Rapid progression of aortic and mitral stenosis in a patient with AA amyloidosis: a case report

Background: Aortic stenosis is a common finding in cardiac amyloidosis (CA). Younger patients often remain asymptomatic. If unrecognized, this can lead to serious complications such as heart failure. Progression of aortic stenosis can be accelerated in patients with chronic kidney disease and need for dialysis. Perioperative risk in these patients is often high due to the underlying systemic disease. Case summary: A 40-year-old Caucasian man with known AA amyloidosis, highly active Ankylosing Spondylitis and need for chronic dialysis due to end-stage chronic renal failure presented for echocardiographic routine exam without reporting any cardiac symptoms. At the last visit 4 years ago, a normal heart valve function was noted and no echocardiographic follow-up was performed in the following. Now, rapid progression with severe aortic valve and mitral valve stenosis was stated and the patient underwent combined aortic and mitral surgical valve replacement following discussion in the multidisciplinary cardiology meeting. Macroscopic examination of the valves revealed significant calcification and histological examination showed the high presence of amyloid by Congo-red staining and immunohistological staining for AA-Amyloid. Both valve prosthetic devices showed normal function as well as a normal left ventricular ejection fraction in initial post-operative transoesophageal echocardiography. After prolonged and complicated post-operative course in the intensive care unit the patient died 3 months after surgery due to intractable multiorgan failure in combined severe abdominal septic and cardiogenic shock. Discussion: Concomitant CA and chronic dialysis can accelerate the onset of severe aortic valve stenosis. Young patients, as in this case, often stay asymptomatic, perioperative risk increases with duration of chronic dialysis and severity of valve stenosis. This increases the need for regular short-term echocardiographic examinations even in clinical stable patients

Impact of inferior caval valve implantation on severity of tricuspid regurgitation and right heart function

Aims: Severe tricuspid regurgitation (TR) is a common finding in heart failure patients and associated with increased mortality. New interventional therapeutic options are needed as many heart failure patients are unfit for surgery. The TRICAVAL study compared valve implantation into the inferior vena cava (CAVI) with optimal medical therapy (OMT) in patients with severe TR. Here, we report details on the impact of CAVI on TR severity as well as right heart function and morphology. Methods and results: We randomized 28 patients with severe TR to CAVI (n = 14) with transfemoral implantation of an Edwards Sapien XT valve into the inferior vena cava or OMT (n = 14). Inclusion and exclusion criteria were based on anatomical and clinical parameters. Echocardiographic measurements were performed at baseline, at the first postoperative day and one, three, and twelve months after randomization. As proof of concept of an effective sealing of the inferior vena cava, we detected a significant decrease in systolic hepatic vein reflux volume (11.0 [6.2-21.9] mL vs 3.5 [0.6-8.5] mL,P = .016) and hepatic vein diameter (11.5 [10.0-14.8] mm vs 10.0 [9.3-11.8] mm,P = .034) at thirty-day follow-up. However, CAVI had no significant impact on TR, cardiac function, and morphology. Conclusions: Caval valve implantation significantly reduced systolic reflux into the hepatic veins but was not associated with an improvement in cardiac function, morphology, or TR severity

Renal and hepatic function of patients with severe tricuspid regurgitation undergoing inferior caval valve implantation

Due to progressive abdominal-venous congestion severe tricuspid regurgitation (TR) is a common cause of cardiorenal and cardiohepatic syndrome. We initiated the TRICAVAL study to compare interventional valve implantation into the inferior vena cava (CAVI) versus optimal medical therapy (OMT) in severe TR. In the present subanalysis, we aimed to evaluate the effects of CAVI on clinical signs of congestion, renal and hepatic function. TRICAVAL was an investigator-initiated, randomized trial. Twenty-eight patients with severe TR were randomized to OMT or CAVI using an Edwards Sapien XT valve. Probands who completed the 3-month follow-up (CAVI [n = 8], OMT [n = 10]) were evaluated by medical history, clinical examination, and laboratory testing at baseline, 3 and 12 months. After 3 months, the CAVI group exhibited a significant reduction of body weight (from 80.7 [69.0-87.7] kg to 75.5 [63.8-84.6] kg, p < 0.05) and abdominal circumference (from 101.5 +/- 13.8 cm to 96.3 +/- 15.4 cm, p <= 0.01) and a trend to lower doses of diuretics compared to OMT. Renal and hepatic function parameters did not change significantly. Within a short-term follow-up, CAVI led to an improvement of clinical signs of venous congestion and a non-significant reduction of diuretic doses compared to OMT

