Two-dimensional echocardiographic right heart ratios for assessment of right heart size in dogs: Reference intervals and reproducibility.

BACKGROUND: Reference intervals for simple body weight-independent measurements of right heart size and function are limited. OBJECTIVES: Generate reference intervals for measurements of right heart size indexed to the long-axis aortic valve diameter (AoD) or corresponding left heart structure (right heart ratios) and describe the reproducibility of these indices. ANIMALS: Ninety healthy adult dogs of variable body weight. METHODS: Prospective study. All dogs underwent an echocardiogram performed by the same operator. Numerous linear 2-dimensional measurements of right heart size and function from different imaging planes were performed. Eight dogs underwent repeated echocardiograms by the same operator on 3 different days, and 3 different operators performed repeated echocardiograms on the same day. Reference intervals were generated using the Clinical Laboratory Standards Institute method. Reproducibility was quantitated using coefficients of variation (CVs) and reproducibility coefficients. RESULTS: Reference intervals for right heart ratios were generated and allow simple assessments of right heart size and function that do not require a scaling exponent or body weight table. Right heart ratios did not show clinically relevant associations with body weight. All CVs were <22.6%. In general, CVs for right heart measurements indexed to AoD were lower compared with right heart measurements indexed to the corresponding left heart structure. CONCLUSIONS AND CLINICAL IMPORTANCE: Reference intervals for simple body weight-independent right heart ratios are available to help detect abnormalities of right heart size and function. Reproducibility coefficients might be useful to help identify meaningful changes in right heart size during serial evaluations.

The Relationship Between Right Ventricular Longitudinal Strain and Adverse Outcome in Hypertensive Patients: 10-year Follow-up.

INTRODUCTION: Previous studies showed the importance of right ventricular (RV) remodeling in patients with arterial hypertension and RV longitudinal strain was recognized as very sensitive parameter for detection of subtle cardiac impairment. However, its clinical importance in arterial hypertension has not been established so far. AIM: The present study aimed to evaluate the association between RV longitudinal strain (global and free-wall) on adverse outcomes measured by MACE in the large group of hypertensive patients who were followed for mean period of 10 years. METHODS: This retrospective study finally included 544 hypertensive patients who underwent full echocardiographic examination including 2D speckle tracking imaging. between January 2010 and December 2014. MACE was considered as the primary outcome and it was defined by all-cause mortality, cardiovascular mortality, myocardial infarction, coronary artery by-pass, coronary stent implantation, stroke, development of heart failure, and occurrence of atrial fibrillation during follow-up. RESULTS: Patients who experienced MACE were older than those who did not. There was no difference in demographic and clinical parameters between MACE and non-MACE patients. There was no difference in RV diameter, but MACE patients had higher RV wall thickness. RV systolic function parameters were similar between the two groups. RV global and free-wall longitudinal strain were significantly lower in MACE patients (-22.3 ± 3.6 vs. -24.7 ± 3.9%, p < 0.001 and - 25.8 ± 4.2 vs. -28.1 ± 4.5%, p < 0.001; respectively). Reduced RV GLS [OR 1.10; 95%: 1.02-1.20] and reduced RV free-wall longitudinal strain [OR 1,21; 95%CI: 1.05-1.39] were independently of clinical and echocardiographic parameters related with adverse outcome measured by MACE. CONCLUSION: RV GLS and RV free-wall longitudinal strain were independently related with adverse outcomes during 10-year follow-up in initially uncomplicated hypertensive patients.

Hypertrophic cardiomyopathy and left ventricular non-compaction: Distinct diseases or variant phenotypes of a single condition?

Hypertrophic cardiomyopathy (HCM) is a genetically determined myocardial disease characterized by an increased thickness of the left ventricle (LV) wall that cannot be solely attributed to abnormal loading conditions. HCM may present with an intraventricular or LV outflow tract obstruction, diastolic dysfunction, myocardial fibrosis and/or ventricular arrhythmias. Differentiating HCM from other diseases associated with LV hypertrophy, such as hypertension, aortic stenosis, or LV non-compaction (LVNC), can at times be challenging. LVNC is defined by excessive LV trabeculation and deep recesses between trabeculae, often accompanied by increased LV myocardial mass. Previous studies indicate that the LVNC phenotype may be observed in up to 5% of the general population; however, in most cases, it is a benign finding with no impact on clinical outcomes. Nevertheless, LVNC can occasionally lead to LV systolic dysfunction, manifesting as a phenotype of dilated or non-dilated left ventricular cardiomyopathy, with an increased risk of thrombus formation and arterial embolism. In extreme cases, where LVNC is associated with a very thickened LV wall, it can even mimic HCM. There is growing evidence of an overlap between HCM and LVNC, including similar genetic mutations and clinical presentations. This raises the question of whether HCM and LVNC represent different phenotypes of the same disease or are, in fact, two distinct entities.

