Repaired Tetralogy of Fallot: Have We Understood the Right Timing of PVR?

Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR.

The mechanics of congenital heart disease: from a morphological trait to the functional echocardiographic evaluation.

Advances in pediatric cardiac surgery have resulted in a recent growing epidemic of children and young adults with congenital heart diseases (CHDs). In these patients, congenital defects themselves, surgical operations and remaining lesions may alter cardiac anatomy and impact the mechanical performance of both ventricles. Cardiac function significantly influences outcomes in CHDs, necessitating regular patient follow-up to detect clinical changes and relevant risk factors. Echocardiography remains the primary imaging method for CHDs, but clinicians must understand patients' unique anatomies as different CHDs exhibit distinct anatomical characteristics affecting cardiac mechanics. Additionally, the use of myocardial deformation imaging and 3D echocardiography has gained popularity for enhanced assessment of cardiac function and anatomy. This paper discusses the role of echocardiography in evaluating cardiac mechanics in most significant CHDs, particularly its ability to accommodate and interpret the inherent anatomical substrate in these conditions.

Multimodality Imaging Assessment of Tetralogy of Fallot: From Diagnosis to Long-Term Follow-Up.

Tetralogy of Fallot (TOF) is the most common complex congenital heart disease with long-term survivors, demanding serial monitoring of the possible complications that can be encountered from the diagnosis to long-term follow-up. Cardiovascular imaging is key in the diagnosis and serial assessment of TOF patients, guiding patients' management and providing prognostic information. Thorough knowledge of the pathophysiology and expected sequalae in TOF, as well as the advantages and limitations of different non-invasive imaging modalities that can be used for diagnosis and follow-up, is the key to ensuring optimal management of patients with TOF. The aim of this manuscript is to provide a comprehensive overview of the role of each modality and common protocols used in clinical practice in the assessment of TOF patients.

The Role of Multimodality Imaging in Pediatric Cardiomyopathies.

Cardiomyopathies are a heterogeneous group of myocardial diseases representing the first cause of heart transplantation in children. Diagnosing and classifying the different phenotypes can be challenging, particularly in this age group, where cardiomyopathies are often overlooked until the onset of severe symptoms. Cardiovascular imaging is crucial in the diagnostic pathway, from screening to classification and follow-up assessment. Several imaging modalities have been proven to be helpful in this field, with echocardiography undoubtedly representing the first imaging approach due to its low cost, lack of radiation, and wide availability. However, particularly in this clinical context, echocardiography may not be able to differentiate from cardiomyopathies with similar phenotypes and is often complemented with cardiovascular magnetic resonance. The latter allows a radiation-free differentiation between different phenotypes with unique myocardial tissue characterization, thus identifying the presence and extent of myocardial fibrosis. Nuclear imaging and computed tomography have a complementary role, although they are less used in daily clinical practice due to the concern related to the use of radiation in pediatric patients. However, these modalities may have some advantages in evaluating children with cardiomyopathies. This paper aims to review the strengths and limitations of each imaging modality in evaluating pediatric patients with suspected or known cardiomyopathies.

Telemedicine in Adult Congenital Heart Disease: Usefulness of Digital Health Technology in the Assistance of Critical Patients.

The number of adults with congenital heart disease (ACHD) has progressively increased in recent years to surpass that of children. This population growth has produced a new demand for health care. Moreover, the 2019 coronavirus pandemic has caused significant changes and has underlined the need for an overhaul of healthcare delivery. As a result, telemedicine has emerged as a new strategy to support a patient-based model of specialist care. In this review, we would like to highlight the background knowledge and offer an integrated care strategy for the longitudinal assistance of ACHD patients. In particular, the emphasis is on recognizing these patients as a special population with special requirements in order to deliver effective digital healthcare.

