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An increasing number of medical services dedicated to the diagnosis, treatment and follow-up of pulmonary hypertension (PH) in children are being established. This has, in turn, increased the need to adapt current guidelines for the treatment of PH to be more relevant to paediatric patients with PH. This article will summarise the data obtained so far from paediatric registries, national cohorts and clinical trials and discuss the best approach for developing a treatment algorithm designed for children with different types of PH. The many unanswered questions, challenges and issues relating to the PH in the paediatric population will also be discussed.

Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD). Greater numbers of patients with PAH associated with CHD (PAH-CHD) are now surviving into adulthood, many with increasingly complex cardiac defects. Patients with cardiac defects which result in left-to-right shunting are at risk of developing PAH, owing to the increased shear stress and circumferential stretch induced by increased pulmonary blood flow, which leads to endothelial dysfunction and progressive vascular remodelling and, thus, increased pulmonary vascular resistance. Although PAH-CHD shares similar lung histology with idiopathic PAH, differences do exist between these aetiologies. Management of PAH-CHD can involve surgical correction of the cardiac defect and/or treatment of the PAH, depending on the underlying cardiac defect and status of disease progression. Transplantation surgery can be curative but is not without limitations. The timing of intervention in patients with PAH-CHD is important, but the optimums are sometimes difficult to define, with limited robust data to inform management decisions. Uncontrolled studies suggest that prostacyclin analogues and phosphodiesterase type-5 inhibitors may have benefits in advanced pulmonary vascular disease. In the only randomised controlled trial dedicated to end-stage PAH-CHD, bosentan significantly reduced pulmonary vascular resistance and significantly increased 6-minute walk distance without compromising peripheral oxygen saturation, in patients with Eisenmenger syndrome. These data suggest that targeted therapies are beneficial in the PAH-CHD population, and warrant further research.

Recent advances in the field of pulmonary hypertension (PH) have provided clinicians with a range of treatment options, but effective disease management in children presents a unique challenge. The present article will discuss the steps being taken to address unmet needs in paediatric PH. Understanding the epidemiology of paediatric PH is essential to guide management decisions, but such epidemiological data are scarce. The first international paediatric PH registry, Tracking Outcomes in Paediatric Pulmonary Hypertension (TOPP), promises to become a vital resource. Studies of PH therapies are rare in children, and treatment of paediatric PH is generally guided by the adult treatment algorithm, with some adaptations. However, invasive management options, such as continuous prostacyclin infusion, even if effective, are challenging in children, and further research is required to develop appropriate treatment strategies, formulations and doses for paediatric PH. Measures of treatment success must also be defined, and the applicability of end-points from adult clinical studies remains an open question. In summary, further epidemiological and treatment data are needed for paediatric pulmonary hypertension. The international TOPP registry will provide a valuable insight, but this must be complemented by research and development of adapted paediatric therapies. Dedicated childhood pulmonary hypertension services would optimise the diagnosis and management of this life-threatening disease.

