Publications by authors named "Antonio Amodeo"

96 Publications

Telemedicine for adult congenital heart disease patients during the first wave of COVID-19 era: a single center experience.

J Cardiovasc Med (Hagerstown) 2021 Apr 20. Epub 2021 Apr 20.

Dipartimento di Scienze Cardiovascolari e Toraciche, Fondazione Policlinico Universitario A. Gemelli IRCCS Catholic University of the Sacred Heart Department of Pediatric Cardiology and Cardiac Surgery - Bambino Gesù Hospital, Rome, Italy.

Aim: To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of COVID-19.

Methods: This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination.

Results: A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29-51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5-11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab.

Conclusion: During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2459/JCM.0000000000001195DOI Listing
April 2021

Infant miniaturized continuous-flow pumps and permanent support in Pediatrics.

Ann Cardiothorac Surg 2021 Mar;10(2):278-280

Heart Failure, Transplant and Mechanical Cardiocirculatory Support Unit, Department of Pediatric Cardiology and Cardiac Surgery, Heart Lung Transplantation, ERN GUARD HEART: Bambino Gesù Hospital and Research Institute, Rome, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/acs-2020-cfmcs-15DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033256PMC
March 2021

Deciphering Genetic Variants of Warfarin Metabolism in Children With Ventricular Assist Devices.

Pediatr Cardiol 2021 Apr 10. Epub 2021 Apr 10.

Heart Failure, Transplant and Mechanical Assistance Program Unit, Bambino Gesù Children Hospital, Rome, Italy.

Warfarin is prescribed in patients with ventricular assist devices (VADs). Dosage depends on several factors including the underlying genotype. These include polymorphisms of genes encoding cytochrome P450 enzymes, the main ones being CYP2C9, VKORC1, and CYP4F2. The objectives of this study were to evaluate the prevalence of CY2CP9 1*2*3*, VKORC1, and CYP4F2 in children with VADs and the time to reach the target international normalized ratio. We performed a retrospective/prospective study in children with VADs. We recorded polymorphisms, disease, type of VAD, ethnicity, age, gender, height, weight, INR values, bleeding, and thromboembolic episodes. Informed consent was obtained. We enrolled 34 children (19 male, 15 female), with a median age of 2 years (range 0.3-17 years) and median weight of 6.9Kg. The Berlin Heart was the most commonly implanted VAD (22/34; 64%), and the most common diagnosis was dilated cardiomyopathy. Statistical analysis confirmed a significant partial correlation with VKORC1 CC (p = 0.019). The CYP2C9*2 CT genotype showed a late rise in target INR values (p = 0.06), while the CYP2C9*2 CC showed a tendency toward an early INR rise (p = 0.024). We provide new information on the contribution of the warfarin polymorphisms in children with VAD implantation. Pharmacogenomic dosing for children using warfarin has the potential to improve clinical care in VAD patients. Patients with the CYP2C9*2 CT genotype may need more time or higher doses to reach target INR, while clinicians may need to be aware of the potential for a rapid rise in INR in patients with the CYP2C9*2 CC genotype.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-021-02585-2DOI Listing
April 2021

Remember friedreich ataxia even in a toddler with apparently isolated dilated (not hypertrophic!) cardiomyopathy: revisited.

Minerva Pediatr (Torino) 2021 Apr 2. Epub 2021 Apr 2.

European Reference Network for rare, low prevalence and complex diseases of the heart - ERN GUARD-Heart HCP, Pediatric Cardiology and Arrhythmia/Syncope Units, Bambino Gesù Children Hospital and Research Institute, Rome, Italy -

Background: Friedreich Ataxia (FRDA) is the most common form of ataxia in late childhood. Neurological manifestations often precede cardiac involvement, presenting mainly as hypertrophic cardiomyopathy.

Methods: We describe a toddler with apparently isolated severe heart failure, successfully managed with heart transplant (HT). Although well described in adolescents and adults, onset of FRDA is very uncommon in toddlers and neurological ataxic features are predominant. The presenting symptom of cardiomyopathy is very rare. Similar history is rarely reported in literature, that we described, including an aggressive cardiomyopathy in children younger than 5years-old.

Results: Our patient was diagnosed with FRDA at a postoperative stage due to minimal neurological manifestations. Moreover, the novelty of this study lies in demonstrating a major DNA triplet repeat expansion in skeletal muscle compared to DNA from peripheral blood leukocytes. These results support the concept that triplet repeat expansion is variable among different tissues in FRDA, and in our case it was more expanded in the post mitotic muscular tissue than in blood cells.

Conclusions: We believe on the importance of taking in consideration this rare condition even in a toddler with apparently isolated cardiomyopathy and especially when conventional investigations give negative results. We discuss potential trigger effect of heart transplant as a precipitating factor in manifesting neurological symptoms. This observation corresponds to our experience and relates to three patients described so far (the third patient died suddenly). Early onset cardiomyopathy with FRDA should increase awareness of this rare condition and we highlight HT successful outcome. Further reports are needed to delineate this rare condition in youngsters.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S2724-5276.21.05969-3DOI Listing
April 2021

Use of carotid artery cannulation during redo sternotomy in congenital cardiac surgery: a single-centre experience.