Myocardial work in children with Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White Syndrome (WPW) has been associated with reduced local myocardial deformation, and when left ventricular dysfunction is present, catheter ablation of the accessory pathway may be required, even in asymptomatic patients. We aimed to evaluate the diagnostic value of non-invasive myocardial work in predicting subtle abnormalities in myocardial performance in children with WPW.Seventy-five paediatric patients (age 8.7 ± 3.5 years) were retrospectively recruited for the study: 25 cases with manifest WPW and 50 age- and sex- matched controls (CTR). Global myocardial work index (MWI) was measured as the area of the left ventricle (LV) pressure-strain loops. From MWI, global Myocardial Constructive Work (MCW), Wasted Work (MWW), and Work Efficiency (MWE) were estimated. In addition, standard echocardiographic parameters of LV function were evaluated. Despite normal LV ejection fraction (EF) and global longitudinal strain (GLS), children with WPW had worse MWI, MCW, MWW, and MWE. At multivariate analysis, MWI and MCW were associated with GLS and systolic blood pressure, and QRS was the best independent predictor of low MWE and MWW. In particular, a QRS > 110 ms showed good sensitivity and specificity for worse MWE and MWW values. In children with WPW, myocardial work indices were found significantly reduced, even in the presence of normal LV EF and GLS. This study supports the systematic use of myocardial work during the follow-up of paediatric patients with WPW. Myocardial work analysis may represent a sensitive measure of LV performance and aid in decision-making.

Italian Association of Hospital Cardiologists practical guidance for sodium-glucose cotransporter 2 inhibitors use in patients with heart failure.

Sodium-glucose cotransporter 2 inhibitors (SGLT2-is) have recently been included among the first-line drugs for the treatment of heart failure with reduced ejection fraction. International guidelines recommend SGLT2-i use in association with neuro-hormonal modulators (renin-angiotensin blockers, beta blockers, and aldosterone antagonists). Although SGLT2-is are well tolerated, it is important to know potential side effects and conditions that may lead to an increased risk of adverse events in order to maximize clinical benefits. The aim of this Italian Association of Hospital Cardiologists document is to briefly report clinical evidence that supports SGLT2-i use in patients with heart failure and provide practical indications for clinical implementation.

Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study.

BACKGROUND: Long-term data on COVID-19 vaccine safety, immunogenicity, and acceptance in adults with CHD are lacking. METHODS: This is a prospective study including adults with CHD patients undergoing COVID-19 vaccination from January 2021 to June 2022. Data on adverse events, antispike IgG titre, previous or subsequent COVID-19 infection, booster doses, and patients' attitude towards vaccination were collected. RESULTS: Four hundred and ninety CHD patients (36 ± 13 years, 53% male, 94% with moderate/complex defects) were prospectively included: 433 (88%) received a Pfizer-BioNTech mRNA vaccine, 31 (6%) Moderna mRNA vaccine, 23 (5%) AstraZeneca-Oxford ChAdOx1 nCov-19 vaccine, and 3 (0.6%) Janssen Vaccine; 310 (63%) received a booster dose. Median follow-up after vaccination was 1.53 [1.41-1.58] years. No major adverse event was reported. Eighty-two fully vaccinated patients contracted COVID-19 during follow-up after a median of 5.4 [4.3-6.5] months from the last dose. One patient with Ebstein's disease died from severe COVID-19. Symptoms' duration in patients who tested positive after vaccination was significantly shorter than in the group tested positive before vaccination (5.5 [3-8] versus 9 [2.2-15] days, p = 0.04). Median antispike IgG titre measured in 280 individuals (57%) at a median of 1.4 [0.7-3.3] months from the last dose was 2381 [901-8307] BAU/ml. Sixty patients (12%) also showed positive antinucleocapsid antibodies, demonstrating previous SARS-COV2 exposure. Twenty-nine percent appeared to have concerns regarding vaccine safety and 42% reported fearing potential effects of the vaccine on their cardiac disease before discussing with their CHD cardiologist. CONCLUSION: COVID-19 vaccines appear safe in the mid-term follow-up in adults with CHD with satisfactory immunogenicity and reduction of symptoms' duration in case of infection.