Introduction: Patients with congenital heart disease (ConHD) are at increased risk for adverse cardiac events. Predicting long-term outcomes and guidance of patient management might benefit from a range of (new) biomarkers. This is a rapidly evolving field with potentially large consequences for clinical decision making. With a systematic review of available biomarkers in ConHD we identified the clinical role of these markers, knowledge gaps and future research directions., Methods: We systematically reviewed the literature on associations between blood biomarkers and outcome measures (mortality or composite adverse outcomes in patients with ConHD., Results: The inclusion criteria were met by 102 articles. Biomarkers assessed in more than 3 studies are discussed in the main text, those studied in 3 or less studies are summarized in the supplement. Thus, we discuss 15 biomarkers from 92 studies. These biomarkers were studied in 32,399 / 10,735 patients for the association with mortality and composite adverse outcomes, respectively. Biomarkers that were studied most and had statistically significant associations with mortality or composite adverse outcomes were (NT-pro)BNP, MELD-XI score, Hs-CRP, creatinine, albumin and sodium. Most of these biomarkers are involved in intracardiac processes associated with inflammation or are markers of renal function., Conclusion: For (NT-pro)BNP, clinical value for prediction of mortality and composite adverse outcomes in adult and paediatric ConHD has been shown. For MELD-XI, hs-CRP, albumin, creatinine, sodium, RDW, and GDF-15, correlations with mortality and composite adverse outcomes have been demonstrated in patient groups with mixed types of ConHD, but clinical utility needs additional exploration., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background : In adults, epicardial adipose tissue (EAT) is associated with metabolic syndrome (MS) and coronary artery disease. EAT thickness is increased in obese youth, but total EAT volume and its correlation with cardiovascular risk factors have not been studied. Objectives : To determine EAT volume in adolescents and its association with obesity and cardiovascular risk factors. Methods: We performed a cross-sectional study including 48 pubertal adolescents (24 obese and 24 lean subjects, aged 13.6 ± 1.5 yr). EAT volume as well as visceral and subcutaneous abdominal adipose tissue volumes were obtained by magnetic resonance imaging. Anthropometrical parameters; blood pressure (BP); fasting serum triglycerides; total and low- and high-density lipoprotein (HDL-C) cholesterol; glucose; and insulin levels were measured. Results : Obese adolescents had higher EAT volume compared to lean controls (49.6 ± 18.0 vs. 17.6 ± 6.7 cm 3 , p < 0.0005). They also had significantly increased visceral abdominal fat volumes, systolic BP, serum triglycerides, and insulin levels, and decreased HDL-C concentration. EAT volume was significantly associated with anthropometrical indices and cardiovascular risk factors: waist circumference, systolic BP, triglycerides, HDL-C levels, and insulin resistance indices. Metabolic syndrome was present in 25% of obese adolescents. EAT volume was significantly higher in obese adolescents with MS compared to those without MS (63.5 ± 21.4 vs. 44.9 ± 14.6 cm 3 , p = 0.026). Conclusions : EAT volume, which is known to contribute to atherogenesis in adults, is increased in obese adolescents, and is associated with abdominal visceral fat, cardiovascular risk factors, and MS. Excessive EAT early in life may contribute to the development of premature cardiometabolic disease.

Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps. An updated risk stratification tool and treatment algorithm is provided, now also including strategies for patients with associated cardiopulmonary conditions. Treatment of paediatric PH continues to be hindered by the lack of randomised controlled clinical trials. The challenging management of children failing targeted PAH therapy is discussed, including balloon atrial septostomy, lung transplantation and pulmonary-to-systemic shunt (Potts). A novel strategy using a multimodal approach for the management of PAH associated with congenital heart diseases with borderline pulmonary vascular resistance is included. Advances in diagnosing neonatal PH, especially signs and interpretation of PH by echocardiography, are highlighted. A team approach to the rapidly changing physiology of neonatal PH is emphasised. Challenges in drug approval are discussed, particularly the challenges of designing accurate paediatric clinical trials with age-appropriate end-points and adequate enrolment., Competing Interests: Conflict of interest: D. Ivy reports grants from the National Institutes of Health, GSK and Janssen, consultancy fees to the University of Colorado and support for attending meetings from Bayer, Merck and Janssen, and a leadership role with the Association of Pediatric Pulmonary Hypertension. E.B. Rosenzweig reports grants from National Institutes of Health, Bayer, Janssen, Insmed and SonVie, and is Director, PPHNet and Board Member, Team Phenomenal Hope. S.H. Abman reports consultancy fees from Chiasi and Oak Hills Bio, and leadership roles with BPD Collaborative and Pediatric Pulmonary Hypertension Network. M. Beghetti reports consultancy fees from Actelion/Janssen, MSD, Merck, Gossamer, GSK and Orpha, payment or honoraria for lectures, presentations, manuscript writing or educational events and support for attending meetings from Actelion/Janssen and MSD, and participation on a data safety monitoring board or advisory board with GSK, Actelion/Janssen, Gossamer, Altavant and MSD. D. Bonnet reports consultancy fees from Janssen, MSD and Novartis, and participation on a data safety monitoring board or advisory board with Lupin. J.M. Douwes has no potential conflicts of interest to disclose. A. Manes reports grants from Janssen/Actelion and Merck, payment or honoraria for lectures, presentations, manuscript writing or educational events from Janssen/Actelion, support for attending meetings from Dompè, and participation on a data safety monitoring board or advisory board with AOP Health Italy. R.M.F. Berger reports grants from Johnson & Johnson, consultancy fees from Johnson & Johnson, GSK and Ferrer, payment for educational events from Johnson & Johnson, Ferrer, AOP, MSD, Heart Medical, Occlutech Salveo, Bayer and Gossamerbio, participation on a data safety monitoring board or advisory board with MSD, and leadership roles with TOPP-registry, PPHNet and the ESC/ERS 2022 guidelines for diagnosis and treatment of pulmonary hypertension task force., (Copyright ©The authors 2024.)

