Multimodality Imaging of Intimal Sarcoma Causing Both Severe Mitral Stenosis and Mitral Regurgitation.

Intimal sarcomas (IS) are rare, malignant, rapidly progressive mesenchymal tumors that typically occur in the tunica intima of larger vessels, and they rarely involve the heart. IS are frequently misdiagnosed during the initial clinical presentation. This case report describes an uncommonly located IS, highlighting specific findings obtained through multimodality imaging.

Combination anticoagulation strategy in pregnancy with mechanical valves: The KYBELE study.

BACKGROUND: Optimal first-trimester anticoagulation is still challenging in pregnant women with mechanical heart valves (MHVs) requiring high-dose warfarin. This multicenter prospective study aims to determine the optimal anticoagulation regimens for pregnant patients with MHVs. METHODS: All women were allocated to one of three treatment options during first trimester including lone low-molecular-weight heparin (LMWH), combination of LMWH + 2.5 mg warfarin, and LMWH+4 mg warfarin. Primary maternal outcome included a combination of death, thromboembolism, severe bleeding, and need for treatment of mechanical valve thrombosis (MVT). Any fetal loss was determined as primary fetal outcome. RESULTS: The study included 78 pregnancies in 65 women with MHVs. Primary maternal outcome rate was 44%, 12.5%, 3.5%, respectively. The rates of primary maternal outcome (44 vs 3.5%, P < .001), obstructive MVT (16 vs 0%, P = .04), MVT requiring treatment (28 vs 0%, P = .003), and cerebral embolism (24 vs 3.4%, P = .041) were found to be significantly higher in lone LMWH group compared to LMWH + 4 mg warfarin group. Moreover, the rates of primary maternal outcome (12.5 vs 44%, P = .015) and treatment for MHV thrombus (4.2 vs 28%, P = .049) were significantly lower in LMWH + 2.5 mg warfarin group compared to lone LMWH group. The incidences of fetal loss were 8 (32%) in the lone LMWH group, 8 (33.3%) in LMWH + 2.5 mg warfarin group, and 11 (37.9%) in LMWH + 4 mg warfarin group (P = .890 for 3-group).Warfarin related-embryopathy was not observed in any case. CONCLUSIONS: The combined anticoagulation strategy of LMWH plus low-dose warfarin during the first trimester of pregnancy may result in less maternal complications with comparable fetal outcomes in patients with MHVs. CONDENSED ABSTRACT: Low-molecular-weight heparin (LMWH) is thought to be safer for the fetus, however it is suspected to be less protective for the mother. To solve this dilemma, the authors suggested a novel anticoagulation strategy in pregnant women with prosthetic valves. Seventy-eight pregnancies of 65 women (median age 32 [27-35] years) were included in the study. A combination of LMWH and a reduced dose warfarin were associated with low rates of thrombus-related complications in pregnant patients with mechanical heart valves.

Transcatheter Closure or Surgery for Symptomatic Paravalvular Leaks: The Multicenter KISS Registry.

BACKGROUND: The optimal treatment of symptomatic paravalvular leak (PVL) remains controversial between transcatheter closure (TC) and surgery. This large-scale study aimed to retrospectively evaluate the long-term outcomes of the patients who underwent reoperation or TC of PVLs. METHODS AND RESULTS: A total of 335 (men, 209 [62.4%]; mean age, 58.15±12.77 years) patients who underwent treatment of PVL at 3 tertiary centers between January 2002 and December 2021 were included. Echocardiographic features, procedure details, and in-hospital or long-term outcomes were assessed. The primary end point was defined as the all-cause death during follow-up. The regression models were adjusted by applying the inverse probability weighted approach to reduce treatment selection bias. The initial management strategy was TC in 171 (51%) patients and surgery in 164 (49%) cases. Three hundred cases (89.6%) had mitral PVL, and 35 (10.4%) had aortic PVL. The mean left ventricular ejection fraction was 52.03±10.79%. Technical (78.9 versus 76.2%; P=0.549) and procedural success (73.7 versus 65.2%; P=0.093) were similar between both groups. In both univariate and multivariable logistic regression analysis, the in-hospital mortality rate in the overall population was significantly higher (15.9 versus 4.7%) in the surgery group compared with the TC group (unadjusted odds ratio, 3.13 [95% CI, 1.75-5.88]; P=0.001; and adjusted odds ratio (inverse probability-weighted), 4.55 [95% CI, 2.27-10.0]; P<0.001). However, the long-term mortality rate in the overall population did not differ between the surgery group and the TC group (unadjusted hazard ratio [HR], 0.86 [95% CI, 0.59-1.25]; P=0.435; and adjusted HR (inverse probability-weighted), 1.11 [95% CI, 0.67-1.81]; P=0.679). CONCLUSIONS: The current data suggest that percutaneous closure of PVL was associated with lower early and comparable long-term mortality rates compared with surgery.

