Publications by authors named "Gianluigi Perri"

42 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.
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http://dx.doi.org/10.2459/JCM.0000000000001195DOI 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.
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http://dx.doi.org/10.1093/icvts/ivab060DOI Listing
March 2021

Commentary: "Which Outcome for Adult Repair of Partial Atrioventricular Septal Defects?"

Semin Thorac Cardiovasc Surg 2021 Feb 15. Epub 2021 Feb 15.

Pediatric Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children Hospital, Rome, Italy. Electronic address:

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http://dx.doi.org/10.1053/j.semtcvs.2020.12.017DOI Listing
February 2021

Commentary: Looking for the ideal pulmonary valve.

J Thorac Cardiovasc Surg 2020 08 11;160(2):485-486. Epub 2020 May 11.

Pediatric Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children Hospital, Rome, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2020.04.135DOI Listing
August 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.).

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

Anomalous Origin of Left Common Carotid Artery From Left Pulmonary Artery in a 22q11.2 Deletion Syndrome Newborn With Right Aortic Arch and Aberrant Left Subclavian Artery.

Circ Cardiovasc Imaging 2020 03 5;13(3):e010087. Epub 2020 Mar 5.

Department of Pediatric Cardiology and Cardiac Surgery (W.V., G.P., S.F, F.S.I.), Bambino Gesù Children's Hospital, Rome, Italy.

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http://dx.doi.org/10.1161/CIRCIMAGING.119.010087DOI Listing
March 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.
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http://dx.doi.org/10.1177/0391398820906840DOI Listing
October 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:

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

Outcome following heart transplant assessment in adults with congenital heart disease.

Heart 2019 11 5;105(22):1741-1747. Epub 2019 Jul 5.

Adult Congenital and Pediatric Heart Unit, Freeman Hospital Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.

Objectives: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT).

Methods: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV).

Results: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period.

Conclusions: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.
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http://dx.doi.org/10.1136/heartjnl-2019-314711DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6855839PMC
November 2019

Italian survey on cardiac surgery for adults with congenital heart disease: which surgery, where and by whom?

Interact Cardiovasc Thorac Surg 2019 Mar 25. Epub 2019 Mar 25.

Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, Division of Cardiac Surgery, University of Verona, Verona, Italy.

Objectives: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom.

Methods: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units.

Results: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons.

Conclusions: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases.
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http://dx.doi.org/10.1093/icvts/ivz045DOI Listing
March 2019

Aortic Valve Replacement in Elderly Patients With Small Aortic Annulus: Results With Three Different Bioprostheses.

Innovations (Phila) 2019 Feb 7;14(1):27-36. Epub 2019 Mar 7.

1 Cardiovascular Sciences Department, Foundation Polyclinic University A. Gemelli IRCCS, Rome, Italy.

Objectives: Aortic valve replacement (AVR) in patients with small aortic annulus (diameter ≤21 mm) is considered a challenging scenario because of technical aspects and the high risk of patient-prosthesis mismatch (PPM). The choice of the appropriate prosthesis is crucial, and at the moment, an ideal device has yet to be identified. We compare clinical and hemodynamic results after AVR with three bioprostheses with different design and characteristics.

Methods: We retrospectively evaluated 76 consecutive patients from two cardiac surgery centers who underwent AVR (Trifecta = 24; Edwards INTUITY Elite valve system = 26, and Perceval = 26) for severe aortic stenosis between 2013 and 2017. Patients selected were older than 75 years and with an annulus diameter ≤21 mm at preoperative echocardiogram. Reinterventions and combined procedures were excluded. Minimally invasive AVR was performed in 44 (57.8%) patients. Telephonic interview was obtained at 2.9 ± 0.5 years and echocardiographic follow-up at 2.2 ± 0.8 years.

Results: Clinical outcome was similar in the three groups. At follow-up, Trifecta patients presented significantly higher peak and mean transprosthetic pressure gradients ( P = 0.04 and 0.01). Effective orifice area and left ventricular mass regression were comparable, although an advantage was observed in Perceval patients without reaching the statistical significance. Incidence of moderate ( P = 0.2) and severe PPM ( P = 0.7) was comparable.

