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A significant risk of maternal and fetal morbidity and mortality has been shown to be associated with congenital heart disease or heart disease occurring during pregnancy. Given the increasing number of patients with corrected congenital heart disease who reach fertile age and the more and more common advanced maternal age associated with preexisting or intercurrent comorbidities, a higher incidence of cardiac complications in pregnancy has been reported in the last decades. Improvement in maternal and neonatal outcomes is influenced by a multidisciplinary strategy. The purpose of this review is to assess the role of the Pregnancy Heart Team which should ensure careful pre-pregnancy counseling, pregnancy monitoring, and delivery planning for both congenital heart disease and other cardiac or metabolic disorders.

Over recent years, thanks to remarkable advances in pediatric cardiology, cardiac surgery and catheter interventions, survival of children with congenital heart disease has significantly increased with the majority of patients surviving into adulthood. Therefore, the prevalence of adult patients with congenital heart disease has dramatically increased, as well as the need for specific and dedicated programs. Acute heart failure, infective endocarditis and arrhythmias represent the most common causes of visit in the emergency department in this population. Our task force aimed at guiding physicians taking care of this peculiar cohort of patients in the emergency department.

Dabigatran is a novel target specific oral anticoagulant for stroke prevention in non valvular atrial fibrillation. Little is still known about its real-world effectiveness and safety in the italian population. Aim of our study was to evaluate the efficacy and safety of dabigatran in a large single-center cohort of "real-life" italian population with non-valvular AF and to compare the results with those obtained from the RE-LY trial and the Medicare study.We studied a prospective cohort of 2108 patients (1119 male; mean age 69.4 ± 9.4 years) who started the oral anticoagulant treatment with dabigatran 110 mg twice-daily (DAB 110; N = 1075; 51%) or 150 mg twice-daily (DAB 150; N = 1033; 49%). Follow-up data were obtained trough outpatients visits each 3-6 months for assessing the clinical status, adherence to treatment, occurrence of side effects and major cardiovascular complications.In DAB 150 group the mean age was 64.9 ± 8.8 years, 56.8% of patients was male. CHA2DS2Vasc Score was ≥ 3 in 94.3% and HAS-BLED was ≥ 3 in 59.7%. In DAB 110 group (N = 1075) the mean age was 73.9 ± 7.5 years; 49.5% of patients was male. CHA2DS2Vasc Score was ≥ 3 in 73.4% and HAS-BLED was ≥ 3 in 87.4% of DAB 110 patients. One patient taking Dabigatran 110 mg bid had ischemic stroke without significantly neurological sequelae. In both groups, no patient experienced hemorrhagic stroke during the follow-up period. 147 patients (6.9%) of MonaldiCare population reported adverse effects from treatment with dabigatran, of whom 121 patients (5.7%) discontinued therapy. We reported one case of subarachnoid hemorrhage (0.05%) in a patient with high thrombo-embolic and high hemorrhagic risk score who was taking dabigatran 150 mg bid and one case (0.05%) of bladder bleeding in a patient who was taking dabigatran 110 mg bid. No major gastrointestinal bleeding was observed in the MonaldiCare population.MonaldiCare registry showed a safety profile of both dosages of dabigatran regarding major of fatal bleeding in a "real life" single center italian population at high thromboembolic and hemorrhagic risk. The majority of MonaldiCare patients tolerated dabigatran treatment without significant side effects. The efficacy of dabigatran was demonstrated by very low prevalence of ictus/TIA, also when patients underwent electrical AF cardioversion independently of the transesophageal examination.

Background: Implantable cardioverter defibrillators (ICD) are widely accepted therapy in children and adolescents who are survivors of cardiac arrest or for high-risk patients with inheritable channelopathies, cardiomyopathies, or congenital heart disease. Initial experience with subcutaneous ICD (S-ICD) systems has shown a high efficacy in adults. However, the use of S-ICD in children and adolescents implies some specific considerations, as the safety for these patients is unknown and recommendations among physicians may vary widely. Methods: We reviewed the data and studied the indications for S-ICD in children and adolescents and discuss the preliminary clinical experience. Results: From a cohort of 297 patients enrolled in the S-ICD “Monaldi care” registry that encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 21 consecutive children and adolescents (mean age 13.9 years, range 8-18 years, mean body weight 59.3 kg, range 38-100 kg) who underwent S-ICD implant from April 2014 to June 2020. Mean follow-up was 41.9±21.9 months. Only one patient presented, 6 weeks after implantation, skin erosion at the inferior parasternal incision that resolved after antibiotic therapy, without the necessity of any system revision. Two patients experienced appropriate shocks and four inappropriate shocks, due to T wave oversensing or atrial arrhythmia. Only one patient, with arrhythmogenic right ventricular dysplasia, required a system revision after 36 months of the first implantation and then a reintervention with a replacement of the S-ICD by a conventional ICD system. Conclusions: Our experience suggests that the S-ICD device can be used in some children over the age of 8 as well as adults, with a similar rate of unwanted side effects, and early evidence of apparent efficacy.

