Intracorporeal LVAD implantation in pediatric patients: A single-center 10 years' experience.

BACKGROUND: Mechanical cardiac support is currently an effective strategy to reduce morbidity and mortality in pediatric patients. However, solid evidence regarding the feasibility of intracorporeal devices in children still needs to be provided. We report our 10-year experience with intracorporeal left ventricular assist devices (LVAD) in children. MATERIALS AND METHODS: We included all patients undergoing intracorporeal, continuous-flow LVAD implantation between 2012 and 2022. Baseline and postoperative data were collected from the institutional database. RESULTS: Seven HeartWare and 4 HeartMate3 were implanted in 11 patients (median age 13.9 years, median body surface area - BSA - 1.42 m2, IQR 1.06-1.68). The most frequent indication to LVAD implant was dilated cardiomyopathy (72.7%). All candidates underwent a thorough preoperative advanced imaging. Three-dimensional reconstructions and implant fit simulation were performed when BSA was <1.2 m2, weight <30 kg, or internal transverse thoracic diameter <20 cm. There was no operative death. The most common postoperative complication was surgical re-exploration due to bleeding (27.3%). One patient died of severe neurological complications after about 3 months of hospitalization. No late deaths or unplanned re-hospitalizations occurred in the remaining 10, 6 of whom were discharged home. There were no major complications at the follow-up. All survivors underwent successful heart transplantation. CONCLUSIONS: Intracorporeal LVAD implantation proved to be a potentially feasible and safe option in young teenagers and children whose BSA was >1.0 m2. In borderline cases, the 3D reconstruction with implant fit simulation can effectively help to identify those patients who can safely undergo intrathoracic LVAD implantation.

A native mitral valve mass beyond imagination.

The authors report a case of a patient with a history of IgA nephropathy that, during the admission for pneumonia, was found to have an incidental finding of a huge mitral valve (MV) mass on transthoracic echocardiography. The differential diagnosis was challenging because the clinical scenario raised the suspicion of possible infective endocarditis and the imaging features were suggestive of a myxoma or vegetation. The patient underwent urgent excision of the mass with MV replacement due to the high risk of embolism. Intraoperative findings were consistent with clot or vegetation. Pathology result of thrombus was beyond our imagination and at the best of our knowledge one case only has been reported. Awareness about native MV thrombosis and its etiologic factors, workup, and management is key for better medical and surgical management planning because this condition is extremely rare and challenging in the clinical and imaging arena.

Role of three-dimensional transesophageal echocardiography in cardiac myxomas: an imaging challenge.

Nowadays, the diagnosis of cardiac myxomas (CM), particularly the histological types, remains a challenge. Two-dimensional (2D) transthoracic (TT) and transesophageal (TEE)  echocardiography (ECHO) represent the first steps in the imaging pathway. 3D ECHO,  implemented in imaging practice, appears to be an emerging diagnostic technique that overcomes some of the limitations of 2D ECHO while integrating the information provided by magnetic resonance (MRI). However, its role in the imaging arena is still debatable. Analyzing 17 myxomas in 13 patients, the study uncovers a diverse anatomical spectrum. Classical CM morphology is a minority, with most myxomas being sessile and originating from unexpected locations (right ventricular outflow tract and left atrial appendage). Texture and size variations are also noted. Comparing imaging, 2D TEE outperforms 2D TT in visualizing anatomical features, especially attachment types. 3D TEE confirms 2D TT findings and offers more detailed assessments, identifying peduncles missed in four cases by 2D TEE. Two small recurrent myxomas were exclusively detected by 3D TEE, not by 2D TEE or MRI. Two patients have papillary myxomas, one has an embolism. Another patient with a solid myxoma also suffers an embolism, with a clot found at the apex during surgery. Our study showed that CM has a wide anatomical spectrum beyond the typical features, making the diagnosis challenging. Therefore, a multimodality imaging approach is essential for distinguishing CM from other cardiac masses and differentiating myxoma histological types. These findings stress the importance of incorporating 3D ECHO alongside other imaging techniques for a comprehensive evaluation.

Unveiling the gothic aortic arch and cardiac mechanics: insights from young patients after arterial switch operation for d-transposition of the great arteries.

