Systemic Ventricle Research Articles

Biventricular performance in adults with a systemic right ventricle: new insights from myocardial work analysis.

To evaluate biventricular mechanics by means of echo-derived myocardial work (MW) analysis in patients with a systemic right ventricle (sRV). Comprehensive echo data were collected in all patients with a sRV who underwent transthoracic echocardiography at our tertiary centre between 2020 and 2021 including sRV function indices, global longitudinal strain (GLS) of right and left ventricle (RV/LV), biventricular MW, and atrial strain in those with congenitally corrected transposition of the great arteries (ccTGA). Fifty-six patients (37 [30.97-45.87]years, 59% male) and 49 healthy individuals matched per age and sex were included for comparison. Global work index (GWI:1106 [869.80-1293.10] Vs 314.2 [281.5-358.2]mmHg%,p < 0.0001) and global constructive work(GCW: 1542.50 [1338.9-1718.50] Vs 416.4 [365.70-464]mmHg%,p < 0.0001) were both increased for sRV compared to normal RV, reflecting exposition to a systemic afterload, with a contemporary raise in wasted work (GWW:197 [138.50-322.20] Vs 26.09 [17.80-43.48]mmHg%,p < 0.0001) and impaired efficiency (GWE:89 [83-93.54] Vs 93.67 [91.67-96] %,p < 0.0001). Conversely, sRV showed reduced MW indices in comparison to normal LV(p < 0.0001 for all). Non-systemic LV demonstrated normal GLS values (19.51 ± 3.9%), but reduced GWI (479 [368-665] Vs 2172 [1978-2386]mmHg%,p < 0.0001) and GCW (708 [490-815]mmHg% Vs 86.5 [59.25-118],p < 0.0001). Nevertherless, non-systemic LV showed also impaired efficiency (91 [88-94] Vs 95 [94-97]%,p < 0.0001). LVGLS values were related to RVGLS (R = 0.5,p = 0.00019), suggesting a consistent interventricular dependency. Atrial strain could be assessed in 16 out of 20 (80%) patients with ccTGA: both atria had reduced strain values compared to their normal counterparts. Moreover, pulmonary atrial strain during the reservoir phase was related to LVGWE (R = 0.58,p = 0.047) and inversely related to LVGLS (R=-0.71,p = 0.0043). MW analysis is feasible in sRV and may provide additional clinical data. In our cohort MW revealed biventricular impairment, in particular for non-systemic LV, in spite of normal GLS values.

Progress in Managing Heart Failure: an in-depth Review of Sacubitril/Valsartan Combination Therapy

Abstract Heart failure is a severe cardiovascular condition when the heart fails to adequately pump blood or oxygen to support the body’s organs. Sacubitril/valsartan, an emerging angiotensin receptor-neprilysin inhibitor (ARNI) known for its superior efficacy to conventional treatments, has revolutionized the management of heart failure. The United States Food and Drug Administration has approved the drug’s use in treating patients with chronic heart failure with reduced ejection fraction (HFrEF). Clinical trials have demonstrated its effectiveness in reducing hospitalizations and mortality rates associated with heart failure across various patient populations, including those with low ejection fraction. Numerous significant clinical trials have investigated the drug’s safety and efficacy in treating individuals with heart failure and a failing systemic right ventricle. This review delves into the current landscape of cardiovascular medicine, the role of pharmacology, and the safety profile of this medication.

Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction. However, in adult congenital heart disease, the clinical significance of deformation imaging has not been systematically assessed and remains unclear. According to the Preferred Reporting Items for Systematic Reviews checklist, this systematic review included studies that reported on the prognostic value of echocardiographic left and/or right ventricular strain by 2-dimensional speckle tracking for hard clinical end-points (death, heart failure hospitalization, arrhythmias) in the most frequent forms of adult congenital heart disease. In total, 19 contemporary studies were included. Current data shows that left ventricular and right ventricular global longitudinal strain (GLS) predict heart failure, transplantation, ventricular arrhythmias and mortality in patients with Ebstein's disease and tetralogy of Fallot, and that GLS of the systemic right ventricle predicts heart failure and mortality in patients post atrial switch operation or with a congenitally corrected transposition of the great arteries. Deformation imaging can potentially impact the clinical decision making in ACHD patients. Further studies are needed to establish disease-specific reference strain values and ranges of impaired strain that would indicate the need for medical or structural intervention.

