Isolated aneurysmal disease as an underestimated finding in individuals with JAG1 pathogenic variants.

Pathogenic variants in JAG1 are known to cause Alagille syndrome (ALGS), a disorder that primarily affects the liver, lung, kidney, and skeleton. Whereas cardiac symptoms are also frequently observed in ALGS, thoracic aortic aneurysms have only been reported sporadically in postmortem autopsies. We here report two families with segregating JAG1 variants that present with isolated aneurysmal disease, as well as the first histological evaluation of aortic aneurysm tissue of a JAG1 variant carrier. Our observations shed more light on the pathomechanisms behind aneurysm formation in JAG1 variant harboring individuals and underline the importance of cardiovascular imaging in the clinical follow-up of such individuals.

Percutaneous Closure of PFO in Patients with Reduced Oxygen Saturation at Rest and during Exercise: Short- and Long-Term Results.

BACKGROUND: A patent foramen ovale (PFO) is a rare cause of hypoxemia and clinical symptoms of dyspnea. Due to a right-to-left shunt, desaturated blood enters the systemic circulation in a subset of patients resulting in dyspnea and a subsequent reduction in quality of life (QoL). Percutaneous closure of PFO is the treatment of choice. OBJECTIVES: This retrospective multicentre study evaluates short- and long-term results of percutaneous closure of PFO in patients with dyspnea and/or reduced oxygen saturation. METHODS: Patients with respiratory symptoms were selected from databases containing all patients percutaneously closed between January 2000 and September 2018. Improvement in dyspnea, oxygenation, and QoL was investigated using pre- and postprocedural lung function parameters and two postprocedural questionnaires (SF-36 and PFSDQ-M). RESULTS: The average follow-up period was 36 [12-43] months, ranging from 0 months to 14 years. Percutaneous closure was successful in 15 of the 16 patients. All patients reported subjective improvement in dyspnea immediately after device deployment, consistent with their improvement in oxygen saturation (from 90 ± 6% to 94 [92-97%] on room air and in upright position) (p < 0.05). Both questionnaires also indicated an improvement of dyspnea and QoL after closure. The two early and two late deaths were unrelated to the procedure. CONCLUSION: PFO-related dyspnea and/or hypoxemia can be treated successfully with a percutaneous intervention with long-lasting benefits on oxygen saturation, dyspnea, and QoL.

Abnormal longitudinal peak systolic strain in asymptomatic patients with type I diabetes mellitus.

AIMS: In addition to increased risk of cardiovascular disease, the prevalence of diabetic cardiomyopathy is increasingly recognized in patients with type 1 diabetes mellitus (T1DM). We aimed to identify the occurrence of subclinical markers of cardiovascular risk and cardiac dysfunction and assess their relation to clinical parameters in asymptomatic patients with T1DM. METHODS AND RESULTS: A total of 102 patients (mean age 46 years [20-73], 62% male) with a history of T1DM ranging from 5 to 47 years underwent standard 2D and pulse-wave tissue Doppler echocardiography (Philips iE33) and computerized tomography for assessment of coronary calcium score (CACS) and visceral fat. Global peak longitudinal strain (GPLSS, speckle tracking) was calculated by offline analysis (Qlab 9.0). Whereas systolic function was preserved in all patients (LVEF > 50%), subclinical dysfunction (defined as global longitudinal peak systolic strain [GLPSS] of >-20%) was present in 39% and 66% had diastolic dysfunction. Fifty patients had a CACS above the 50th percentile according to age and gender. These patients were older, more obese, had higher levels of visceral fat, higher SBP and increased levels of LDL cholesterol. Higher CACS meant increased risk of diastolic and subclinical systolic dysfunction. However, decreased GLPSS was also detected in 30% of patients with CACS of <50th percentile. Stepwise linear regression analysis indicated visceral fat as a strong predictor of abnormal GPLSS and CACS. CONCLUSION: Subclinical left ventricular dysfunction and atherosclerosis were highly prevalent in asymptomatic T1DM. Abnormal GPLSS was noted with or without associated increase in CACS. Visceral fat was a strong predictor of increased CACS as well as abnormal GLPSS.

