Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

: Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: -0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (-0,14 ± 1.24 vs. -0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

488 Candidacy for heart transplantation in adult congenital heart disease patients: a single-centre, retrospective, cohort study

Abstract Aims End-stage heart failure (HF) is the leading cause of death in adult congenital heart disease (ACHD) population. Heart transplantation (HTx) improves prognosis in ACHD end-stage HF but candidacy evaluation, referral pattern, and correct listing timing are not fully elucidated in this population. To evaluate factors associated to refusal from Htx in ACHD patients with end-stage HF referred for HTx evaluation. Methods and results This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients undergone HTx between 2000 and 2013. Refusal from HTx served as primary study endpoint. Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013. The main indication to HTx in patients with single ventricle physiology was Fontan failure, while in patients with systemic left ventricle and systemic right ventricle physiology, it was systemic ventricular dysfunction. We compared clinical, anatomical and demographic data of 41 patients accepted for transplantation with 15 patients refused after screening. Risk factors for refusal were: coexistence of multiple high risk features [odds ratio (OR): 3.6; 95% confidence interval (CI): 1.1–12.9; P 0.048]; anatomical factors (OR: 14.5; 95% CI: 3.1–68.4; P 0.001), out-of-centre ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). Survival in patients accepted for HTx was significantly higher than survival in patients declined from HTx with landmark comparison at 20, 40 and 60 months of 87%, 78%, and 72% vs. 70%, 59%, and 20%, respectively. HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1–8.3; P 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, coexistence of multiple conventional HTx high risk factors and out-of-centre referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy and to reduce referral delay in tertiary centre are strongly necessary for this growing population

New classification of geometric patterns considering left ventricular volume in patients with chronic aortic valve regurgitation: Prevalence and association with adverse cardiovascular outcomes

Background: Left ventricular (LV) remodeling due to aortic regurgitation (AR) often leads to maladaptive responses. We assessed the prevalence and clinical implications of LV remodeling considering LV volume, mass, and relative wall thickness at the time of AR diagnosis. Methods and Results: Between 2008 and 2017, 370 consecutive patients (mean age 67.3 ± 16.1 years, 56.5% males), with moderate or severe AR, were retrospectively analyzed. LV geometric patterns and clinical outcomes (cardiovascular death, hospitalization for heart failure, or aortic valve replacement) were evaluated. LV dilatation (LV end-diastolic volume >75 mL/m2) was present in 228 patients (61.6%). Applying the new LV remodeling classification system, 40 (10.8%) patients had normal geometry, 14 (3.8%) concentric remodeling, 43 (11.6%) concentric hypertrophy (LVH), 45 (12.2%) indeterminate LVH, 38 (10.3%) mixed LVH, 93 (25.1%) dilated LVH, 54 (14.6%) eccentric LVH, and 43 (11.6%) eccentric remodeling. During a median follow-up of 3.48 years (25th–75th percentile 0.91–5.57), 97 (26.2%) had the combined endpoint. LV dilation (P < 0.001), LVH (P < 0.001), and LV remodeling patterns were significantly associated with the combined endpoint. After multivariable adjustment for age, EF, aortic stenosis, CAD history, and moderate mitral regurgitation, dilated LVH (HR 7.61, IC 95% 1.82–31.80; P = 0.005) and eccentric LVH (HR 7.91, IC 95% 1.82–34.38; P = 0.006) were associated with adverse outcome compared to eccentric remodeling, that showed the best event-free survival rate. Conclusions: In a contemporary cohort of patients with AR, applying the new LV remodeling classification system, only a minority had normal geometry. Dilated LVH and eccentric LVH showed distinct outcome penalty after adjustment for confounders

Hypertrophic Cardiomyopathy with Biventricular Involvement and Coronary Anomaly: A Case Report

Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) involvement has also been reported, though still not extensively characterized. We present a case of biventricular HCM with significant RV involvement in the absence of a left intraventricular gradient: RV outflow tract gradient due to hypertrophy and near obliteration of the RV cavity. Significant RV hypertrophy may cause reduced RV diastolic filling and/or RV outflow obstruction, with potentially increased incidence of symptoms of heart failure, arrhythmias, and pulmonary thromboembolism. The optimal treatment for these patients is unclear. Our patient underwent complete treatment and elimination of right ventricular obstruction, resulting in improved symptoms and a significant reduction in postoperative gradients. Direct relief of outflow tract obstruction can be achieved with low morbidity and good intermediate- to long-term results. Conventional surgery may provide significant symptomatic improvement and should thus be considered in the setting of HCM with outflow obstruction