Papillary Craniopharyngioma: an integrative and comprehensive review.

Papillary craniopharyngioma (PCP) is a rare type of tumor, comprising ∼20% of all craniopharyngioma (CP) cases. It is now recognized as a separate pathological entity from the adamantinomatous type. PCPs are benign tumors, classified as WHO grade 1, characterized by non-keratinizing squamous epithelium. They typically grow as solid and round papillomatous masses or as unilocular cysts with a cauliflower-like excrescence. PCPs primarily occur in adults (95%), with increased frequency in males (60%) and predominantly affect the hypothalamus. Over 80% of these tumors are located in the third ventricle, expanding either above an anatomically intact infundibulum (strictly third ventricle tumors) or within the infundibulo-tuberal region of the third ventricle floor. Clinical manifestations commonly include visual deficits and a wide range of psychiatric disturbances (45% of patients), such as memory deficits and odd behavior. MRI can identify up to 50% of PCPs by the presence of a basal duct-like recess. Surgical management is challenging, requiring complex approaches to the third ventricle and posing significant risk of hypothalamic injury. The endoscopic endonasal approach allows radical tumor resection and yields more favorable patient outcomes. Of intriguing pathogenesis, over 90% of PCPs harbor the somatic BRAFV600E mutation, which activates the mitogen-activated protein kinase (MAPK/ERK) signaling pathway. A phase 2 clinical trial has demonstrated that PCPs respond well to BRAF/MEK inhibitors. This comprehensive review synthesizes information from a cohort of 560 well-described PCPs and 99 large CP series including PCP cases published from 1856-2023 and represents the most extensive collection of knowledge on PCPs to date.

Bedside right ventricle quantification using three-dimensional echocardiography in children with congenital heart disease: A comparative study with cardiac magnetic resonance imaging.

BACKGROUND: Accurate quantification of right ventricular (RV) volumes and function is crucial for the management of congenital heart diseases. AIMS: We aimed to assess the feasibility and accuracy of bedside analysis using new RV quantification software from three-dimensional transthoracic echocardiography in children with or without congenital heart disease, and to compare measurements with cardiac magnetic resonance imaging. METHODS: We included paediatric patients with congenital heart disease (106 patients) responsible for RV volume overload and a control group (30 patients). All patients underwent three-dimensional transthoracic echocardiography using a Vivid E95 ultrasound system. RV end-diastolic and end-systolic volumes and RV ejection fraction were obtained using RV quantification software. Measurements were compared between RV quantification and cardiac magnetic resonance imaging in 27 patients. RESULTS: Bedside RV quantification analysis was feasible in 133 patients (97.8%). Manual contour adjustment was necessary in 126 patients (93%). The mean time of analysis was 62±42s. RV end-diastolic and end-systolic volumes were larger in the congenital heart disease group than the control group: median 85.0 (interquartile range 29.5) mL/m2 vs 55.0 (interquartile range 20.5) mL/m2 for RV end-diastolic volume and 42.5 (interquartile range 15.3) mL/m2 vs 29.0 (interquartile range 11.8) mL/m2 for RV end-systolic volume, respectively. Good agreement for RV end-diastolic and end-systolic volumes and RV ejection fraction was found between RV quantification and magnetic resonance imaging measurements. RV quantification software underestimated RV end-diastolic volume/body surface area by 3mL/m2 and RV ejection fraction by 2.1%, and overestimated RV end-systolic volume/body surface area by 0.2mL/m2. CONCLUSIONS: We found good feasibility and accuracy of bedside RV quantification analysis from three-dimensional transthoracic echocardiography in children with or without congenital heart disease. RV quantification could be a reliable and non-invasive method for RV assessment in daily practice, facilitating appropriate management and follow-up care.

The effects of standardized intravenous treprostinil in pulmonary arterial hypertension patients after total cavo-pulmonary connection procedure.