High-sensitivity troponin increase after exercise in patients with a systemic right ventricle.

High-sensitivity troponin increase after exercise in patients with a systemic right ventricle.

Experience of Morphologic Left Ventricle Training in Congenitally Corrected Transposition of the Great Arteries in Children: Evaluation of the Operation Results

The aim. To evaluate the efficacy of morphologic left ventricle (mLV) training through pulmonary artery banding (PAB) in pediatric patients with congenitally corrected transposition of the great arteries.&#x0D; Materials and methods. From 2003 to 2023, 12 patients underwent PAB for mLV training at the Ukrainian Children`s Cardiac Center. The mean age of the patients was 30.3 ± 25.1 months, the mean weight was 11.6 ± 5.2 kg. Hemodynamic parameters were assessed to determine the size of the band applied to the pulmonary artery (PA) trunk during the operation. Intraoperative transesophageal echocardiography was performed in 6 patients. Transthoracic echocardiography (TTE) was performed in the early postoperative period. In the remote period, all the patients underwent TTE and X-ray angiography.&#x0D; Results. During PAB, the mLV pressure averaged 58 ± 12.6% (from 40% to 80%) of the systemic arterial pressure. PAB dilatation was required for one patient in the early postoperative period (postoperative day 2) because of significant hemodynamic changes. In the early postoperative period, the maximum gradient across PA measured by TTE averaged 39.3 ± 10.0 mm Hg (from 25 to 60 mm Hg). There was no hospital mortality.&#x0D; The follow-up period averaged 47.2 ± 26.0 months (8 to 113 months). In the long-term observation period, 2 patients (16.6%) died, one 8 months and the other 5 years after the operation. The double switch operation was performed in 6 patients, on average, 48.2 ± 6.9 months after PAB. According to angiography, the mLV pressure before the double switch operation was 90.2 ± 11.6% of that in the systemic right ventricle. The end-diastolic pressure in the mLV averaged 14 ± 4.1 mm Hg. The peak pressure gradient across the PA measured by echo was 81.3 ± 28.8 mm Hg. The mLV mass index averaged 62.9 ± 8.8 g/m2, the mLV ejection fraction averaged 61.2 ± 2.9%. Overall radiographic and echocardiographic data in these patients indicated that mLV is trained and capable of performing the role of a systemic ventricle after anatomical correction.&#x0D; Conclusions. PA banding as a method of mLV training can be used in patients with congenitally corrected transposition of the great arteries with intact ventricular septum, restrictive ventricular septal defects and severe tricuspid regurgitation.&#x0D; To evaluate the effectiveness of PAB for mLV training, a multimodal diagnostic approach is used, including non-invasive and invasive methods such as TTE, transesophageal echocardiography, and cardiac catheterization. The optimal age for PAB in patients of this group for the purpose of training the LV was on average 2-2.5 years.

Myocardial work of the systemic right ventricle and its association with outcomes.

We aimed to evaluate clinical and prognostic significance of myocardial work parameters of the systemic right ventricle (SRV). Thirty-eight patients with the SRV underwent echocardiographic assessment of the SRV systolic function including 3D-echocardiography derived ejection fraction, 2D longitudinal strain and myocardial work analysis. The study endpoint was the combination of all-cause mortality and heart transplantation. Global constructive work (GCW) and global work index (GWI) demonstrated moderate correlation with the 3DE-derived SRV ejection fraction (EF) (Rho 0.64, p<0.0001 and Rho 0.63, p<0.0001, respectively). GCW showed the strongest correlation with the BNP level (Rho -0.77, p<0.0001), closely followed by GWI, 4-chamber longitudinal strain and 3DE EF (all Rho -0.73, p<0.0001). GCW and GWI were significantly lower in patients with moderate or severe tricuspid regurgitation compared with less than moderate regurgitation (1226±439 vs 1509±264mmHg%, p=0.02, and 984±348 vs 1259±278mmHg%, p=0.01, respectively). During a follow-up of 3.5 (2.8-3.9) years, seven patients (18%) died and one received transplantation (3%). They had significantly lower GCW and GWI compared with patients who did not reach the study endpoint (908±255 vs 1433±%, p<0.001 and 721±210 vs 1173±315 mmHg%, p<0.001, respectively). In Cox regression analysis, GCW, GWI, 3DE SRV volumes and EF were the best-fit models based on the Akaike Information Criterion, outperforming longitudinal strain parameters. GWI and GCW, novel echocardiographic parameters of myocardial work, provided reliable quantification of the SRV systolic function. GWI, GCW and 3DE-derived SRV parameters were closely associated with all-cause mortality and heart transplantation in patients with the SRV.