DiGeorge syndrome (DGS), also known as "22q11.2 deletion syndrome" (22q11DS) (MIM # 192430 # 188400), is a genetic disorder caused by hemizygous microdeletion of the long arm of chromosome 22. In the last decades, the introduction of fluorescence in situ hybridization assays, and in selected cases the use of multiplex ligation-dependent probe amplification, has allowed the detection of chromosomal microdeletions that could not be previously identified using standard karyotype analysis. The aim of this review is to address cardiovascular and systemic involvement in children with DGS, provide genotype-phenotype correlations, and discuss their medical management and therapeutic options.

A 3-months-old infant was urgently admitted for drowsiness and lack of appetite started 24 h before. The ECG showed sinus rhythm with a prolonged AV interval (200 ms) and very large QRS complexes (280 ms) due to Flecainide overdosing following incorrectly administration for poor communication between parents resulted in both giving a dose to the infant. Flecainide serum level was 1.2μg/ml, confirming the diagnosis of an accidental drug intoxication. The patient started continue hydration with a close monitoring. Three hours later a significant narrowing of the QRS complex (150 ms) was observed, then over the following 24 h, the QRS almost completely normalized.

Coarctation of the aorta is a leading cause of morbidity and mortality among adults with congenital heart disease (ACHD). Lifelong surveillance is mandatory to screen for possible long-term cardiovascular events.Left ventricular systolic dysfunction has been reported in association with recoarctation, and association with dilated cardiomyopathy (DCMP) is very rare. Herein, we report the case of a 19-year-old boy with coarctation of the aorta who complained of mild exertional dyspnea. Cardiac magnetic resonance revealed a moderately dilated, hypokinetic left ventricle (LV), with mildly reduced EF (45%), and residual isthmic coarctation was excluded. Genetic tests revealed a heterozygous missense variant in TNNT2 (NM_001001430.2): c.518G>A (p. Arg173Gln). This case highlights the role of careful history taking: a family history of cardiomyopathy should not be overlooked even when the clinical setting seems to suggest a predisposition to hemodynamic factors for LVSD.

The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the USA alone the number is estimated to exceed 1 million. In this population, many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counselling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge. This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.

Transvenous pacemaker (PM) catheters can be unintentionally placed in the left ventricle (LV) during the implantation procedure. An 8-year-old girl was discovered with a malpositioned pm wire, seven years after the implant. Trans-thoracic echocardiogram revealed the lead traversing the inter-atrial septum, crossing the mitral valve and embedded in the basal lateral wall of the LV. This is a report of a 14-year long follow-up after the surgical extraction of the malpositioned PM lead. come easier. Although nowadays a transvenous lead can be placed in children, there is a greater risk of complication in small patients [3]. One extremely rare complication associated with transvenous PM implantation is the inadvertent positioning of pacing lead in the left ventricle [4]. The greater risks depend on the delay of diagnosis of lead malpositions. This report concerns a case of long standing malpositioned PM wire in a paediatric patient and the successive long term follow up after the successful surgical extraction.

Background: The subcutaneous implantable cardioverter defibrillator (S-ICD) is a relatively novel alternative to the transvenous ICD for the treatment of life-threatening ventricular arrhythmias, and is currently used in the clinical practice of several centers. The aim of this analysis was to describe current Italian practice regarding S-ICD implantation and its evolution over the years. Methods: We analyzed 607 consecutive patients (78% male, 48 ± 16 years) who underwent S-ICD implantation in 39 Italian centers from 2013 to 2017. Results: Structural cardiomyopathy was present in 78% of patients and 30% of patients received their device for secondary prevention. The proportion of patients with dilated cardiomyopathy and with left ventricular ejection fraction ≤35% increased from ≤2014 to 2017 (from 38% to 58%, from 33% to 53%, respectively; both p < 0.05). Almost all procedures (97%) were performed in electrophysiology laboratories. Over the last 4 years, the 2-incision implantation technique has been widely adopted, with sub- or inter-muscular positioning of the generator, under local anesthesia or deep sedation (≤2014 versus 2017: all p < 0.001). Defibrillation testing was performed in 81% of patients. Shock energy of ≤65 J was successful in 93.9% of patients and the overall cardioversion success rate at ≤80 J was 99.8%. Conclusions: Our analysis confirmed that the S-ICD continues to be preferentially used in specific patients (younger, less frequently with dilated cardiomyopathy and low ejection fraction.). Nonetheless, we noted a trend toward the wider use of S-ICD in patients with dilated cardiomyopathy and systolic dysfunction over the years. Novel approaches have been adopted while the acute efficacy of the system has remained stably high.

