Cardiac autonomic dysfunction in adult congenital heart disease.

PURPOSE: Due to recent advances in diagnosis and treatment, the number of adults with congenital heart disease (ACHD) has substantially increased. This achievement is mitigated by rhythm disorders. Here, we sought to determine alterations in heart rate variability (HRV) and their prognostic value in ACHD. METHODS: Ninety seven ACHD patients (39.2 ± 14.1 years, 51.5% female) and 19 controls (39.7 ± 15.0 years, 47.4% female) underwent 24-h Holter monitoring. RESULTS: As compared to controls, ACHD patients offered a significantly higher burden of premature ventricular contractions (p = 0.02) and decreased HRV indices (natural logarithmic transformation of very low frequency (lnVLF): 7.46 ± 0.76 ms2 vs. 7.91 ± 0.92ms2, p = 0.03; natural logarithmic transformation of low frequency (lnLF): 6.39 ± 0.95ms2 vs. 7.01 ± 1.07ms2, p = 0.01; natural logarithmic transformation of the ratio of low to high frequency spectra (lnLF/HF): 0.81 ± 0.74 vs. 1.17 ± 0.51, p = 0.04). No differences in HRV measures were observed across ACHD lesion groups. NT-proBNP levels were significantly related to both time and frequency domain indices (natural logarithmic transformation of the standard deviation of NN intervals (lnSDNN): Spearman´s rho = -0.32, p = 0.001; natural logarithmic transformation of the standard deviation of the average NN intervals for each 5-min segment of a 24-h Holter monitoring (lnSDANN): Spearman´s rho: -0.33, p = 0.001; natural logarithmic transformation of the total power (lnTP): Spearman´s rho: -0.25, p = 0.01; lnVLF: Spearman´s rho: -0.33, p = 0.001; lnLF: Spearman´s rho: -0.35, p < 0.001; lnLF/HF: Spearman´s rho: -0.34, p = 0.001). After a mean follow-up of 3.9 ± 0.7 years, 8 patients died and 3 patients survived sudden cardiac death (SCD). Several HRV parameters were significantly higher in event-free ACHD patients than in those who died or survived SCD (natural logarithmic transformation of the average of the standard deviations of NN intervals for each 5-min segment of a 24-h Holter monitoring (lnASDNN): p = 0.04; lnPNN30: p = 0.04; lnVFL: p = 0.03; lnLF: p < 0.01). On univariate Cox regression analysis, the time domain indices lnSDNN, lnASDNN and lnPNN30, as well as the frequency domain parameters lnTP, lnVLF and lnLF were associated with death and survived cardiac arrest. CONCLUSION: ACHD is accompanied by HRV impairment that carries prognostic implications on ACHD mortality and survived SCD.

Preclinical atherosclerotic burden in carotid and lower extremity arteries in adults with congenital heart disease.

Background: Over 90% of patients with congenital heart defects now reach adulthood, due to significant medical advances in recent decades. With advancing age, the risk of acquired cardiovascular diseases increases in addition to the already existing risk due to the congenital defect. The aim of this study was to evaluate the prevalence of atherosclerotic lesions in carotid and lower extremity arteries in adults with congenital heart disease (ACHD). Patients and methods: A total number of 108 ACHD patients (40.6±15.0 years, 50.0% male) and 22 healthy controls (39.3±16.6 years, 40.9% male) were included in this prospective study and underwent a comprehensive angiological examination that included vascular strain analysis on the common carotid artery. Results were stratified by the underlying ACHD lesion groups: shunt lesions (n=26), left-sided obstructive lesions (n=29), right-sided lesions (n=26) and complex lesions (n=27). Results: Colour-coded duplex sonography revealed atherosclerotic lesions in lower extremity arteries in 19 ACHD patients (17.6%). This prevalence did not significantly differ from the one assessed in controls (13.6%, p=0.77). All cases were asymptomatic and therefore classified as Fontaine stage I. 20.4% of ACHD patients presented atherosclerotic lesions in extracranial carotid arteries; amongst controls, the corresponding proportion was 18.4% (p=1.00). No significant differences were observed in atherosclerotic burden in extracranial carotid and lower limb arteries across the four ACHD patient groups (p=0.67 and p=0.89, respectively). Vascular strain analysis revealed no differences between patients and controls. Though circumferential strain varied over ACHD groups (p<0.05), comparison of strain measurements across all specific underlying defect subgroups revealed no significant difference for any of the studied strain parameters. Conclusions: ACHD patients present an atherosclerotic burden in extracranial carotid and lower limb arteries and a vascular stiffness that is comparable to healthy controls.