Percutaneous closure of patent foramen ovale for the secondary prevention of decompression illness in sports divers: mind the gap.

OBJECTIVE: To evaluate the efficiency of percutaneous patent foramen ovale (PFO) closure on the recurrence of decompression illness (DCI). DESIGN: Retrospective, observational study with interview and questionnaire. SETTING: Tertiary referral center. POPULATION: 59 scuba divers with a history of DCI who received a percutaneous PFO closure. MAIN OUTCOME MEASUREMENTS: Questionnaire about health status, dive habits and recurrence of DCI after PFO closure. RESULTS: A total of 59 divers with DCI were included. The most common manifestations of DCI were cutaneous or vestibular DCI. Procedural complications occurred in four patients but none with long-term consequences. Four patients had recurrence of DCI after closure during a 10-year follow-up. In three of these cases there was residual shunting, all of which were initially considered closed. The fourth patient had aggravating factors for his recurrent DCI. A quarter of the patients stated to have changed their diving habits. Four patients quit diving. CONCLUSION: Percutaneous PFO closure for secondary prevention of DCI is associated with few, but not negligible, complications. As a large portion of our cohort changed their diving habit after closure it is difficult to ascertain the efficiency of PFO closure for secondary prevention of DCI. However, the study shows that PFO closure does not fully protect against DCI, emphasizing that the relationship between PFO and DCI is but an association. As such it is imperative that divers be counseled to ensure they understand the risks as well as the benefits of percutaneous PFO closure in their specific case.

How to Size ASDs for Percutaneous Closure.

Percutaneous closure is the treatment of choice for secundum-type atrial septal defects (ASD). Balloon sizing (BS) has been the method of choice for deciding on device size. Improved 2D- and 3D-transesophageal echocardiographic (TEE) imaging challenged the necessity of BS. Balloon sizing was performed with two additional techniques to measure the stretched dimension of the ASD. The 1st method uses a stiff guide wire which stretches the ASD and 2D TEE. The second technique uses 3D TEE. Two hundred and thirty-six patients with minimum 1-year follow-up were enrolled. The population was classified into three groups: BS (group 1) n = 90, 2D-TEE (group 2) n = 87, and 3D-TEE (group 3) n = 59. All groups showed a distinct correlation between the maximum baseline dimensions and the device size (R = 0.821). The relative expansion rate did not differ between BS and 3D-TEE. Group 2 (2D-TEE) showed a significantly lower expansion rate. Procedural success and complications did not differ statistically between the 3 groups. 2D TEE sizing was the simplest method without loss of accuracy. 3D sizing offers the advantage of accurate and fast shape assessment, but resulted in more undersizing. Accurate sizing of ASDs with a floppy septum remains a challenge.

Tumor necrosis factor-α impairs adiponectin signalling, mitochondrial biogenesis, and myogenesis in primary human myotubes cultures.

Skeletal muscle metabolic changes are common in patients with chronic heart failure (HF). Previously, we demonstrated a functional skeletal muscle adiponectin resistance in HF patients with reduced left ventricular ejection fraction (HFrEF). We aimed to examine the impact of adiponectin receptor 1 (AdipoR1) deficiency and TNF-α treatment on adiponectin signaling, proliferative capacity, myogenic differentiation, and mitochondrial biogenesis in primary human skeletal muscle cells. Primary cultures of myoblasts and myotubes were initiated from the musculus vastus lateralis of 10 HFrEF patients (left ventricular ejection fraction; 31.30 ± 2.89%) and 10 age- and gender-matched healthy controls. Healthy control cultures were transfected with siAdipoR1 and/or exposed to TNF-α (10 ng/ml; 72 h). Primary cultures from HFrEF patients preserved the features of adiponectin resistance in vivo. AdipoR1 mRNA was negatively correlated with time to reach maximal cell index (r = -0.7319, P = 0.003). SiRNA-mediated AdipoR1 silencing reduced pAMPK (P < 0.01), AMPK activation (P = 0.046), and myoblast proliferation rate (xCELLigence Real-Time Cellular Analysis; P < 0.0001). Moreover, TNF-α decreased the mRNA expression of genes involved in glucose (APPL1, P = 0.0002; AMPK, P = 0.021), lipid (PPARα, P = 0.025; ACADM, P = 0.003), and mitochondrial (FOXO3, P = 0.018) metabolism, impaired myogenesis (MyoD1, P = 0.053; myogenin, P = 0.048) and polarized cytokine secretion toward a growth-promoting phenotype (IL-10, IL-1ß, IFN-γ, P < 0.05 for all; Meso Scale Discovery Technology). Major features of adiponectin resistance are retained in primary cultures from the skeletal muscle of HFrEF patients. In addition, our results suggest that an increased inflammatory constitution contributes to adiponectin resistance and confers alterations in skeletal muscle differentiation, growth, and function.