Detection of occult thrombosis in individuals with Fontan circulation by cardiac MRI.

Identifying thrombus formation in Fontan circulation has been highly variable, with reports between 17 and 33%. Initially, thrombus detection was mainly done through echocardiograms. Delayed-enhancement cardiac MRI is emerging as a more effective imaging technique for thrombus identification. This study aims to determine the prevalence of occult cardiac thrombosis in patients undergoing clinically indicated cardiac MRI. A retrospective chart review of children and adults in the Duke University Hospital Fontan registry who underwent delayed-enhancement cardiac MRI. Individuals were excluded if they never received a delayed-enhancement cardiac MRI or had insufficient data. Demographic characteristics, native heart anatomy, cardiac MRI measurements, and thromboembolic events were collected for all patients. In total, 119 unique individuals met inclusion criteria with a total of 171 scans. The median age at Fontan procedure was 3 (interquartile range 1, 4) years. The majority of patients had dominant systemic right ventricle. Cardiac function was relatively unchanged from the first cardiac MRI to the third cardiac MRI. While 36.4% had a thrombotic event by history, only 0.5% (1 patient) had an intracardiac thrombus detected by delayed-enhancement cardiac MRI. Despite previous echocardiographic reports of high prevalence of occult thrombosis in patients with Fontan circulation, we found very low prevalence using delayed-enhancement cardiac MRI. As more individuals are reaching adulthood after requiring early Fontan procedures in childhood, further work is needed to develop thrombus-screening protocols as a part of anticoagulation management.

The effect of comprehensive management of heart failure in adult with systemic right ventricle.

The effect of comprehensive management of heart failure in adult with systemic right ventricle.

Single Ventricular Assist Device Care and Outcomes for Failed Stage I Palliation: A Single-Center Decade of Experience.

Single ventricular assist device (SVAD) use before and after stage I palliation (S1P) is increasing with limited data on outcomes. To address this knowledge gap, we conducted a single-center retrospective review to assess pre- and post-SVAD clinical status, complications, and outcomes. We leveraged a granular, longitudinal, local database that captures end-organ support, procedural interventions, hematologic events, laboratory data, and antithrombotic strategy. We identified 25 patients between 2013 and 2023 implanted at median age of 53 days (interquartile range [IQR] = 16-130); 80% had systemic right ventricles and underwent S1P. Median SVAD days were 54 (IQR = 29-86), and 40% were implanted directly from ECMO. Compared to preimplant, there was a significant reduction in inotrope use ( p = 0.013) and improved weight gain ( p = 0.008) post-SVAD. Complications were frequent including bleeding (80%), stroke (40%), acute kidney injury (AKI) (40%), infection (36%), and unanticipated catheterization (56%). Patients with in-hospital mortality had significantly more bleeding complications ( p = 0.02) and were more likely to have had Blalock-Thomas-Taussig shunts pre-SVAD ( p = 0.028). Survival to 1 year postexplant was 40% and included three recovered and explanted patients. At 1 year posttransplant, all survivors have technology dependence or neurologic injury. This study highlights the clinical outcomes and ongoing support required for successful SVAD use in failed single-ventricle physiology before or after S1P.

Impact of preoperative left pulmonary artery stenting on the Fontan procedure: a retrospective multicentre study.

Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. A multicentre retrospective analysis was performed on 304 consecutive Fontan patients. The study population was divided into 2 groups (LPA stented, n = 62 vs not stented, n = 242); pre-and postoperative data were compared. LPA-stented patients had a higher prevalence of systemic right ventricle (P = 0.01), hypoplastic left heart syndrome (P = 0.042), complex neonatal palliations (Norwood/Damus-Kaye-Stansel) and surgical LPA patch repair at Glenn (P < 0.001). No differences were found in cross-clamp rates, early (P = 0.29) and late survival (94.6% vs 98.4, P = 0.2) or complications (P = 0.14). Complex palliations on ascending aorta/aortic arch (P = 0.013) and surgical LPA repair at Glenn (P < 0.001) proved to be risk factors for LPA stenting before Fontan at multivariable analysis. The LPA-stented group showed similar outcomes in terms of survival and complications rate compared to patients without LPA stenosis; however, they significantly differ in their higher preoperative risk profile and in their more complex anatomy. Complex neonatal palliations involving ascending aorta or aortic arch may increase the risk of pulmonary branches stenosis requiring stenting; therefore, preoperative stenting of LPA stenoses could help to reduce the surgical risk of complex Fontan procedure by avoiding the need for cross-clamp or complex mediastinal dissections to perform a high-risk surgical repair.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The studyis aretrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1years) than those with d-TGA (37.5 ± 8.3years) or complex ccTGA (40.8 ± 13.4years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Multi-modality imaging of the systemic right ventricle in congenital heart disease.

A comprehensive and structured imaging approach in the evaluation of the systemic right ventricle (sRV) in patients with complete transposition of the great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of the great arteries (ccTGA) is a key for their optimal lifelong surveillance. Despite the improvements in cardiovascular imaging of adults with congenital heart disease (ACHD), the imaging of sRV remains an ongoing challenge due to its complex morphology and the difficulty in applying the existing knowledge for the systemic left ventricle. While cardiac magnetic resonance (CMR) is considered the gold standard imaging method, echocardiographic evaluation is primarily preferred in everyday clinical setting. Although qualitative assessment of its systolic function is primarily used, the introduction of advanced echocardiographic techniques, such as speckle tracking echocardiography (STE) and three-dimensional echocardiography (3DE), has provided new insights into the optimal assessment of the sRV. Standardized quantitative parameters remain to be elucidated, and morphometric and mechanistic studies are warranted to validate reference ranges for the sRV. This review highlights the challenges in the optimal evaluation of sRV and summarizes the available imaging tools. HIGHLIGHTS: CMR is the gold standard imaging method of sRV. Qualitative assessment of the systolic function of sRV is primarily used. Advanced echocardiographic techniques (STE and 3DE) provide optimal sRV assessment. Reference ranges for the sRV indices are warranted to be validated.

Kiosk 3R-TA-10 - t1mapping Predicts Outcome in Patients with Systemic Right Ventricles

Kiosk 3R-TA-10 - t1mapping Predicts Outcome in Patients with Systemic Right Ventricles

Kiosk 3R-TA-11 - t1mapping in Patients with a Systemic Right Ventricle: A Multicentric Study

Kiosk 3R-TA-11 - t1mapping in Patients with a Systemic Right Ventricle: A Multicentric Study

СОСТОЯНИЕ ДЫХАТЕЛЬНОЙ СИСТЕМЫ У ДЕТЕЙ С ГЕМОДИНАМИКОЙ ФОНТЕНА

Fontan procedure is a surgical operation for patients who were born with one working heart ventricle that involves complete separation of the pulmonary and the systemic circulation systems, which significantly improves their quality of life and becomes the preferred method of palliative surgery for congenital heart defects with univentricular hemodynamics. Despite all of its advantages this procedure is associated with a number of limitations dictated by the unique type of blood circulation. The uniqueness of Fontan hemodynamics lies in the fact that it is based on passive (non-pulsatile) pulmonary blood flow, which ensures gas exchange and preload of a single (systemic) ventricle. Pulmonary blood flow is determined by central venous pressure (CVP) and pulmonary vascular resistance. Therefore, systemic ventricular preload and cardiac output depend on well-running lung function coupled with low values of pulmonary vascular resistance. Various pulmonary anatomical or pathophysiological changes may compromise the optimal Fontan circulation. This bibliographical review represents currently known data on cardiopulmonary interactions within Fontan circulation, the causes for and the variants of the pulmonary dysfunction.

Outcomes of cardiac resynchronization therapy in congenital heart disease: A meta-analysis and systematic review.