Associations of 2D speckle tracking echocardiography-based right heart deformation parameters and invasively assessed hemodynamic measurements in patients with pulmonary hypertension

Background: We aimed to evaluate associations of right atrial (RA) and right ventricular (RV) strain parameters assessed by 2D speckle tracking echocardiography (2D STE) with invasively measured hemodynamic parameters in patients with and without pulmonary hypertension (PH). Methods: In this study, we analyzed 78 all-comer patients undergoing invasive hemodynamic assessment by left and right heart catheterization. Standard transthoracic echocardiographic assessment was performed under the same hemodynamic conditions. RA and RV longitudinal strain parameters were analyzed using 2D STE. PH was defined as invasively obtained mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest and was further divided into pre-capillary PH (pulmonary capillary wedge pressure [PCWP] ≤ 15 mmHg), post-capillary PH (PCWP > 15 mmHg) and combined PH (PCWP > 15 mmHg and difference between diastolic PAP and PCWP of ≥7 mmHg). Correlation analyses between variables were calculated with Pearson's or Spearman's correlation coefficient as applicable. Results: Out of 78 patients, 45 presented with PH. Within the PH group, 39 had post-capillary, five had combined pre- and post-capillary PH, and one had pre-capillary PH. Patients with PH had a significantly increased RA area (PH 22.0 ± 9.2 cm2, non-PH 17.3 ± 10.7 cm2; p = 0.003) and end-systolic RV area (PH 14.7 ± 6.1, non-PH 11.9 ± 4.8 cm2; p = 0.022). RV mid strain was significantly reduced in PH (PH -17.4 ± 7.8, non-PH: - 21.6 ± 5.5; p = 0.019). Average peak systolic RA strain (RAS) and average peak systolic RV strain (RVS) showed a significant association with mPAP (r = - 0.470, p = 0.001 and r = 0.490, p = 0.001, respectively) and with PCWP (r = - 0.296, p = 0.048 and r = 0.365, p = 0.015, respectively) in patients with PH. Furthermore, RV apical, mid and basal strain as well as RV free wall strain showed moderate associations with mPAP. In patients without PH, there were no associations detectable between RA or RV strain parameters and mPAP and PCWP. Conclusion: In an all-comer cohort, RA and RV strain parameters showed significant associations with invasively assessed mPAP and PCWP in patients with predominantly post-capillary PH. These associations may be useful in clinical practice to assess the impact of post-capillary PH on myocardial right heart function

Early right ventricular systolic dysfunction in patients with systemic sclerosis without pulmonary hypertension: a Doppler Tissue and Speckle Tracking echocardiography study

<p>Abstract</p> <p>Background</p> <p>Isovolumetric acceleration (IVA) is a novel tissue Doppler parameter for the assessment of systolic function. The aim of this study was to evaluate IVA as an early parameter for the detection of right ventricular (RV) systolic dysfunction in patients with systemic sclerosis (SSc) without pulmonary hypertension.</p> <p>Methods</p> <p>22 patients and 22 gender- and age-matched healthy subjects underwent standard echocardiography with tissue Doppler imaging (TDI) and speckle tracking strain to assess RV function.</p> <p>Results</p> <p>Tricuspid annular plane systolic excursion (TAPSE) (23.2 ± 4.1 mm vs. 26.5 ± 2.9 mm, p < 0.006), peak myocardial systolic velocity (Sm) (11.6 ± 2.3 cm/s vs. 13.9 ± 2.7 cm/s, p = 0.005), isovolumetric contraction velocity (IVV) (10.3 ± 3 cm/s vs. 14.8 ± 3 cm/s, p < 0.001) and IVA (2.3 ± 0.4 m/s²vs. 4.1 ± 0.8 m/s², p < 0.001) were significant lower in the patient group. IVA was the best parameter to predict early systolic dysfunction with an area under the curve of 0.988.</p> <p>Conclusion</p> <p>IVA is a useful tool with high-predictive power to detect early right ventricular systolic impairment in patients with SSc and without pulmonary hypertension.</p

Comparative analysis of phasic left atrial strain and left ventricular posterolateral strain pattern to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy

Background: "Classical" echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH), posterolateral strain impairment (PLSI), and papillary muscle hypertrophy may be of limited diagnostic accuracy in clinical practice. Our aim was to evaluate the diagnostic value of left atrial (LA) strain impairment compared to "classical" echocardiographic findings to discriminate FC. Methods: In standard echocardiographic assessments, we retrospectively analyzed the diagnostic value of the "classical" red flags of FC as well as LA strain in 20 FC patients and in 20 subjects with other causes of LVH. Receiver operating characteristic (ROC) curve analysis was performed to assess the respective diagnostic accuracy. Results: FC was confirmed in 20 patients by genetic testing. In the LVH group, 12 patients were classified by biopsy to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six LVH combined with borderline myocarditis. Global and regional left ventricular (LV) strain was not significantly different between groups while LA strain was significantly impaired in FC (Left atrial reservoir strain (LASr) 19.1%±8.4 in FC and 25.6%±8.9 in LVH, p = 0.009; left atrial conduction strain (LAScd) -8.4%±4.9 in FC and -15.9%±8.4 in LVH, p < 0.01). LAScd, with an area under the curve (AUC) of .81 (95% confidence interval [CI] .66-.96) showed the highest diagnostic accuracy to discriminate FC. The PLSI pattern showed an AUC of .49, quantification of papillary muscle hypertrophy an AUC of .47. Conclusion: Adding LA strain analysis to a comprehensive echocardiographic work-up of unclear LVH may be helpful to identify FC as a possible cause

Implementation of seven echocardiographic parameters of myocardial asynchrony to improve the long-term response rate of cardiac resynchronization therapy (CRT)

<p>Abstract</p> <p>Background</p> <p>Cardiac resynchronization Therapy (CRT) is an effective therapy for chronic heart failure with beneficial hemodynamic effects leading to a reduction of morbidity and mortality. The responder rates, however, are low. There are various and contentious echocardiographic parameters of myocardial asynchrony. Patient selection by echocardiographic assessment of asynchrony is thought to improve responder rates.</p> <p>Methods</p> <p>In this small single-center pilot-study, seven established parameters of myocardial asynchrony were used to select patients for CRT: (1) interventricular electromechanical delay (IMD, cut-off ≥ 40 ms), (2) Septal-to-posterior wall motion delay (SPWMD, ≥ 130 ms), (3) maximal difference in time-to-peak velocities between any two of twelve LV segments (Ts-12 ≥ 104 ms), (4) standard deviation of time to peak myocardial velocities (Ts-12-SD, ≥ 34.4 ms), (5) difference between the septal and basal time-to-peak velocity (TDId, ≥ 60 ms), (6) left ventricular electromechanical delay (LVEMD, > 140 ms) and (7) delayed longitudinal contraction (DLC, > 2 segments).</p> <p>16 chronic heart failure patients (NYHA III–IV, LVEF < 0.35, QRS ≥ 120 ms) at least two out of seven parameters of myocardial asynchrony received cardiac resynchronization therapy (CRT-ICD). Follow-up echo examination was after 6 months. The control group was a historic group of CRT patients (n = 38) who had not been screened for echocardiographic signs of myocardial asynchrony prior to device implantation.</p> <p>Results</p> <p>Based on reverse remodeling (relative reduction of LVESV > 15%, relative increase of LVEF > 25%), the responder rate to CRT was 81.2% in patients selected for CRT according to our protocol as compared to 47.4% in the control group (p = 0.04). At baseline, there were on average 4.1 ± 1.6 positive parameters of asynchrony (follow-up: 3.7 [± 1.6] parameters positive, p = 0.52). Only the LVEMD decreased significantly after CRT (p = 0.027). The remaining parameters showed a non-significant trend towards reduction of myocardial asynchrony.</p> <p>Conclusion</p> <p>The implementation of different markers of asynchrony in the selection process for CRT improves the hemodynamic response rate to CRT.</p

Myocardial Work Assessment for the Prediction of Prognosis in Advanced Heart Failure