The evolving role of adiponectin as an additive biomarker in HFrEF.

Heart failure (HF) is a growing health problem. Despite improved management and outcome, the number of patients with HF is expected to keep rising in the following years. In recent research, adiponectin was shown to exert beneficial effects in the cardiovascular system, but the protein was also implicated in the development and progression of HF. The objective of this review is to provide an overview of current knowledge on the role of adiponectin in HF with reduced ejection fraction. We discuss the cardioprotective and (anti-) inflammatory actions of adiponectin and its potential use in clinical diagnosis and prognosis.

Primary skeletal muscle myoblasts from chronic heart failure patients exhibit loss of anti-inflammatory and proliferative activity.

BACKGROUND: Peripheral skeletal muscle wasting is a common finding with adverse effects in chronic heart failure (HF). Whereas its clinical relevance is beyond doubt, the underlying pathophysiological mechanisms are not yet fully elucidated. We aimed to introduce and characterize the primary culture of skeletal muscle cells from individual HF patients as a supportive model to study this muscle loss. METHODS AND RESULTS: Primary myoblast and myotubes cultures were successfully propagated from the m. vastus lateralis of 6 HF patients with reduced ejection fraction (HFrEF; LVEF <45 %) and 6 age and gender-matched healthy donors. HFrEF cultures were not different from healthy donors in terms of morphology, such as myoblast size, shape and actin microfilament. Differentiation and fusion indexes were identical between groups. Myoblast proliferation in logarithmic growth phase, however, was attenuated in the HFrEF group (p = 0.032). In addition, HFrEF myoblasts are characterized by a reduced TNFR2 expression and IL-6 secretion (p = 0.017 and p = 0.016; respectively). CONCLUSION: Biopsy derived primary skeletal muscle myoblasts of HFrEF patients produce similar morphological and myogenic differentiation responses as myoblasts of healthy donors, though demonstrate loss of anti-inflammatory and proliferative activity.

Unveiling women's lived experiences with care provided by a pregnancy heart team: A qualitative phenomenological study.

OBJECTIVE: The purpose of this study was to gain an in-depth understanding of the lived experiences of women with CVD regarding their care by a pregnancy heart team (PHT) during pregnancy and the immediate postpartum period. METHODS: Using a qualitative phenomenological study, data were collected through semi-structured interviews with 13 women receiving follow-up care from a PHT at a large tertiary center. Data were collected between December 2022 and September 2023, and thematic content analysis was conducted. RESULTS: The lived experiences of women with CVD were reflected in the PHT by two main themes: "emotional distress" and "(dis-)organization of care." The "emotional distress" theme had sub-themes of "awareness and impact of CVD on pregnancy," "loneliness," and "need for psychological support." The theme of "(dis)organization of care" was expressed through the sub-themes of "(dis-)continuity of care" and "(expected) skills of healthcare providers." CONCLUSION: The current study findings highlight the impact of CVD on pregnancy and the emotional challenges faced by women with CVD during the course of their pregnancy. Improvements in accessibility, timeliness, reciprocity and shared decision-making, and psychological support could contribute to more patient-centered care.