Telemonitoring with Electronic Devices in Patients with a Single Ventricle Anatomy.

BACKGROUND: A growing number of patients with a single ventricle anatomy, who had a Fontan palliation as a child, are now reaching adulthood. Many need an epimyocardial pacemaker system with an optional telemonitoring (TM) unit, which evaluates the collected data and sends it via Internet to the patient's physician. There are no data on the reliability and clinical relevance of these systems in this patient group. METHODS: We analyzed data in 48 consecutive patients (mean age 18 years, standard deviation 9 years) with a Fontan or Fontan-like palliation who received a cardiac implantable electronic device with a TM unit from Biotronik (Home Monitoring) or Medtronic (CareLink) between 2005 and 2020 with regard to the reliability and clinical relevance of the downloaded data. RESULTS: The observation period was from 4 months to 14 years (mean 7 years, standard deviation 3.9 years). A total of 2.9 event messages (EMs)/patient/month and 1.3 intracardiac electrogram recordings/patient/month were received. Two patients died during follow-up. The combination of regularly arriving statistical data and 313 clinically relevant EMs led to the modification of antiarrhythmic or diuretic medication, hospitalization with cardioversion or ablation, and cortisone therapy to avoid exit block in 21 (44%) patients. CONCLUSION: TM is an instrument to receive functional and physiologic parameters of our Fontan patients. It provides the ability to respond early for signs of system failure, or arrhythmia, even if the patient is not experiencing any problems. It is a useful tool to manage this difficult patient population without frequent hospital visits.

Performance of epimyocardial leads in patients with a single ventricle circulation.

BACKGROUND: Cardiac pacing can be challenging after a Fontan operation, and limited data exist regarding strategies to plan these epimyocardial systems while minimizing the number of surgical procedures. METHODS: A retrospective review of all our 47 patients (mean age 18 years, standard deviation 9 years) with a Fontan palliation who received an epimyocardial cardiac implantable electronic device (CIED) between 2002 and 2020 with regard to the stability of the epimyocardial lead parameters and the incidence of system revisions. RESULTS: Over the last 18 years, 84 implantations or revisions of the epimyocardial CIED in 47 Fontan patients were performed. Mean age at time of the first implantation was 9.4 (range 0.28-29.3) years. Follow-up period ranges from 0.11 to 18.2 (mean 7.7, standard deviation 4.2) years. A total of 123 pacing leads were implanted of which 99 are still active. From 2010 triple lead cardiac resynchronization devices were used in 17 patients to better cope with lead problems. The initial pacing threshold of the leads inactivated during this study period proved significantly higher (mean 1.66 V) than in the "all leads" group (mean 1.27 V, p = .0005) or the group of the still active leads (mean 1.17 V, p = .00004). CONCLUSIONS: When implanted with a low pacing threshold, the bipolar epimyocardial electrodes show stable and good long-term results in young patients with a Fontan circulation. Resynchronization pacing systems and the prospective implantation of reserve leads may help to reduce the rate of resternotomies and provide a flexible concept to deal with lead failure.

Impact of left ventricular conduction defect with or without need for permanent right ventricular pacing on functional and clinical recovery after TAVR.

BACKGROUND: Left ventricular conduction disturbances (VCD) with or without need for pacemaker (PM) implantation are common after transcatheter aortic valve replacement (TAVR). Its effect on patients' functional recovery after TAVR is unclear. METHODS AND RESULTS: 212 patients (Age 80.8 ± 6.4 years, logEuroscore 28.95 ± 17.37 %) underwent TAVR with the self-expanding CoreValve prosthesis and completed 9-month follow-up (FU). After TAVR 125 (59 %) patients were diagnosed with VCD. This group consists of 41 (19 %) patients with a new PM after TAVR, 33 (16 %) patients with PM prior TAVR and markedly increased ventricular stimulation rate, 48 (23 %) patients with new LBBB post-TAVR and three (1 %) patients with LBBB prior TAVR. After FU, the presence of VCD alone was associated with worse recovery of left ventricular ejection fraction (LVEF) (VCD: LVEFbaseline 51.7 ± 18.2 %, LVEFFU 53.9 ± 13.0 %; p = 0.8; noVCD: LVEFbaseline 53.8 ± 12.9 %, LVEFFU 63.4 ± 10.1 %; p < 0.01) but had no impact on functional outcomes after TAVR (p > 0.05). Especially patients with VCD caused by permanent RV pacing showed worse functional outcomes presenting with higher functional NYHA classes (p < 0.05), and higher NT-proBNP levels (p < 0.05). 20.4 % of patients with need for PM after TAVR remained in NYHA class ≥3, as compared to 5 % of patients without PM (VCD but no PM: 4.7 %, p < 0.001; noVCD: 5.3 %, p < 0.001). VCD with or without need for PM had no impact on survival after FU. CONCLUSION: The occurrence of VCD after TAVR is common and associated with unfavorable left ventricular functional recovery. However, only the combination of VCD with permanent right ventricular pacing has adverse impact on heart failure-related symptoms after TAVR.