Transcatheter mitral paravalvular closure: a single centre experience with techniques and outcomes.

BACKGROUND: In patients with symptomatic mitral PVL, successful transcatheter reduction of the PVL to less than mild is associated with significant improvement in short- and midterm survival. OBJECTIVES: In this study, we present our single-centre, same operators' experience on percutaneous paravalvular leak closure with techniques and outcomes. METHODS: In this retrospective observational designed study, we retrieved hospital records of patients with a surgical history of mechanical or biological prosthetic valve replacement and who subsequently underwent transcatheter mitral paravalvular leak closure (TMPLC). All procedures were performed by the same operators. RESULTS: A total of 45 patients with 58 PVDs underwent TMPLC using 60 devices. All patients had moderate or severe mitral paravalvular regurgitation associated with symptomatic HF (15.6%), clinically significant haemolytic anaemia (57.8%) or both (26.7%). The technical success rate was 91.4%, with 53 defects successfully occluded. The clinical success rate was 75.6%. Among the clinical success parameters, the preprocedural median ejection fraction increased from 45% (35-55) to 50% (40-55) (p = .04). Mitral gradients decreased from max/mean 18/8 mmHg to max/mean 16/7 mmHg; p = .02). Haemoglobin levels increased from 9.9 (8.5-11.1) to 11.1 (3-13); p = .003. LDH levels decreased from 875 (556-1125) to 435 (314-579); p: <.001. All-cause 30-day and in-hospital mortality rates were the same at 8.9%. CONCLUSION: This single-centre study with a limited number of patients confirmed that TMPLC is a safe and effective procedure to improve symptoms and severity of PVL.

3D TEE imaging in a patient with severe tricuspid regurgitation with dextrocardia and tricuspid commissural prolapse.

Although dextrocardia is a rare congenital anomaly, it may be accompanied by numerous pathologies. Tricuspid valve prolapse is an extremely uncommon cause of primary tricuspid valve disease, as is its association with dextrocardia. In this instance, we wished to share our knowledge of tricuspid commissural prolapse together with dextrocardia, which we augmented with 3D images.

Impact of Naples Prognostic Score on midterm all-cause mortality in patients with decompensated heart failure.

Aim: This work was designed to investigate the relationship between cardiac outcomes and Naples Prognostic Score (NPS) among heart failure (HF) patients. Materials & methods: This retrospective observational study enrolled 298 consecutive individuals hospitalized for New York Heart Association class 3-4 HF. The primary outcome was all-cause mortality. Secondary outcomes were rehospitalization and in-hospital death. Results: The high NPS group had a statistically greater rate of all-cause mortality (p < 0.001). In Cox regression analysis, integrating NPS considerably improved the performance of the full model over the baseline model (adjusted hazard ratio = 2.28; p = 0.004). Based on time-dependent receiver operating characteristic curve analysis, the NPS model outperformed the baseline and CONUT score models in discriminatory power in predicting the probability of survival. Conclusion: NPS was associated with short- and midterm mortality as well as rehospitalization.

Heart failure is a serious condition that affects millions of individuals around the world. This study was designed to investigate whether there is a relationship between Naples Prognostic Score (NPS) and worse outcomes in heart failure patients. A total of 298 patients with advanced heart failure were included in the study. Patients with a high NPS are more likely to pass away and need to be readmitted to the hospital. NPS also predicted survival more accurately than some other variables at an average of 15 months follow-up. In conclusion, NPS was found to be useful in predicting short- and medium-term mortality and readmissions in patients with advanced heart failure.

Does papillary muscle free strain has predictive value in risk stratification of patients with hypertrophic cardiomyopathy?