12-year Temporal Trend in Referral Pattern and Test Results of Stress Echocardiography in a Tertiary Care Referral Center with Moderate Volume Activities and Cath-lab Facility

Background: Data on stress echocardiography (SE) time-related changes in referral patterns and diagnostic yield for detection of inducible ischemia could enhance Echo Lab quality benchmarks and performance measures. Aim: This study aims to evaluate temporal trends in SE test results among ambulatory patients with suspected or known coronary artery disease (CAD) in a tertiary care referral center with moderate (>100/year) volume SE activities and Cath-Lab facility. Methods: From January 2004 to December 2015, 1954 patients (mean age 62 ± 12 years, 42% women, 27% with known CAD) underwent SE (1673 exercise SE, 86%, 246 pharmacological SE, 12%, 35 pacing SE, 2%). Time was grouped into three 4 year periods, where clinical data and test results were evaluated. Results: Our series comprised low-to-intermediate pretest probability of CAD throughout the observation period (overall pretest probability of CAD 19% ± 15%). A progressive decline over time in the rate of pharmacological SE instead of a dramatic increment of exercise SE (79%-96%, P < 0.0001) was noted. The use of beta-blockers increased (from 43% to 66%, P < 0.0001), while the use of nitrates decreased (from 11% to 4%, P < 0.0001) over time. We noted a very uncommon occurrence of abnormal test results with a further decrease in the last period (from 11% to 3%, P < 0.0001). Conclusions: We observed, over a 12-year period, a progressive decrease in the frequency of inducible myocardial ischemia among patients with known or suspected CADe referred to our Echo Lab for SE with Cath-Lab facility, and this trend was parallel to changes in SE referral practice. These findings are particularly relevant if we consider the practical implications on diagnostic SE accuracy and risk assessment

Consultation-liaison psychiatry and the "Woman Wellness Project": a model of integrated services for female mental health

wellness, integrated care, female

Stress Echo 2030: the new ABCDE protocol defining the future of cardiac imaging

Functional testing with stress echocardiography is based on the detection of regional wall motion abnormality with two-dimensional echocardiography and is embedded in clinical guidelines. Yet, it under-uses the unique versatility of the technique, ideally suited to describe the different functional abnormalities underlying the same wall motion response during stress. Five parameters converge conceptually and methodologically in the state-of-the-art ABCDE protocol, assessing multiple vulnerabilities of the ischemic patient. The five steps of the ABCDE protocol are (1) step A: regional wall motion; (2) step B: B-lines by lung ultrasound assessing extravascular lung water; (3) step C: left ventricular contractile reserve by volumetric two-dimensional echocardiography; (4) step D: coronary flow velocity reserve in mid-distal left anterior descending coronary with pulsed-wave Doppler; and (5) step E: assessment of heart rate reserve with a one-lead electrocardiogram. ABCDE stress echo offers insight into five functional reserves: epicardial flow (A); diastolic (B), contractile (C), coronary microcirculatory (D), and chronotropic reserve (E). The new format is more comprehensive and allows better functional characterization, risk stratification, and personalized tailoring of therapy. ABCDE protocol is an 'ecumenic' and 'omnivorous' functional test, suitable for all stresses and all patients also beyond coronary artery disease. It fits the need for sustainability of the current era in healthcare, since it requires universally available technology, and is low-cost, radiation-free, and nearly carbon-neutral

Left ventricle function after arterial switch procedure for D-transposition of the great arteries: Long term evaluation by speckle-tracking analysis

