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

Do not throw away anything from the pig.

Among the surgical sciences, paediatric cardiac surgery is one of the closest to the concept of reconstruction. Despite the common use of the verb \u2018to recycle\u2019 in the third millennium, this notion cannot always be applied to our field and a multitude of materials, patches and conduits have to be used, often leading to rejection, aneurysm formation, calcification and stenosis. Moreover, our population is very particular for the potential of growing inherent to the age of patients. For these reasons we are forced to use materials that can give the best results in short-term follow-up but that cannot remain effective in the longer term. The opportunity to use living materials, growing patches or valves, opens up exciting possibilities\u2014opens up incredible possibilities for our patients. The extracellular matrix (ECM) is the naturally-occurring bioscaffold that surrounds cells in almost all tissues and organ structures. It is an acellular biomaterial that is gradually remodelled, leaving behind organized and healthy tissue. When implanted as a patch, the ECM acts as a scaffold into which the patient's cells migrate and integrate until it is gradually replaced. ECMs originating from various organs have been tested and good results reported. Among the types available, the CorMatrix\u2122 patch (CorMatrix\uae, Alpharetta, Georgia, USA), which is derived from porcine small intestinal submucosa extracellular matrix (SIS-ECM), is one of the most promising options among those commercially available. In this issue of the Journal [1], Witt and colleagues report their experience in the use of the SIS-ECM CorMatrix patch in 37 paediatric patients. Their patient population was divided into four groups, depending on the implant location: septal defect patching, vascular patching, outflow tract patching or valve reconstruction. There were four deaths (10.8%) at a follow-up time of 411 \ub1 225 days (range 62\u2013757 days), with no death attributed to the implanted SIS-ECM, even though, in one patient with coronary ostial stenosis after supravalvular aortic patch enlargement, the suspicion of a relationship is legitimate. The best results were reported in the septal closure group. Unfortunately, a septal closure can be accomplished perfectly with many other cheaper materials and the potential of SIS-ECM is probably greatly reduced in this situation. In adult patients, where the extent of the patch is bigger, SIS-ECM is probably justified and could provide better results in terms of the possibility of restoring suitable cardiac muscle. In pulmonary or aortic enlargement plasty, the growth potential of SIS-ECM is interesting. In Witt's experience [1], vascular patching accounted for more than 50% of SIS-ECM patch applications (26 locations), with just one re-operation for pulmonary artery residual stenosis. No aneurysm formation was reported when used for aortoplasty [1]. This promising experience confirms that of others. Indeed, Padalino implanted SIS-ECM patch on the abdominal aorta in 15 rats [2]. Graft re-population was demonstrated as early as 15 days after implantation, while it was almost completely remodelled 180 days after implantation. No significant graft aneurysmatic dilation or detachment was present. The new aortic wall presented with an intima incorporating an endothelial lining, a media with smooth muscle cells and an adventitia containing vessels and fibroblasts [2]. The same encouraging results were not obtained in the clinical experience by McCready, who reported seven episodes of patch aneurysm among 76 patients who had undergone patch angioplasty of the carotid artery following endoarterectomy [3]. The patch used in this latter study was another SIS-ECM product (Surgisis\uae by Cook Surgical, Bloomington, IN, USA) that was thinner than CorMatrix. However, aneurysmal dilation of the graft patch has been described when the CorMatrix was used in carotid patch angioplasty. As regards right ventricular outflow tract (RVOT) reconstruction, Witt reported on six patients with one re-operation. In three patients, a unicusp SIS-ECM valve was created but, unfortunately, one patient died from low cardiac output and the other two showed moderate-to-severe valve incompetence. School reported four unicusp valves implanted in pulmonary outflow tract reconstruction with three of them competent at an average follow-up of 9 months: too short to be of significance [4]. Quarti reported 26 cases treated with SIS-ECM patch reconstruction, nine of which were cusp extension valvular repairs: five aortic, two tricuspid, one mitral and one pulmonary valve. At a mean follow-up of 12.5 months no patients had undergone re-operation and no more than mild incompetence was evident [5]. A large variability of results is evident among all the reported experiences. An interesting experimental study by Tottey confirms that differences exist in the composition, structure and mechanical properties of SIS-ECM prepared from tissues harvested from animals of different ages. In their conclusion, SIS-ECMs harvested from pigs aged 12 weeks are suitable for withstanding substantial mechanical loading after in vivo implantation and remodelling into load-bearing or force-generating tissues, while animals aged >52 weeks will yield scaffolds that may persist longer after in vivo implantation [6]. The interactions between the CorMatrix patch and the surrounding tissues can also be affected by the implantation techniques, the suture materials and other factors, potentially explaining the extreme variability of the results. In our experience in Bologna, the SIS-ECM CorMatrix patch was used in 19 locations on 16 patients (septal patch in 6; pulmonary artery patch enlargement in 5; valve reconstruction in 5 [3 aortic, 1 mitral and 1 tricuspid]; Senning procedure as part of double switch operation in 2 and aortic arch reconstruction in 1). All patients are alive at a mean follow-up of 15 months. One patient underwent re-operation for recurrent incompetence 24 months after repair of a dysplastic aortic valve. The explanted patch appeared pliable and without any calcification. Our impression is that the SIS-ECM CorMatrix patch is very useful in pulmonary artery enlargement and valve cusp extension by reason of its pliability and thinness, whilst it is probably not necessary for septal closure. Since clinical studies so far have limited follow-up, it is not possible to confirm the ability of the patch to regenerate normal tissue in anatomical and functional terms, although this has been demonstrated in experimental studies. However, the absence of calcific degeneration is encouraging and sufficiently important to recommend its use. If the material proves capable of allowing for growth, previously unimagined possibilities will open up, forever solving all the problems related to the use of non-living materials