BACKGROUND: Papillary muscle free strain has not been evaluated previously in hypertrophic cardiomyopathy (HCMP) patients. Our aim was to evaluate free papillary muscle free strain in HCMP patients and to find whether it has a value for prediction of sudden cardiac death (SCD) risk score. METHODS: Transthoracic echocardiography with tissue Doppler imaging, 2-D speckle tracking imaging (STI) of 55 HCMP patients and 45 controls were performed. HCMP patients were further divided into two groups according to their SCD risk score. Patients with risk score of less than 6 points constituted low/intermediate risk group, whereas patients with risk score of greater or equal to 6 points constituted high risk group. RESULTS: Interventricular septum, posterior wall, and left ventricular mass index were significantly higher, whereas mitral E/A ratio was significantly lower in HCMP patients compared to controls. Longitudinal apical 4C, 2C, 3C, global longitudinal LV strain, anterolateral papillary muscle (ALPM), posteromedial papillary muscle (PMPM) free strain were significantly reduced in HCMP group compared to control group. Global longitudinal strain and ALPM free strain were significantly lower in patients with high SCD risk score (-14.6 (-17.4 - -13.1) vs -11.6 (-13.2 - -10.1), p = 0.001 and -17.1 (-20.3 - -14.0) vs -9.2 (-12.6 - -7.5), p<0.001, respectively. Global longitudinal strain and ALPM free strain were statistically significantly correlated with SCD risk score (r = 0.480, p<0.001 and r = 0.462, p<0.001, respectively). Global longitudinal strain value of -12.60% had a sensitivity of 73.3% and specificity of 82.5% for predicting high SCD risk score (AUC: 0.787, 95% CI: 00.643-0.930, p = 0.001). ALPM free strain value of -12.95% had 66.7% sensitivity and 77.5% specificity for predicting high SCD risk score (AUC: 0.766, 95% CI: 0.626-0.905, p = 0.003). CONCLUSION: Papillary muscle free strain was reduced in HCMP patients. It might be used in risk stratification of these patients.

Gender-Dependent Comparison of Coronary Computed Tomography Angiographic Characteristics among Patients with Suspected Atherosclerosis: A Single-Center Experience.

OBJECTIVE: In this study, we aimed to examine gender-based differences in coronary artery disease (CAD) risk factors, the presence and severity of atherosclerosis, and the distribution of plaque type in patients presenting with chest pain. METHODS: A total of 1496 patients who applied to our cardiology outpatient clinic with chest pain and underwent computed coronary tomographic angiography (CTA) between August 2020 and October 2021 were included in the study. Plaque characteristics, Agatston score, and Coronary Artery Disease-Reporting and Data System (CAD-RADS) score obtained from the patients' CTAs were compared by gender. RESULTS: Of the 1496 patients evaluated, 47.9% were female. Coronary atherosclerosis was detected in 35.4% of females and 52.9% of males (P <0.001). Diabetes mellitus [155 (21.8%) vs. 123 (15.7%); P <0.001] and hypertension [271 (38.1%) vs. 249 (32%); P <0.001] rates were higher in females than in males. Plaque burden and high-risk plaque rate were found to be higher in males (P <0.001). Next, the rate of moderate-to-high coronary artery stenosis (CAD-RADS ≥3) was observed at 21.6% in men and 12.2% in women (P <0.001). Agatston score was found to be higher in males than in females for all age groups (P <0.001). The severity of CAD increased sharply with age in females (P interaction = 0.003). CONCLUSION: Although female patients demonstrated higher rates of traditional risk factors, the male gender was associated with increased coronary plaque burden, high-risk plaque, CADRADS, and Agatston scores. Therefore, patient-based approaches that consider gender-related differences could provide effective treatment and follow-up.

A rare cause of pulmonary hypertension: Idiopathic fibrosing mediastinitis obstructing right pulmonary artery and pulmonary veins.

Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) may occur as a severe complication of the disease. Herein, we report a patient diagnosed with fibrosing mediastinitis and pulmonary hypertension, whose imaging tests showed obstruction of right pulmonary artery and veins.

Double trouble during the left atrial appendage occlusion procedure.

Percutaneous structural interventions are being performed at increased numbers and rare findings or complications occur more frequently. Lipomatous hypertrophy of the interatrial septum (LHIS) is a relatively uncommon finding on transthoracic echocardiogram (TTE). The major challenge is the difficulty in performing transseptal puncture. We aimed to report the difficulties that were experienced during the left atrial appendage (LAA) closure in a case with an extreme form of LHIS.

The factors associated with progression of tricuspid regurgitation after left-sided double valve replacement in propensity score matched analysis.