Interact Cardiovasc Thorac Surg 2021 Mar 2. Epub 2021 Mar 2.

Department of Pediatric Cardiac Surgery, Bambino Gesù Children's Hospital, Rome, Italy.

Objectives: Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures.

Methods: We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery. We classified our population study on the basis of the primary diagnosis and the type of surgery. The primary outcomes of our analysis were the evaluation of the safety of the procedure in terms of survival and freedom from neurological events.

Results: We analysed 22 patients who had undergone previous complex operations. The median age and weight at the time of reintervention were 130.35 (range 0.46-435) months and 31.5 (range 2.2-85) kg, respectively. Composite graft carotid cannulation provided adequate arterial flow in all patients with a median arterial flow of 3.5 l/min/m2 (range 0.6-6). One major cardiac injury occurred during sternotomy when emergency cardiopulmonary bypass (CPB) was initiated. Moreover, during their hospital stays, all patients had an uneventful recovery without neurological or vascular complications and no cervical wound infections.

Conclusions: Carotid cannulation using interposition of a side graft on the common carotid artery for arterial inflow is a reliable and safe method for initiation of CPB in complex redo surgeries in patients with congenital disease. Complications directly associated with this type of cannulation are uncommon and allow surgical re-entry with overall low risks.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/icvts/ivab060DOI Listing
March 2021

A retrospective multicentric analysis on testicular torsion: is there still something to learn?

Scand J Urol 2021 Feb 23:1-7. Epub 2021 Feb 23.

Urology Clinic, Department of Surgical, Oncological and Gastroenterological Sciences, Padova University, Italy.

Introduction And Aim: Speedy diagnosis are mandatory in testicular torsion, nevertheless some cases of irreversible ischemia still occur. In this study we analysed the results of patients undergoing surgical exploration for acute scrotum.

Materials And Methods: A multicentric retrospective clinical evaluation was carried out on patients who underwent urgent scrotal exploration at 12 different departments in North-Eastern Italy. Data included complete anagraphic information, clinical presentation, numeric pain rating scale, previous testicular surgery, Doppler serial ultrasonography (US) evaluation and concordance with surgical findings, testicular mobility, surgical treatment, staged or concurrent treatment of the contralateral gonad. Statistical analysis was conducted both for descriptive and inferential statistics with SPSS v26.

Results: Three hundred and sixty-eight cases were collected between January 2010 and June 2019. The time between symptom onset and ER access time was within 6 h in majority of patients. However, 17.4% of subject presented after more than 12 h. In patients undergoing US, this showed signs of ischemia in 237 patients (77.2%) and normal vascularisation in 70 (22.8%) of whom 26 had signs of testicular torsion at surgical exploration. Overall, the US data were concordant with the surgical findings in 254 cases (82.7%). A significant association was found between time-to-evaluation and time-to-treatment and the need for orchiectomy ( < 0.01).

Conclusion: Testicular torsion management is still challenging in terms of time-saving decision making. Scrotal US is helpful, but even in the contemporary its sensitivity is low era in a non-neglectable number of cases, therefore surgical exploration is warranted in acute scrotum when torsion cannot be ruled out, even when US shows vascularisation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/21681805.2021.1889026DOI Listing
February 2021

Neonatal respiratory and cardiac ECMO in Europe.

Eur J Pediatr 2021 Jun 5;180(6):1675-1692. Epub 2021 Feb 5.

NICU, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Della Commenda 12, 20122, Milan, Italy.

Neonatal extracorporeal membrane oxygenation (ECMO) is a life-saving procedure for critically ill neonates suffering from a potentially reversible disease, causing severe cardiac and/or respiratory failure and refractory to maximal conventional management. Since the 1970s, technology, management, and clinical applications of neonatal ECMO have changed. Pulmonary diseases still represent the principal neonatal diagnosis, with an overall 74% survival rate, and up to one-third of cases are due to congenital diaphragmatic hernia. The overall survival rate in cardiac ECMO is lower, with congenital heart defect representing the main indication. This review provides an overview of the available evidence in the field of neonatal ECMO. We will address the changing epidemiology, basic principles, technologic advances in circuitry, and monitoring, and deliver a current multidisciplinary management framework, focusing on ECMO applications, complications, and long-term morbidities. Lastly, areas for further research will be highlighted.Conclusions: ECMO is a life support with a potential impact on long-term patients' outcomes. In the next years, advances in knowledge, technology, and expertise may push neonatal ECMO boundaries towards more premature and increasingly complex infants, with the final aim to reduce the burden of ECMO-related complications and improve overall patients' outcomes. What is Known: • ECMO is a life-saving option in newborns with refractory respiratory and/or cardiac failure. • The multidisciplinary ECMO management is challenging and may expose neonates to complications with an impact on long-term outcomes. What is New: • Advances in technology and biomaterials will improve neonatal ECMO management and, eventually, the long-term outcome of these complex patients. • Experimental models of artificial placenta and womb technology are under investigation and may provide clinical translation and future research opportunities.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-020-03898-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7864623PMC
June 2021

Long-term veno-arterial extracorporeal membrane oxygenation as a bridge to heart-lung transplant.