OBJECTIVE: Total cavo-pulmonary connection (TCPC) is a palliative treatment for single ventricular malformations. For high-risk patients (preoperative mean pulmonary arterial pressure, mPAP > 15 mmHg), between the inhaled and oral targeted medications, the application of intravenous treprostinil as a bridge therapy to achieve "seamless" management is core postoperative treatment. This study intends to explore the effect of different administration regimens on early postoperative recovery. METHODS: This was a retrospective cohort study. High-risk pediatric patients (age ≤ 14 years) who underwent TCPC procedure in Fu Wai Hospital from 2015 to 2022 were included. Since the regimen of treprostinil was standardized in our center in 2021, the patients in 2020 and before were included in group 1, patients in 2021 and 2022 were included in group 2. The hemodynamic parameters were compared before and after the maintenance dose of treprostinil. The differences of demographic characteristics, surgical data and postoperative recovery were compared between the two groups. RESULTS: A total of 51 pediatric patients were included. Group 1 included 35 patients who received treprostinil at 1-3 postoperative days and an average dose of 12 ± 4 ng/(kg·min). Group 2 included 16 patients who received treprostinil within postoperative 1 day and an average dose of 22 ± 7 ng/(kg·min). There were no significant differences between the two groups in terms of age, weight, preoperative percutaneous oxygen saturation and mPAP,  heterotaxy syndrome, TCPC procedure type, other concurrent procedure, cardiopulmonary bypass time and aortic cross-clamp proportion (p > 0.05). After 24 h of treprostinil treatment, the mPAP in group 1 reduced from 17 ± 3 mmHg to 15 ± 2 mmHg (p < 0.001), and in group 2 from 17 ± 2 mmHg to 14 ± 2 mmHg (p < 0.001), with no difference between groups. In the postoperative recovery, patients in Group 2 exhibited a reduced duration of mechanical ventilation, 19 (11, 25) hours vs 69 (23, 189) hours, p = 0.001; a shorter stay in the ICU, 8 (6, 12) days vs 16 (9,26) days, p = 0.006; and a shorter postoperative length of stay, 27 (17,55) days vs 39 (29,58) days, p = 0.032. Patients in Group 2 also exhibited a lower incidence of thromboembolic events, 0 (0/26) vs 26% (9/35), p = 0.043; and the need for renal replacement therapy, 0 (0/26) vs 31% (11/35), p = 0.011. CONCLUSION: Treprostinil reduces pulmonary artery pressure after TCPC procedure. The standardized application of treprostinil may improve the postoperative recovery which should be proven by randomized controlled trials or matched cohort studies in the future.

Fontan Conversion in an Adult With Hypoplastic Left Heart Syndrome: A 38-Year-Old Norwood Success.

A 38-year-old man with a functional single ventricle secondary to hypoplastic left heart syndrome presented with exertional fatigue. His last palliation was an intra-atrial conduit Fontan procedure. Comprehensive evaluation showed elevated liver enzyme values and a small, calcified conduit. Successful conversion to a nonfenestrated extracardiac conduit Fontan was performed with normalization of his liver enzyme values.

Prenatally Diagnosed Case of Successful Survival of an Unguarded Mitral Valve Orifice.

An unguarded mitral valve orifice is a rare condition characterized by a thinned, hypocontractile left ventricle on fetal echocardiogram. This is the first report of an unguarded mitral valve orifice with a double-outlet right ventricle and intact ventricular septum diagnosed prenatally from these typical ultrasound features.

Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease.

BACKGROUND: Ineffective right ventricular (RV) adaptation to increasing pulmonary arterial (PA) afterload in pulmonary vascular disease (PVD) significantly contributes to morbidity and mortality. PVD in systemic sclerosis (SSc) arises through various mechanisms, yet detecting abnormal contractile response remains challenging. Here, we examine whether echocardiographic RV-PA coupling metrics correlate with invasive pressure-volume (PV) loops, enhancing the prediction of adverse clinical outcomes in SSc-PVD patients. METHODS: Prospectively enrolled patients with SSc-PVD with paired echocardiogram and PV loops were included. Linear regression and receiver-operating curve (ROC) analysis were used to assess the relationship between tricuspid annular plane systolic excursion (TAPSE)/PA systolic pressure (PASP), fractional area change (FAC)/PASP, tissue Doppler velocity (TDI S')/PASP, RV free wall strain (RVFWS)/PASP and coupling thresholds defined by end-systolic to end-arterial elastance (Ees/Ea), obtained by the multi-beat method. The contribution of right atrial strain (RAS) to RV-PA coupling parameters was also investigated. Kaplan-Meier analysis was used to identify the relationship between coupling ratios and composite outcomes including clinical worsening, lung transplant, and death. RESULTS: 42 patients with SSc were studied with mean age 59 ± 12 years, 91% female and varying degrees of PVD: mPAP 29.5 ± 12.8 mmHg, PVR 4.7 ± 4.2 WU, PCWP 10.3 ± 4.1 mmHg. Echocardiographic coupling metrics including TAPSE/PASP, FAC/PASP, TDI S'/PASP, RVFWSglobal and RVFWSbasal/PASP, and RASreservoir/PASP were linearly associated with Ees/Ea. At cut-points obtained through ROC analysis, all ratios were predictive of RV-PA uncoupling, defined by Ees/Ea, and composite outcomes. Additionally, RASreservoir/RVFWS correlated with Ees/Ea even after adjustment for PASP, suggesting that diminished RAS further impacts RV performance and coupling. CONCLUSION: Echocardiographic RV-PA coupling ratios strongly correlate with invasive Ees/Ea and predict adverse clinical outcomes in SSc patients across the spectrum of PVD. Further, we demonstrate how RAS impacts RV performance. These findings may refine risk stratification and prognostication in this at-risk cohort.