The arterial switch operation (ASO) has become the standard surgical treatment for patients with d-transposition of the great arteries. While ASO has significantly improved survival rates, a subset of patients develop a unique anatomical anomaly known as the gothic aortic arch (GAA). Understanding cardiac mechanics in this population is crucial, as altered mechanics can have profound consequences for cardiac function and exercise capacity. The GAA has been associated with changes in ventricular function, hemodynamics, and exercise capacity. Studies have shown a correlation between the GAA and decreased ascending aorta distensibility, loss of systolic wave amplitude across the aortic arch, and adverse cardiovascular outcomes. Various imaging techniques, including echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography, play a crucial role in assessing cardiac mechanics and evaluating the GAA anomaly. Despite significant advancements, gaps in knowledge regarding the prognostic implications and underlying mechanisms of the GAA anomaly remain. This review aims to explore the implications of the GAA anomaly on cardiac mechanics and its impact on clinical outcomes in young patients after ASO. Advancements in imaging techniques, such as computational modeling, offer promising avenues to enhance our understanding of cardiac mechanics and improve clinical management.

Coronary artery calcium score: we know where we are but not where we may be.

Cardiac computed tomography angiography (CCTA) has emerged as a cost-effective and time-saving technique for excluding coronary artery disease. One valuable tool obtained by CCTA is the coronary artery calcium (CAC) score. The use of CAC scoring has shown promise in the risk assessment and stratification of cardiovascular disease. CAC scores can be complemented by plaque analysis to assess vulnerable plaque characteristics and further refine risk assessment. This paper aims to provide a comprehensive understanding of the value of the CAC as a prognostic tool and its implications for patient risk assessment, treatment strategies, and outcomes. CAC scoring has demonstrated superior ability in stratifying patients, especially asymptomatic individuals, compared to traditional risk factors and scoring systems. The main evidence suggests that individuals with a CAC score of 0 have a good long-term prognosis, while an elevated CAC score is associated with increased cardiovascular risk. Finally, the clinical power of CAC scoring and the development of new models for risk stratification could be enhanced by machine learning algorithms.

Right-sided infective endocarditis and pulmonary embolism: a multicenter study.

The incidence of right-sided infective endocarditis (RSIE) is steadily increasing and it has been reported to be associated with high risk of embolic events (EE). Aim of our study was to identify the clinical characteristics of patients with RSIE complicated by PE. Indeed, the identification of patients at high risk of significant PE who will benefit from a more aggressive therapeutic strategy may improve the prognosis. From January 2015 to September 2020, 176 patients (Pts) in 6 centers were found to have definite RSIE complicated by PE. Advanced imaging for PE including computed tomography pulmonary angiography (CTPA) was performed in 28 pts (16%) who represent our study group (24 male, mean age 50.6 ±18.29 years). They all underwent transesophageal echocardiography (TEE), in 12 cases (43%) also three-dimensional (3D) TEE, and 27 patients (99%) had both TEE and transthoracic echocardiography (TTE). A total of 53 vegetations (V) were detected. In 18 pts (64%) two or more vegetations were found. Native tricuspid valve was the most frequently involved valve (38 V, 71.7%), followed by catheter (5 V, 9.4%), tricuspid valve prosthesis (4 V, 7.5%), chordae and papillary muscle (2 V, 3.8%) and one vegetation (9%) in each of the following: pulmonic valve, inferior vena cava, eustachian valve, and right atrium. The most common location for vegetations was the anterior leaflet of the tricuspid valve (19 V, 35.8 %) followed by the posterior leaflet (11 V, 20.8%). The most common vegetations morphology was raceme-like shaped (35.8%). Staphylococcus aureus (S. aureus) was the most common causative pathogen (14 pts, 50%). The incidence of PE was very high in patients with vegetation length above 1.5 cm (median 17.6±6.5 mm by TEE). Our results suggest that a routine CTPA should be advised in the presence of vegetations larger than 1.5 cm and with S. aureus infection. This behavior would identify patients at high risk of PE who will benefit from a more aggressive therapeutic strategy, leading to an improvement in the prognosis. Further prospective studies are required to better confirm our hypothesis.

Thyroid dysfunction on the heart: clinical effects, prognostic impact and management strategies.

Thyroid hormones have a considerable influence on cardiac function and structure. There are direct and indirect effects of thyroid hormone on the cardiovascular system, which are prominent in both hypothyroidism and hyperthyroidism. In this review, we discuss how thyroid dysfunction impacts cardiovascular pathophysiology and the underlying molecular mechanisms.

Indirect ultrasound evaluation of left ventricular outflow tract diameter implications for heart failure and aortic stenosis severity assessment.