Acute changes of mitral valve geometry during interventional edge-to-edge repair with the MitraClip system are associated with midterm outcomes in patients with functional valve disease: preliminary results from a prospective single-center study.

BACKGROUND: Transcatheter mitral valve repair (TMVR) is a treatment option in patients with symptomatic functional or degenerative mitral regurgitation (DMR) at high surgical risk. The acute effect of MitraClip procedure on mitral valve (MV) annular geometry and its relation to functional outcomes is unclear. We sought to assess immediate effect of TMVR on MV annular geometry with 3-dimensional (3D) transesophageal echocardiography and the association of MV diameter reduction with functional response after 6 months. METHODS AND RESULTS: Consecutive patients (n = 111; age, 78.3 ± 8.1 years) at high surgical risk (logistic EuroSCORE [European System for Cardiac Operative Risk Evaluation], 29.8±21.5%) underwent TMVR. The procedure was completed successfully in 107 (96%) patients with 3D reconstruction of MV annular geometry immediately before and after clip implantation. Only patients with functional mitral regurgitation (n = 71) experienced an acute reduction of anterior-posterior MV diameters (4.0 ± 0.6 and 3.6 ± 0.6 cm; P < 0.0001), MV annulus areas (2D annulus area, 13.9 ± 3.8 and 12.8 ± 3.4 cm(2); P < 0.0001 and 3D annulus area, 14.4 ± 3.9 and 12.9 ± 3.4 cm(2); P < 0.0001), and MV annular geometry (MV sphericity index, 0.9 ± 0.1 and 0.8 ± 0.1; P < 0.0001); the lateral medial MV diameters remained unchanged (4.3 ± 0.7 and 4.4 ± 0.6 cm; P = 0.13). In subjects with DMR, all MV annular geometry-defining values were not significantly altered after TMVR (n = 36; P > 0.05). Acute anterior-posterior diameter reduction was associated with clinical response to TMVR after 6 months of follow-up (cutoff value, ≥ 6.4%; area under the curve, 0.81; P = 0.002; sensitivity, 81.6%; specificity, 81.8%), which was confirmed by additional regression analysis (P = 0.007). CONCLUSIONS: Three-dimensional transesophageal echocardiography enables assessment of acute changes of MV geometry in patients undergoing the MitraClip procedure. Only patients with functional mitral regurgitation experienced significant reduction of MV annular dimensions, which was associated with clinical response to TMVR.

Impact of untreated obstructive sleep apnea on left and right ventricular myocardial function and effects of CPAP therapy.

BACKGROUND: Obstructive sleep apnea (OSA) has deteriorating effect on LV function, whereas its impact on RV function is controversial. We aimed to determine the effect of OSA and continuous positive airway pressure (CPAP) treatment on left and right ventricular (LV, RV) function using transthoracic echocardiography (TTE) and 2 dimensional speckle tracking (2D ST) analysis of RV deformation capability. METHODS AND RESULTS: 82 patients with OSA and need for CPAP therapy were prospectively enrolled and underwent TTE at study inclusion and after 6 months of follow up (FU). Multivariate regression analysis revealed an independent association between baseline apical right ventricular longitudinal strain (RV-Sl), BMI and the severity of OSA (apical RV-Sl: P = 0.0002, BMI: P = 0.02). After CPAP therapy, LV functional parameters (LVEF: P<0.0001, LV performance index: P = 0.03, stroke volume: P = 0.042), and apical RV-Sl (P = 0.001) improved significantly. The effect of CPAP therapy was related to severity of OSA (LVEF: AHI 5-14, 66.4 ± 8.8%, 68.5 ± 10.6% [P = ns]; AHI 15-30:59.8 ± 7.7%, 68.6 ± 9.3% [P = 0.002]; AHI>30:54.1 ± 12.4%, 68.2 ± 13.6%[P<0.0001]; apical RV-Sl: AHI 5-14: -17.3 ± 8.7%, -16.0 ± 10.8% [P = ns], AHI 15-30: -9.8 ± 6.0%, -15.4 ± 10.9% [P = 0.028], AHI>30: -6.3 ± 5.7%, -17.9 ± 11.2% [P<0.0001]). CONCLUSIONS: OSA seems to have deteriorating effect on LV and RV function. We found a beneficial effect of CPAP on LV and RV functional parameters predominately in patients with severe OSA. 2D speckle tracking might be of value to determine early changes in global and regional right ventricular function.