Purpose: Children with congenital heart disease (CHD) from low- to middle-income countries (LMIC) are suspected to have a high prevalence of antibiotic-resistant microorganisms (ARMOs) carriage, but data are currently lacking. Carriage of ARMOs could impact the post-operative course in pediatric intensive care unit (PICU). The aim of the study was to assess the prevalence of ARMOs carriage in children with CHD from LMIC and its impact on post-operative outcomes., Methods: This was a retrospective monocentric study from 01/2019 to 12/2022. Included patients were children (0-18 years) from a LMIC admitted after CHD surgery and with AMRO screening performed the week before. Infections and post-operative evolution were compared based on ARMOs carriage status., Findings: Among 224 surgeries (median age 38.5 months (IQR 22-85.5)), ARMOs carriage was evidenced in 95 cases (42.4%). Main organisms isolated were Extended Spectrum Beta-Lactamase (ESBL) producing E. coli (75/224) 33.5%)) and ESBL-K. pneumoniae (30/224) 13.4%)). Median mechanical ventilation duration was 1 day (IQR 0-1), PICU stay 3 days (IQR 2-4) and hospital stay 6.5 days (IQR 5-10). A total of 17 infectious episodes occurred in 15 patients, mostly consisting in hospital-acquired pneumonia (HAP) (12/17). Only two infections were caused by a colonizing ARMO. Occurrence of infections and patients' outcome were similar between ARMO carriers and non-carriers. Higher use of carbapenems (6 (6.3%) vs 1 (0.8%), p = 0.04) and a trend to a higher use of vancomycin (14 (13.7%) vs 9 (6.9%), p = 0.04) in case of ARMOs carriage. Applying current guidelines, negative swab screening could have led to sparing most of empirical vancomycin therapy (11/12) for HAP based on current guidelines., Conclusion: Prevalence of AMROs carriage is high in children from LMIC and has a limited impact on patients' outcome. However, ARMOs carriage leads to higher consumption of antibiotics. Screening may help saving use of broad-spectrum antibiotic in non-carrier patients., (© 2024. The Author(s).)

Introduction: Pediatric pulmonary hypertension is a rare condition. Survival remains poor in the current management era. There is a lack of data regarding the medical management of pediatric pulmonary hypertension and most pulmonary vasodilators are used off-label in children., Areas Covered: Pediatric pulmonary hypertension clinical trials' design and realization face many hurdles, including poor recruitment, limited available pharmacologic and physiologic data in children of various ages, ethical issues, and the lack of validated trial endpoint. Innovative clinical trial designs have emerged and may allow us to overcome some of these issues. Extrapolation of adult data to children, with additional pharmacokinetic and safety data, remains extremely important and valid in etiologies where the pediatric and the adult pathophysiologies are believed to be similar., Expert Opinion: Close collaboration between sponsors, regulators, patients, caregivers, physicians and researchers is necessary to develop efficacious and safe drugs for pediatric pulmonary hypertension. The increasing involvement of patients' and caregivers' participation in the development of clinical trials should help shape future research that is feasible and meaningful to the patients.