Conclusions: Despite higher postoperative pressure gradients observed with the Trifecta valve, all three prostheses (Trifecta, Edwards INTUITY Elite, and Perceval) have proven to be reliable when implanted in small aortic annuli, with good clinical outcome and favorable left ventricular mass regression.
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http://dx.doi.org/10.1177/1556984519826430DOI Listing
February 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.
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http://dx.doi.org/10.1016/j.ijcard.2019.01.052DOI Listing
April 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.
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http://dx.doi.org/10.1111/aor.13302DOI Listing
January 2019

Mechanical Circulatory Support for Single Ventricle Failure.

Front Cardiovasc Med 2018 28;5:115. Epub 2018 Aug 28.

Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, United States.

Mechanical circulatory support (MCS) for failing single ventricle (SV) physiology is a complex and challenging problem, which has not yet been satisfactorily addressed. Advancements in surgical strategies and techniques along with intensive care management have substantially improved the outcomes of neonatal palliation for SV physiology, particularly for hypoplastic left heart syndrome (HLHS). This is associated with a steady increase in the number of SV patients who are susceptible to develop heart failure (HF) and would potentially require MCS at a certain stage in their palliation. We have reviewed the literature regarding the reported modalities of MCS use in the management of SV patients. This includes analysis of various devices and strategies used for failing circulation at distinct stages of the SV pathway: after neonatal palliation, after the superior cavo-pulmonary connection (SCPC), and after total cavo-pulmonary connection (TCPC).
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http://dx.doi.org/10.3389/fcvm.2018.00115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6122112PMC
August 2018

Acute heart failure related to a large left atrial myxoma.

Proc (Bayl Univ Med Cent) 2018 Jul 9;31(3):331-333. Epub 2018 Apr 9.

Cardiovascular Sciences Department, Catholic University of The Sacred Heart, Agostino Gemelli Policlinic, Rome, Italy.

An association between atrial myxoma and left ventricular failure is rarely described, is not completely understood, and may have multiple etiologies. We present a 49-year-old man with no history of cardiovascular disease who was admitted to our hospital with pulmonary edema. He was in atrial fibrillation with rapid ventricular response. Echocardiography showed a 10.5-cm left atrial myxoma, which had been asymptomatic until the onset of congestive heart failure in the presence of severe left ventricular systolic dysfunction. Left ventricular inflow obstruction associated with the giant atrial mass could not be the only cause for acute heart failure.
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http://dx.doi.org/10.1080/08998280.2018.1446641DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5997048PMC
July 2018

A New 2D Echocardiographic Approach to Evaluate the Membrane and Valve Movement of the Berlin Heart EXCOR VAD Chamber in Pediatric VAD Patients.

Artif Organs 2018 Apr;42(4):451-456

Department of Pediatric Cardiology and Cardiac Surgery, -Ospedale Pediatrico Bambino Gesù, Rome, Italy.

The use of Berlin Heart EXCOR VAD (BH) is a validated therapy to bridge pediatric patients to heart transplant. Serial echocardiographic (ECHO) assessment of VAD patients is necessary to support patients' management. This work aims at developing an innovative strategy to evaluate the BH device functioning by ECHO and its interaction with the native heart in a pediatric population. ECHO evaluation of BH membrane movement, and inflow and outflow valves was performed in 2D, 2D-color Doppler, M-mode, and M-mode color Doppler to assess the functioning of the device by direct positioning of the ECHO probe on the BH cannulas and membranes. Forty Berlin Heart EXCOR VAD were analyzed in 18 patients. Seven BH were placed as RVAD and 33 as LVAD. Results evidenced that 14 (21) inflow (outflow) valves presented a mild regurgitation, while 5 inflow (3 outflow) valves presented a moderate regurgitation. In three cases, we observed severe valve regurgitation with back flow in the left ventricle/right atrium. In both cases, the BH chambers were substituted, but we observed that in one case the regurgitation was due to cannulas compression, while in the other case it was due to valve malfunctioning. The M-mode and the ECHO of the membranes and valves permitted to appreciate the beat phenomenon to assess if the native heart and the BH are working in opposite or in the same phase. The membrane ECHO permits evaluation of minimal changes in membrane movement to assure the completely empty-completely fully work modality. Systematic ECHO assessment of BH chamber might support the BH programming and the detection of anomalous VAD-heart interaction.
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http://dx.doi.org/10.1111/aor.13122DOI Listing
April 2018

Evolution of Biventricular Loading Condition in Pediatric LVAD Patient: A Prospective and Observational Study.

Artif Organs 2018 Apr 12;42(4):386-393. Epub 2017 Dec 12.