Background: This study aims to investigate the association of progression of tricuspid regurgitation following double-valve replacement by comparing the tricuspid valve repair and no repair groups, and to analyze outcomes of patients with non-repaired mild-to-moderate tricuspid regurgitation. Methods: Between January 2014 and September 2017, a total of 157 patients (74 males, 83 females; mean age: 51.7±13.7 years; range, 18 to 78 years) who underwent aortic and mitral valve replacements with/without concomitant tricuspid valve repair were retrospectively analyzed. The patients were divided into two groups: no-repair (n=78) and repair groups (n=79). The primary outcome measure was development of more than moderate tricuspid regurgitation during follow-up. Results: The data were evaluated according to propensity score matched analysis. The progression of tricuspid regurgitation was significantly increased in the no-repair group (p=0.006). Rheumatic etiology was independently associated with the presence of postoperative moderateto- severe tricuspid regurgitation (p=0.004, odds ratio: 3.40). There was no statistically significant difference between the groups in terms of the potential complications and mortality and survival rates. A multivariable subgroup analysis for the baseline mild-to-moderate tricuspid regurgitation without repair showed that rheumatic etiology was an independent factor for the progression of postoperative tricuspid regurgitation (p=0.01). Conclusion: Our study results demonstrated that rheumatic etiology was an independent marker for increased tricuspid regurgitation and it was also independently associated with increased tricuspid regurgitation in patients with mild-to-moderate non-repaired patients. The degree of tricuspid regurgitation was improved in the repair group during follow-up.

A combination of severe complications in a case of infective endocarditis: Dehiscence of prosthetic aortic valve, aortic dissection, pseudoaneurysm, and hematoma causing right ventricular collapse.

Prosthetic valve endocarditis with mechanical complications causing pulmonary edema is fatal, therefore it needs to be diagnosed early and should be treated surgically in emergency setting. Transesophageal echocardiogram is crucial for recognizing the mechanical complications, which can be encountered on daily practice, but the coexistence of complications occurring on different mechanism is rather uncommon. Herein, we report a 21-year-old gentleman presenting with acute heart failure, whose imaging tests showed a combination of dehiscence of mechanical aortic valve prosthesis, aortic dissection, pseudoaneurysm, and hematoma causing right ventricular collapse.

Serum Levels of Cholesterol and Lipoproteins in Patients With Symptomatic Paravalvular Leaks.

Paravalvular leak (PVL) is a complication of valve replacement surgery which may lead to serious clinical consequences including hemolytic anemia. This study aimed to retrospectively evaluate the effect of successful intervention on serum lipid parameters in patients with PVL. A total of 106 patients (mean age: 57.2 ± 13.6 years, male: 67) who underwent surgical or transcatheter closure for symptomatic PVL were enrolled in this study. During the follow-up period, hemolysis and lipid parameters were evaluated at each clinical visit. This is the first study describing the effects of PVL on lipid metabolism after surgical or transcatheter closure. In the study, 18 patients (17%) had aortic PVL, 84 patients (79%) had mitral PVL, and 4 patients (3.8%) had both aortic and mitral PVL. A total of 59 patients underwent transcatheter closure and 47 patients were treated surgically. Technical success of the procedures was 83%. After successful PVL closure, hemoglobin and haptoglobin levels increased significantly (9.5 ± 1.3 vs 11.9 ± 2.1 g/dl, p <0.001 and 16.6 ± 7.9 vs 34.1 ± 19.9 mg, p <0.001, respectively). A significant increase in total cholesterol (158.9 ± 42.7 vs 209.3 ± 58.7 mg/dl, p <0.001), low-density lipoprotein cholesterol (99.1 ± 33.8 vs 133.9 ± 45.7 mg/dl, p <0.001), and high-density lipoprotein cholesterol (39.8 ± 12.4 vs 44.8 ± 11.7 mg/dl, p <0.001) levels was observed after successful PVL closure. In conclusion, symptomatic patients with PVL had hypocholesterolemia, reflected by low serum lipoprotein levels. After successful PVL closure, an increase in serum lipoprotein levels was observed. The recovery in levels of lipoproteins could be used as a marker of successful PVL closure, and absence of recovery of lipoprotein levels may indicate incomplete closure.

Diagnosis of Pulmonary Artery Sarcoma with Multi-modality Imaging.