BACKGROUND: Whereas dependency of left ventricular outflow tract diameter (LVOTD) from body surface area (BSA) has been established and a BSA-based LVOTD formula has been derived, the relationship between LVOTD and aortic root and LV dimensions has never been explored. This may have implications for evaluation of LV output in heart failure (HF) and aortic stenosis (AS) severity. METHODS: A cohort of 540 HF patients who underwent transthoracic echocardiography was divided in a derivation and validation subgroup. In the derivation subgroup (N = 340), independent determinants of LVOTD were analyzed to derive a regression equation, which was used for predicting LVOTD in the validation subgroup (N = 200) and compared with the BSA-derived formula. RESULTS: LVOTD determinants in the derivation subgroup were sinuses of Valsalva diameter (SVD, beta = 0.392, P < .001), BSA (beta = 0.229, P < .001), LV end-diastolic diameter (LVEDD, beta = 0.145, P = .001), and height (beta = 0.125, P = .037). The regression equation for predicting LVOTD with the aforementioned variables (LVOTD = 6.209 + [0.201 × SVD] + [1.802 × BSA] + [0.03 × LVEDD] + [0.025 × Height]) did not differ from (P = .937) and was highly correlated with measured LVOTD (R = 0.739, P < .001) in the validation group. Repeated analysis with LV end-diastolic volume instead of LVEDD and/or accounting for gender showed similar results, whereas BSA-derived LVOTD values were different from measured LVOTD (P < .001). CONCLUSION: Aortic root and LV dimensions affect LVOTD independently from anthropometric data and are included in a new comprehensive equation for predicting LVOTD. This should improve evaluation of LV output in HF and severity of AS when direct LVOTD measurement is difficult or impossible.

Single coronary artery originating from right sinus. Role of MDCT and a review of literature.

SCA from the right sinus is the rarest coronary anomaly. We describe 2 cases: 1 with SCA type-1RI; 2 with SCA type-2RII-A. Appropriate and successful treatment (CABG in case-1; PTCA in case-2) was chosen relying on accurate morphological description provided by MDCT, in order to recognize all the possible mechanisms of myocardial ischemia.

Features and behavior of valvular abnormalities in adolescent and adult patients in mucopolysaccharidosis: an echocardiographic study.

Mucopolysaccharidoses, a rare inherited disorder of lysosomal storage, account for less than 0.1% of all genetic diseases.  The penetrance is highly variable and clinically it varies from severe fetal-neonatal forms to attenuated diseases diagnosed in adult individuals. The majority of the patients have been reported to show cardiac abnormalities since pediatric age, however, there is a minority of patients with attenuated disease diagnosed in the adolescent and adult age. The haematopoietic stem cell transplantation and enzyme replacement therapy are the current therapies for these disorders. Thanks to these treatments, Mucopolysaccharidoses patients live longer than in the past. Even though enzyme replacement therapy has been demonstrated to reduce left ventricular mass in patients with cardiomyopathy, the efficacy on valve abnormalities has not been clearly demonstrate yet. Furthermore, thanks to the current therapy, to better understanding and to the advent on new technologies, an increasing number of adolescent and adult patients diagnosed with MPS are followed up in the adult echocardiographic laboratory. Indeed, a systematic descriptive study describing the echocardiographic features of valvular involvement and their evolution in adolescent and adult patients lacks of medical literature and this was the aim of our investigation. Our results showed that all the valves are affected, mainly the mitral valve with a higher prevalence compared to the pediatric age.  The echocardiographic features of MPS differs from other valvular disease of adolescent and adult age, and knowing them can avoid misdiagnosis. Our observations also suggest that the progression of cardiac involvement slows after the initiation of the therapy in our group of age. Further studies on larger population are required to confirm our results.

Left Ventricular Mechanics in Patients with Abnormal Origin of the Left Main Coronary Artery from the Pulmonary Trunk Late after Successful Repair.