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

The aim of this study was to describe the echocardiographic trend of left ventricular (LV) and right ventricular (RV) function after implantation of a pulsatile flow left ventricular assist device (LVAD) in children. From 2013 to 2016, we prospectively evaluated 13 consecutive pediatric Berlin Heart EXCOR LVAD patients. Clinical and echocardiographic data were collected at baseline, within 24 h after implantation and monthly until LVAD explant. Median age and weight at the implantation was 8 (4-23) months and 5 (4.6-8.3) kg at the time of implantation, respectively. All were affected by dilated cardiomyopathy. Average LVAD support time was 226.2 ± 121.2 days. Nine (70%) were transplanted, 4 (30%) died. LV end-systolic and end-diastolic volumes were reduced until the follow up of two months (P = 0.019 and P = 0.001). A progressive increase in RV dimensions was observed. After 4 months of follow up, RV fractional area change worsening was statistically related with the deterioration of LV unloading (P = 0.0036). Four patients needed prolonged inotropic support for RV failure. Pulsatile LVAD in pediatrics is followed by an early and mid-term LV unloading, as expressed by a decrease in LV volumes and diameters at echocardiogram. The effects of unloading do not remain stable at long term follow up. RV function improved in the acute phase, but a progressive dilatation of RV was noted over time. In some patients, RV failure might lead to the need of an increase of inotropic support at long term follow up.
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http://dx.doi.org/10.1111/aor.13050DOI Listing
April 2018

A current approach to heart failure in Duchenne muscular dystrophy.

Heart 2017 11 1;103(22):1770-1779. Epub 2017 Jul 1.

Department of Cardiovascular Medicine, Catholic University of the Sacred Heart, Rome, Italy.

Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by the long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually loose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Echocardiography and ECG are standardly used for screening and detection of cardiovascular abnormalities in these patients, although these tools are not always adequate to detect an early, clinically asymptomatic phases of disease progression. In this regard, cardiovascular magnetic resonance (CMR) with late gadolinium enhancement is emerging as a promising method for the detection of early cardiac involvement in patients with DMD. The early detection of cardiac dysfunction allows the therapeutic institution of various classes of drugs such as corticosteroids, beta-blockers, ACE inhibitors, antimineralocorticoid diuretics and novel pharmacological and surgical solutions in the multimodal and multidisciplinary care for this group of patients. This review will focus on these challenges and available options for HF in patients with DMD.
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http://dx.doi.org/10.1136/heartjnl-2017-311269DOI Listing
November 2017

Concomitant pulsatile and continuous flow VAD in biventricular and univentricular physiology: a comparison study with a numerical model.

Int J Artif Organs 2017 Mar 11;40(2):74-81. Epub 2017 Feb 11.

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

Introduction: To develop and test a lumped parameter model to simulate and compare the effects of the simultaneous use of continuous flow (CF) and pulsatile flow (PF) ventricular assist devices (VADs) to assist biventricular circulation vs. single ventricle circulation in pediatrics.

Methods: Baseline data of 5 patients with biventricular circulation eligible for LVAD and of 5 patients with Fontan physiology were retrospectively collected and used to simulate patient baselines. Then, for each patient the following simulations were performed: (a) CF VAD to assist the left ventricle (single ventricle) + a PF VAD to assist the right ventricle (cavo-pulmonary connection) (LCF + RPF); (b) PF VAD to assist the left ventricle (single ventricle) + a CF VAD to assist the right ventricle (cavo-pulmonary connection) (RCF + LPF).

Results: In biventricular circulation, the following results were found: cardiac output (17% RCF + LPF, 21% LCF + RPF), artero-ventricular coupling (-36% for the left ventricle and -21.6% for the right ventricle), pulsatility index (+6.4% RCF + LPF, p = 0.02; -8.5% LCF + RPF, p = 0.00009). Right (left) atrial pressure and right (left) ventricular volumes are decreased by the RCF + LPF (by RPF + LCF). Pulmonary arterial pressure decreases in the LCF + RPF configuration. In Fontan physiology: cardiac output (LCF + RPF 35% vs. 8% in RCF + LPF), ventricular preload (+4% RCF + LPF, -10% LCF + RPF), Fontan conduit pressure (-5% RCF + LPF, +7% LCF + RPF), artero-ventricular coupling (-14% RCF + LPF vs. -41% LCF + RPF) and pulsatility (+13% RCF + LPF, - 8% LCF + RPF).