Cardiac resynchronization therapy (CRT) is a standard treatment for patients with heart failure with reduced ejection fraction. However, there is still a gap of evidence in congenital heart disease (CHD) patients regarding resynchronization therapy. We performed a meta-analysis and systematic review of CHD patients who received CRT implantation. We comprehensively searched the databases of MEDLINE, EMBASE, and Cochrane database from inception to June 2023. Studies that reported response rate to CRT, total mortality rate, change in QRS duration, change in left ventricular ejection fraction, and change in New York Heart Association functional class were included. A total of 14 studies were included in the study. There were 10 studies that reported response rates after implantation. The overall response rate to CRT in CHD patients was 68% (95% confidence interval [CI] 61%-75%, I2 32%). The response rates in patients with systemic right ventricle (RV), systemic left ventricle (LV), and single ventricle were 58% (95% CI 46%-70%, I2 0%), 80% (95% CI 74%-86% I2 14%), and 67% (95% CI 49%-80% I2 0%). Response to CRT in systemic RV was inferior to systemic LV with an odds ratio of 0.38 (95% CI 0.15-0.95, I2 38%). The total mortality rate from sevenstudies was 12% (95% CI 8%-18%, I2 55%). The parameters which represented ventricular dyssynchrony improved after CRT implantation. The overall response rate to CRT in CHD was 68%. Patients with systemic RV had a lower response rate to CRT when compared to patients with systemic LV. The total mortality rate after CRT implantation was 12%.

Techniques for Cardiac Resynchronization Therapy in Patients with Congenital Heart Disease.

Cardiac resynchronization therapy (CRT) for congenital heart disease has shown promising suucess as an adjunct to medical therapy for heart failure. While cardiac conduction defects and need for ventricular pacing are common in congential heart disease, CRT indications, techniques and long term outcomes have not been well establaished. This is a review of the techniques nad short term outcomes of CRT for the following complex congenital heart disease conditions: single ventricle physiology, systemic right ventricle, and the subpulmonic right ventricle.

Prognostic utility of a risk prediction model for predischarge major residual lesions or unplanned reinterventions following congenital mitral valve repair

ObjectiveWe sought to develop a risk prediction model for predischarge major mitral valve (MV) residual lesions or unplanned MV reinterventions following congenital MV repair. MethodsPatients who underwent congenital MV repair (excluding primary repair, but including secondary repair, of canal-type defects) at a single institution from January 2000 to December 2020 and survived to discharge were retrospectively reviewed. The primary outcome was major MV residua (mean gradient >6 mm Hg or moderate or greater regurgitation on the discharge echocardiogram) or predischarge unplanned MV reintervention. Risk factors of interest included age, single-ventricle physiology, preoperative and intraoperative postrepair MV stenosis and regurgitation severity, MV annular diameter z score, systemic ventricle ejection fraction, unfavorable anatomy, concomitant left-heart procedure, and various technique-related categories. Logistic regression was used to develop a weighted risk score for the primary outcome. Internal validation using bootstrap-resampling was performed. ResultsOf 866 patients who underwent congenital MV repair at a median age of 2.7 years (interquartile range, 0.7-9.1 years), 202 (23.3%) patients developed the primary outcome. The final risk prediction model had a C-statistic of 0.82 (95% confidence interval, 0.78-0.85). A weighted risk score was formulated per the variables in this model. The median risk score was 8 (interquartile range, 6-11) points. Patients were categorized as low (score 0-5), medium (score 6-10), high (score 11-15), or very high (score ≥16) risk. The probability of the primary outcome was 5.0 ± 1.7%, 15.2 ± 6.7%, 45.9 ± 12.6%, and 76.7 ± 8.8% for low-, medium-, high-, and very-high-risk patients, respectively. ConclusionsOur risk prediction model may guide prognostication of patients following congenital MV repair.