Hepatic T1 Mapping as a Novel Cardio-Hepatic Axis Imaging Biomarker Early after STEMI.

BACKGROUND: The hepatic response after ST-elevation myocardial infarction (STEMI) may be associated with mortality and morbidity. We aimed to assess the cardio-hepatic axis post-STEMI using cardiovascular magnetic resonance (CMR). METHODS: This prospective, observational, single-centre study included consecutive STEMI patients who underwent CMR after primary angioplasty from January 2015 to January 2019. Standard infarct characteristics were analysed, and hepatic T1 and hepatic extracellular volume (ECV) were assessed using pre- and post-contrast T1-mapping sequences. The primary endpoint was the relationship between native hepatic T1-values and ischemic right ventricular (RV) involvement, determined by RV ejection fraction (EF) dysfunction and/or the presence of RV acute myocardial infarction (AMI). The diagnostic performance of hepatic T1 values for detecting RV involvement was assessed using the area under the receiver operating characteristic curve (AUC). RESULTS: Of 177 consecutive STEMI patients undergoing CMR, 142 were included. Patients with RV ischemic involvement, compared to those without, had significantly higher native hepatic T1 (p < 0.001) and hepatic ECV (p = 0.016). Hepatic T1 values demonstrated a good diagnostic performance in detecting RV involvement (AUC 0.826, p < 0.001) and correlated positively with NT-proBNP values (r = 0.754, p < 0.001). Patients with high hepatic T1 values (>605 ms) had significantly higher NT-proBNP levels (<0.001), larger RV end-diastolic volume (p < 0.001), lower RV-EF (p < 0.001), and a higher prevalence of RV AMI (p = 0.022) compared to those with hepatic T1 ≤ 605 ms, while left ventricular EF and infarct size were similar. Multivariable logistic regression analysis identified RV-EF (p = 0.010) and NT-proBNP values (p < 0.001) as independent predictors of increased hepatic T1 values. Patients with increased hepatic T1 values had a higher rate of re-hospitalization for heart failure at 17-month follow-up (12.1% vs 2.0%, p = 0.046). CONCLUSIONS: Hepatic T1 mapping has emerged as a possible novel imaging biomarker of the cardio-hepatic axis in STEMI, being associated with RV involvement and increased NT-proBNP values.

Assessment of Biventricular Systolic and Diastolic Function Using Conventional and Strain Echocardiography in Children with Sickle Cell Disease Surviving 1-year After Hematopoietic Stem Cell Transplant.

