Cardiac fibromas in adult patients: a case series focusing on rhythmology and radiographic features.

Background: Fibromas are rare primary benign cardiac tumours that can become symptomatic due to expansive growth, ventricular rhythm disturbances, and sudden cardiac death. Distinguishing fibromas from other (malign) cardiac masses is essential for accurate diagnosis and treatment. While there is some experience in management of cardiac fibromas in children, management of adult patients is unknown. Case summary: We present three cases of cardiac fibroma in adult patients diagnosed by echocardiography, cardiovascular magnetic resonance (CMR), and computed tomography (CT): (1) a 55-year-old male with a left ventricular fibroma leading to reduced left ventricular ejection fraction and mitral regurgitation. He had family history of sudden cardiac death, showed premature ventricular contractions (PVCs), and was treated with a primary preventive subcutaneous implantable cardiac defibrillator (S-ICD); (2) a 39-year-old male with right ventricular fibroma as an incidental finding. He complained of episodes of PVC. Due to a low PVC burden, decision was made against ablation and the patient was planned for follow-up; and (3) an 18-year-old female with left ventricular apex fibroma detected by CMR shortly after birth and confirmed by surgical biopsy. Being asymptomatic, conservative management was pursued and follow-up by CMR planned. Discussion: Cardiac fibromas can show various clinical presentations and hence being detected late in life. Given potential complications of surgical biopsy, diagnosis of cardiac fibromas is primarily based on echocardiography, CT, and CMR. Rhythm disturbances as PVCs are common. Due to association with ventricular arrhythmias and sudden cardiac death, preventive ICD placement might be appropriate on an individual basis.

Performance of Transcatheter Direct Annuloplasty in Patients With Atrial and Nonatrial Functional Tricuspid Regurgitation.

BACKGROUND: A novel echocardiography-based definition of atrial functional tricuspid regurgitation (A-FTR) has shown superior outcomes in patients undergoing conservative treatment or tricuspid valve transcatheter edge-to-edge repair. Its prognostic significance for transcatheter tricuspid valve annuloplasty (TTVA) outcomes is unknown. OBJECTIVES: This study sought to investigate prognostic, clinical, and technical implications of A-FTR phenotype in patients undergoing TTVA. METHODS: This multicenter study investigated clinical and echocardiographic outcomes up to 1 year in 165 consecutive patients who underwent TTVA for A-FTR (characterized by the absence of tricuspid valve tenting, midventricular right ventricular [RV] dilatation, and impaired left ventricular ejection fraction) and nonatrial functional tricuspid regurgitation (NA-FTR). RESULTS: A total of 62 A-FTR and 103 NA-FTR patients were identified, with the latter exhibiting more pronounced RV remodeling. Compared to baseline, the tricuspid regurgitation (TR) grade at discharge was significantly reduced (P < 0.001 for both subtypes), and TR ≤II was achieved more frequently in A-FTR (85.2% vs 60.8%; P = 0.001). Baseline TR grade and A-FTR phenotype were independently associated with TR ≤II at discharge and 30 days. In multivariate analyses, A-FTR phenotype was a strong predictor (OR: 5.8; 95% CI: 2.1-16.1; P < 0.001) of TR ≤II at 30 days. At 1 year, functional class had significantly improved compared to baseline (both P < 0.001). One-year mortality was lower in A-FTR (6.5% vs 23.8%; P = 0.011) without significant differences in heart failure hospitalizations (13.3% vs 22.7%; P = 0.188). CONCLUSIONS: Direct TTVA effectively reduces TR in both A-FTR, which is a strong and independent predictor of achieving TR ≤II, and NA-FTR. Even though NA-FTR showed more RV remodeling at baseline, both phenotypes experienced similar symptomatic improvement, emphasizing the benefit of TTVA even in advanced disease stages. Additionally, phenotyping was of prognostic relevance in patients undergoing TTVA.

Highly compressed SENSE accelerated relaxation-enhanced angiography without contrast and triggering (REACT) for fast non-contrast enhanced magnetic resonance angiography of the neck: Clinical evaluation in patients with acute ischemic stroke at 3 tesla.