J Card Surg 2021 Mar 27;36(3):798-799. Epub 2021 Jan 27.

Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesú Children's Hospital IRCCS, Rome, Italy.

En bloc heart-lung transplantation still represents definitive therapy for end-stage cardiopulmonary failure. However, patients may critically decompensate while awaiting suitable donor organs and necessitate veno-arterial extracorporeal membrane oxygenation. In this article, we describe the combined use of central cannulation with the Berlin Heart EXCOR ventricular assist device cannulae and the CentriMag centrifugal pump as an extended bridge to heart-lung transplantation in three pediatric patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/jocs.15369DOI Listing
March 2021

Persistent myocardial atrophy despite LV reverse remodeling in Duchenne cardiomyopathy treated by LVAD.

Pediatr Transplant 2021 Mar 26;25(2):e13890. Epub 2020 Oct 26.

Pediatric Cardiology and Cardiac Arrhythmias/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

DCM is the leading cause of death in Duchenne patients. LVADs are considered as therapeutic options as DT in advanced HF. The aim of our study was to evaluate LV remodeling of Duchenne after LVADs and chronic therapy. Demographic and echocardiographic data of 8 Duchenne patients implanted with LVADs were reviewed and analyzed. All measures were collected before LVAD implantation, after 1 month and 1 year. All patients were affected by end-stage DCM, and mean age at implantation was 16.9 ± 2.9 years. Patients were treated with maximal medical therapy. One-year post-implantation HR decreased from a mean of 110 ± 19 bpm to 82 ± 2 bpm (P = .002), and a significant decrease in LV volumes and diameters LVEDD P = .03, LVESD P = .02, EDV P = .01, and ESV P = .02) was noticed together with a significant increase in EF (P = .0036). However, RWT did not change over time, showing an eccentric remodeling pattern pre- and post-LVADs. Our data showed that cardiac atrophy is persistent in Duchenne cardiomyopathy despite the improvement of LV function secondary to a significant ventricular unloading due to LVADs coupled with chronic therapy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/petr.13890DOI Listing
March 2021

Serum lactate at 24 hours is associated with outcome in children requiring extracorporeal membrane oxygenation for pulmonary causes - a retrospective, observational study.

Swiss Med Wkly 2020 Oct 6;150:w20358. Epub 2020 Oct 6.

Division of Congenital Cardiovascular Surgery, University Children's Hospital Zurich, Switzerland / Children's Research Centre, University Children's Hospital Zurich, Switzerland.

Objectives: Extracorporeal membrane oxygenation (ECMO) has become an essential life-saving tool. Being resource-intensive, judicious use and optimising the outcomes of this precious resource is important. This retrospective, explanatory, observational study aimed to quantify associations between factors and outcome after pulmonary ECMO in children.

Methods: This study included 39 consecutive ECMO runs in 38 children performed for pulmonary indications at our hospital from 2008 to 2018. Indications included acute respiratory distress syndrome, infection, drowning, meconium aspiration and pulmonary hypertension, among others. Depending on the need for haemodynamic support, 21 patients (53.8%) received veno-venous ECMO, while 18 (46.2%) received veno-arterial ECMO. We sought to compare the 11 non-survivors with the 27 survivors with respect to time-independent and time-dependent variables. Logistic regression models and Cox proportional hazards models were used. Threshold analysis was done using the “minimum p-value approach”.

Results: 27/39 (69%) ECMO runs could be weaned; 27/38 (71%) patients were discharged. 20/27 (74%) survivors had unremarkable neurological status, six (22%) had mild findings (convulsions, muscular hypotony, neuropathy) and one (4%) had a hemi-syndrome at discharge. Univariate analyses showed a hazard ratio (HR) of 0.48 for log(pH) (95% confidence interval [CI] 0.22 to 1.02, p = 0.055) and an HR of 4.48 for log(lactate) (95% CI 1.92 to 10.48, p = 0.0005). Multivariate models showed an HR of 0.99 for log(pH) (95% CI 0.43 to 2.26, p = 0.98) and an HR of 4.44 for log(lactate) (95% CI 1.65 to 11.95, p = 0.003). Threshold analysis showed lactate >4.1 to be associated with mortality, with an HR of 32.7 (95% CI 4.8 to 221.7, p = 0.0002). This threshold should, however, be interpreted very cautiously. Evidence of an association between serum lactate at 24 hours and mortality was found (difference between survivors and non-survivors: −2.78, 95% CI −5.36 to −0.20, p = 0.037).

Conclusions: The results of ECMO for pulmonary indications are very good. Serum lactate may be an early prognostic indicator.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4414/smw.2020.20358DOI Listing
October 2020

Duchenne Dilated Cardiomyopathy: Cardiac Management from Prevention to Advanced Cardiovascular Therapies.