Hematopoietic stem cell transplant (HSCT) is a potentially curative therapy for children with sickle cell disease (SCD). The effects of HSCT on ventricular function are not well characterized in children with SCD. Echocardiograms from children with SCD who underwent HSCT between 2007 and 2017 were retrospectively analyzed before and 1-year after HSCT. Left ventricular (LV) volumes, mass, and ejection fraction were calculated by the 5/6 area*length method. LV end-diastolic and systolic dimensions, septal, and posterior wall thickness, and fractional shortening were measured by M-mode. Mitral and tricuspid inflow Dopplers (E and A waves) as well as mitral, tricuspid, and septal tissue Dopplers (E', A') were assessed. E/A, E'/A' and E/E' ratios were calculated. Biventricular strain imaging was performed using speckle-tracking echocardiography. Peak global systolic longitudinal and circumferential LV strain, and global longitudinal right ventricular strain, as well as early and late diastolic strain rate, were measured on LV apical 4-chamber, LV short-axis mid-papillary, and RV apical views, respectively. Forty-seven children (9.7 ± 5.5 years, 60% male) met inclusion criteria. Pre-HSCT, subjects had mild LV dilation with normal LV systolic function by conventional measure of ejection fraction and fractional shortening. There was a significant reduction in LV volume, mass, and ejection fraction after HSCT, but measurements remained within normal range. LV longitudinal and circumferential strain were normal pre-HSCT and showed no significant change post-HSCT. RV strain decreased after HSCT, but the absolute change was small, and mean values were normal both pre- and post-HSCT. Conventional measures of diastolic function were all normal pre-HSCT. Post-HSCT there was a reduction in select parameters, but all parameters remained within normal range. Early and late diastolic strain rate parameters showed no significant change from pre- to post-HSCT. At one-year after HSCT in children with SCD conventional measures of systolic and diastolic function are within normal limits. Except for a small decrease in RV systolic strain with values remaining within normal limits, systolic strain and diastolic strain rate values did not significantly change 1-year after HSCT.

Distinction of papillary and adamantinomatous craniopharyngioma: Clinical features, surgical nuances and hypothalamic outcomes.

OBJECTIVE: Understanding the differences of suprasellar papillary and adamantinomatous craniopharyngiomas (PCPs/ACPs) is pivotal for target therapy, surgical strategy or postoperative management. Here, the clinical features, surgical nuances and postoperative hypothalamic outcomes of PCPs were systematically recapitulated. METHODS: 24 PCPs and 52 ACPs underwent initial surgery were retrospectively reviewed. Clinical data, quantified third ventricle (3rd V) occupation and optic chiasm distortion were compared, as well as intra-operative findings, operating notes and prognosis. Moreover, analysis of tumor/3rd V relationship and hypothalamic outcomes were also performed. RESULTS: Tumors were more likely to occupies the 3rd V cavity in PCPs. Chiasm distortion of "compressed forward" was the most common pattern (45.8 %) in PCPs, whereas "stretched forward" pattern accounted the highest (42.5 %) in ACPs. Besides, round-shaped with less calcification, duct-like recess, solid consistency, rare subdiaphragmatic invasion, visible lower stalk and improved postoperative visual outcome were more frequently observed in PCPs. The basal membranes of the tumor epithelium and the reactive gliosis were separated by a layer of collagen fibers in most PCPs, which differs from ACPs in the morphological examination of tumor/3rd V floor interface. In daytime sleepiness and memory difficulty, the PCPs showed significantly better outcomes than the ACPs groups, and PCPs suffered less postoperative weight gain (p < 0.05) than ACPs among adult-onset cases. CONCLUSION: PCPs are different from ACPs regards the clinical features, operative techniques and outcomes. If necessary, PCPs are suggested more amenable to total removal since its less invasiveness to the 3rd V floor and better hypothalamic outcomes.

High on sparsity: Interbin compensation of cardiac motion for improved assessment of left-ventricular function using 5D whole-heart MRI.

PURPOSE: Cardiac magnetic resonance is the gold standard for evaluating left-ventricular ejection fraction (LVEF). Standard protocols, however, can be inefficient, facing challenges due to significant operator and patient involvement. Although the free-running framework (FRF) addresses these challenges, the potential of the extensive data it collects remains underutilized. Therefore, we propose to leverage the large amount of data collected by incorporating interbin cardiac motion compensation into FRF (FRF-MC) to improve both image quality and LVEF measurement accuracy, while reducing the sensitivity to user-defined regularization parameters. METHODS: FRF-MC consists of several steps: data acquisition, self-gating signal extraction, deformation field estimations, and motion-resolved reconstruction with interbin cardiac motion compensation. FRF-MC was compared with the original 5D-FRF method using LVEF and several image-quality metrics. The cardiac regularization weight ( λ c $$ {\lambda}_c $$ ) was optimized for both methods by maximizing image quality without compromising LVEF measurement accuracy. Evaluations were performed in numerical simulations and in 9 healthy participants. In vivo images were assessed by blinded expert reviewers and compared with reference standard 2D-cine images. RESULTS: Both in silico and in vivo results revealed that FRF-MC outperformed FRF in terms of image quality and LVEF accuracy. FRF-MC reduced temporal blurring, preserving detailed anatomy even at higher cardiac regularization weights, and led to more accurate LVEF measurements. Optimized λ c $$ {\lambda}_c $$ produced accurate LVEF for both methods compared with the 2D-cine reference (FRF-MC: 0.59% [-7.2%, 6.0%], p = 0.47; FRF: 0.86% [-8.5%, 6.7%], p = 0.36), but FRF-MC resulted in superior image quality (FRF-MC: 2.89 ± 0.58, FRF: 2.11 ± 0.47; p < 10-3). CONCLUSION: Incorporating interbin cardiac motion compensation significantly improved image quality, supported higher cardiac regularization weights without compromising LVEF measurement accuracy, and reduced sensitivity to user-defined regularization parameters.