Interarm Blood Pressure Difference Revealing a Right-Sided Aortic Arch and Occluded Aberrant Left Subclavian Artery.

We present the case of a 33-year-old patient with a rare combination of a right-sided aortic arch and occluded asymptomatic aberrant left subclavian artery diagnosed after the coincidental finding of an interarm blood pressure difference. Because there were no symptoms of local compression or subclavian steal, conservative management was suggested.

First diagnosis of severe coarctation of the aorta necessitating percutaneous intervention during pregnancy: a case report.

Background: Coarctation of the aorta (CoA) is a common congenital heart defect that affects about 3-4 in every 10 000 live births. Despite clear signs on clinical examination, the diagnosis is sometimes not made until adulthood. An increasing number of patients with CoA are reaching child-bearing age. Unrepaired CoA or severe recurrent stenosis during pregnancy is a significant concern, as it poses a high risk of maternal and foetal complications and even death. Case summary: A 21-year-old woman was referred to the cardiology department at 17 weeks' gestation for management of arterial hypertension and unexplained systolic murmur. She had been diagnosed with hypertension elsewhere the year before presentation, but unfortunately, this remained unexplored. She had been started on labetalol early in the pregnancy. Clinical examination showed a loud systolic heart murmur extending from parasternal to subclavicular and scapular areas. Pulses in the lower extremities were very weak, and blood pressure was slightly elevated with a significant gradient between the upper and lower extremities. Echocardiography showed remarkable absence of pulsatile flow in the abdominal aorta and narrowing just distal to the subclavian artery with typical diastolic tail pattern on suprasternal imaging. Cardiac magnetic resonance confirmed the presence of a severe coarctation distal to the subclavian artery and presence of multiple collaterals allocating this patient in the extremely high-risk category with a risk of up to 40%-100% of maternal cardiac event during pregnancy. An extensive multidisciplinary team meeting was convened. After initial medical optimization, increased claudication and signs of placental hypoperfusion necessitated an endovascular procedure under general anaesthesia at 23 weeks' gestation. A Bentley BeGraft Plus stent (16 × 38 mm) was successfully placed. Postoperative ultrasound showed biphasic placental perfusion and normalization of blood pressure and ankle-brachial indices. At 36 weeks' gestation, the patient gave birth to a healthy child. Discussion: Coarctation of the aorta should be considered in any young patient with arterial hypertension. Altered maternal haemodynamics during pregnancy resulted in severe symptomatic CoA and reduced placental flow necessitating percutaneous intervention during pregnancy. A multidisciplinary pregnancy heart team is essential for optimal treatment management in these high-risk patients.

Peripheral Microvascular Dysfunction in Children and Adults with Congenital Heart Disease: A Literature Review.

Although there is a continually growing number of patients with congenital heart disease (CHD) due to medical and surgical advances, these patients still have a poorer prognosis compared to healthy individuals of similar age. In patients with heart failure, microvascular dysfunction (MVD) has recently emerged as a crucial modulator of disease initiation and progression. Because of the substantial pathophysiological overlap between CHD and heart failure induced by other etiologies, MVD could be important in the pathophysiology of CHD as well. MVD is believed to be a systemic disease and may be manifested in several vascular beds. This review will focus on what is currently known about MVD in the peripheral vasculature in CHD. Therefore, a search on the direct assessment of the vasodilatory capacity of the peripheral microcirculation in patients with CHD was conducted in the PubMed database. Since there is little data available and the reported studies are also very heterogeneous, peripheral MVD in CHD is not sufficiently understood to date. Its exact extent and pathophysiological relevance remain to be elucidated in further research.

Adiponectin: key role and potential target to reverse energy wasting in chronic heart failure.