[Degenerative aortic valve stenosis: looking for a pharmacological prevention]. / Stenosi valvolare aortica degenerativa: alla ricerca di una prevenzione farmacologica.

Degenerative calcific aortic valve stenosis (CAVS) is a chronic disease whose prevalence has increased over the last decade because of the aging of the general population. CAVS pathogenesis is characterized by complex molecular and cellular mechanisms that promote valve fibro-calcific remodeling. During the first phase, referred to as initiation, the valve undergoes collagen deposition and lipid and immune cell infiltration due to mechanical stress. Subsequently, during the progression phase, the aortic valve undergoes chronic remodeling through osteogenic and myofibroblastic differentiation of interstitial cells and matrix calcification. Knowledge of the mechanisms underlying CAVS development supports the resort to potential therapeutic strategies that interfere with fibro-calcific progression. Currently, no medical therapy has demonstrated the ability to significantly prevent CAVS development or slow its progression. The only treatment available in symptomatic severe stenosis is surgical or percutaneous aortic valve replacement. The aim of this review is to highlight the pathophysiological mechanisms involved in CAVS pathogenesis and progression and to discuss potential pharmacological treatments able to inhibit the main pathophysiological mechanisms of CAVS, including lipid-lowering treatment with lipoprotein(a) as emergent therapeutic target.

Implantable Loop Recorder with Long Sensing Vector: Safety, Acceptability, and Sensing Performance in Pediatric Patients.

Implantable loop recorders (ILRs) are effective tools for detecting arrhythmias by long-term continuous heart rhythm monitoring. Benefits have been demonstrated even in pediatric patients. ILR with a long sensing vector has recently been designed to improve signal quality in terms of P wave visibility and R wave amplitude. However, there are no data on its use in pediatric patients. We considered a series of pediatric patients implanted with a long sensing vector ILR. Sensing performance, including R wave amplitude and P wave visibility, device-related complications, and diagnostic yield were collected. During follow-up, each patient guided by his/her parents/guardians was also asked to complete a brief questionnaire to assess patient acceptability of the device. Twenty-five consecutive pediatric patients (mean age 11.3 ± 3.5 years, 72% male) were enrolled. The insertion success rate was 100% on the first attempt with no complications. The median amplitude of the R wave was 1.15 mV (interquartile range, 1.01-1.42) with no significant differences between patients aged ≤ or > 10 years (p = 0.726) and between female and male (p = 0.483). P wave was classified as 'always visible' in 24/25 patients (96%). ILR was generally well accepted and tolerated by all involved patients. During a median follow-up of 297 days (117-317), we achieved in 5 patients a correlation between symptoms and rhythm disorders (20%) and ruled out significant arrhythmias in 6 symptomatic children (24%). Long sensing vector ILR showed to be well accepted, with good signal quality and an excellent safety profile even in pediatric patients.

Non-Invasive Imaging Assessment in Patients with Aortic Coarctation: A Contemporary Review.

Coarctation of the aorta (CoA) is a congenital abnormality characterized by a narrowing of the aortic lumen, which can lead to significant morbidity and mortality if left untreated. Even after repair and despite significant advances in therapeutic management, these patients have overall reduced long-term survival due to the consequences of chronic afterload increase. Cardiovascular imaging is key from the first diagnosis to serial follow-up. In recent years, novel imaging techniques have emerged, increasing accessibility to advanced imaging modalities and enabling early and non-invasive identification of complications after repair. The aim of this paper is to provide a comprehensive review of the role of different imaging techniques in the evaluation and management of patients with native or repaired CoA, highlighting their unique strengths and limitations.

Role of Cardiovascular Imaging in the Follow-Up of Patients with Fontan Circulation.