Trans-septal left ventricular endocardial lead in a patient with extensive anterior myocardial infarction and left ventricle (LV) apical endoventriculoplasty using a Vascutek patch-case report.

Background: Cardiac resynchronization therapy (CRT) implantation has significantly improved quality of life and reduced overall mortality due to heart failure. The conventional method of CRT implantation is implanting a left ventricle (LV) lead into a side branch of the coronary sinus (CS) tributary to pace the epicardial surface and capture the LV. This is safe, and well tolerated with a high success rate. The rate of failure to place an LV lead has decreased over time, however, there are still challenging cases where a conventional CRT implant fails and alternative techniques are being considered, one such technique is trans-septal endocardial LV lead placement used to capture the LV, endocardially but its use is limited due to lack of evidence, practice uptake and clinical trials. Case Description: We present, a case report of a patient for whom we successfully used a trans-septal left ventricle (TSLV) endocardial lead implantation approach following a failed LV lead implant via the CS to get effective cardiac resynchronisation. Conclusions: Post-TSLV lead implantation follow-up checks were normal with good electrical parameters and appropriate biventricular pacing. No post-procedural complications were reported, and echocardiographic parameters improved at follow-up. We believe, although, TSLV lead implant is more complex and often double operators are required, in selected patients, it can be a safe alternative following a failed traditional LV lead implant via the CS.

Right ventricular function in mitral stenosis: plays a fundamental role.

The importance of the right ventricle (RV) was neglected or forgotten for decades. The RV has an essential function in cardiovascular physiology and pathology. The RV dysfunction is one of the causes of morbidity and mortality in valvular heart disease (VHD), especially in mitral stenosis (MS). Right ventricular systolic and diastolic function are important for the determination of clinical symptoms, exercise functional capacity, pre-procedure survival, and post-procedure outcome in patients with MS. Right ventricular dysfunction in MS with a sign of systemic venous congestion is easy to recognize, but MS without clinical evidence of RV dysfunction has not been studied. Detecting RV dysfunction earlier in MS is important in clinical practice.

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.

OBJECTIVE: We aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation. METHODS: All infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index. RESULTS: A total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (p=0.926), right PA index (p=0.692), left PA index (p=0.297) and the symmetry index (p=0.650) were similar between the groups. At stage III Fontan completion, PA index (p=0.115), right PA index (p=0.868), and the symmetry index (p=0.144) were similar between the groups. However, left PA index (60 vs. 74mm2/m2, p=0.015) was lower, and the incidence of veno-venous collaterals (24.2 vs. 7.8%, p=0.036) was higher in DS group compared to SPS group. Freedom from PA interventions between stage II and III palliation was lower in DS group compared to SPS group (p=0.009). CONCLUSIONS: In patients after DS in infants with univentricular heart, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared to those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

Zebrafish as a Model System for Brugada Syndrome.

Brugada syndrome (BrS) is an inheritable cardiac arrhythmogenic disease, associated with an increased risk of sudden cardiac death. It is most common in males around the age of 40 and the prevalence is higher in Asia than in Europe and the United States. The pathophysiology underlying BrS is not completely understood, but several hypotheses have been proposed. So far, the best effective treatment is the implantation of an implantable cardioverter-defibrillator (ICD), but device-related complications are not uncommon. Therefore, there is an urgent need to improve diagnosis and risk stratification and to find new treatment options. To this end, research should further elucidate the genetic basis and pathophysiological mechanisms of BrS. Several experimental models are being used to gain insight into these aspects. The zebrafish (Danio rerio) is a widely used animal model for the study of cardiac arrhythmias, as its cardiac electrophysiology shows interesting similarities to humans. However, zebrafish have only been used in a limited number of studies on BrS, and the potential role of zebrafish in studying the mechanisms of BrS has not been reviewed. Therefore, the present review aims to evaluate zebrafish as an animal model for BrS. We conclude that zebrafish can be considered as a valuable experimental model for BrS research, not only for gene editing technologies, but also for screening potential BrS drugs.