The concept of skeletal muscle myopathy as a main determinant of exercise intolerance in chronic heart failure (HF) is gaining acceptance. Symptoms that typify HF patients, including shortness of breath and fatigue, are often directly related to the abnormalities of the skeletal muscle in HF. Besides muscular wasting, alterations in skeletal muscle energy metabolism, including insulin resistance, have been implicated in HF. Adiponectin, an adipocytokine with insulin-sensitizing properties, receives increasing interest in HF. Circulating adiponectin levels are elevated in HF patients, but high levels are paradoxically associated with poor outcome. Previous analysis of m. vastus lateralis biopsies in HF patients highlighted a striking functional adiponectin resistance. Together with increased circulating adiponectin levels, adiponectin expression within the skeletal muscle is elevated in HF patients, whereas the expression of the main adiponectin receptor and genes involved in the downstream pathway of lipid and glucose metabolism is downregulated. In addition, the adiponectin-related metabolic disturbances strongly correlate with aerobic capacity (VO2 peak), sub-maximal exercise performance and muscle strength. These observations strengthen our hypothesis that adiponectin and its receptors play a key role in the development and progression of the "heart failure myopathy". The question whether adiponectin exerts beneficial rather than detrimental effects in HF is still left unanswered. This current research overview will elucidate the emerging role of adiponectin in HF and suggests potential therapeutic targets to tackle energy wasting in these patients.

Unraveling new mechanisms of exercise intolerance in chronic heart failure: role of exercise training.

Despite remarkable progress in the therapeutic approach of patients with chronic heart failure (CHF), exercise intolerance remains one of the hallmarks of the disease. During the past two decades, evidence has accumulated to underscore the key role of both endothelial dysfunction and skeletal muscle wasting in the process that gradually leads to physical incapacity. Whereas reverse ventricular remodeling has been attributed to aerobic exercise training, the vast majority of studies conducted in this specific patient population emphasize the reversal of peripheral abnormalities. In this review, we provide a general overview on underlying pathophysiological mechanisms. In addition, emphasis is put on recently identified pathways, which contribute to a deeper understanding of the main causes of exercise tolerance and the potential for reversal through exercise training. Recently, deficient bone marrow-related endothelial repair mechanisms have received considerable attention. Both acute exercise bouts, as well as exercise training, affect the mobilization of endothelial progenitor cells and their function. The observed changes following exercise training are believed to significantly contribute to improvement of peripheral endothelial function, as well as exercise capacity. With regard to skeletal muscle dysfunction and energy deprivation, adiponectin has been suggested to play a significant role. The demonstration of local skeletal muscle adiponectin resistance may provide an interesting and new link between the insulin resistant state and skeletal muscle wasting in CHF patients.

Elevated oxidative stress in patients with congenital heart disease and the effect of cyanosis: a meta-analysis.

Oxidative stress is an important pathophysiological mechanism in the development of numerous cardiovascular disorders. To improve therapy and preventive strategies, clinicians need a better understanding of the underlying pathophysiological mechanisms of congenital heart diseases (CHD). The objective of this meta-analysis was to determine whether oxidative stress is elevated in patients with CHD compared to healthy controls, and to evaluate whether a difference in oxidative stress parameters can be observed between patients with cyanotic (cCHD) and acyanotic CHD (aCHD). Therefore, 21 studies investigating oxidative stress in peripheral blood of both children and adults with CHD were reviewed. Different methods to assess the oxidant status were compared and divided into three categories: pro-oxidative or anti-oxidative stress markers and the ratio of pro-to-anti oxidative stress markers. This meta-analysis showed elevated oxidative stress levels in patients with CHD, and more specifically in patients with cCHD. Moreover, this indicates that there could be potential in oxidative stress measurements as a new biomarker of disease severity. Further research will be needed to clarify the exact role of oxidative stress and its contributors in CHD in order to get a better and more in-depth understanding of the underlying pathophysiology of CHD, especially the higher susceptibility of the right ventricle (RV) to progress to heart failure (HF). This could facilitate the development of antioxidant treatments and RV-specific HF therapies, which are necessary to improve survival in these patients and could be of particular importance in cCHD.

Phenotype of COL3A1/COL5A2 deletion patients.