Diagnostic value of echocardiography in the diagnosis of pulmonary hypertension.

AIMS: To determine the value of echocardiography including tissue Doppler imaging (TDI) and right ventricular (RV) speckle tracking analysis for the diagnosis of pulmonary hypertension (PH) and discrimination between pre- and postcapillary PH. METHODS AND RESULTS: 155 consecutive patients (mean age 70.5±13.0 years, 81 [52%] male gender, BMI 27.2±6.1 kg/m(2)) with PH undergoing right heart catheterization (RHC) and transthoracic echocardiography (TTE) with TDI between January 2008 and December 2009 were retrospectively evaluated including offline speckle tracking analysis of RV contractility. After RHC 23.2% of patients (36) were diagnosed with precapillary PH. Invasive results from RHC were significantly correlated to TTE measurements (E/é, postcapillary wedge pressure [PCWP], r=0.61, P<0.001; mean, systolic pulmonary arterial pressure [mPAP, sPAP], r=0.43, P<0.001). Single echocardiographic parameters were of good predictive value for final PH-diagnosis (sPAP, area under the curve [AUC] 0.63, P=0.025; lateral apical RV longitudinal strain [RVaSl)], AUC 0.76, P=0.001; E/é, AUC 0.84, P<0.001) which could be increased by combining most predictive parameters after receiver operating curves (ROC) cut off analysis (sPAP>69 mmHg, E/é<12, RVaSl ≥-8.4%). TTE had a sensitivity of 33.33% and a specificity of 100% to identify patients with precapillary PH, and a negative predictive value of 84.72% to rule out precapilary PH. CONCLUSION: Echocardiography allows feasible and reliable estimation of PH and seems helpful to distinguish between pre-and postcapillary PH. Further prospective studies on patients with different manifestations of PH must validate the predictive value of echocardiography in this clinical setting.

Three-dimensional speckle-tracking analysis of left ventricular function after transcatheter aortic valve implantation.

BACKGROUND: The acute and long-term effects of transcatheter aortic valve implantation (TAVI) in patients with aortic valve stenosis on left ventricular (LV) function are controversial. The aim of this study was to determine the effect of TAVI on LV function with two-dimensional (2D) and three-dimensional (3D) speckle-tracking analysis of LV deformation capability. METHODS: Patients underwent standardized 2D and 3D transthoracic echocardiography before TAVI and after 6 months of follow-up, including 3D and 2D LV deformation imaging. RESULTS: Forty-four patients (mean age, 81.7 ± 5.5 years; 21 men [47.7%]; mean body mass index, 26.3 ± 5.1 kg/m(2); mean logistic European System for Cardiac Operative Risk Evaluation score, 24.4 ± 13.7%) undergoing TAVI were prospectively included. After follow-up, mean 3D LV ejection fraction (LVEF) (35.4 ± 13.1% vs 40.6 ± 12.6%, P = .004), 3D LV volumes (end-systolic volume, 85.9 ± 41.8 vs 65.9 ± 33.7 mL, P < .001; end-diastolic volume, 127.6 ± 40.7 vs 106.4 ± 40.9 mL, P = .001), 3D global longitudinal strain (-9.9 ± 3.7% vs -12.6 ± 4.2%, P < .001), and 3D LV twist (6.1 ± 4.3° vs 8.5 ± 6.9°, P = .025) were relevantly improved. LV improvement was pronounced in patients with decreased baseline LV function (area under the curve, 0.78; P < .001), with a cutoff value for 3D LVEF of ≤37% to identify functional responders to TAVI. After follow-up, patients with 3D LVEFs ≤ 37% showed a significant improvements in 3D LVEF (26.0 ± 7.6% vs 35.9 ± 11.7%, P < .001), 3D LV volumes (end-diastolic volume, 147.4 ± 40.6 vs 117.1 ± 45.5 mL, P = .001; end-systolic volume, 110.9 ± 39.2 vs 77.5 ± 37.2 mL, P < .001), 3D global longitudinal strain (-7.8 ± 2.7% vs -11.3 ± 4.2%, P < .001), and 3D LV twist (5.6 ± 4.2° vs 8.0 ± 5.6°, P = .047), whereas in patients with 3D LVEFs > 37%, only 3D global longitudinal strain was relevantly altered (-12.5 ± 3.1% vs -14.2 ± 3.8%, P = .04). Compared with 2D transthoracic echocardiography, 3D LV functional imaging allowed significantly faster image acquisition and data analysis (P < .0001). New York Heart Association functional class improved significantly in both groups (3D LVEF ≤ 37%, from 3.1 ± 0.5 to 2.0 ± 0.6, P < .001; 3D LVEF > 37%, from 2.7 ± 6.7 to 1.5 ± 0.7, P < .001), whereas a significant amelioration of N-terminal pro-brain natriuretic peptide was observed only in patients with baseline 3D LVEFs ≤ 37% (10,314.64 ± 11,682.2 vs 3,398.7 ± 3,598.9 pg/mL, P = .02; 3D LVEF > 37%, 10,306.4 ± 32,000.5 vs 2,868.0 ± 3,816.7 pg/mL, P = .12). CONCLUSIONS: Our results indicate significant improvements of LV global and longitudinal function and clinical parameters 6 months after TAVI that are pronounced in patients with impaired baseline LV function. Compared with 2D LV functional imaging, 3D speckle-tracking imaging allowed significantly faster image acquisition and data analysis.