Pulmonary artery sarcoma is an extremely uncommon malignancy with a poor prognosis. It is often difficult to distinguish it from pulmonary thromboembolic disease because of nonspe cific signs and symptoms as well as similar imaging findings. We present a 46-year-old man who had initially been diagnosed with presumed asthma that later proved to be pulmonary artery sarcoma. The patient was evaluated with multi-modality imaging studies which showed a mass in the pulmonary artery, its extension, mobility and invasion, and attachment to the artery wall. Pulmonary artery mass was excised and pulmonary artery endarterectomy was performed. The histopathological diagnosis was undifferentiated sarcoma with pleomorphic morphology.

Thrombolysis or Surgery in Patients With Obstructive Mechanical Valve Thrombosis: The Multicenter HATTUSHA Study.

BACKGROUND: Prosthetic valve thrombosis (PVT) is one of the life-threatening complications of prosthetic heart valve replacement. Due to the lack of randomized controlled trials, the optimal treatment of PVT remains controversial between thrombolytic therapy (TT) and surgery. OBJECTIVES: This study aimed to prospectively evaluate the outcomes of TT and surgery as the first-line treatment strategy in patients with obstructive PVT. METHODS: A total of 158 obstructive PVT patients (women: 103 [65.2%]; median age 49 years [IQR: 39-60 years]) were enrolled in this multicenter observational prospective study. TT was performed using slow (6 hours) and/or ultraslow (25 hours) infusion of low-dose tissue plasminogen activator (t-PA) (25 mg) mostly in repeated sessions. The primary endpoint of the study was 3-month mortality following TT or surgery. RESULTS: The initial management strategy was TT in 83 (52.5%) patients and surgery in 75 (47.5%) cases. The success rate of TT was 90.4% with a median t-PA dose of 59 mg (IQR: 37.5-100 mg). The incidences of outcomes in surgery and TT groups were as follows: minor complications (29 [38.7%] and 7 [8.4%], respectively), major complications (31 [41.3%] and 5 [6%], respectively), and the 3-month mortality rate (14 [18.7%] and 2 [2.4%], respectively). CONCLUSIONS: Low-dose and slow/ultraslow infusion of t-PA were associated with low complications and mortality and high success rates and should be considered as a viable treatment in patients with obstructive PVT.

A tricuspid mitral valve with severe regurgitation.

Tricuspid mitral valve is a rare entity, distinct from isolated clefts of the mitral leaflets. Echocardiographically, it manifests as the presence of three commissures, separate papillary muscles and concordant atrioventricular or ventriculoarterial connections. Three-dimensional transesophageal echocardiography helps distinguish the three cusps from clefts. Herein, we present a case of tricuspid mitral valve that led to severe mitral regurgitation.

Crochetage sign may predict late atrial arrhythmias in patients with secundum atrial septal defect undergoing transcatheter closure.

BACKGROUND: Atrial arrhythmias are well-known complications of atrial septal defect (ASD), and associated with substantial morbidity. After ASD closure, right atrial and ventricular enlargement regresses, however, the risk of atrial arrhythmia development continues. In this study, we aimed to investigate the relationship between the Crochetage sign, which is a possible reflection of heterogeneous ventricular depolarization due to long-term hemodynamic overload, and the development of late atrial arrhythmia after ASD closure. METHODS: This retrospective study included a total of 314 patients (mean age: 39.5 (30-50) years; male: 115) who underwent percutaneous device closure for secundum ASD. The study population was divided into two groups according to the presence or absence of the Crochetage sign. The Crochetage sign was defined as an M-shaped or bifid pattern notch on the R wave in one or more inferior limb leads. Cox-regression analysis was performed to determine independent predictors of late atrial arrhythmia development. RESULT: Fifty-seven patients (18.1%) presented with late atrial arrhythmia. Of these 57 patients, 30 developed new-onset atrial fibrillation/atrial flutter (AF/AFL), and 27 patients with pre-procedure paroxysmal AF/AFL had a recurrence of AF/AFL during follow-up. History of paroxysmal AF/AFL before the procedure (HR: 4.78; 95% CI 2,52-9.05; p < 0.001), the presence of Crochetage sign (HR: 3.90; 95% CI 2.05-7.76; p < 0.001), and older age at the time of ASD closure (HR: 1.03; 95% CI 1.01-1.06; p = 0.002) were found as independent predictors for late atrial arrhythmia. CONCLUSION: The presence of Crochetage sign may be used to predict the risk of late atrial arrhythmia development after transcatheter ASD closure.