INTRODUCTION: The diagnosis of Ehlers-Danlos syndrome is usually based on well-defined diagnostic criteria and the result of DNA investigation. Classical (cEDS) and vascular type (vEDS) are the most prevalent subtypes and are caused by heterozygous pathogenic variants in COL5A1, COL5A2, COL1A1 or, respectively, in COL3A1. We describe 3 cases with contiguous deletions resulting in haploinsufficiency of both genes with relative mild features of connective tissue disease. PATIENTS AND METHODS: Information on medical history, physical information, genetic results (CNV-analysis) and imaging were obtained from the medical file. RESULTS: The first patient was a 31 yr old female, diagnosed during pregnancy after the NIPT result showed an interstitial deletion of 2.3 Mb on chromosome 2q32.2, confirmed by XON array. She had normal aortic diameters. She had no signs of cEDS or vEDS except for a relatively thin skin with increased visibility of the veins. Her father died suddenly of a type A/B dissection at the age of 62 years. The second patient was diagnosed at the age of 10 years after she was referred because of her intellectual disability, autism and constipation. She was known with a thin and vulnerable skin and had a bleeding after tooth extraction. Array showed a 14,5 Mb deletion of 2q31.3q32.3 (de novo). Imaging (latest age 17 years) did not show any abnormalities. The third patient, aged 28 years, was diagnosed during pregnancy with an interstitial deletion of circa 6 Mb on chromosome 2q31.1q32.2 3, previously shown in the fetus with bilateral club feet and hydronephrosis. She had no vEDS facial features and the skin was relatively thin. She has thoracolumbar scoliosis and dural ectasia. Imaging did not reveal any vascular abnormalities. Her son, born at 37 weeks 3 days. had club feet but not other clinical signs suggestive of classical or vascular EDS. DISCUSSION: Three patients are described with a contiguous deletion of varying size encompassing the COL3A1 and COL5A2 gene. Due to the mild phenotype a diagnosis of EDS was not suspected and was found coincidental. Since two of the patients were pregnant without major complications these patients may require a less defensive, approach to pregnancy/delivery.

Circulating Reactive Oxygen Species in Adults with Congenital Heart Disease.

Oxidative stress is an important pathophysiological mechanism in the development of numerous cardiovascular disorders, but few studies have examined the levels of oxidative stress in adults with congenital heart disease (CHD). The objective of this study was to investigate oxidative stress levels in adults with CHD and the association with inflammation, exercise capacity and endothelial function. To this end, 36 adults with different types of CHD and 36 age- and gender-matched healthy controls were enrolled. Blood cell counts, hs-CRP, NT-proBNP, fasting glucose, cholesterol levels, iron saturation and folic acid concentrations were determined in venous blood samples. Levels of superoxide anion radical in whole blood were determined using electron paramagnetic resonance spectroscopy in combination with the spin probe CMH. Physical activity was assessed with the IPAQ-SF questionnaire. Vascular function assessment (EndoPAT) and cardiopulmonary exercise testing were performed in the patient group. Superoxide anion radical levels were not statistically significantly different between adults with CHD and the matched controls. Moreover, oxidative stress did not correlate with inflammation, or with endothelial function or cardiorespiratory fitness in CHD; however, a significant negative correlation with iron saturation was observed. Overall, whole blood superoxide anion radical levels in adults with CHD were not elevated, but iron levels seem to play a more important role in oxidative stress mechanisms in CHD than in healthy controls. More research will be needed to improve our understanding of the underlying pathophysiology of CHD.

Functional adiponectin resistance and exercise intolerance in heart failure.