OBJECTIVE: Our aim was to evaluate left ventricular (LV) mechanics by using speckle tracking echocardiography (STE) in asymptomatic patients with abnormal origin of the left main coronary artery from the pulmonary trunk (ALCAPA), late after successful repair, in the presence of LV ejection fraction (EF) >50%. METHODS: We studied 30 ALCAPA patients (median age 4 years, range 1-25 years, NYHA class I, LVEF >50%) and 16 healthy age- and sex-matched controls (median age 5 years, range 1-25 years). All underwent standard echocardiographic evaluation and STE. RESULTS: LV dimensions and LVEF (63.6 ± 8.2% vs. 64.1 ± 5.1%, p = 0.826) were not different between patients and controls. Diastolic parameters were significantly abnormal in our patients versus controls (E/e' average: 11.9 ± 5.8 vs. 6.6 ± 3.0, p = 0.0014). Global LV longitudinal strain was significantly lower in ALCAPA patients versus controls (-17.6 ± 3.5% vs. -23.4 ± 3.1%, p < 0.0001). LV torsion (9.1 ± 4.9° vs. 11.9 ± 3.3°, p = 0.046) was significantly impaired in ALCAPA patients. CONCLUSIONS: After successful repair in asymptomatic ALCAPA patients, despite an LVEF >50%, diastolic function, LV longitudinal deformation and LV torsion remain impaired. We suggest including a detailed study of the diastolic function and cardiac mechanics in the clinical follow-up of these patients to identify the subgroup of patients at higher risk.

Atrial function in heart transplant recipients operated with the bicaval technique.

OBJECTIVES: In heart transplantation (HTx) with the bicaval technique the whole right atrium (RA) is donor tissue while the left atrium (LA) consists of both donor and recipient tissue. The aims of the study were to describe atrial function in comparison with healthy controls and to identify determinants of dysfunction. DESIGN: Forty-three patients and 30 controls were retrospectively included. Echocardiography was performed within 24 h of right heart catheterization (RHC) (HTx recipients). RESULTS: The peak longitudinal atrial systolic strain (ɛ) described the atrial reservoir function. The LA and RA reservoir function were reduced versus controls (LA-ɛ 18 ± 8 versus 44 ± 10%; RA-ɛ 22 ± 10 versus 69 ± 17%, p < 0.001). There were moderate relationships between atrial-ɛ and ventricular filling pressure (r = -0.64 for LA-ɛ; r = -0.57 for RA-ɛ). In a multiple regression analysis the LA-ɛ was determined by pulmonary capillary wedge pressure (PCWP) and LA minimum volume index (r = -0.71) while RA-ɛ was dependent on the right ventricular ɛ (r = -0.77). CONCLUSIONS: Atrial reservoir function is markedly reduced in HTx recipients related to elevated PCWP and LA-enlargement in the LA and in the RA impaired longitudinal right ventricular function.

Intermittent Malfunction of a Prosthetic Valve-A Diagnostic Challenge.

Intermittent malfunction of a mechanical valve prosthesis is a rare condition that carries serious clinical implications. It results from the periodic entrapment of a prosthetic disk in either an open or closed position leading to transient intravalvular regurgitation or obstruction to flow. The intermittent nature of the malfunction poses a diagnostic challenge, particularly in the era of digital echocardiography. In this manuscript, we describe the Doppler and two-dimensional echocardiographic findings in a patient with intermittent prosthetic mitral valve malfunction.

The various hemodynamic profiles of the patent ductus arteriosus in adults.

The patent ductus arteriosus (PDA) has diverse clinical and hemodynamic manifestations depending on its size and the degree of the ensuing left-to-right shunt. A small PDA that causes minor shunting has no major hemodynamic consequences. Conversely, a large PDA with a significant left-to-right shunt may lead to various hemodynamic abnormalities. These include left-sided volume overload that may result in heart failure and/or pulmonary hypertension, the latter being a flow-dependent and mostly reversible phenomenon. The most feared complication is the development of severe and irreversible pulmonary hypertension (Eisenmenger physiology). In this manuscript, we provide examples of the various hemodynamic profiles of PDA as assessed by echocardiography in the adult population.

Echocardiography detects elevated left ventricular filling pressures in heart transplant recipients.

Diastolic dysfunction is a recognized complication in heart transplant (HTx) recipients that limits exercise capacity and is a risk factor for mortality. We investigated the ability of echocardiography to detect elevated pulmonary capillary wedge pressure (mean PCWP>15 mmHg) in HTx recipients. This retrospective study comprised HTx recipients with echocardiography and right heart catheterization within 24 hours (n = 100, 113 investigations). Echocardiographic assessment was performed using mitral inflow (E/A ratio, deceleration time [DT], isovolumic relaxation time [IVRT]), tissue Doppler (E/E' lateral) parameters, and the Doppler-estimated pulmonary artery systolic pressure (Doppler PASP). The right atrial pressure (RAP) was estimated based on size and the effect of respiration or sniffing on the inferior vena cava diameter. Cutoff values were determined from a derivation group (n = 57, receiver operator characteristic curve analysis) and evaluated in a test group (n = 56). Elevated PCWP were found in 38%. The RAP and PCWP were both normal in 58 investigations and elevated in 39 investigations (concordance rate of 86.6%). The presence of signs of increased RAP by echocardiography or with three of five parameters (E/A, DT, IVRT, E/E' lateral, and Doppler PASP) reaching the cutoff values ruled in elevated PCWP with positive likelihood ratios ranging from 15.3 to 9. With normal RAP by echocardiography or none of the other parameters reaching cutoff values elevated PCWP can be ruled out with negative likelihood ratios ranging from 0.07 to 0.19. In conclusion, elevated PCWP in HTx recipients can be assessed using echocardiography.