J Clin Med 2020 Oct 1;9(10). Epub 2020 Oct 1.

Heart Failure Clinic-Heart Failure, Heart Transplant, Mechanical Circulatory Support Unit, Department of Pediatric Cardiology and Cardiac Surgery, Heart and Lung Transplant, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Duchenne muscular dystrophy (DMD) cardiomyopathy (DCM) is characterized by a hypokinetic, dilated phenotype progressively increasing with age. Regular cardiac care is crucial in DMD care. Early recognition and prophylactic use of angiotensin converting enzyme inhibitors (ACEi) are the main stay therapeutic strategy to delay incidence of DMD-DCM. Pharmacological treatment to improve symptoms and left ventricle (LV) systolic function, have been widely implemented in the past years. Because of lack of DMD specific drugs, actual indications for established DCM include current treatment for heart failure (HF). This review focuses on current HF strategies to identify, characterize, and treat DMD-DCM.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/jcm9103186DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7600130PMC
October 2020

Pathophysiology and molecular signalling in pediatric heart failure and VAD therapy.

Clin Chim Acta 2020 Nov 17;510:751-759. Epub 2020 Sep 17.

Institute of Clinical Physiology, CNR, Pisa, Italy. Electronic address:

Heart Failure (HF) is a progressive clinical syndrome characterized by molecular and structural abnormalities that result in impaired ventricular filling and a reduced blood ejection. In pediatric patients, HF represents an important cause of morbidity and mortality, but underlying cause, presentation and disease course remains unclear in many cases. It is evident that a child is not a "small adult" and findings are not comparable. The adoption of a standardized clinical and surgical tools as well as increased biomolecular research and therapeutic trials targeting pediatric patients with HF would greatly improve the management of this special class of patients. This review examines the most current information about the pathophysiology and molecular mechanisms related to HF in children to identify gaps in our knowledge base to further improve clinical care and outcomes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.cca.2020.09.010DOI Listing
November 2020

Ethics and extracorporeal membrane oxygenation during coronavirus disease 2019 outbreak.

Perfusion 2020 09 25;35(6):562-564. Epub 2020 Jun 25.

Pediatric Cardiac Intensive Care Unit, Children's Hospital Bambino Gesù, Rome, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0267659120937545DOI Listing
September 2020

Undiagnosed Severe Late Complications of Repaired Tetralogy of Fallot.

Circ Cardiovasc Imaging 2020 06;13(6):e010273

Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy (R.L., E.P., F.G., A.M.L., M.G., F.C., M.M.).

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCIMAGING.119.010273DOI Listing
June 2020

Variations of circulating miRNA in paediatric patients with Heart Failure supported with Ventricular Assist Device: a pilot study.

Sci Rep 2020 04 3;10(1):5905. Epub 2020 Apr 3.

Institute of Clinical Physiology, CNR, Pisa, Italy.

Circulating miRNAs (c-miRNAs) are promising biomarkers for HF diagnosis and prognosis. There are no studies on HF pediatric patients undergoing VAD-implantation. Aims of this study were: to examine the c-miRNAs profile in HF children; to evaluate the effects of VAD on c-miRNAs levels; to in vitro validate putative c-miRNA targets. c-miRNA profile was determined in serum of HF children by NGS before and one month after VAD-implant. The c-miRNA differentially expressed were analyzed by real time-PCR, before and at 4 hrs,1,3,7,14,30 days after VAD-implant. A miRNA mimic transfection study in HepG2 cells was performed to validate putative miRNA targets selected through miRWalk database. Thirteen c-miRNAs were modified at 30 days after VAD-implant compared to pre-VAD at NSG, and, among them, six c-miRNAs were confirmed by Real-TimePCR. Putative targets of the validated c-miRNAs are involved in the hemostatic process. The in vitro study confirmed a down-regulatory effect of hsa-miR-409-3p towards coagulation factor 7 (F7) and F2. Of note, all patients had thrombotic events requiring pump change. In conclusion, in HF children, the level of six c-miRNAs involved in the regulation of hemostatic events changed after 30 days of VAD-treatment. In particular, the lowering of c-miR-409-3p regulating both F7 and F2 could reflect a pro-thrombotic state after VAD-implant.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41598-020-62757-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7125126PMC
April 2020

Left ventricular unloading during extracorporeal membrane oxygenation - Impella versus atrial septal defect: A simulation study.

Int J Artif Organs 2020 Oct 22;43(10):663-670. Epub 2020 Feb 22.

Department of Pediatric Cardiology and Cardiac Surgery, Pediatric Hospital Bambino Gesù, Rome, Italy.

Background: Atrial septal defect and Impella have been proposed for left ventricular unloading in venoarterial extracorporeal membrane oxygenation patients. This work aims at evaluating the haemodynamic changes in venoarterial extracorporeal membrane oxygenation patients after Impella implantation or atrial septal defect realization by a simulation study.