BACKGROUND AND PURPOSE: Long acquisition times limit the feasibility of established non-contrast-enhanced MRA (non-CE-MRA) techniques. The purpose of this study was to evaluate a highly accelerated flow-independent sequence (Relaxation-Enhanced Angiography without Contrast and Triggering [REACT]) for imaging of the extracranial arteries in acute ischemic stroke (AIS). MATERIALS AND METHODS: Compressed SENSE (CS) accelerated (factor 7) 3D isotropic REACT (fixed scan time: 01:22 min, reconstructed voxel size 0.625 × 0.625 × 0.75 mm3) and CE-MRA (CS factor 6, scan time: 1:08 min, reconstructed voxel size 0.5 mm3) were acquired in 76 AIS patients (69.4 ± 14.3 years, 33 females) at 3 Tesla. Two radiologists assessed scans for the presence of internal carotid artery (ICA) stenosis and stated their diagnostic confidence using a 5-point scale (5 = excellent). Vessel quality of cervical arteries as well as the impact of artifacts and image noise were scored on 5-point scales (5 = excellent/none). Apparent signal- and contrast-to-noise ratios (aSNR/aCNR) were measured for the common carotid artery (CCA) and ICA (C1-segment). RESULTS: REACT provided a sensitivity of 88.5% and specificity of 100% for clinically relevant (≥50%) ICA stenosis with substantial concordance to CE-MRA regarding stenosis grading (Cohen's kappa 0.778) and similar diagnostic confidence (REACT: mean 4.5 ± 0.4 vs. CE-MRA: 4.5 ± 0.6; P = 0.674). Presence of artifacts (3.6 ± 0.5 vs. 3.5 ± 0.7; P = 0.985) and vessel quality (all segments: 3.6 ± 0.7 vs. 3.8 ± 0.7; P = 0.004) were comparable between both techniques with REACT showing higher scores at the CCA (4.3 ± 0.6 vs. 3.8 ± 0.9; P < 0.001) and CE-MRA at V2- (3.3 ± 0.5 vs. 3.9 ± 0.8; P < 0.001) and V3-segments (3.3 ± 0.5 vs. 4.0 ± 0.8; P < 0.001). For all vessels, REACT showed a lower impact of image noise (3.8 ± 0.6 vs. 3.6 ± 0.7; P = 0.024) while yielding higher aSNR (52.5 ± 15.1 vs. 37.9 ± 12.5; P < 0.001) and aCNR (49.4 ± 15.0 vs. 34.7 ± 12.3; P < 0.001) for all vessels combined. CONCLUSIONS: In patients with acute ischemic stroke, highly accelerated REACT provides an accurate detection of ICA stenosis with vessel quality and scan time comparable to CE-MRA.

Incidence and clinical impact of renal failure and bleeding following transcatheter tricuspid valve annuloplasty.

BACKGROUND: Bleeding is the most common complication after percutaneous leaflet-based tricuspid valve repair and associated with acute kidney injury (AKI) and adverse outcome. TTVA with the Cardioband system is a technically more complex procedure; however, frequency and prognostic impact of postinterventional bleeding and renal complications have not been thoroughly examined. AIMS: This study was performed to determine the incidence and clinical impact of bleeding complications (MVARC criteria) and acute kidney injury (KDIGO criteria) following transcatheter tricuspid valve annuloplasty (TTVA). METHODS: In a bi-center retrospective analysis of patients undergoing TTVA between 2018 and 2022, we examined frequency, predictors, and clinical impact of bleeding and renal failure. RESULTS: In 145 consecutive patients, the incidence of any MVARC bleeding was 20.7% (n = 30), whereas major MVARC bleeding occurred in 6.9% (n = 10). The incidence of AKI was 18.6% (n = 27). Risk factors for bleeding events included low baseline hemoglobin and elevated baseline creatinine levels. Risk factors for AKI included diabetes mellitus, arterial hypertension, high body mass index, and elevated baseline creatinine levels. Neither procedure duration nor amount of contrast media was associated with AKI or bleeding. Both bleeding and AKI led to a longer hospital stay. At 3 months, 10.0% (n = 3) of patients with bleeding and 7.8% (n = 9) of patients without bleeding complications died (p = 0.70). Additionally, mortality rate was 7.4% (n = 2) in patients with AKI compared to 8.5% (n = 10) without AKI (p = 0.83). CONCLUSION: While about a fifth of patients undergoing TTVA suffered from postinterventional AKI or bleeding, none of these complications was associated with higher mortality at short-term follow-up. One important risk factor for both complications was chronic renal dysfunction, indicating a high-risk patient population. The most frequent bleeding localizations were the femoral access site, pericardial hemorrhage, and the esophagus, which need explicit attention in periprocedural management.

A Case Series of Concomitant Cardiac Electrical Disease among Takotsubo Syndrome Patients and Literature Review.