Conclusions: A numerical model supports clinicians in defining and innovating the VAD implantation strategy to maximize the hemodynamic benefits. Results suggest that the hemodynamic benefits are maximized by the LCF + RPF configuration.
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http://dx.doi.org/10.5301/ijao.5000562DOI Listing
March 2017

The Use of Berlin Heart EXCOR VAD in Children Less than 10 kg: A Single Center Experience.

Front Physiol 2016 6;7:614. Epub 2016 Dec 6.

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

Despite the improvement in ventricular assist device (VAD) therapy in adults and in adolescents, in infant population only Berlin Heart EXCOR (BHE) is licensed as long term VAD to bridge children to Heart Transplantation (HTx). Particularly demanding in terms of morbidity and mortality are smallest patients namely the ones implanted in the first year of life or with a lower body surface area. This work aims at retrospective reviewing a single center experience in using BHE in children with a body weight under 10 kg. Data of all pediatric patients under 10 kg undergoing BHE implantation in our institution from March 2002 to March 2016 were retrospectively reviewed. Of the 30 patients enrolled in the study, 53% were male, 87% were affected by a dilated cardiomyopathy with an average weight and age at the implantation of 6.75 ± 2.16 Kg and 11.57 ± 10.12 months, respectively. Three patients (10%) required a BIVAD implantation. After the implantation, 7 patients (23%) required re-intervention for bleeding and 9 patients (30%) experienced BHE cannulas infection. A total of 56 BHE pump were changed for thrombus formation (1.86 BHE pump for patient). The average duration of VAD support was 132.8 ± 94.4 days. Twenty patients (67%) were successfully transplanted and 10 patients (33%) died: 7 for major neurological complication and 3 for sepsis. Mechanical support in smaller children with end stage heart failure is an effective strategy for bridging patients to HTx. The need for BIVAD was relegated, in the last years, only to restrictive cardiomiopathy. Further efforts are required in small infants to improve anticoagulation strategy to reduce neurological events and BHE pump changes.
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http://dx.doi.org/10.3389/fphys.2016.00614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5138210PMC
December 2016

Hybrid treatment: the left anterior descendant artery to the surgeon - all the rest to the interventional cardiologist.

J Cardiovasc Med (Hagerstown) 2017 01;18 Suppl 1:e141-e144

Cardiosurgery Unit, Policlinico Agostino Gemelli Foundation, Rome, Italy.

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http://dx.doi.org/10.2459/JCM.0000000000000458DOI Listing
January 2017

Left ventricular assist device as destination therapy in cardiac end-stage dystrophinopathies: Midterm results.

J Thorac Cardiovasc Surg 2017 03 28;153(3):669-674. Epub 2016 Aug 28.

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

Objective: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end-stage dystrophinopathies.

Methods: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2-23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach.

Results: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow-up time of 21.7 months (range, 3-45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months.

Conclusions: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end-stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options.
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http://dx.doi.org/10.1016/j.jtcvs.2016.08.016DOI Listing
March 2017

Mini-aortic surgery with percutaneous cannulation and rapid-deployment valve.

Asian Cardiovasc Thorac Ann 2016 Jul 9;24(6):535-40. Epub 2016 Jun 9.

Department of Cardiovascular Sciences, Catholic University, Rome, Italy.

Background: We aimed to evaluate the results of the combined use of rapid-deployment valves, percutaneous cardioplegia delivery and left heart venting during minimally invasive aortic valve replacement surgery.

Methods: We identified 2 propensity-matched cohorts of patients who underwent primary elective isolated minimally invasive aortic valve surgery at our center over a 3-years period: 30 patients in group A had a conventional valve prosthesis and 30 patients in group B received a rapid-deployment valve using percutaneous cardioplegia delivery and percutaneous left heart venting. Skin incision length, intraoperative times, postoperative hospital outcomes, and 30-day echocardiographic results were compared between the 2 groups.

Results: Patients in group B had significantly shorter operative times and shorter skin incisions compared to group A (total operative time 196.0 ± 40.6 vs. 225.1 ± 30.8 min, respectively, p < 0.003; cardiopulmonary bypass time 79.9 ± 10.6 vs. 92.9 ± 17.2 min respectively, p < 0.001; crossclamp time 52.3 ± 9.6 vs. 74.9 ± 10.2 min, respectively, p < 0.001; incision length 3.6 ± 0.5 vs. 6.0 ± 0.6 cm, respectively, p < 0.001). Postoperative hospital outcomes and echocardiographic evaluation showed no significant differences.