The contribution of skeletal muscle myopathy to the phenotype of patients with chronic heart failure (CHF) has become generally accepted. Besides the macro- and microscopic changes that develop during the progressive process of muscular wasting, functional abnormalities manifest in an earlier stage. Analogous to the failing heart, alterations in skeletal muscle energy metabolism, including insulin resistance, are increasingly recognized. In the search for factors causing this observed myopathy, adipokines receive growing attention. In particular, adiponectin is of special interest due to its fundamental role in skeletal muscle energy metabolism. In strong contrast with patients at risk for cardiovascular disease, circulating adiponectin levels are increased in patients with CHF, and this finding is associated with adverse outcome. Recently, the concept of functional skeletal muscle adiponectin resistance has been suggested to explain compensatory elevated adiponectin levels in CHF. Unraveling of adiponectin's complex downstream signalling pathways and insights into the concept of adiponectin resistance hopefully will disengage the road for targeted therapeutic interventions.

Multi-plane echocardiographic assessment of right ventricular function in adults with repaired Tetralogy of Fallot.

In patients with repaired Tetralogy of Fallot (ToF), detailed assessment of right ventricular (RV) function is important for management and timing of possible pulmonary valve re-intervention. The aim of this study was to evaluate RV function using two-dimensional multi-plane echocardiography (2D MPE), a novel four-wall imaging method obtained from one apical acoustic window utilising electronic plane rotation. In sixty-two ToF patients (aged - 28 [22, 39] years, 65% male), systolic function of four different RV walls (lateral, anterior, inferior and inferior coronal) were evaluated using MPE. Tricuspid annular plane systolic excursion (TAPSE), tricuspid annular peak systolic velocity (RV-S') and RV wall longitudinal strain (RV-LS) measurements were compared with those of matched healthy individuals. 2D MPE measurements were highly feasible across the four RV walls (93.5-100% for TAPSE/S'; 66.1-95.1% for RVLS) and could be performed more reliably than 3D RV ejection fraction (RVEF - 56.5%). All functional values were significantly reduced when compared to the control group (p < 0.001). Higher RV-LS values were seen in the lateral (- 17.8 ± 4.5%) and inferior (- 17.8 ± 4.2%) walls compared to the anterior (- 15.9 ± 3.8%) and inferior coronal (- 15.1 ± 3.9%) walls. 3D RVEF correlated strongest with RV-LS values from the lateral (r - 0.50; p = 0.002) and anterior walls (r - 0.74; p < 0.001) and furthermore the four-wall average (r - 0.57; p = 0.001). 2D MPE evaluation of the RV is highly feasible in ToF patients. This novel method provides new insights into regional RV wall function, enabling a more comprehensive and quantitative approach to RV assessment in daily clinical practice.

Quantitative assessment of the entire right ventricle from one acoustic window: An attractive approach in patients with congenital heart disease in daily practice.

BACKGROUND: Right ventricular (RV) function is recognized as an important prognostic factor in adult congenital heart disease (ACHD). The accuracy of established parameters including tricuspid annular plane systolic excursion (TAPSE), RV fractional area change (RVFAC) and tissue Doppler imaging (TDI S') is limited as only a small RV region is reflected. We previously introduced a novel four-view approach with different RV walls visualized from one apical view using electronic plane rotation, also known as iRotate. AIM: To evaluate the entire RV function using electronic plane rotation echocardiography within the spectrum of ACHD compared with healthy subjects. METHODS AND RESULTS: One hundred and forty-two ACHD patients were recruited from the outpatient clinic and 89 healthy subjects. All subjects underwent a transthoracic echocardiogram with evaluation of TAPSE, TDI S' and peak systolic longitudinal RV strain (RV-LS) from all RV walls using the four-view electronic plane rotation model. With exception of TDI S' in inferior coronal view, all parameters were lower in ACHD vs healthy subjects (p < 0.001). Within the ACHD patients, RV strain was lower in anterior (-15.9 ± 4.9) and inferior coronal view (-15.1 ± 4.5) versus lateral (-17.6 ± 5.0) and inferior wall (-17.2 ± 4.7) (p < 0.05). RV-LS values of systemic RV were lower (p < 0.05), but no difference was observed between subpulmonic RV loading conditions. CONCLUSION: The four-view electronic plane rotation model represents a reproducible, easily applicable and complete RV assessment in daily practice. RV function is significantly decreased in the ACHD group using both regional and global assessment parameters. Complete RV strain analysis reveals regional differences.