The pathophysiology of Takotsubo Syndrome (TTS) is not completely understood and the trigger of sudden cardiac death (SCD) in TTS is not clear either. We therefore sought to find an association between TTS and primary electrical diseases. A total of 148 TTS patients were analyzed between 2003 and 2017 in a bi-centric manner. Additionally, a literature review was performed. The patients were included in an ongoing retrospective cohort database. The coexistence of TTS and primary electrical diseases was confirmed in five cases as the following: catecholaminergic polymorphic ventricular tachycardia (CPVT, 18-year-old female) (n = 1), LQTS 1 (72-year-old female and 65-year-old female) (n = 2), LQTS 2 (17-year-old female) (n = 1), and LQTS in the absence of mutations (22-year-old female). Four patients suffered from malignant tachyarrhythmia and recurrent syncope after TTS. Except for the CPVT patient and one LQTS 1 patient, all other cases underwent subcutaneous ICD implantation. An event recorder of the CPVT patient after starting beta-blocker did not detect arrhythmias. The diagnosis of primary electrical disease was in 80% of cases unmasked on a TTS event. This diagnosis triggered a family clinical and genetic screening confirming the diagnosis of primary electrical disease. A subsequent literature review identified five cases as the following: a congenital atrioventricular block (n = 1), a Jervell and Lange-Nielsen Syndrome (n = 1), and a family LQTS in the absence of a mutation (n = 2), LQTS 2 (n = 1). A primary electrical disease should be suspected in young and old TTS patients with a family history of sudden cardiac death. In suspected cases, e.g., ongoing QT interval prolongation, despite recovery of left ventricular ejection fraction a family screening is recommended.

Impact of Chronobiological Variation in Takotsubo Syndrome: Prognosis and Outcome.

Background: A considerable amount of evidence has shown that acute cardiovascular diseases exhibit specific temporal patterns in their onset. Aim: This study was performed to determine if takotsubo syndrome (TTS) shows chronobiological variations with short and long-term impacts on adverse events. Design: Our institutional database constituted a collective of 114 consecutive TTS patients between 2003 and 2015. Methods: Patients were divided into groups defined by the onset of TTS as per time of the day, day of the week, month and quarter of year. Results: TTS events were most common afternoon and least common in the night, indicating a wave-like pattern (p = 0.001) of manifestation. The occurrence of TTS events was similar among days of the week and weeks of the month. TTS patients diagnosed in the month of November and subsequently in the fourth quarter showed a significantly longer QTc interval. These patients also revealed a significantly lower event-free-survival over a 1-year follow-up. In a multivariate Cox regression analysis, TTS events occurring in the fourth quarter of year (HR 6.8, 95%CI: 1.3-35.9; p = 0.02) proved to be an independent predictor of lower event-free-survival. Conclusions: TTS seems to exhibit temporal preference in its onset, but nevertheless this possibly coincidental result needs to be analyzed in a large multicenter registry.

Association Between Mortality and Left Ventricular Ejection Fraction in Patients With Takotsubo Syndrome Versus Acute Coronary Syndrome.

BACKGROUND/AIM: The association between ejection fraction (EF) and mortality in TTS patients as compared to ACS is limited. This study aims to investigate the association between EF and clinical outcomes in patients with TTS as compared to ACS. PATIENTS AND METHODS: This study compared in-hospital, and long-term incidence of clinical outcomes for 5 years in patients with TTS and ACS. The study was composed of two groups EF≥35% and EF<35%. RESULTS: The long-term mortality of the EF≥35% for 5 years was significantly higher in TTS patients as compared to ACS (18.1% vs. 7.7%, log-Rank; p<0.01). Irrespective of EF, a non-cardiovascular death was significantly higher in TTS as compared to ACS patients with EF≥35 (6.4% vs. 2.1%; p=0.02) and with EF<35% (21.4% vs. 7.5%; p=0.03). CONCLUSION: The long-term mortality is significantly higher in TTS as compared to ACS dominated by a non-cardiovascular cause of death at 5-years-follow-up.

The Use of Beta Blockers in Takotsubo Syndrome as Compared to Acute Coronary Syndrome.