Since its first description in 1971, the Fontan procedure and its modifications have led to a substantial improvement in the survival rates of patients with a variety of types of complex Congenital Heart Disease (CHD) characterised by the presence of a single, dominant ventricle. However, despite the significant improvement of the prognosis over the years, Fontan patients are still exposed to several cardiovascular and systemic complications. It is, therefore, important to fully understand the pitfalls hidden behind a Fontan anatomy and the potential predictors of ventricular failure. Cardiovascular imaging plays a key role in this context, allowing for the early identification of complications with important prognostic implications. Echocardiography remains the first-line imaging modality for serial evaluation of Fontan patients. However, there is a growing role of cardiovascular magnetic resonance and cardiac computed tomography from pre-operative assessment to longitudinal follow-up. The aim of this paper will be to provide a comprehensive overview of the role, strengths, and weaknesses of each imaging modality in the assessment of congenital cardiac conditions palliated with the Fontan procedure.

[Cardiology as seen through social media]. / La cardiologia vista attraverso i social media.

The use of social media (SoMe) has spread worldwide among doctors, scientific societies, and researchers. SoMe offer a powerful platform to accelerate or create new contacts, spread scientific news, and increase visibility. A social media promotion strategy for cardiovascular medicine papers seems to be associated with increased online visibility and a higher number of citations. This effect is independent of the type of article and the total number of followers of the authors. Indeed, SoMe are democratic and even non-senior researchers may be popular on Twitter: your title is not as important as what you do on Twitter. Nevertheless, some physicians may be over-celebrated due to their presence on SoMe. This is why a new author index, the K-index, has been proposed. The K-index correlates the citations of a scientist with the number of Twitter followers. Even scientific journals and societies have recognized the importance of SoMe and in the last years they have appeared on SoMe with official accounts. Therefore, besides the classic impact factor, publishers now pay more and more attention to other parameters, such as the Altmetric score, which takes into account the number of citations, but also the number of downloads, mentions on SoMe, newspapers and tv news, web sites, and blogs. The COVID-19 pandemic has boosted the importance of SoMe for scientific content distribution, particularly for congresses. For all these reasons, it is important to understand the pros and cons of SoMe. It is also possible that SoMe will become a new education medium for continuing medical education.

Echocardiographic Normal Reference Ranges for Non-invasive Myocardial Work Parameters in Pediatric Age: Results From an International Multi-Center Study.

Aims: This international multi-center study aimed to demonstrate the feasibility and reliability of non-invasive myocardial work (MW) parameters in the pediatric population, and to provide normal reference ranges for this useful echocardiographic tool in this specific subset of patients. Methods and Results: In this retrospective multi-center study involving three pediatric laboratories, 150 healthy children and adolescents (mean age of 10.6 ± 4.5, 91 males) were enrolled. A complete echocardiographic examination has been performed, including global longitudinal strain (GLS) assessment. The following parameters of non-invasive MW have been obtained through a dedicated software: global work index (GWI), global constructive work (GCW), global work waste (GWW), and global work efficiency (GWE), using left ventricular (LV) strain loops and non-invasive brachial artery cuff pressure values. The lowest expected values were for GWI 1,723 mmHg% in males and 1,682 mmHg% in females, for GCW 2,089 and 2,106 mmHg%, for GWE 95.9 and 95.5% whereas the highest expected value for GWW was 78 mmHg% in men and 90 mmHg% in women. The univariable and multivariable analysis showed significant associations between either GWI or GCW with SBP (ß coefficient = 0.446, p < 0.001; ß coefficient = 0.456, p < 0.001, respectively) and LV GLS (ß coefficient = -0.268, p = 0.001; ß coefficient = -0.233, p = 0.003, respectively). Inter- and intra-observer variability showed good reproducibility of non-invasive MW parameters. Conclusion: Non-invasive MW parameters were feasible and reliable in the pediatric population. This study provided normal reference ranges of these useful echocardiographic indices.

Maternal cardiovascular adaptation to pregnancy in women with previous bariatric surgery.