Competing Interests: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

This open-label, extension study assessed long-term safety, tolerability, and efficacy of ambrisentan in a pediatric population (age 8- < 18 years) with pulmonary arterial hypertension (PAH). Following completion of a 6-month, randomized study, participants entered the long-term extension at individualized ambrisentan dosages (2.5/5/7.5 or 10 mg/day). Safety assessments included adverse events (AEs), AEs of special interest, and serious AEs (SAEs); efficacy outcomes included 6-min walking distance (6MWD) and World Health Organization functional class (WHO FC). Thirty-eight of 41 (93%) randomized study participants entered the extension; 21 (55%) completed (reaching age 18 years). Most participants received concomitant phosphodiesterase-5 inhibitors (n = 25/38, 66%). Median ambrisentan exposure was 3.5 years. Most participants experienced ≥ 1 AE (n = 34/38, 89%), and 21 (55%) experienced SAEs, most commonly worsening PAH (n = 3/38, 8%), acute cardiac failure, pneumonia, or anemia (n = 2/38; 5% each); none considered ambrisentan-related. Seven participants (18%) died, with recorded reasons (MedDRA preferred term): cardiac failure (n = 2), PAH (n = 2), COVID-19 (n = 1), acute right ventricular failure (n = 1), and failure to thrive (n = 1); median time to death: 5.2 years. Anemia and hepatotoxicity AEs were generally mild to moderate and did not require ambrisentan dose adjustment. Assessed at study end in 29 participants (76%), mean 6MWD improved by 17% (standard deviation: 34.3%), and all (29/29, 100%) had improved or unchanged WHO FC.    Conclusion: Long-term weight-based ambrisentan dosing, alone or combined with other PAH therapies in children with PAH aged 8- < 18 years, exhibited tolerability and clinical improvements consistent with prior randomized study results.    Trial registration: NCT01342952, April 27, 2011. What is Known: • The endothelin receptor antagonist, ambrisentan, is indicated for treatment of pulmonary arterial hypertension (PAH). Previous studies have shown similar efficacy and tolerability in pediatric patients as in adults. What is New: • This open-label extension study assessed the long-term use of ambrisentan in pediatric patients (8-<18 years) with PAH, most of whom were also receiving recommended background PAH treatment. • Weight-based dosing of ambrisentan, given alone or in combination with other PAH therapies, was well tolerated with clinical improvements consistent with prior randomized study results., (© 2024. The Author(s).)

Competing Interests: Disclosures None.

Aims: A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification. We aimed to analyse the applicability of an adapted clinical classification of PAH-CHD to pediatric patients using the TOPP-1 registry (Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension) and focus on atrial septal defects (ASD) and transposition of the great arteries (TGA)., Methods and Results: Hemodynamic and clinical data of all patients with PAH-CHD in the TOPP cohort were reviewed. Patients were classified according to predefined ABCDE categories (A: Eisenmenger syndrome, B: left-to-right shunt, C: coincidental defects, including all ASDs, D: corrected CHD, E: TGA), or as complex CHD (group 5), by 2 independent investigators. In case of disagreement, a third reviewer could either settle a final decision, or the patient was deemed not classifiable. Survival curves were calculated for each group and compared to idiopathic PAH patients of the registry. A total of 223 out of 531 patients in the registry had PAH-CHD, and 193 were categorized to the following groups: A 39(20%), B 27(14%), C 62(32%) including 43 ASDs, D 58(30%), E 7(4%), whereas 6 patients were categorized as group 5, and 10 patients were unable to be classified. No survival difference could be demonstrated between the groups., Conclusions: This modified classification seems to be more applicable to pediatric PAH-CHD patients than the previous classification, but some patients with PAH-CHD who never had a shunt remain unclassifiable. The role of ASD in pediatric PH should be reconsidered., Competing Interests: The M3C-Necker contracts with Actelion, Bayer, Novartis, MSD and Lilly for DB to perform consultant activities and to participate in steering committees for clinical trials. MB has received grants from and contracted as consultant for Actelion and Bayer-Schering; and served as a consultant and participated in the steering committee for Actelion/Janssen, Acceleron, AOP, Bayer-Schering, GlaxoSmithKline, and Eli Lilly, The University of Colorado contracts with Acceleron, Actelion/Janssen, Altavant, Bayer, Gilead, Gossamer Bio, Lilly, MSD and United Therapeutics for DI to be a consultant. The University Medical Center Groningen contracts with Actelion, GlaxoSmithKline, MSD, and Lilly for RB to perform consultant activities and to participate in steering committees for clinical trials. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Wacker, Humpl, Berger, Ivy, Bowers, Bonnet and Beghetti.)

Vasoreactive pulmonary arterial hypertension (PAH) in children is a form of idiopathic PAH that responds to vasoreactive testing with nitric oxide (NO) by a significant decrease of pulmonary vascular resistances and pressure. Oral calcium channel antagonists (CCA) that allow pulmonary arterial vasodilation are the treatment of choice. The therapeutic effect is strongly depending on adequate drug intake. In growing children, drug dose must be adapted to weight. In case of unavailability of low-dose pharmaceutical preparations, officinal formulations become mandatory. Officinal formulations may be related to a multitude of errors at different steps including prescription, transcription, preparation and administration. This may have life-threatening consequences for the child.To illustrate this, we report a case of a compounding error with underdosage of CCA, leading to acute cardiovascular failure in an adolescent with vasoreactive PAH.

