GUCH POPULATION: CARDIOLOGIC AND SURGICAL DEMANDS

To evaluate the cardiologic and surgical demands of the GUCH population followed up in our Department, a retrospective analysis of 709 hospital admissions (HA) of 536 pts (mean age 34?16, range 16-79 years), carried out from 1997 to 2002, has been performed. In 293 pts the basic cardiopathy was simple (SC), in 158 of moderate complexity (MCC) and in 85 of great complexity (GCC). The reason for 169 HA was cardiac surgery and for 537 HA cardiologic issues. The overall HA/pt was 1.3 (1.1 in SC,1.3 in MCC, 2.1 in GCC). In 166 surgical pts, 171 operations were performed. First operations (n=105, 62%) were most commonly for atrial septal defect (n=50, 48%), aortic valve replacement(n=11,10%), ventricular septal defect (n=8, 8%). Reoperations (n=64, 38%) were divided among reintervention after corrective repair (n=45, 70%), further palliation (n=17, 26%), first palliation (n=2, 4%) and the most frequent were: Fontan operation (n=11, 17%), RV-PA conduit replacement (n=8, 12%), aortic valve replacement (n=6, 9%). The mean hospital stay was 19?17 days. Pleuro-pericardial effusion (n=32,18%), bleeding (n=11, 6%) and arrhythmias(n=11, 6%) were the most prevalent complications. Early mortality (n=7/171, 4%) was influenced by cyanosis (6 of 33,18% in cyanotic pts; 1 of 136, 2% in acyanotic, p<0,001) and by complexity of cardiopathy (n=6 of 43 GCC, 14%; n=1 of 72 MCC, 1%; 0 of 54 SC,0%, p<0.001) that was a risk factor also for more serious complications (20% in GCC, with respect of 9% in MCC, p<0,05 and 4% in SC, p<0.001). The principal reasons for 537 cardiologic HA in 432 pts, were: interventional procedures (n=209, 39%), diagnostic catheterisation (n=155, 29%), non invasive evaluation (n=95, 18%), arrhythmias (n=46, 10%). Closure of atrial septal discontinuity (n=167, 80%), patent ductus arteriosus (n=21, 10%), pulmonary valvuloplasty (n=8, 4%) and angioplasty of Coarctation (n=6, 3%), were more prevalent interventional procedures. In pts with GCC, heart failure and cyanosis were more commonly cause of HA than in MGC and SC (p<0.001). Despite the progress of pediatric cardiology and cardiac surgery, medical demands of GUCH remain numerous and complex throughout their lives. Therefore, it is extremely important to provide specialized surgical and cardiologic care for this complicated population

Cumulative patient effective dose and acute radiation-induced chromosomal DNA damage in children with congenital heart disease

Background The seventh Committee on "Biological Effects of Ionizing Radiation" (BEIR VII, 2006) underlines "the need of studies of infants who are exposed to diagnostic radiation because catheters have been placed in their hearts". Objective To determine the lifetime attributable risk (LAR) of cancer associated with the estimated cumulative radiological dose in 59 children (42 male, age 2.863.2 years) with complex congenital heart disease, and to assess chromosomal DNA damage after cardiac catheterisation procedures. Methods In all patients, the cumulative exposure was estimated as effective dose in milliSievert (mSv), and LAR cancer was determined from the BEIR VII report. In a subset of 18 patients (13 male, age 5.265.7 years) micronucleus as a biomarker of DNA damage and longterm risk predictor of cancer was assayed before and 2 h after catheterisation procedures. Doseearea product (Gy cm2) was assessed as a measure of patient dose. Results The median life time cumulative effective dose was 7.7 mSv per patient (range 4.6e41.2). Cardiac catheterisation procedures and CT were responsible for 95% of the total effective dose. For a 1-year-old child, the LAR cancer was 1 in 382 (25th to 75th centiles: 1 in 531 to 1 in 187) and 1 in 156 (25th to 75th centiles: 1 in 239 to 1 in 83) for male and female patients, respectively. Median micronucleus values increased significantly after the procedure in comparison with baseline (before 6&vs after 9&, p?0.02). The median doseearea product value was 20 Gy cm2 (range 1e277). Conclusion Children with congenital heart disease are exposed to a significant cumulative dose. Indirect cancer risk estimations and direct DNA data both emphasise the need for strict radiation dose optimisation in children

Echocardiographic nomograms for chamber diameters and areas in Caucasian children

