Cardio-respiratory and hepatic functions evaluation in single ventricle patients treated with total cavo-pulmonary connection operation

L'intervento di connessione cavo-polmonare totale (TCPC) nei pazienti portatori di cuore univentricolare, a causa della particolare condizione emodinamica, determina un risentimento a carico di numerosi parenchimi. Scopo della ricerca è di valutare l'entità di questo danno ad un follow-up medio-lungo. Sono stati arruolati 115 pazienti, sottoposti ad intervento presso i centri di Cardiochirurgia Pediatrica di Bologna (52 pz) e Torino (63 pz). Il follow-up medio è stato di 125±2 mesi. I pazienti sono stati sottoposti ad indagine emodinamica (88 pz), test cardiopolmonare (75 pz) e Fibroscan ed ecografia epatica (47 pz). La pressione polmonare media è stata di 11.5±2.6mmHg, ed in 12 pazienti i valori di pressione polmonare erano superiori a 15mmHg. La pressione atriale media era di 6.7±2.3mmHg ed il calcolo delle resistenze vascolari polmonari indicizzate (RVP) era in media di 2±0.99 UW/m2. In 29 pazienti le RVP erano superiori a 2 UW/m2. La VO2 max in media era pari a 28±31 ml/Kg/min, 58±15 % del valore teorico. La frequenza cardiaca massima all'apice dello sforzo era di 151±22 bpm, pari al 74±17% del valore teorico. Il Fibroscan ha fornito un valore medio di 17.01 kPa (8-34.3kPa). Cinque pazienti erano in classe F2, 9 pazienti in classe F3 e 33 pazienti risultavano in classe F4. Nei pazienti con follow-up maggiore di 10 anni il valore di stiffness epatica (19.6±5.2kPa) è risultato significativamente maggiore a quello dei pazienti con follow-up minore di 10 anni (15.1±5.8kPa, p<0.01). La frequenza cardiaca massima raggiunta durante lo sforzo del test cardiopolmonare è risultata significativamente correlata alla morfologia del ventricolo unico, risultando del 67.8±14.4% del valore teorico nei pazienti portatori di ventricolo destro contro il 79.6±8.7% dei portatori di ventricolo sinistro (p=0.006). L'intervento di TCPC determina un risentimento a carico di numerosi parenchimi proporzionale alla lunghezza del follow-up, e necessita pertanto un costante monitoraggio clinico-strumentale multidisciplinare.Single ventricle patients are treated with total cavo-pulmonary connection (TCPC) operation that, for the particular hemodynamic condition, can cause a multiorgan damage. Aim of the research is to evaluate the amount of this damage at a medium-long term follow-up. One hundred fifteen patients, operated in the Pediatric Cardiac Surgery Department of Bologna (52 pts) and Torino (63 pts), were enrolled. Mean follow-up was 125±2 months. Patients underwent hemodynamic evaluation (88 pts), cardiopulmonary exercise testing (CPET) (75 pts) and Fibroscan and hepatic echography (47 pts). Mean pulmonary artery pressure (PAP) was 11.5±2.6mmHg, and in 12 patients was more than 15mmHg. Mean atrial pressure was 6.7±2.3 mmHg and mean pulmonary vascular resistances (PVR) was 2±0.99 WU/m2. In 29 patients, PVR were more than 2 WU/m2. Mean peak VO2 was 28±31 ml/Kg/min, 58±15 % of the theoretic value. Mean peak heart rate during exercise was 151±22 bpm, 74±17% of the theoretic value. Mean hepatic stiffness evaluation at Fibroscan was 17.01 kPa (8-34.3 kPa). Five patients were in Metavir class F2, 9 in F3 e 33 in F4 class. Among patients with a longer than 10 years follow-up, hepatic stiffness evaluation was significantly higher (19.6±5.2 kPa) than in patients with shorter follow-up (15.1±5.8 kPa, p<0.01). Peak heart rate at CPET was significantly related to single ventricle morphology, resulting 67.8±14.4% of the theoretic value in right single ventricle patients and 79.6±8.7% in left single ventricle patients (p=0.006). TCPC operation cause a multiorgan damage proportional to follow-up length, and for this reason deserve a constant multidisciplinary clinico-instrumental monitoring