Infective endocarditis due to Kingella kingae is a rare but serious invasive infection that occurs mostly in children. Recent advances in nucleic acid amplification testing as well as in cardiac imaging have enabled more accurate diagnosis. A good understanding of the epidemiology and virulence factors remains crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, pathophysiological insights, complications, and therapy regarding Kingella kingae endocarditis in children and adults. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.

Background and Aims: The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global network established to gain insights into the disease course and long-term outcomes of paediatric pulmonary arterial hypertension (PAH). Previously published cohorts in paediatric PAH are obscured by survival bias due to the inclusion of both prevalent (previously diagnosed) and incident (newly diagnosed) patients. The current study aims to describe long-term outcome and its predictors in paediatric PAH, exclusively of newly diagnosed patients., Methods and Results: Five hundred thirty-one children with confirmed pulmonary hypertension, aged ≥3 months and <18 years, were enrolled in the real-world TOPP registry at 33 centres in 20 countries, from 2008 to 2015. Of these, 242 children with newly diagnosed PAH with at least one follow-up visit were included in the current outcome analyses. During long-term follow-up, 42 (17.4%) children died, 9 (3.7%) underwent lung transplantation, 3 (1.2%) atrial septostomy, and 9 (3.7%) Potts shunt palliation (event rates: 6.2, 1.3, 0.4, and 1.4 events per 100 person-years, respectively). One-, three-, and five-year survival free from adverse outcome was 83.9%, 75.2%, and 71.8%, respectively.Overall, children with open (unrepaired or residual) cardiac shunts had the best survival rates. Younger age, worse World Health Organization functional class, and higher pulmonary vascular resistance index were identified as independent predictors of long-term adverse outcome. Younger age, higher mean right atrial pressure, and lower systemic venous oxygen saturation were specifically identified as independent predictors of early adverse outcome (within 12 months after enrolment)., Conclusion: This comprehensive analysis of survival from time of diagnosis in a large exclusive cohort of children newly diagnosed with PAH describes current-era outcome and its predictors., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Mutations in the TTN gene, encoding the muscle filament titin, are a major cause of inherited dilated cardiomyopathy. Early-onset skeletal muscle disorders due to recessive TTN mutations have recently been described, sometimes associated with cardiomyopathies., Case Description: We report the case of a boy with congenital core myopathy due to compound heterozygosity for TTN variants. He presented in infancy with rapidly evolving restrictive cardiomyopathy, requiring heart transplantation at the age of 5 years with favorable long-term cardiac and neuromuscular outcome., Conclusion: Heart transplantation may have a role in selected patients with TTN-related congenital myopathy with disproportionally severe cardiac presentation compared to skeletal and respiratory muscle involvement., (© 2023 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)

Purpose: Malignant stroke is a life-threatening emergency, with a high mortality rate (1-3). Despite strong evidence showing decreased morbidity and mortality in the adult population, decompressive hemicraniectomy (DCH) has been scarcely reported in the pediatric stroke population, and its indication remains controversial, while it could be a potential lifesaving option., Methods and Results: We performed an extensive literature review on pediatric malignant arterial ischemic stroke (pmAIS) and selected 26 articles reporting 97 cases. Gathering the data together, a 67% mortality rate is observed without decompressive therapy, contrasting with a 95.4% survival rate with it. The median modified Rankin score (mRS) is 2.1 after surgery with a mean follow-up of 31.8 months. For the 33% of children who survived without surgery, the mRS is 3 at a mean follow-up of 19 months. As an illustrative case, we report on a 2-year-old girl who presented a cardioembolic right middle cerebral artery stroke with subsequent malignant edema and ongoing cerebral transtentorial herniation in the course of a severe myocarditis requiring ECMO support. A DCH was done 32 h after symptom onset. At the age of 5 years, she exhibits an mRS of 3., Conclusion: Pediatric stroke with malignant edema is a severe condition with high mortality rate if left untreated and often long-lasting consequences. DCH might minimize the vicious circle of cerebral swelling, increasing intracranial pressure and brain ischemia. Our literature review underscores DCH as an efficient therapeutic measure management of pmAIS even when performed after a significant delay; however, long-lasting morbidities remain high., (© 2023. The Author(s).)