Aim The objective of this study was to assess left ventricle (LV) function in patients underwent arterial switch procedure (ASO) for transposition of great arteries (TGA) in long-term follow-up. Methods We studied 59 asymptomatic patients (43 male) who have undergone single-stage ASO for TGA, aged 13.9 ± 4.8 years, with a normal LV ejection fraction, compared to healthy peers. We evaluated LV volume, function and myocardial deformation in asymptomatic patients with normal ejection fraction by using speckle-tracking echocardiography (STE). Results Global longitudinal strain (GLS) was lower in patients compared to healthy peers throughout all age groups (5–9 years: −20.03 ± 0.65% vs 21.00 ± 1.30%, p = 0.083; 10–14 years: −19.43 ± 1.75% vs −21.80 ± 1.30%, p < 0.0001; 15–19 years: −19.05 ± 1.65% vs −22.50 ± 1.30%, p < 0.0001; 20–24 years: −17.90 ± 0.85% vs −20.90 ± 1.30%, p < 0.0001; >25 years: −18.60 ± 0.42% vs 20.60 ± 1.20%, p = 0.041). At the univariate analysis GLS resulted significantly related only to the presence of restrictive patent foramen ovale at birth (p = 0.0016). At the multivariate analysis GLS was significantly related to prenatal diagnosis, restrictive patent foramen ovale and by-pass time. Conclusion Children and young adults late after ASO demonstrate normal ejection fraction, but present subclinical signs of myocardial dysfunction, such as reduction of longitudinal strain. Our findings support the usefulness of STE to detect it precociously

Moderate and Severe Congenital Heart Diseases Adversely Affect the Growth of Children in Italy: A Retrospective Monocentric Study

Children with congenital heart disease (CHD) are at increased risk for undernutrition. The aim of our study was to describe the growth parameters of Italian children with CHD compared to healthy children. We performed a cross-sectional study collecting the anthropometric data of pediatric patients with CHD and healthy controls. WHO and Italian z-scores for weight for age (WZ), length/height for age (HZ), weight for height (WHZ) and body mass index (BMIZ) were collected. A total of 657 patients (566 with CHD and 91 healthy controls) were enrolled: 255 had mild CHD, 223 had moderate CHD and 88 had severe CHD. Compared to CHD patients, healthy children were younger (age: 7.5 ± 5.4 vs. 5.6 ± 4.3 years, p = 0.0009), taller/longer (HZ: 0.14 ± 1.41 vs. 0.62 ± 1.20, p < 0.002) and heavier (WZ: −0,07 ± 1.32 vs. 0.31 ± 1.13, p = 0.009) with no significant differences in BMIZ (−0,14 ± 1.24 vs. –0.07 ± 1.13, p = 0.64) and WHZ (0.05 ± 1.47 vs. 0.43 ± 1.07, p = 0.1187). Moderate and severe CHD patients presented lower z-scores at any age, with a more remarkable difference in children younger than 2 years (WZ) and older than 5 years (HZ, WZ and BMIZ). Stunting and underweight were significantly more present in children affected by CHD (p < 0.01). In conclusion, CHD negatively affects the growth of children based on the severity of the disease, even in a high-income country, resulting in a significant percentage of undernutrition in this population

Candidacy for heart transplantation in adult congenital heart disease patients: A cohort study

Objective The object of the present study is to evaluate factors precluding heart transplantation (HTx) in adult congenital heart disease patients (ACHD) with end-stage heart failure (HF) referred for HTx evaluation. Methods This retrospective cohort study enrolled consecutive ACHD patients considered for HTx in our institution between 2014 and 2020 and patients receiving HTx between 2001 and 2013. HTx refusal due to poor candidacy status for excess risk of mortality after transplantation served as the main study outcome. Results Between 2014 and 2020, 46 ACHD patients were evaluated for HTx, 14 ACHD patients underwent HTx between 2001 and 2013 (final sample size 60 patients). We compared clinical, anatomical and demographic data of 41 patients suitable for transplantation with 15 patients refused after screening (excluding 4 patients with ongoing screening). Risk factors for refusal were: multiple high risk features (odds ratio [OR]: 3.6; 95% confidence interval [CI]: 1.1 to 12.9; p 0.048); anatomical factors (OR: 14.5; 95% CI: 3.1 to 68.4; p 0.001), out-of-center ACHD/HTx program referral (OR: 5.3; 95% CI: 1.5 to 19.0; p 0.01). HTx refusal identifies a high risk ACHD patient subgroup (hazard ratio for overall mortality: 3.1; 95% CI: 1.1 to 8.3; p 0.02). Conclusions In our study risk factors for refusal from HTx are adverse anatomical features, multiple conventional HTx high risk factors and out-of-center referral. ACHD patients refused from HTx present shorter time to death. Efforts to increase HTx candidacy are strongly necessary for this growing population