Objectives: The aim of this study was to investigate whether echocardiographic assessment of myocardial work is a predictor of outcome in advanced heart failure. Background: Global work index (GWI) and global constructive work (GCW) are calculated bymeans of speckle tracking, blood pressuremeasurement, and a normalized reference curve. Their prognostic value in advanced heart failure is unknown. Methods: Cardiopulmonary exercise testing and echocardiography with assessment of GWI and GCW was performed in patients with advanced heart failure caused by ischemic heart disease or dilated cardiomyopathy (n = 105). They were then followed up repeatedly. The combined endpoint was all-cause death, implantation of a left ventricular assist device, or heart transplantation. Results: The median patient age was 54 years (interquartile range [IQR]: 48–59.9). The mean left ventricular ejection fraction was 27.8 ± 8.2%, the median NT-proBNP was 1,210 pg/ml (IQR: 435–3,696). The mean GWI was 603 ± 329 mmHg% and the mean GCW was 742 ± 363 mmHg%. The correlation between peak oxygen uptake and GWI as well as GCW was strongest in patients with ischemic cardiomyopathy (r = 0.56, p = 0.001 and r = 0.53, p = 0.001, respectively). The median follow-up was 16 months (IQR: 12–18.5). Thirty one patients met the combined endpoint: Four patients died, eight underwent transplantation, and 19 underwent implantation of a left ventricular assist device. In themultivariate Cox regression analysis, only NYHA class, NT-proBNP and GWI (hazard ratio [HR] for every 50 mmHg%: 0.85; 95% CI: 0.77–0.94; p = 0.002) as well as GCW (HR for every 50 mmHg%: 0.86; 95% CI: 0.79–0.94; p = 0.001) were identified as independent predictors of the endpoint. The cut-off value for predicting the outcome was 455 mmHg% for GWI (AUC: 0.80; p < 0.0001; sensitivity 77.4%; specificity 71.6%) and 530 mmHg% for GCW (AUC: 0.80; p < 0.0001; sensitivity 74.2%; specificity 78.4%). Conclusions: GWI and GCW are powerful predictors of outcome in patients with advanced heart failure

Cardiac and renal function in a large cohort of amateur marathon runners

Background Participation of amateur runners in endurance races continues to increase. Previous studies of marathon runners have raised concerns about exercise-induced myocardial and renal dysfunction and damage. In our pooled analysis, we aimed to characterize changes of cardiac and renal function after marathon running in a large cohort of mostly elderly amateur marathon runners. Methods A total of 167 participants of the BERLIN-MARATHON (female n = 89, male n = 78; age = 50.3 ± 11.4 years) were included and cardiac and renal function was analyzed prior to, immediately after and 2 weeks following the race by echocardiography and blood tests (including cardiac troponin T, NT- proBNP and cystatin C). Results Among the runners, 58% exhibited a significant increase in cardiac biomarkers after completion of the marathon. Overall, the changes in echocardiographic parameters for systolic or diastolic left and right ventricular function did not indicate relevant myocardial dysfunction. Notably, 30% of all participants showed >25% decrease in cystatin C-estimated glomerular filtration rate (GFR) from baseline directly after the marathon; in 8%, we observed a decline of more than 50%. All cardiac and renal parameters returned to baseline ranges within 2 weeks after the marathon. Conclusions The increase in cardiac biomarkers after completing a marathon was not accompanied by relevant cardiac dysfunction as assessed by echocardiography. After the race, a high proportion of runners experienced a decrease in cystatin C-estimated GFR, which is suggestive of transient, exercise-related alteration of renal function. However, we did not observe persistent detrimental effects on renal function

Automated quantification of mitral valve tenting volume in functional mitral regurgitation by three‐dimensional echocardiography

Background: Tenting of the mitral leaflets is a major pathophysiological factor contributing to functional mitral regurgitation (FMR). A novel software tool allows automated quantification of the tenting volume (TnV) by 3D transesophageal echocardiography (TEE). The aims of this study are to investigate the correlations of biometric patient characteristics with the TnV and whether a threshold value for the diagnosis of a moderate or severe FMR can be calculated for the TnV. Methods: This explorative and hypothesis-generating study analyzed the TnV of the mitral valve obtained by clinically indicated TEE. The mid-systolic, threefold calculated and averaged TnV from 80 patients with no or mild FMR and 27 patients with moderate or severe FMR was determined using the TomTec 4D MV Assessment tool. Results: The TnV correlated significantly with the body size (r = 0.341), the weight (r = 0.272), and the body surface area (r = 0.320). After the adjustment to the body size, a threshold value of 1.25 cm(3)/m was determined for the TnV by using a receiver-operating characteristic curve. This value distinguished moderate to severe from none to mild FMR with a sensitivity of 85% and a specificity of 71%. The intra-observer variability and inter-observer variability were determined to be 0.96 and 0.85, respectively. Conclusions: Automated assessment of TnV has the potential to support the diagnostic evaluation of FMR. Further studies are needed to validate this result, detect additional factors influencing the size of the TnV, and determine further thresholds for any degree of FMR