BACKGROUND: Takotsubo syndrome (TTS) and acute coronary syndrome (ACS) patients have a similar mortality rate. In this study, we sought to determine the short- and long-term outcome of TTS patients as compared to ACS patients both treated with beta-blockers. OBJECTIVES: In the present study we described the data of 5 years of follow up of 103 TTS and 422 ACS patients both treated with beta-blockers. METHODS: Data from TTS patients were included retrospectively and prospectively, ACS patients were included retrospectively. All retrospectively included patients have been followed up for 5 years. The end point in this study was the occurrence of death. RESULTS: TTS affected significantly more women (87.4%) than ACS (34.6%) (p < 0.01). TTS patients suffered significantly more often from thromboembolic events (14.6% versus 2.1%; p < 0.01) and cardiogenic shock (11.9% versus 3.6%; p < 0.01) than the ACS group. TTS patients had a significantly higher long-term mortality (within 5 years) as compared to ACS patients (17.5% versus 3.6%) (p < 0.01). Patients of the TTS group compared to the ACS group did not benefit from combination of beta-blockers and ACE-inhibitors in terms of long-term mortality (p < 0.01). As we compare TTS patients who were treated with beta-blockers and ACE-inhibitors versus single use of beta-blockers there was no difference in long-term mortality (p = 0.918). CONCLUSION: TTS patients had a significantly higher long-term mortality (within 5 years) than patients with an ACS.

Improved Outcome of Cardiogenic Shock Triggered by Takotsubo Syndrome Compared With Myocardial Infarction.

BACKGROUND: Cardiogenic shock (CS) is a severe complication of myocardial infarction (MI) or of takotsubo syndrome (TTS). For both diseases, CS is related to a worse long-term outcome. The outcome of CS has not been studied in a direct comparison of patients with MI and patients with TTS. METHODS: Mortality and cardiovascular complications were compared in patients presenting with CS based on MI or TTS between 2003 and 2017 during a follow-up of 5 years. A total of 138 patients with TTS and 532 patients with MI were included. Of these, 66 patients with MI and 25 patients with TTS developed CS (12% vs 18%, P = 0.08). RESULTS: Patients with MI and CS had more often malignant arrhythmias (74% vs 28%, P < 0.01), and need for resuscitation (80% vs 24%, P < 0.01) or death (71% vs 24%, P < 0.01) than patients with TTS and CS during the first 30 days. Although the overall rate of death remained higher in MI than in TTS (75.8% vs 52%, log rank, P < 0.01), deaths occurred in TTS constantly throughout the follow-up time, but not in MI. The incidence of heart failure increased in MI but not in TTS (31.8% vs 4%, P < 0.01) during follow-up. CONCLUSIONS: Patients with MI and CS have a worse prognosis than patients with TTS and CS. This is driven by cardiovascular events or death during the first 30 days after the index event. However, patients with TTS and CS show high mortality as well, especially during long-term follow-up.

Takotsubo syndrome and cardiac implantable electronic device therapy.

Recent studies have reported that takotsubo syndrome (TTS) patients are suffering from life-threatening arrhythmias. The aim of our study was to understand the short and long-term usefulness of cardiac implantable electronic devices in TTS patients.We constituted a collective of 142 patients in a bi-centric study diagnosed with TTS between 2003 and 2017. The patient groups, divided according to the treatment with (n = 9, 6.3%) or without cardiac devices (n = 133, 93.7%), were followed-up to determine the importance of devices and its complications. One patient was treated with a permanent pacemaker, five patients with a wearable cardioverter defibrillator, two patients with a subcutaneous defibrillator and one patient with a transvenous defibrillator. Regular device check-up was documented in all patients, presenting an ongoing high-degree AV-block. Neither device complications nor life-threatening tachyarrhythmias were documented after acute TTS event. However, patients comprising the device group suffered significantly more often from a highly reduced EF (30 ± 7.7% versus 39.1 ± 9.7%; p < 0.05), cardiogenic shock with use of inotropic agents (66.6% versus 16.6%; p < 0.05) and cardiopulmonary resuscitation (44.4% versus 5.3%; p < 0.05). Our data confirm the usefulness of pacemaker in TTS patients. However, the cardioverter defibrillator including wearable cardioverter defibrillator may not be recommended.

Short- and Long-Term Incidence of Thromboembolic Events in Takotsubo Syndrome as Compared With Acute Coronary Syndrome.

Thromboembolic events are a common complication in Takotsubo syndrome (TTS). However, their long-term incidence compared with acute coronary syndrome (ACS) is lacking. In-hospital and long-term incidence of thromboembolic events of 138 consecutive patients with TTS were compared with 138 sex- and age-matched patients with ACS. Predictors of events were analyzed. The incidence of thromboembolic events in TTS was 2-fold higher than ACS (21% vs 9%; P < .01) over a mean follow-up of 5 years. Although the left ventricular ejection fraction (LVEF) at event was significantly lower in TTS compared with ACS (38% [9%] vs 54% [11%]; P < .01), the follow-up LVEF was comparable. Patients with TTS suffering from thromboembolic events were more often treated with anticoagulation compared with ACS (44.8% vs 8.3%, P = .03). However, more patients presenting with ACS (100% vs 48.3%; P < .01) were discharged on aspirin. Only elevated C-reactive protein was a predictor of thromboembolic events using multivariate analysis (hazard ratio 1.1, 95% confidence interval, 1.0-1.2; P < .01). In conclusion, the risk of thromboembolic events in TTS was significantly higher than the risk of thromboembolic events in ACS over a mean follow-up of 5 years.