Methods: A lumped parameter model of the cardiovascular system was adapted to this study. Atrial septal defect was modelled as a resistance between the two atria. Venoarterial extracorporeal membrane oxygenation and Impella were modelled starting from their pressure-flow characteristics. The baseline condition of a patient undergoing venoarterial extracorporeal membrane oxygenation was reproduced starting from haemodynamic and echocardiographic data. The effects of different atrial septal defect size, Impella and venoarterial extracorporeal membrane oxygenation support were simulated.

Results: Impella caused an increment of mean arterial pressure up to 67%, a decrement in mean pulmonary arterial pressure up to 8%, a decrement in left ventricular end systolic volume up to 11% with a reduction up to 97% of left ventricular cardiac output. Atrial septal defect reduces left atrial pressure (19%), increases right atrial pressure (22%), increases mean arterial pressure (18%), decreases left ventricular end systolic volume (11%), increases right ventricular volume (33%) and decreases left ventricular cardiac output (55%).

Conclusion: Impella has a higher capability in left ventricular unloading during venoarterial extracorporeal membrane oxygenation in comparison to atrial septal defect with a lower right ventricular overload.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0391398820906840DOI Listing
October 2020

ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation.

J Heart Lung Transplant 2020 04 31;39(4):331-341. Epub 2020 Jan 31.

Heart Institute, Joe Dimaggio Children's Hospital, Hollywood, Florida.

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.healun.2020.01.1345DOI Listing
April 2020

Behavioral economics-A framework for donor organ decision-making in pediatric heart transplantation.

Pediatr Transplant 2020 05 27;24(3):e13655. Epub 2020 Jan 27.

Division of Pediatric Cardiology, University of Texas Southwestern Medical Center, Children's Medical Center, Dallas, TX, USA.

The high discard rate of pediatric donor hearts presents a major challenge for children awaiting heart transplantation. Recent literature identifies several factors that contribute to the disparities in pediatric donor heart usage, including regulatory oversight, the absence of guidelines on pediatric donor heart acceptance, and variation among transplant programs. However, a likely additional contributor to this issue are the behavioral factors influencing transplant team decisions in donor offer scenarios, a topic that has not yet been studied in detail. Behavioral economics and decision psychology provide an excellent foundation for investigating decision-making in the pediatric transplant setting, offering key insights into the behavior of transplant professionals. We conducted a systematic review of published literature in pediatric heart transplant related to behavioral economics and the psychology of decision-making. In this review, we draw on paradigms from these two domains in order to examine how existing aspects of the transplant environment, including regulatory oversight, programmatic variation, and allocation systems, may precipitate potential biases surrounding donor offer decisions. Recognizing how human decision behavior influences donor acceptance is a first step toward improving utilization of potentially viable pediatric donor hearts.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/petr.13655DOI Listing
May 2020

First human implantation of a miniaturized axial flow ventricular assist device in a child with end-stage heart failure.

J Heart Lung Transplant 2020 01 11;39(1):83-87. Epub 2019 Sep 11.

Department of Pediatrics, Stanford University School of Medicine, Palo Alto, California. Electronic address:

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.healun.2019.09.003DOI Listing
January 2020

Principlism and Personalism. Comparing Two Ethical Models Applied Clinically in Neonates Undergoing Extracorporeal Membrane Oxygenation Support.

Front Pediatr 2019 30;7:312. Epub 2019 Jul 30.

Department of Critical Care, The Hospital for Sick Children, Toronto, ON, Canada.

Extracorporeal membrane oxygenation (ECMO) is a technology used to temporarily assist critically ill patients with acute and reversible life-threatening cardiac and/or respiratory failure. This technology can often be lifesaving but is also associated with several complications that may contribute to reduced survival. Currently, neonates supported with ECMO are complex and bear an increased risk of mortality. This means that clinicians must be particularly prepared not only to deal with complex clinical scenarios, but also ethical issues associated with ECMO. In particular, clinicians should be trained to handle unsuccessful ECMO runs with attention to high quality end of life care. Within this manuscript we will compare and contrast the application of two ethical frameworks, used in the authors' institutions (Toronto and Rome). This is intended to enhance a broader understanding of cultural differences in applied ethics which is useful to the clinician in an increasingly multicultural and diverse patient mix.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fped.2019.00312DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6682695PMC
July 2019

Predicting the pressure of the total cavopulmonary connection: clinical testing of a mathematical equation.

Cardiol Young 2019 Aug 23;29(8):1066-1071. Epub 2019 Jul 23.

Department of Pediatric Cardiology and Cardiac Surgery, Pediatric Hospital Bambino Gesù, Rome, Italy.

Introduction: Some authors advocate the use of a dedicated formula to predict the Fontan pressure starting from pre-Fontan catheterisation data. This paper aims at testing the predictive value of the mentioned formula through a retrospective clinical study.

Methods And Results: Pre-Fontan catheterisation data and Fontan pressure measured at the completion were retrospectively collected. Pre-Fontan data were used to calculate the predicted pressure in the Fontan system. The predicted values were compared to the Fontan pressure measured at the Fontan completion and with the needs for fenestration. One hundred twenty-four Fontan patients were retrospectively enrolled (At Fontan: median age 30.73 [24.70-37.20] months, median weight 12.00 [10.98-14.15] kg). Fontan conduit was fenestrated in 78 patients. A poor correlation (r2 = 0.05128) between the measured and predicted data for non-fenestrated patients was observed. In the case of Fontan-predicted pressure <17.59 mmHg, the formula identified a good short-term clinical outcome with a sensitivity of 92%.