Ross-Kabbani Operation in an Infant with Mitral Valve Dysplasia

Background. Mitral valve replacement can be very difficult to obtain in infants because the valve annulus diameter can be smaller than the available prosthesis. Case Report. We describe the case of a 2-month-old female weighing 3.5 kg affected by mitral valve dysplasia leading to severe valve stenosis. Despite full medication, the clinical conditions were critical and surgery was undertaken. The mitral valve was unsuitable for repair and the orifice of mitral anulus was 12 mm, too small for a mechanical prosthesis. Therefore, a Ross-Kabbani operation was undertaken, replacing the mitral valve with the pulmonary autograft and reconstructing the right ventricular outflow tract with an etherograft. Results. The postoperative course was uneventful and the clinical conditions are good at 4-month follow-up. Conclusion. The Ross-Kabbani operation can be an interesting alternative to mitral valve replacement in infants when valve repair is not achievable and there is little space for an intra-annular mechanical prosthesis implant

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS)

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (≤19 years of age) performed from 1 January 2000 to 1 July 2019 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-nine hospitals contributed 398 registered implants in 353 patients (150 female, 203 male) to the registry. The most frequent aetiology of heart failure was any form of cardiomyopathy (61%), followed by congenital heart disease and myocarditis (16.4% and 16.1%, respectively). Competing outcomes analysis revealed that a total of 80% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 20% died while on support. At 12 months, 46.7% received transplants, 8.7% were weaned from their device and 18.5% died. The 3-month adverse events rate was 1.69 per patient-year for device malfunction including pump exchange, 0.48 for major bleeding, 0.64 for major infection and 0.78 for neurological events. CONCLUSIONS: The overall survival rate was 81.5% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age) (P = 0.01) and lower weight (<20 kg) (P = 0.015). Transplant rates at 6 months contin

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe

[Background] The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection.[Methods] This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included.[Results] A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8–12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died.[Conclusions] Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.This research was partially supported by the Institute of Health Carlos III, Proyectos de Investigacion en Salud (ISCIII PI17/01409) financed by European Development Regional Fund ‘A way to achieve Europe’, Operative program Intelligent Growth 2014-2020.Peer reviewe

Pulmonary artery banding

Summary Pulmonary artery banding (PAB) is a simple surgical technique to reduce pulmonary overcirculation in some congenital heart disease. In the beginning, when the use of cardiopulmonary bypass was affected by many deleterious effects, this technique played a fundamental role in the treatment of patients with congenital heart defects and an intracardiac left-to-right shunt. The use of PAB has decreased during the last two decades, due to the increasing popularity of early complete intracardiac repair, which results have shown to be superior to staged repair, even in low body weight patients. Moreover, several authors have emphasized the negative effects of PAB such as pulmonary arterial branch distortion, abnormal right ventricular hypertrophy, pulmonary valve insufficiency, sub-aortic obstruction and decreased ventricular compliance in patients with univentricular heart. For all these reasons, this procedure has been placed in the dark corner of surgery, representing, between 2002 and 2005, ~2% of the total amount of cardiac surgery procedures. In a more recent era, PAB has been performed in instances other than classic univentricular heart, as palliation in small infants with cardiac defects with a left-to-right shunt and pulmonary overcirculation, thus gaining some time prior to a planned staged repair. Recently, the role of PAB is becoming more important in selected subsets of congenital cardiac defects: L-transposition of the great arteries, D-transposition of the great arteries, hypoplastic left heart syndrome, moderately hypoplastic left ventricle (congenitally corrected transposition of the great arteries). This renewed interest in the banding procedure is spurring all surgeons and cardiologists to find new solutions for an easier banding procedure while making debanding less traumatic

Aortopulmonary Window and Anomalous Coronary Artery: An Exceptional Association

Aortopulmonary window is rarely associated with other cardiac anomalies, such as anomalous origin of one coronary artery. This exceptional association has been reported in 15 patients, with different surgical solutions. We describe 2 patients with aortopulmonary window with anomalous origin of the right coronary artery, one of which presented with tetralogy of Fallot-pulmonary atresia. A neonatal modified Blalock-Taussig shunt and ligature of the distal pulmonary artery at the level of pulmonary bifurcation was performed in the first patient, and the second patient underwent aortopulmonary window division with anomalous coronary artery reimplantation. Both patients had an uneventful postoperative cours