Multimodality imaging in arrhythmogenic cardiomyopathy - From diagnosis to management.

Arrhythmogenic Cardiomyopathy (AC), an inherited cardiac disorder characterized by myocardial fibrofatty replacement, carries a significant risk of sudden cardiac death (SCD) due to ventricular arrhythmias. A comprehensive multimodality imaging approach, including echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (CCT), allows for accurate diagnosis, effective risk stratification, vigilant monitoring, and appropriate intervention, leading to improved patient outcomes and the prevention of SCD. Echocardiography is primary tool ventricular morphology and function assessment, CMR provides detailed visualization, CCT is essential in early stages for excluding congenital anomalies and coronary artery disease. Echocardiography is preferred for follow-up, with CMR capturing changes over time. The strategic use of these imaging methods aids in confirming AC, differentiating it from other conditions, tracking its progression, managing complications, and addressing end-stage scenarios.

[Paradoxical low flow-low gradient aortic stenosis: lights and shadows]. / Stenosi aortica "paradoxical low flow-low gradient": luci ed ombre.

Paradoxical low flow-low gradient aortic valve stenosis (AVS) is an increasing phenotype in the general population, particularly after the seventh decade of life. It is an AVS in which, despite the preserved ejection fraction, the mean transvalvular gradient is not suggestive of severe AVS (<40 mmHg). The pathophysiology is often intertwined with conditions resulting in heart failure with preserved ejection fraction, such as arterial hypertension and cardiac amyloidosis. Its management is rather controversial about the diagnosis and therapeutic management. The aims of this focus are: to clarify the role and reliability of the main available diagnostic methods, the efficacy of surgical and percutaneous treatments, and to develop a diagnostic-therapeutic algorithm for managing this condition in clinical practice. This algorithm will involve a multi-parametric evaluation, integrating standard echocardiographic assessment with three-dimensional planimetric valve area calculation, determination of the energy loss index), and calcium score calculation by computed tomography scan. This approach aims to ascertain the severity of the stenosis and determine the appropriate therapeutic management.

Phenotypic Expression and Clinical Outcomes in Patients With Arrhythmogenic Cardiomyopathies.

BACKGROUND: In recent years, it has become evident that arrhythmogenic cardiomyopathy (ACM) displays a wide spectrum of ventricular involvement. Furthermore, the influence of various clinical phenotypes on the prognosis of the disease is currently being assessed. OBJECTIVES: The purpose of this study was to evaluate the impact of phenotypic expression in ACM on patient outcomes. METHODS: We conducted an analysis of 446 patients diagnosed with ACM. These patients were categorized into 3 groups based on their phenotype: arrhythmogenic right ventricular cardiomyopathy (ARVC) (right-dominant ACM), arrhythmogenic left ventricular cardiomyopathy (ALVC) (left-dominant ACM), and biventricular arrhythmogenic cardiomyopathy (BIV). We compared clinical, instrumental, and genetic findings among these groups and also evaluated their outcomes RESULTS: Overall, 44% of patients were diagnosed with ARVC, 23% with ALVC, and 33% with BIV forms. Subjects showing with ARVC and BIV phenotype had a significantly higher incidence of life-threatening ventricular arrhythmias compared with ALVC (P < 0.001). On the other hand, heart failure, heart transplantation, and death caused by cardiac causes were more frequent in individuals with BIV forms compared to those with ALVC and ARVC (P < 0.001). Finally, patients with an ALVC phenotype had a higher incidence of hot phases compared with those with ARVC and BIV forms (P = 0.013). CONCLUSIONS: The comparison of ACM phenotypes demonstrated that patients with right ventricular involvement, such as ARVC and BIV forms, exhibit a higher incidence of life-threatening ventricular arrhythmias. Conversely, ACM forms characterized by left ventricular involvement, such as ALVC and BIV, show a higher incidence of heart failure, heart transplantation, and hot phases.