Conclusion: The proposed formula showed a poor capability in estimating the actual pressure into the Fontan system and in identifying patients needing fenestration. As the pressure into the Fontan system is determined by multiple factors, the tested formula could be an additional data in a multi-parametric approach.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1017/S1047951119001513DOI Listing
August 2019

Changes in left and right ventricular two-dimensional echocardiographic speckle-tracking indices in pediatric LVAD population: A retrospective clinical study.

Int J Artif Organs 2019 Dec 26;42(12):711-716. Epub 2019 Jun 26.

Department of Cardiology and Cardiac Surgery, Bambino Gesù Pediatric Hospital, Rome, Italy.

Echocardiographic strain and strain-rate imaging is a promising tool for the evaluation of myocardial segmental function, for the early detection of myocardial dysfunction, and for the prediction of reverse remodeling. We aimed at studying the changes in left and right ventricular function in pulsatile left ventricular assist device pediatric patients by two-dimensional echocardiography and two-dimensional speckle-tracking echocardiography. Echocardiographic and clinical data of patients implanted with a pulsatile-flow left ventricular assist device from 2011 to 2018 were retrospectively reviewed before and after implantation at 1, 3, and 6 months. A total of 18 patients were enrolled. Median age and weight at implantation were 9 months (5-23 months) and 5.85 kg (4.85-8.75 kg), respectively; median left ventricular assist device support was 181 (114.5-289.5) days. 13 patients (73%) were transplanted and 5 patients (27%) died. At follow-up: left ventricular ejection fraction increase at 1 month (p = 0.001) and 3 months (p = 0.01), left ventricular global longitudinal strain improvement at 1 month (p = 0.0008) and 3 months (p = 0.02), and right ventricular free-wall longitudinal strain increase at 1 month (p = 0.01). At short term after left ventricular assist device implantation, both left ventricular and right ventricular mechanics improved. The temporary benefit seems to decrease over time. The worsening of left ventricular function has been followed by a worsening of right ventricular function probably due to the ventricular interdependence.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/0391398819857446DOI Listing
December 2019

Heart rate reduction strategy using ivabradine in end-stage Duchenne cardiomyopathy.

Int J Cardiol 2019 04 17;280:99-103. Epub 2019 Jan 17.

Department of Pediatric Cardiology and Cardiac Surgery, Cardiology Unit, Bambino Gesù Hospital & Research Institute, Rome, Italy.

Background: End-stage dilated cardiomyopathy (DCM) is the leading cause of morbidity and mortality in patients with Duchenne Muscular Dystrophy (DMD). No studies are available on the effect of ivabradine on long-term outcomes in end-stage DMD/DCM.

Methods: We prospectively enrolled a cohort of end-stage DMD/DCM patients with LV ejection fraction <40%, on chronic HF treatment with an ACE inhibitor referred consecutively from 2012 to 2017 to Bambino Gesù Children's Hospital. In each patient, before starting HRR strategy and after 1 year, we collected medical records comprehensive of clinical, demographic and imaging parameters, BNP levels, neurological and respiratory assessment.

Results: Twenty male patients with DMD/DCM with a mean age of 15.0 ± 3.5 (13-19 IQR) years were enrolled and divided into 2 groups according to ivabradine therapy. This group showed a higher incidence of MACEs compared to others in treatment with ivabradine (87.5% vs 12.5%, p = 0.025). At Kaplan Meier survival analysis curves, the rate free from MACEs was higher in patients treated with ivabradine (log rank p = 0.017). At multivariate Cox regression analysis, ivabradine therapy was an independent predictor of freedom from MACEs (H.R. 0.078, 95% CI 0.007-0.877, p = 0.039).

Conclusion: HRR strategy, whether achieved by beta blockers alone or in combination with ivabradine, seemed to be effective in reducing the incidence of acute adverse events, reaching optimal target heart rate and improving left ventricular function in DMD/DCM patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijcard.2019.01.052DOI Listing
April 2019

Improvement of survival in low-weight children on the Berlin Heart EXCOR ventricular assist device support†.

Eur J Cardiothorac Surg 2019 May;55(5):913-919

Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada.

Objectives: Publications on the paediatric Berlin Heart EXCOR ventricular assist device have revealed that low body weight <10 kg is a significant risk factor for mortality with children weighing <5 kg being at the highest risk. However, these studies are limited to implantation periods prior to 2011. Since then, progress has been made in the optimization of patient selection and management. This study investigated whether the survival of children weighing <10 kg supported with the EXCOR assist device has improved in recent years and sought to determine the risk factors for mortality.

