluation of myocardial abnormalities and perfusion defect in this clinical setting. Moreover, beyond ischemia assessment, it can provide complementary diagnostic and prognostic information in valvular heart disease and cardiomyopathies. When performed using cardiovascular magnetic resonance, it could detect, in addition, myocardial fibrosis and infarction, increasing the diagnostic yield. Several imaging modalities are currently available for the evaluation of stress myocardial perfusion. Advances in technologies have also increased the feasibility, safety and availability of these modalities in the pediatric age group. However, despite the established role of stress imaging and its increasing use in daily clinical practice, there are currently no specific guidelines, and little data are available in the literature on this topic. The aim of this review is to summarize the most recent evidence on pediatric stress imaging and its clinical application with a focus on the advantages and limitations of each imaging modality currently available. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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Other

8 pages, 1672 KiB  
Case Report
Idiopathic Pulmonary Arterial Hypertension in Paediatrics Represents Still a Serious Challenge: A Case Series Study
by Pier Paolo Bassareo, Paola Argiento, Colin Joseph McMahon, Esme Dunne, Kevin Patrick Walsh, Maria Giovanna Russo and Michele D’Alto
Children 2023, 10(3), 518; https://doi.org/10.3390/children10030518 - 06 Mar 2023
Cited by 1 | Viewed by 1346
Abstract
Introduction: Paediatric pulmonary hypertension (PH) represents a heterogeneous illness that is responsible for high morbidity and mortality if left without treatment. Idiopathic pulmonary arterial hypertension (IPAH) is a subtype of PAH rarely seen in paediatrics. Limited long-term data are available. Methods: Over a [...] Read more.
Introduction: Paediatric pulmonary hypertension (PH) represents a heterogeneous illness that is responsible for high morbidity and mortality if left without treatment. Idiopathic pulmonary arterial hypertension (IPAH) is a subtype of PAH rarely seen in paediatrics. Limited long-term data are available. Methods: Over a period of 20 years, 10 paediatric patients were enrolled at two tertiary centres. Their clinical, echocardiographic, and right heart catheterisation (RHC) features and outcome were evaluated. Results: The mean age at first diagnosis was 5.7 ± 5.7 years. The age at the last follow-up was 12.4 ± 6.1 years. The average follow-up was 6.6 ± 0.8 years. There was a female prevalence of 60% (p < 0.05) in this case series. Regarding the NYHA functional class, 80% of IPAH subjects were in class III or IV. The mean saturation was 91 ± 5%. In this regard, 70% of the patients were on a combination of three drugs, with sildenafil (90%) included. On echocardiography, longitudinal right ventricular contractility (TAPSE) was slightly reduced (13.4 ± 2.6 mm), whilst RVSP was severely elevated (101 ± 19 mmHg). The RHC data showed that mPAP was 61.8 ± 23.1 mmHg (p = 0.0017 with RVSP on echocardiography), mRAP was 10.7 ± 3.8 mmHg, CI was 2.6 ± 1 L·min−1·m−2, PVRi was 16.8 ± 12.6 WU·m2, and SVO2 was 63.6 ± 14.8%. Regarding the outcome, two male IPAH patients (20%) died, and 50% underwent lung transplant or were on transplant assessment or already on the waiting list for lung transplantation. One patient underwent a ductus arteriosus stenting (reverse Potts shunt) and another underwent atrial septostomy and stenting. Conclusions: Notwithstanding the progress in medical therapy, IPAH continues to represent a serious challenge, particularly in the paediatric population, with the need for lung transplantation and significant mortality. Full article
(This article belongs to the Special Issue Research Progress of the Pediatric Cardiology: 2nd Edition)
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