Aim: To determine whether right atrial (RA) function has prognostic value in patients with idiopathic pulmonary arterial hypertension (PAH).Methods and results: Overall, 104 patients (70 female, mean age 58 +/- 13 years) with idiopathic PAH underwent standard Doppler echocardiography and strain and strain rate (SR) analysis before right heart catheterization. At a mean follow-up of 22 +/- 7 months, 30 patients (29%) had clinical worsening. On Cox multivariable proportional-hazards regression analysis, RA reservoir function measured as peak longitudinal SR (hazard ratio [HR] 0.5; P < 0.0001), RA area (HR 1.2; P < 0.01), right ventricular (RV) SR (HR 0.6; P < 0.0001), cardiac index (HR 0.79; P < 0.01), and mixed venous oxygen saturation (HR 0.82; P < 0.01) were found to be independent correlates of cardiac events. A RA SR reservoir cut-off value of

AIMS A simple echocardiographic score was designed for diagnosing precapillary vs postcapillary pulmonary hypertension and for discriminating between isolated postcapillary pulmonary hypertension (Ipc-PH) and combined precapillary and postcapillary pulmonary hypertension (Cpc-PH). METHODS The score comprised 7 points (2 for E/e' ratio ≤10, 2 for a dilated non-collapsible inferior vena cava, 1 for a left ventricular eccentricity index ≥1.2, 1 for a right-to-left heart chamber dimension ratio >1 and 1 for the right ventricle forming the heart apex) and was applied to 230 consecutive patients referred for evaluation of pulmonary hypertension. RESULTS Precapillary pulmonary hypertension and postcapillary pulmonary hypertension were diagnosed in 160 and 70 patients, respectively. In the latter, Ipc-PH was found in 51 and Cpc-PH in 19. The echo score was higher in precapillary vs postcapillary pulmonary hypertension patients (4.2 ± 1.7 vs 1.6 ± 1.7, P

Background Fluid challenge may help in the differential diagnosis between pre- and postcapillary pulmonary hypertension (PH). However, the test is still in need of standardization and better defined clinical relevance. Methods Two hundred twelve patients referred for PH underwent a right-sided heart catheterization with measurements before and after rapid infusion of 7mL/kg of saline. PH was defined as mean pulmonary artery pressure≥ 25mmHg, and postcapillary PH was defined as pulmonary artery wedge pressure (PAWP) > 15mmHg. An increase in PAWP≥ 18mmHg was considered diagnostic for postcapillary PH. At baseline, 66 patients received a diagnosis of no PH; 22, of postcapillary PH; and 124, of precapillary PH (mostly pulmonary arterial hypertension). Results After fluid challenge, five of 66 patients with no PH (8%) and eight of 124 with precapillary PH (6%) had the diagnosis reclassified as postcapillary PH. Fluid challenge was associated with an increase in PAWP by 7 ± 2mmHg in postcapillary PH and 3 ± 1mmHg in both precapillary PH and no-PH groups. Between-group differences were significant, but there was overlap. There were no adverse events related to fluid challenge. Prediction bands calculated from quadratic fits of the PAWP responses in pooled control subjects with no PH and patients with precapillary PH helped confirm 18mmHg as the cutoff for diagnosing postcapillary PH. Conclusions Fluid challenge with 7mL/kg saline increases PAWP, more in postcapillary than in precapillary PH or in control subjects with no PH. A cutoff value of 18mmHg allows reclassification of 6%to 8%of patients with precapillary PH or normal hemodynamic characteristics at baseline.

Background: Exercise-induced increase in pulmonary artery systolic pressure (PASP) as a possible measure of right ventricular (RV) contractile reserve has been shown to predict survival in severe pulmonary hypertension. However, RV contractile reserve can also be measured by changes in stroke volume (SV), tricuspid annular plane systolic excursion (TAPSE), or tricuspid annular systolic velocity (S'). The limits of normal values and the functional significance of these changes in healthy subjects are not well known. Methods: In this prospective study, 90 healthy subjects (45 male, mean age 39 ± 13years) underwent exercise stress echocardiography with measurement of TAPSE, S', TAPSE/PASP, SV, and PASP at rest and peak exercise. Maximum and minimum normal values were reported for all indices. Results: Normal values of exercise-induced changes (Δ) were 4 to 10mm for TAPSE, 6 to 14cm/s for S', 12 to 57mm Hg for PASP, 0 to 96mL for SV, and −1.2 to 0mm/mm Hg for TAPSE/PASP. At peak exercise, women showed lower ΔTAPSE/PASP, ΔPASP, ΔS', and ΔSV, but higher TAPSE/PASP than men. Aging was associated with decreased ΔTAPSE/PASP, ΔTAPSE, ΔS', ΔPASP, and ΔSV. In addition, ΔS', ΔTAPSE/PASP, ΔPASP, and ΔSV, but not ΔTAPSE, were directly correlated with maximum workload. Conclusions: Our results provide age- and sex-related limits of normal for RV contractile reserve as assessed by exercise stress echocardiography and demonstrate that RV systolic function indices (PASP, TAPSE, S', and TAPSE/PASP) correlate with maximum exercise capacity.

Percutaneous closure is widely recognized as the first therapeutic option in the majority of cases of secundum atrial septal defect (ASD) because of its high effectiveness and safety. Nonetheless, with the progressive increase of implanted devices and follow-up duration, several adverse events, some of them potentially life-threatening, have been reported. We report the case of an asymptomatic aortic erosion that occurred 13 years after the procedure. The main feature of our case is the very late occurrence of a life-threatening asymptomatic complication of ASD percutaneous closure, which should prompt lifelong surveillance in this population.