Methods: The Berlin Heart EXCOR prospective registry (n = 1832) was retrospectively reviewed between 2000 and 2017 to compare the outcomes of different weight cohorts: A (<5 kg; n = 204), B (5-10 kg; n = 633) and C (>10 kg; n = 995) in different eras [era 1: January 2000-December 2012 (n = 1089) and era 2: January 2013-June 2017 (n = 743)].

Results: Overall survival in groups A and B significantly increased from era 1 to era 2 (group A 51% vs 65%, P < 0.001; group B 74% vs 78%, P = 0.001), whereas it remained stable in group C (78% vs 73%). In era 2, the survival of group B was not significantly different from group C. On the multivariable analysis of children weighing <5 kg, congenital heart disease, preoperative extracorporeal life support and biventricular support were independently associated with increased mortality in era 1 [hazard ratio 2.04 (95% confidence interval 1.18-3.53); 2.44 (1.36-4.37) and 1.93 (1.11-3.34), respectively] but not in era 2.

Conclusions: Paediatric EXCOR ventricular assist device therapy has significantly improved for patients weighing <10 kg. Withholding a ventricular assist device is not justified on the basis of the body weight alone.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/ejcts/ezy394DOI Listing
May 2019

Is the New Infant Jarvik 2015 Suitable for Patients<8 kg? In Vitro Study Using a Hybrid Simulator.

Artif Organs 2019 Jan 6;43(1):E1-E8. Epub 2018 Nov 6.

Department of Pediatric Cardiology and Cardiac Surgery, Pediatric Hospital Bambino Gesù, Rome, Italy.

Our aim was to study the feasibility of implanting the Infant Jarvik 2015 in patients weighing less than 8 kg. The Infant Jarvik 2015 left ventricular assist device (LVAD) was tested in a hybrid simulator of the cardiovascular system reproducing specific patients' hemodynamics for different patient weights (2-7 kg). For each weight, the sensitivity of the pump to different circulatory parameters (peripheral resistance, left ventricular elastance, right ventricular elastance, heart rate, and heart filling characteristics) has been tested repeating for each experiment a pump ramp (10 000-18 000 rpm). The increase in the pump speed causes a decrease (increase) in the left (right) atrial pressure, an increase (decrease) in the arterial systemic (pulmonary) pressure, an increase in the right ventricular pressure, a decrease (increase) in the left (right) ventricular volume, a decrease in the left ventricular cardiac output, an increase in the LVAD output and an increase in the right ventricular cardiac output (total cardiac output). Suction was observed for lower weight patients and for higher pump speed in the case of vasodilation, left ventricular recovery, bradycardia, right ventricular failure, and left ventricular hypertrophy. Backflow was observed in the case of left ventricular recovery at lower pump speed. In the hybrid simulator, the Infant Jarvik 2015 could be suitable for the implantation in patients lower than 8 kg because of the stability of the device respect to the cardio/circulatory changes (low frequency of suction and backflow) and because of the capability of the device to maintain adequate patient hemodynamics.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/aor.13302DOI Listing
January 2019

Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine.

J Clin Med 2018 Sep 19;7(9). Epub 2018 Sep 19.

Dipartimento di Scienze Cardiovascolari e Toraciche, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00100 Rome, Italy.

Duchenne's muscular dystrophy is an X-linked neuromuscular disease that manifests as muscle atrophy and cardiomyopathy in young boys. However, a considerable percentage of carrier females are often diagnosed with cardiomyopathy at an advanced stage. Existing therapy is not disease-specific and has limited effect, thus many patients and symptomatic carrier females prematurely die due to heart failure. Early detection is one of the major challenges that muscular dystrophy patients, carrier females, family members and, research and medical teams face in the complex course of dystrophic cardiomyopathy management. Despite the widespread adoption of advanced imaging modalities such as cardiac magnetic resonance, there is much scope for refining the diagnosis and treatment of dystrophic cardiomyopathy. This comprehensive review will focus on the pertinent clinical aspects of cardiac disease in muscular dystrophy while also providing a detailed consideration of the known and developing concepts in the pathophysiology of muscular dystrophy and forthcoming therapeutic options.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/jcm7090291DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6162458PMC
September 2018

First evidence of maternally inherited mosaicism in TGFBR1 and subtle primary myocardial changes in Loeys-Dietz syndrome: a case report.

BMC Med Genet 2018 09 15;19(1):170. Epub 2018 Sep 15.

Mechanical Assistance Device and Artificial Heart Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.

Background: Loeys-Dietz syndrome (LDS) is a rare multisystemic disorder characterized by vascular and skeletal abnormalities, with considerable intra- and interfamilial variability.