Conclusions: The combined use of rapid-deployment valves, percutaneous cardioplegia, and left heart venting is safe and effective and allows a significant reduction of the skin incision together with a significant reduction of intraoperative times without affecting hospital outcomes or hemodynamic performance of the prosthetic valves.
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http://dx.doi.org/10.1177/0218492316654774DOI Listing
July 2016

Repair of Posterior Infarct Ventricular Septal Defect in a Patient with Dextrocardia and Situs Inversus.

J Card Surg 2016 Mar 19;31(3):147-9. Epub 2016 Jan 19.

Cardiac Surgery Institute, Catholic University-A. Gemelli Hospital, Rome, Italy.

We report a patient with situs inversus who developed a large posterior interventricular septum pseudoaneurysm with a septal defect following a myocardial infarction. The ventricular septum was approached through the left ventricle and the entrance of the pseudoaneurysm was repaired with a strip of equine pericardium.
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http://dx.doi.org/10.1111/jocs.12691DOI Listing
March 2016

Mechanically Assisted Total Cavopulmonary Connection With an Axial Flow Pump: Computational and In Vivo Study.

Artif Organs 2016 Jan 14;40(1):43-9. Epub 2015 Dec 14.

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

A relevant number of patients undergoing total cavopulmonary connection (TCPC) experience heart failure (HF). Heart transplant is then the final option when all other treatments fail. The axial flow blood pumps are now the state of the art; however, there is little experience in low-pressure circuits, such as support of the right ventricle or even a TCPC circulation. A new T-shaped model of mechanically assisted TCPC using the "Jarvik Child 2000" axial pump, (flow rates between 1 and 3 L/m in a range of 5000-9000 rpm) was designed, simulated numerically, and then tested in animals. Eight sheep (42-45 kg) were studied: two pilot studies, four pump-supported (PS) TCPC for 3 h, and two not pump-supported (NPS) TCPC. In the PS, the axial pump was set to maintain the baseline cardiac output (CO). Pressures, CO, systemic and pulmonary vascular resistance, lactate levels, and blood gases were recorded for 3 h. Computational fluid dynamics (CFD) study allows us to set the feasible operating condition and the safety margins to minimize the venous collapse risk. In the NPS animals, a circulatory deterioration, with increasing lactate level, occurred rapidly. In the PS animals, there was a stable cardiac index of 2.7 ± 1.4 L/min/m(2), central venous pressure of 12.3 ± 1 mm Hg, and a mean pulmonary artery pressure (PAP) of 18.1 ± 6 after 3 h of support up to 9000 rpm. systemic vascular resistance (SVR), pulmonary vascular resistance (PVR), blood gasses, and arterial lactate levels remained stable to baseline values. No caval collapse occurred. A new pediatric axial flow pump provides normal CO and physiologic stability in a new T-shaped model of TCPC in sheep, in vivo. CFD and in vivo data showed that this experimental arrangement will allow us to evaluate the potential for mechanical support in patients with Fontan failure avoiding major adverse events.
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http://dx.doi.org/10.1111/aor.12641DOI Listing
January 2016

Outcome of Standard and Bicuspidalized Cryopreserved Homografts for Primary Right Ventricular Outflow Tract Reconstruction.

J Heart Valve Dis 2015 Jan;24(1):83-8

Background And Aim Of The Study: The study aim was to analyze the safety and longevity of cryopreserved homografts used for primary right ventricular outflow tract reconstruction (RVOTR), and to compare the outcome using either standard or bicuspidalized allografts.

Methods: Between February 2000 and September 2014, a total of 53 patients underwent primary RVOTR using either a standard (n = 40) or a bicuspidalized (n = 13) cryopreserved homograft. The median age at surgery was 15.5 months (range: 1-419.06 months), and bodyweight was 8.5 kg (range: 4.1-71 kg). The median standard homograft size was 17.5 mm (range: 10-25 mm), while the median bicuspidalized homograft size was 16 mm (range: 14-22 mm). Follow up was complete in 91.4% of patients, with a median duration of 30.11 months (range: 0.26-161.26 months). Data analysis included primary diagnosis, type of surgery, age at surgery, size of conduit, need for reintervention, and survival. Predetermined primary outcomes were represented by survival and freedom from conduit reintervention.