Introduction: Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD., Methods: This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT., Results: The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO 2 slope during CPET and an increased TVR of >2.5 m/s on TTE., Conclusion: DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better., Competing Interests: MB reports grants from Actelion and Bayer, contracted as consultant, and participates in the steering committee for Actelion/Janssen, Bayer, GSK, Gossamer, OrphaCare/AOP Altavant, and Merck/Acceleron, outside the submitted work. FL reports grants, personal fees, and non-financial support from Actelion, personal fees from MSD, and grants and personal fees from Bayer, outside the submitted work. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Joye, Wacker, Nguyen, Hachulla, Maggio, Cimasoni, Lador, Ansari and Beghetti.)

Left transposition of the great arteries with inlet ventricular septal defect and pulmonary stenosis is a relatively uncommon cardiac malformation. Two surgical treatments are available: double switch or physiological correction. The choice of surgical technique depends on the results of a discussion between the family and the surgeon. Choosing the appropriate technique is challenging because all options present various complications and benefits. We present a 'triple switch' aortic and pulmonary root inversion and modified Senning procedure for an anatomically complex left transposition of the great arteries with an inlet ventricular septal defect and pulmonary stenosis., (© The Author 2023. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

This article presents an implantable wireless system for remote hemodynamic monitoring, which enables direct, continuous (24/7), and simultaneous measurement of pulmonary arterial pressure (PAP) and cross-sectional area (CSA) of the artery. The implantable device, which measures 3.2 mm × 2 mm × 10 mm, comprises a piezoresistive pressure sensor, an ASIC implemented in 180-nm CMOS, a piezoelectric ultrasound (US) transducer, and a nitinol anchoring loop. An energy-efficient pressure monitoring system, which employs duty-cycling and spinning excitation technique, achieves 0.44 mmHg resolution in a pressure range from -135 mmHg to +135 mmHg and consumes 1.1 nJ conversion energy. The artery diameter monitoring system utilizes the inductive characteristic of the implant's anchoring loop and achieves 0.24 mm resolution within a diameter range of 20 mm to 30 mm, four times higher than echocardiography lateral resolution. The wireless US power and data platform enables simultaneous power and data transfer employing a single piezoelectric transducer in the implant. The system is characterized with an 8.5 cm tissue phantom and achieves a US link efficiency of 1.8%. The uplink data is transmitted by using an ASK modulation scheme parallel to the power transfer and achieves a modulation index of 26%. The implantable system is tested in an in-vitro experimental setup, which emulates the arterial blood flow, and accurately detects fast pressure peaks for systolic and diastolic pressure changes at both 1.28 MHz and 1.6 MHz US powering frequencies, with corresponding uplink data rates of 40 kbps and 50 kbps.

Congenital and acquired heart diseases can cause pulmonary hypertension (PH) in children, either by increasing pulmonary blood flow (PBF), left atrial pressure (LAp), and/or pulmonary vascular resistance (PVR). Pathophysiological process of pulmonary vascular disease (PVD) in different types of congenital heart diseases (CHDs) are reviewed hereafter. As with other types of PH, a rigorous diagnostic evaluation is mandatory to characterize the etiology of the PH, rule out other or additional causes of PH, and establish a risk profile. Cardiac catheterization remains the gold standard exam for PH diagnosis. Treatment of pulmonary arterial hypertension (PAH) associated with CHD (PAH-CHD) can then be started according to the recent guidelines recommendations, although most of the evidence is extrapolated from studies on other causes of PAH. PH in pediatric heart disease is often multifactorial, and sometimes unclassifiable, making the management of these patients complicated. The operability of patients with a prevalent left-to-right shunt and increase of PVR, the management of children with PH associated with left-sided heart disease, the challenges of pulmonary vascular disorders in children with univentricular heart physiology and the role of vasodilator therapy in failing Fontan patients are some of the hot topics discussed in this review., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-23-64/coif). The column “Pediatric Heart” was commissioned by the editorial office without any funding or sponsorship. FL receives honoraria and consulting fees from Janssen and MSD and receives support for attending meetings and/or travel from Janssen. MB receives grants, honoraria for lectures and support for attending meetings and/or travel from Actelion and Janssen, and receives consulting fees from Actelion, Janssen, Bayer, Gossamer, Merck, Altavant and Orpha. MB also participates on a Data Safety Monitoring Board or Advisory Board of GSK, Orpha, and Merck. The authors have no other conflicts of interest to declare., (2023 Translational Pediatrics. All rights reserved.)