BACKGROUND: Although a quantitative evaluation of cardiac chamber dimensions in pediatric echocardiography is often important, nomograms for these structures are limited. The aim of this study was to establish reliable echocardiographic nomograms of cardiac chamber diameters and areas in a wide population of children. METHODS: A total of 1,091 Caucasian Italian healthy children (age range, 0 days to 17 years; 44.8% female) with body surface areas (BSAs) ranging from 0.12 to 1.8 m(2) were prospectively enrolled. Twenty-two two-dimensional and M-mode measurements of atrial and ventricular chamber diameters and areas were performed. Models using linear, logarithmic, exponential, and square-root relationships were tested. Heteroscedasticity was tested by the White test and the Breusch-Pagan test. Age, weight, height, and BSA, calculated by the Haycock formula, were used as the independent variables in different analyses to predict the mean value of each echocardiographic measurement. The influence of various confounders, including gender, type of delivery, prematurity, and interobserver variability, was also evaluated. Structured Z scores were then computed. RESULTS: The Haycock formula provided the best fit and was used when presenting data as predicted values (mean ? 2 SDs) for a given BSA and within equations relating echocardiographic measurements to BSA. Confounders were not included in the final models, because they did not show significant effects for most of the measurements. CONCLUSIONS: Echocardiographic reference values are presented for chamber area and diameters, derived from a large population of healthy children. These data partly cover a gap in actual pediatric echocardiographic nomograms. Further studies are required to reinforce these data, as well as to evaluate other parameters and ethnicities

Colorectal Cancer Stage at Diagnosis Before vs During the COVID-19 Pandemic in Italy

IMPORTANCE Delays in screening programs and the reluctance of patients to seek medical attention because of the outbreak of SARS-CoV-2 could be associated with the risk of more advanced colorectal cancers at diagnosis. OBJECTIVE To evaluate whether the SARS-CoV-2 pandemic was associated with more advanced oncologic stage and change in clinical presentation for patients with colorectal cancer. DESIGN, SETTING, AND PARTICIPANTS This retrospective, multicenter cohort study included all 17 938 adult patients who underwent surgery for colorectal cancer from March 1, 2020, to December 31, 2021 (pandemic period), and from January 1, 2018, to February 29, 2020 (prepandemic period), in 81 participating centers in Italy, including tertiary centers and community hospitals. Follow-up was 30 days from surgery. EXPOSURES Any type of surgical procedure for colorectal cancer, including explorative surgery, palliative procedures, and atypical or segmental resections. MAIN OUTCOMES AND MEASURES The primary outcome was advanced stage of colorectal cancer at diagnosis. Secondary outcomes were distant metastasis, T4 stage, aggressive biology (defined as cancer with at least 1 of the following characteristics: signet ring cells, mucinous tumor, budding, lymphovascular invasion, perineural invasion, and lymphangitis), stenotic lesion, emergency surgery, and palliative surgery. The independent association between the pandemic period and the outcomes was assessed using multivariate random-effects logistic regression, with hospital as the cluster variable. RESULTS A total of 17 938 patients (10 007 men [55.8%]; mean [SD] age, 70.6 [12.2] years) underwent surgery for colorectal cancer: 7796 (43.5%) during the pandemic period and 10 142 (56.5%) during the prepandemic period. Logistic regression indicated that the pandemic period was significantly associated with an increased rate of advanced-stage colorectal cancer (odds ratio [OR], 1.07; 95%CI, 1.01-1.13; P = .03), aggressive biology (OR, 1.32; 95%CI, 1.15-1.53; P &lt; .001), and stenotic lesions (OR, 1.15; 95%CI, 1.01-1.31; P = .03). CONCLUSIONS AND RELEVANCE This cohort study suggests a significant association between the SARS-CoV-2 pandemic and the risk of a more advanced oncologic stage at diagnosis among patients undergoing surgery for colorectal cancer and might indicate a potential reduction of survival for these patients

Prevalenza, mortalit? e letalit? dei difetti cardiaci congeniti in Toscana, 1992-2009