Left atrial deformation imaging with ultrasound based two-dimensional speckle-tracking predicts the rate of recurrence of paroxysmal and persistent atrial fibrillation after successful ablation procedures.

OBJECTIVE: Since predictors of recurrence of atrial fibrillation (AF) after ablation procedures are poorly defined, this prospective study was conducted to assess the value of left atrial (LA) deformation imaging with two-dimensional speckle-tracking (2D-ST) to predict AF recurrences after successful ablation procedures. METHODS AND RESULTS: One hundred and three consecutive patients (age 58.1 ± 16.6 years, 72.8% male) with AF (76 paroxysmal, 27 persistent) and 30 matched controls underwent transthoracic echocardiography and 2D-ST-LA-deformation analysis with assessment of LA-radial and LA-longitudinal strain (Sr, Sl), and velocities derived from the apical 4- and 2-chamber views (4CV, 2CV). AF recurrence was assessed during 6 months of follow-up. For determination of AF-related LA changes, AF patients were compared to controls and patients with AF recurrences after ablation procedures (n = 30, 29.1%) were compared with patients who maintained sinus rhythm (n = 73, 70.9%). Atrial deformation capabilities were significantly reduced (P < 0.0005) in patients with AF (4CVSl 17.8 ± 13.5%; 4CVSr 22.3 ± 14.9%; 4CV-velocities 2.53 ± 0.97 seconds) when compared with controls (4CVSl 31.3 ± 12.4%; 4CVSr 30.3 ± 9.1%; 4CV-velocities 3.48 ± 1.01 cm/s). Independent predictors for AF recurrence after ablation procedures were 2CV-LA-global-strain (Sr, P = 0.03; Sl, P = 0.003), 4CV-LA-gobal-strain (Sr, P = 0.03; Sl, P = 0.02), and regional LA-septal wall-Sl (P = 0.008). LA-global-strain parameters were superior to regional LA function analysis for the prediction of AF recurrences, with cutoff values (cov), hazard ratios (HR), positive and negative predictive values (PPV, NPV) were: 4CVSl cov, 10.79% (HR 27.8, P < 0.0005; PPV 78.8%, NPV 93.9%), 4CVSr cov, -16.65% (HR 24.8, P < 0.0005; PPV 69.4%, NPV 96.6%), 2CVSl cov, 12.31% (HR 22.7, P < 0.0005; PPV 75.8%, NPV 95.3%), and 2CVSr cov, -14.9% (HR 12.9, P < 0.0005; PPV 64.3%, NPV 93.2%). CONCLUSION: Compared with controls, AF itself seems to decrease LA deformation capabilities. The assessment of global LA strain with 2D-ST identifies patients with high risk for AF recurrence after ablation procedures. This imaging technique may help to improve therapeutic guiding for patients with AF.