BACKGROUND: Obesity in pregnancy is associated with substantial risks, notably hypertensive disorders. Bariatric surgery achieves sustained weight loss and has several cardiovascular benefits, including positive effects on blood pressure, cardiac geometry, and both systolic and diastolic function. Pregnancy following bariatric surgery is also associated with improved outcomes, including a reduced risk of hypertensive disorders. The underlying mechanisms, however, remain uncertain. Maternal cardiovascular adaptation plays a vital role in maintaining a healthy pregnancy, and maladaptation has been associated with adverse pregnancy outcomes. However, to date, the maternal cardiovascular adaptation to pregnancy after bariatric surgery has not been investigated. OBJECTIVE: To investigate the maternal cardiovascular adaptation to pregnancy in women with previous bariatric surgery compared with women with a similar early-pregnancy body mass index, age, and race but no history of weight loss surgery. STUDY DESIGN: This was a prospective, observational, longitudinal study including pregnant women with (n=41) and without (n=41) a history of bariatric surgery. The participants were seen at 3 time points; at 12 to 14, 20 to 24, and 30 to 32 weeks of pregnancy. At each visit, the blood pressure was measured and the maternal cardiovascular system was assessed using transthoracic echocardiography. Two-dimensional speckle tracking was performed to assess the global longitudinal and circumferential strain on a subset of patients (15 in each group). Offline analysis was performed according to the European and American echocardiography guidelines. Multilevel linear mixed-effect models were used for all the comparisons. RESULTS: Compared with the no-surgery group, women with previous bariatric surgery, had lower systolic and diastolic blood pressure, heart rate, and cardiac output across all the trimesters (P<.01 for all comparisons), with an evidence of more favorable diastolic indices, including a higher E-wave/A-wave ratio across the mitral valve (P<.001), higher mitral velocity at the lateral and medial annulus (E') (P=.01 and P=.03, respectively), and a lower left atrial volume (P<.05). Furthermore, women with previous bariatric surgery demonstrated lower global longitudinal (P<.01) and circumferential strain (P=.02), which is suggestive of better systolic function. CONCLUSION: Our findings indicate better cardiovascular adaptation to pregnancy in women with previous bariatric surgery than in pregnant women of a similar early-pregnancy body mass index but no history of surgery.

Prognostic Relevance of Thyroid Disorders in Adults With Congenital Heart Disease.

Adults with congenital heart disease (ACHD) are frequently affected by thyroid diseases (TDs). However, the clinical relevance of TD in ACHD remains unknown. We aimed to describe the prevalence of TD in the ACHD population and to ascertain whether TD are associated with worse outcome. Patients with ACHD >18 years attending our tertiary center for a day-case between 2014 and 2019 were included. Clinical data between patients' first visit and December 2020 were collected. Primary end point was a combination of death, hospitalization for heart failure (HF), and new-onset of arrhythmic events. Secondary end points were each part of the primary outcome as separate end points. A total of 495 patients with ACHD (32.2 [24.5 to 45.6] years; 54% women) were included. Median follow-up was 9.4 (4.5 to 13.1) years. The prevalence of TD was 30%. TD group showed worse clinical status, as demonstrated by N-terminal pro b-type natriuretic peptide values (243.5 [96.5 to 523] vs 94 [45 to 207] pg/ml, p <0.001) and New York Heart Association class (27% vs 13% in class III to IV, p <0.0001) with higher incident rate of adverse events at follow-up (4.45 [3.43 to 5.69] % vs 1.29[0.94 to 1.75] % per person-year, p <0.001). TD were independently associated with higher risk of death (hazard ratio [HR] 4.1, p = 0.009), arrhythmic events (HR 3.8, p <0.0001), and hospitalization for HF (HR 8.02, p <0.0001). There was a fourfold increased risk of primary end point in the TD group even after propensity score matching for clinical variables including age, gender, disease complexity, physiological stage, previous palliative surgery, ventricular function, pulmonary arterial hypertension, cyanosis, and presence of systemic right ventricle (HR 4.47, p <0.0001). In conclusion, TD are predictive of adverse outcome in the ACHD population. Routine screening of thyroid function during follow-up in this population may be helpful to identify those with higher risk of death, arrhythmias, and HF.