,Introduzione: I difetti cardiaci congeniti sono le anomalie pi? comuni alla nascita e rappresentano la principale causa di mortalit? neonatale e di mortalit? e morbilit? infantile. Le cardiopatie congenite rappresentano un\u27area critica di sanit? pubblica in tutto il mondo oltre ad avere un rilevante impatto socio-economico. Obiettivo: stimare la prevalenza, la mortalit? e la letalit? nei nati con difetti cardiaci congeniti e analizzarne l\u27andamento nel tempo confrontando due coorti di nati, secondo il grado di gravit? dell\u27anomalia. Metodi: dal Registro Toscano Difetti Congeniti (RTDC) sono stati estratti i casi con difetto cardiaco congenito registrati tra i nati residenti in Toscana nel periodo 1992-2009. RTDC partecipa dal 1979 al sistema europeo di sorveglianza delle anomalie congenite EUROCAT. Sono stati considerati i difetti cardiaci congeniti totali e escludendo quelli associati ad anomalia cromosomica, classificati in tre classi di gravit? decrescente (SI, SII, SIII), registrati nel 1992-2000 e 2001-2009. La prevalenza ? stata calcolata in nati vivi, morti fetali (nati morti e aborti spontanei > 20a settimana di gestazione), interruzioni di gravidanza per anomalia fetale a qualsiasi et? gestazionale, e nelle cardiopatie diagnosticate in epoca prenatale. Sono state calcolate la mortalit? perinatale, neonatale, infantile e la letalit? neonatale precoce, neonatale e infantile. Risultati: sono stati registrati 3.653 casi con difetti cardiaci congeniti su 488.830 nati, il 95% dei quali non cromosomici. La prevalenza media totale ? stata di 7,47 per 1.000 nati. La prevalenza totale ? diminuita in modo statisticamente significativo, sia per tutti i difetti cardiaci congeniti (Rapporto tra prevalenze (RP)=0,86; IC 95% 0,80-0,91), sia per i non cromosomici (RP=0,86; IC 95% 0,80-0,92), mentre la prevalenza totale delle cardiopatie pi? gravi (classi SI e SII), ? rimasta stabile intorno a 1,56 per 1.000 nati. In questo gruppo ? stato osservato il maggiore aumento nelle interruzioni di gravidanza (RP=3,47; IC 95% 2,08-6,08) e nei casi diagnosticati in epoca prenatale (RP=2,71; IC 95% 2,03-3,67). Tutti i tassi di mortalit? sono diminuiti significativamente nel tempo. La riduzione maggiore ? stata osservata tra le cardiopatie complesse e per la mortalit? neonatale (RP=0,29; IC 95%: 0,16-0,49) rispetto alla mortalit? perinatale (RP=0,37 IC95%:0,19-0,70) e infantile (RP=0,40; IC 95% 0,28-0,56). Tra i difetti cardiaci pi? gravi, la letalit? neonatale precoce si ? ridotta dal 10% al 3%, la neonatale dal 17% al 6%, la infantile dal 24% al 10%. Conclusioni: in base ai risultati ottenuti ? ragionevole ritenere che in Toscana la migliore efficacia della diagnosi prenatale, dei trattamenti e dei processi di cura del feto e del neonato, abbia ridotto l\u27impatto dei difetti cardiaci congeniti sulla mortalit? precoce sullo stato di salute della popolazione. I dati, non presenti in altre regioni, possono essere utili per confronti degli stessi indicatori nel tempo e in altre aree

Un tredicenne con “asma grave” e una curva flusso-volume caratteristica

L’arco aortico destroposto rappresenta una anomalia congenita rara dell’aorta che, nel 50% dei casi, si associa alla presenza di un’arteria succlavia sinistra aberrante che origina da un aneurisma dell’aorta discendente, noto come diverticolo di Kommerell (KD), dando vita a un anello vascolare incompleto che può provocare una compressione della trachea e dell’esofago, con conseguente comparsa di tosse, dispnea e/o disfagia. Ancor più raramente il KD non si associa all’arteria succlavia aberrante e può costituire un rilievo casuale nel corso di accertamenti eseguiti per altri motivi. Tuttavia, la presenza di un KD è una condizione da non sottovalutare a causa dell’alta mortalità legata alla sua dissezione o rottura in età adulta. Descriviamo il caso di un tredicenne con arco aortico destroposto e KD isolato, con morfologia della curva flusso- volume patognomonica, trattato per molti anni per asma grave

Recommendations from the Association of European Paediatric Cardiology for training in diagnostic and interventional cardiac catheterisation

AbstractCardiac catheterisation in congenital heart disease is a developing field. Patients' ages range from foetus to adulthood. This document is a revision and update of the previously published recommendations and summarises the requirements for training in diagnostic and interventional cardiac catheterisation

Italian patent foramen ovale survey (I.P.O.S.): Early results

Summary Background Percutaneous patent foramen ovale (PFO) closure is gaining wide acceptance. Aims of the study were to analyse clinical practice regarding PFO closure in Italy, to study indications, devices, results, and the follow-up of large series of patients treated by percutaneous PFO closure. Methods and patients Italian patent foramen ovale survey (IPOS) is a prospective, observational, multi-centric survey that uses a web-based database. The survey lasted 12 months, (November 2007–October 2008). 50 centres participated. Ongoing follow-up will continue up to 36 months. 1035 patients (m.a. 46 years, 60% females) were included in the registry. Most subjects were treated due to a previous history of TIA/ischemic stroke (∼80% of patients). PFO diagnosis and right-to-left shunt (RLS) were assessed by contrast-enhanced transesophageal (cTEE) and/or transthoracic echocardiography and/or transcranial doppler. An aneurysm of the interatrial septum was associated in 41% of patients. Intraprocedural monitoring was assessed by using cTEE and fluoroscopy in 70% and intracardiac echocardiography in 30% of subjects. Procedures were performed under general anesthesia and local anesthesia/conscious sedation in 54% and 46% of patients respectively. The most used device for PFO closure was Amplatzer (∼70% of cases). Results The procedure was successful in all patients. Early complications occurred in 24/1035 patients (2.3%): 12/24 (50%) of them had cardiac arrhythmias, 1 subject had a TIA. Data regarding both clinical and cardio-neurosonological follow-up were assessed in 444/1035 (43%) subjects. The rate of neurological events and cardiac and extra-cardiac complications were around 3% and 9% up to the 24-month follow-up respectively. A large permanent residual RLS and no RLS were observed in less than 1% and in ∼82% of patients at the 1-year follow-up, respectively. Conclusions Our data confirm that percutaneous PFO closure is a safe procedure. Early complications and those during follow-up are mostly related to arrhythmias. Longer follow-up is under way