Results: Two patients with standard cryopreserved homograft died. during the early postoperative period (3.7%). No deaths were conduit-related. The five-year survival rate and ten-year freedom from reintervention were 91% (range: 74.7-97.2%) and 53.6% (range: 97-33.2%), respectively. RV-PA conduit replacement was performed in 14 patients (26.4%) at a median interval of 44.5 months (range: 14.93-162.46 months). Among these patients, four children (30.7%) received bicuspidalized homografts, and 10 (25%) received standard homografts. Causes of reintervention were conduit stenosis in six cases (43%), severe homograft valve regurgitation in two (14.2%), conduit stenosis and homograft valve regurgitation in two (14.2%), and stenosis of distal anastomosis involving pulmonary bifurcation in four (28.6%). Univariate analyses showed a longer freedom from reintervention for bicuspidalized compared to standard homograft (p = 0.03).

Conclusion: The results obtained suggested that bicuspidalized homograft performance compares well with that of standard allografts in terms of freedom from reintervention. Bicuspidalized homograft use is strongly indicated for primary RVOTR in small children, when a standard homograft of appropriate size is not available.
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January 2015

Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries.

J Card Surg 2015 May 23;30(5):453-8. Epub 2015 Mar 23.

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

Objective: We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) and comment on the treatment performed.

Methods: From 1994 until 2014, 118 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four patients (3.4%) developed severe airflow complications postoperatively. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days to 11 months).

Results: The first patient developed malacia and compression of the left bronchus from the distal RV-PA conduit and was treated with external bronchial stenting with two incomplete costal cartilage rings. The second patient developed recurrent esophagus-left bronchus fistula treated with multiple surgical esophageal and bronchus reconstructions. The third child presented with bilateral bronchial malacia treated with bilateral stenting followed by surgical elongation of the neo-left pulmonary artery to avoid external compression. The last patient developed bilateral bronchomalacia treated with bilateral bronchial stenting followed by RV-PA conduit replacement and endobronchial stenting calibration.

Conclusion: Particular categories of patients with PA, VSD, MAPCAs (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals) may be more predisposed to develop airway compromise. The treatment of the lesion should be individualized according to the pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in case of extrinsic compression.
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http://dx.doi.org/10.1111/jocs.12539DOI Listing
May 2015

Vacuum-assisted closure system in newborns after cardiac surgery.

J Card Surg 2015 Feb 3;30(2):190-3. Epub 2014 Nov 3.

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

Objective: To analyze the effectiveness and the results of the use of a vacuum-assisted closure (VAC) system for the treatment of complex sternal wounds in newborns after cardiac surgery.

Methods: From May 2008 until December 2012, six patients developed post-sternotomy wound problems (large defects of epithelialization or mediastinitis), which were treated with a VAC system. Median age at the time of institution of VAC was 24.5 days (range 16 to 65 days). Median time of treatment was 14 days (range 3 to 42 days).

Results: All patients were newborns and all underwent delayed sternal closure after cardiac surgery. The indications for using the VAC system were: mediastinitis in two patients (33.3%) and impairment of healing without signs of infection in four (66.7%). All children after VAC therapy achieved healing of the sternal wound. VAC therapy was started with high negative pressures (-125 mmHg) continuously then switched to an intermittent modality in all patients.

Conclusion: VAC system with high negative pressure is safe, effective, and is a well-tolerated therapy in newborns with complex sternal wounds.
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http://dx.doi.org/10.1111/jocs.12463DOI Listing
February 2015

"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Pediatr Cardiol 2014 Oct 1;35(7):1162-73. Epub 2014 Jun 1.

Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital IRCCS, P.zza S. Onofrio, 4-00165, Rome, Italy,

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers. The field of pediatric cardiology and cardiac surgery may represent a model of clinical governance of particular interest with regard to programming and to a definition of the quality standards that may be extended to highly specialized institutions and ideally to the entire Health System. The "Baby Heart Project," which represents a model of governance and clinical quality in the field of pediatric cardiology and cardiac surgery, was born from the spontaneous aggregation of a committee of experts, supported by duly appointed Italian Scientific Societies and guided by a national agency for accreditation. The ultimate aim is to standardize both procedures and results for future planning within the national Health System.
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http://dx.doi.org/10.1007/s00246-014-0910-xDOI Listing
October 2014