Impact of ST-segment elevation on the outcome of Takotsubo syndrome.

BACKGROUND: Recent studies have highlighted that Takotsubo syndrome (TTS), mimicking acute coronary syndrome (ACS), is associated with poor clinical outcome. TTS is associated with different repolarization disorders including ST-segment elevation. ST elevation myocardial infarction (STEMI) in ACS is associated with declined prognosis. However, the clinical and prognostic impact of ST-segment elevation on TTS remains lacking. AIM: The aim of this study was to determine the short- and long-term prognostic impact of ST-segment elevation on TTS patients as compared with STEMI patients. PATIENTS AND METHODS: Our institutional database constituted a consecutive cohort of 138 TTS patients and 138 ACS patients matched for age and sex. TTS patients (n=41) with ST-segment elevation were compared with ACS patients with ST-segment elevation (n=64). RESULTS: Chest pain was significantly more documented in STEMI patients as compared with TTS patients (48.8% vs 78.1%; P<0.01). Cardiovascular risk factors such as diabetes mellitus (12.2% vs 29.7%; P=0.02) were significantly more presented in STEMI patients. Although the initial left ventricular ejection fraction (LVEF) was more declined in TTS patients (39%±9% vs 45%±16%; P<0.01), the LVEF was more declined in STEMI patients at follow-up (54%±10% vs 45%±16%; P=0.04). Inhospital complications such as respiratory failure were significantly more presented in TTS patients (68.3% vs 20.3%; P<0.01). The short-term as well as the long-term morality was similar in both groups. In univariate analysis, male sex, ejection fraction (EF) <35%, glomerular filtration rate (GFR) <60 mL/min, cardiogenic shock, inotropic drugs, and history of cancer were predictors of 5-year mortality. CONCLUSION: Rates of the long-term mortality in TTS patients with ST elevations are comparable with STEMI patients.

Impact of T-inversion on the outcome of Takotsubo syndrome as compared to acute coronary syndrome.

BACKGROUND: Previous studies revealed that patients with Takotsubo syndrome (TTS) have a higher mortality rate than the general population and a comparable mortality to acute coronary syndrome (ACS). Repolarisation abnormalities, namely T-wave amplitude, may provide incremental prognostic information, in addition to traditional risk factors in ACS. This study was performed to determine the short- and long-term prognostic impact of inverted T-waves in TTS patients, as compared to ACS patients. METHODS AND RESULTS: Our institutional database constituted a collective of 138 patients diagnosed with TTS from 2003 to 2017, as well as 532 patients suffering from ACS. Patients with TTS or with ACS (n = 138 per group) were matched for age and sex and assessed retrospectively and prospectively and divided into two groups, TTS with inverted T-waves (n = 123) and ACS with inverted T-waves (n = 80). In-hospital complications such as respiratory failure with the need of respiratory support (60.2% vs 6.3%; P < 0.01), thromboembolic events (13.8% vs 2.5%; P < 0.01) and cardiogenic shock (18.9% vs 8.8%; P = 0.05) were significantly more presented in TTS as compared to ACS patients. Among cardiovascular risk factors diabetes mellitus (23.6% vs 45.0%; P < 0.01) and arterial hypertension (57.7% vs 78.8%; P < 0.01) were more presented in ACS patients as compared to TTS patients. Short-term mortality was similar, however the long-term mortality of 5 years was significantly higher in the TTS group (25.2% vs 7.5%; P < 0.01). In univariate analysis were male gender, EF < 35%, GFR < 60 mL/min, cardiogenic shock, inotropic drugs and history of cancer predictors of 5-year mortality. The multivariate analysis showed only male gender (HR 2.7, 95% CI 1.1-6.5; P = 0.02), GFR < 60 mL/min (HR 2.8, 95% CI 1.2-6.0; P = 0.01) and history of cancer (HR 3.6, 95% CI 1.4-9.3; P < 0.01) as independent predictors of 5-year mortality. CONCLUSION: Rates of long-term mortality were significantly higher in TTS patients showing inverted T-waves compared with patients diagnosed with ACS with inverted T-waves. However, T-inversion was not an independent predictor of 5-year mortality in the multivariate analysis.