Case Presentation: We report the case of an 8-year-old male with clinical features of two distinct genetic disorders, namely LDS, manifesting in the first months by progressive aortic root dilatation, arterial tortuosity, bifid uvula, and inguinal hernias and oculocutaneous albinism (OCA) manifesting by white hair and skin that does not tan, nystagmus, reduced iris pigment with iris translucency, and reduced retinal pigment). We identified previously reported, homozygous mutations of TYR, c.1A > G (p.Met1Val) and heterozygous, missense mutation of TGFBR1, c.1460G > A (p.Arg487Gln). Family history revealed that his mother underwent multiple surgical repairs for recurrent hemorrhage originating from the buccal artery. Molecular studies confirmed a maternally inherited low grade TGFBR1 mutation somatic mosaicism (18% in peripheral blood leukocytes, 18% in buccal cells and 10% in hair root cells). Maternal cardiac investigations revealed peculiar cardiovascular features: mild tortuosity at the aortic arch, dilatation of the proximal abdominal aorta, multiple deep left ventricular myocardial crypts, and dysplastic mitral valve. TGFBR2 germline mosaicism has been described in three fathers of children carrying TGFBR2 mutations but, to the best of our knowledge, no case of maternally inherited TGFBR1 mutation mosaicism has been reported so far.

Conclusions: This case report suggests that individuals with somatic mosaicism might be at risk for mild and unusual forms of LDS but germline mosaicism can lead to full blown picture of the disease in offspring.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12881-018-0661-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139163PMC
September 2018

The introduction of a high-fidelity simulation program for training pediatric critical care personnel reduces the times to manage extracorporeal membrane oxygenation emergencies and improves teamwork.

J Thorac Dis 2018 Jun;10(6):3409-3417

Department of Intensive Care, Hôpital Erasme, Université Libre de Bruxelles, ULB, Brussels, Belgium.

Background: Extracorporeal membrane oxygenation (ECMO) is used to support patients with severe respiratory and/or cardiac failure unresponsive to conventional treatments. Despite being one of the most complex supportive therapy used in intensive care unit, there is a still a lack of training programs dedicated to improve both clinical and nonclinical skills. The aim of the current study was to evaluate if the introduction of an ECMO high-fidelity simulation curriculum among personnel reduces the times to manage bedside emergencies and improves the behavioral skills.

Methods: This retrospective study was performed from 2011 to 2016 in a 6-beds general pediatric intensive care unit (PICU) of a tertiary children's hospital. The study population was the PICU personnel. From the beginning of 2011 to the end of 2013, ECMO education was provided without a simulation program. A high-fidelity simulation program instead, was provided from December 2013. Times to manage the most common ECMO emergencies (pump failure, oxygenator change and air embolism management) as well as the behavioral skills of the personnel were evaluated before and after the simulation intervention only in novice learners to reduce the bias related to the natural improvement associated with the bedside practice.

Results: There were a total of 30 ECMO runs and 27 ECMO emergencies over the study period. Ten ECMO emergencies occurred during the pre-simulation period and 17 in the post-simulation period. The median time to change an oxygenator in case of failure was 5.3 (4.80-6.02) min during the pre-simulation period 3.9 (3.50-4.15) min in the post-simulation period (P=0.02). The median time to manage an air embolism emergency was 22 (20.00-23.50) min during the pre-simulation period 15 (13.75-16.50) min in the post-simulation period (P=0.048). Only one episode of pump failure occurred in either the pre-simulation and post-simulation periods. In the pre-simulation period the median cumulative behavioral score was 40 (35.00-44.75) whereas it was 48 (44.5-49.00) in the post-simulation period (P<0.01).

Conclusions: The introduction of a high-fidelity simulation program for pediatric ECMO improved both the times to effective interventions and behavioral skills.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/jtd.2018.05.77DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6051841PMC
June 2018

Time-course of circulating cardiac and inflammatory biomarkers after Ventricular Assist Device implantation: Comparison between paediatric and adult patients.

Clin Chim Acta 2018 Nov 21;486:88-93. Epub 2018 Jul 21.

CNR, Institute of Clinical Physiology, Laboratory of Biochemistry and Molecular Biology, Pisa, Italy. Electronic address:

Background: Ventricular Assist Device (VAD) as bridge to transplantation is a common therapy for adult with heart failure (HF), but VAD use is increasing also in children. Cardiac and inflammatory biomarkers have an important role in the diagnosis and prognosis of HF in adults, but their role in paediatric setting is unknown. The aim of this study was to examine changes in cardiac and inflammatory biomarkers, both in HF paediatric and adult patients, before and following VAD.

Methods: Cardiac (NT-proBNP, cTnI, sST2,Gal-3) and inflammatory (IL-6,IL-8) biomarkers were determined in plasma collected from 12 paediatric patients and 7 adult patients with HF, before and at 4 h,1,3,7,14 and 30 days after VAD implant.

Results: All biomarkers increased up to 1 day after VAD implant and then decreased at pre-VAD levels in 1 month in both groups. Only in children, NT-proBNP decreased significantly after 30 days Post-VAD treatment compared to pre-VAD levels. During the post-operative time-course, NT-proBNP and sST2 were significantly higher in children than adults, while IL-6 was lower.

Conclusions: Cardiac and inflammatory biomarkers were differently modified by VAD implant in children compared to adults. These preliminary data could suggest that different molecular pathways may underlie HF patho-physiology of the two groups, possibly paving the way to a specific and targeted therapeutic intervention in the near future.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.cca.2018.07.036DOI Listing
November 2018