The Risk of Sudden Unexpected Cardiac Death in Children: Epidemiology, Clinical Causes, and Prevention.

"Sudden unexplained death (SUD) is a tragic event for both the family and community, particularly when it occurs in young individuals. Sudden cardiac death (SCD) represents the leading form of SUD and is defined as an unexpected event without an obvious extracardiac cause, occurring within 1 hour after the onset of symptoms. In children, the main causes of SCD are inherited cardiac disorders, whereas coronary artery diseases (congenital or acquired), congenital heart diseases, and myocarditis are rare. The present review examines the current state of knowledge regarding SCD in children, discussing the epidemiology, clinical causes, and prevention strategies."

Diagnosis and Management of Cardiovascular Involvement in Friedreich Ataxia.

Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder caused by a homozygous GAA triplet repeat expansion in the frataxin gene. Cardiac involvement, usually manifesting as hypertrophic cardiomyopathy, can range from asymptomatic cases to severe cardiomyopathy with progressive deterioration of the left ventricular ejection fraction and chronic heart failure. The management of cardiac involvement is directed to prevent disease progression and cardiovascular complications. However, direct-disease therapies are not currently available for FRDA. The present review aims to describe the current state of knowledge regarding cardiovascular involvement of FRDA, focusing on clinical-instrumental features and management of cardiac manifestation.

Chylothorax Due to Superior Vena Cava Obstruction in a Patient With Complex Congenital Heart Disease.

Obstruction of the superior vena cava represents an under-recognized cause of chylothorax in the adult population. Our case report describes the successful conservative management of chylothorax due to bilateral superior vena cava obstruction in an adult patient with complex congenital heart disease. (Level of Difficulty: Beginner.).

Abnormal myocardial work in children with Kawasaki disease.

Kawasaki disease (KD) can be associated with high morbidity and mortality due to coronary artery aneurysms formation and myocardial dysfunction. Aim of this study was to evaluate the diagnostic performance of non-invasive myocardial work in predicting subtle myocardial abnormalities in Kawasaki disease (KD) children with coronary dilatation (CADL). A total of 100 patients (age 8.7 ± 5 years) were included: 45 children with KD and CADL (KD/CADL) (Z-score > 2.5), 45 age-matched controls (CTRL) and, finally, an additional group of 10 children with KD in absence of coronary dilatation (KD group). Left ventricular (LV) systolic function and global longitudinal strain (GLS) were assessed. Global myocardial work index (MWI) was calculated as the area of the LV pressure-strain loops. From MWI, global Constructive Work (MCW), Wasted Work (MWW) and Work Efficiency (MWE) were estimated. Despite normal LV systolic function by routine echocardiography, KD/CADL patients had lower MWI (1433.2 ± 375.8 mmHg% vs 1752.2 ± 265.7 mmHg%, p < 0.001), MCW (1885.5 ± 384.2 mmHg% vs 2175.9 ± 292.4 mmHg%, p = 0.001) and MWE (994.0 ± 4.8% vs 95.9 ± 2.0%, p = 0.030) compared to CTRL. Furthermore, MWI was significantly reduced in children belonging to the KD group in comparison with controls (KD: 1498.3 ± 361.7 mmHg%; KD vs CTRL p = 0.028) and was comparable between KD/CADL and KD groups (KD/CADL vs KD p = 0.896). Moreover, KD/CADL patients with normal GLS (n = 38) preserved significant differences in MWI and MCW in comparison with CTRL. MWI, MCW and MWE were significantly reduced in KD children despite normal LVEF and normal GLS. These abnormalities seems independent from CADL. Thus, in KD with normal LVEF and normal GLS, estimation of MWI may be a more sensitive indicator of myocardial dysfunction.