Continuous monitoring with implantable loop recorder detects arrhythmic risk in adults with systemic right ventricle following atrial switch

Abstract Background Patients with a systemic right ventricular circulation following atrial switch are at high risk of sudden death despite current monitoring strategies and advances in heart failure management. Arrhythmia represents a significant risk factor and early detection and intervention is of great value. The current surveillance approach with intermittent ambulatory monitoring is limited and cumbersome. Purpose We hypothesise that continuous rhythm monitoring with an implantable loop recorder (ILR) in the systemic right ventricle population will allow early detection of clinically significant arrhythmias. Methods Patients under follow up with a systemic right ventricular circulation following atrial switch were offered ILR implantation as an alternative to the standard of care (ambulatory 24-hour ECG). Patients with pre-existing intra-cardiac pacing devices or palliative were excluded. Following implantation, recordings made with symptoms or automatically following detection of significant arrhythmias. This included pauses over 3 seconds, atrioventricular block or supraventricular tachycardia. Transmission were reviewed daily and significant findings discussed in an adult congenital heart disease and electrophysiology MDT. Patients otherwise had routine clinical care. Results 24 patients had implantation of a loop recorder (67% of eligible cohort). They were aged 29-49 years (median 35 years) and 50% had additional lesions. Right ventricular function was normal in 42% and 29% had moderate or severe tricuspid regurgitation. 75% of the cohort were NYHA classification I. All but one patient were in sinus rhythm and 8 (33%) had previous intra-atrial re-entry tachycardia (IART). No patient had previously documented significant conduction disease. Implants were undertaken over 6 years without complications. 18 patients (75%) had automated or symptom activated recordings (52% automated) with a median of 2.8 recordings per patient (range 0 – 8 per patient) over 39.5 months (1.6 – 72.5 months). The recordings were clinically significant in 64% of the cohort (Table). This included 2 (9% of entire cohort) patients with atrioventricular block only, 11 patients (46%) with IART only and 4 patients (17%) with both IART and conduction disease (1 with atrioventricular block). These recordings resulted in 5 pacemaker implants (3 for atrioventricular block) and 2 further patients declined a device (1 subsequent death). 4 patients underwent, or are awaiting, ablation for IART. Risk scoring for sudden death did not predict arrhythmia events or atrioventricular block (1) (Figure). Conclusions Empirical ILR implantation in this low-risk systemic right ventricle cohort was well tolerated and detected a high burden of significant arrhythmias requiring intervention. Arrythmias were not predicted by risk scoring. This monitoring approach is an effective alternative to intermittent ambulatory surveillance however the impact on sudden death is unclear.

Prognostic value and determinants of high sensitive cardiac Troponin levels in patients with a systemic right ventricle: insights from the SERVE-Trial

Abstract Background The prognostic value of high-sensitivity cardiac Troponin T (hs-cTnT) in patients with a systemic left ventricle is well-established. However, the determinants and prognostic value of hs-cTnT among patients with a systemic right ventricle (sRV) are largely unknown. Purpose We aimed to assessed the determinants and prognostic value of hs-cTnT among patients with sRV. Methods Ninety-eight patients from the randomized-controlled SERVE-trial were included. The correlation between baseline hs-cTnT concentrations and bi-ventricular volumes and function quantified by cardiac magnetic resonance was assessed by adjusted linear regression models. The prognostic value of hs-cTnT and other established prognostic markers were assessed by adjusted cox proportional hazards models, survival analysis and c-statistics. The primary outcome was the composite of clinically relevant arrhythmia, hospitalization for heart failure or all-cause death. Results Median age [interquartile range, IQR] was 39 [32, 48] years and 32% were female. Median hs-cTnT concentration was 7 [IQR, 4-11] ng/L. Coefficients of determination for the relationship between hs-cTnT concentrations and right ventricular end-systolic volume index, (RVESVi) and ejection fraction (RVEF) were +0.368, p=0.046; and -0.381, p=0.018, respectively. The sex and aged adjusted hazard ratio [95%, confidence interval, CI] of hs-cTnT at 2- and 4-times the reference level (5 ng/l) for the primary outcome were 2.89 [1.14-7.29] and 4.42 [1.21-16.15], respectively (Figure 1). Patients with hs-cTnT concentrations ≤7 ng/L (median) had better event-free survival (89% vs. 67%, p=0.004). The prognostic performance quantified by the C-statistics [95%, CI] for age and sex adjusted models based on hs-cTnT, RVEF, and peak VO2 predicted (%) were comparable: 0.71 [0.61-0.82], 0.72 [0.59-0.84], and 0.71 [0.59-0.83], respectively (Figure 2). Conclusion Hs-cTnT concentration was significantly correlated with RVEF and RVESVi in patients with a sRV. The prognostic accuracy of this biomarker was comparable to that of the current gold standards (RVEF and peak VO2 predicted).Figure 1Figure 2