Current barriers and recommendations on the diagnosis of transthyretin amyloid cardiomyopathy: a Delphi study.

Objectives: This study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases. Methods: This study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the "agree/strongly agree" or "disagree/strongly disagree" option. Results: The panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM. Conclusion: The diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.

The relation of right ventricular outflow tract measurements with in-hospital clinical outcomes after tricuspid valve surgery.

Right ventricular (RV) function is a determining factor for clinical outcomes in patients undergoing tricuspid valve surgery (TVS). Our aim was to investigate the importance of the function of the right ventricular outflow tract (RVOT), which is an important anatomical region of the RV, in patients underwent TVS. 104 patients who underwent TVS were analyzed retrospectively. Patients with previous cardiac surgery, congenital heart disease, or heart failure were excluded. The parasternal short-axis view at the level of the aortic root was used to measure RVOT dimensions and RVOT fractional shortening (RVOT-FS). The effect of RVOT diameter and function on major adverse cardiac events (MACE) after TVS was investigated. In our study, MACE, consisting of pacemaker implantation, acute kidney injury, postoperative atrial fibrillation and mortality, was developed at 44 (42.3%) patients.We compared the predictive performances of RVOT end-systolic (RVOTs) diameter, RVOT end-diastolic (RVOTd) diameter, RVOT-FS and RV diameters in prediction of MACE. The model including the RVOTs had higher AUC, R2 and likelihood ratio X2 values (0.775, 0.287 and 25.0, respectively) than RVOTd (0.770, 0.279 and 24.2, respectively) and RVOT-FS (0.750, 0.215 and 18.1, respectively). RVOT diameters showed better performance in predicting MACE than RV diameters. Moreover, there was statistically significant association between RVOTs, RVOTd and MACE (p value were 0.014 and 0.027, respectively), while no association between RVOT-FS and MACE (p value was 0.177). In summary, we determined that the RVOT diameters are important predictors for the in-hospital clinical outcomes of patients who underwent TVS.

Contribution of Three-Dimensional Echocardiography Xplane Mode in the Evaluation of Semilunar Cusps: Two Different Case Reports.

Orthogonal-biplane imaging (X-plane) is relatively a new tooL and provides comprehensive information about mitral valve and left atrial appendage evaluation. On the other hand, X-plane method is a promising method in the diagnosis of semilunar valve pathologies. Below, we report 2 cases, in which X-plane method was used, in order to clarify diagnosis. Case 1: Case 1 was a 63-year-old male patient who was admitted to our clinic with dyspnea. Right ventricular dilatation and hypertrophy were assessed. In order to determine the exact reason for right ventricular pressure overload, the pulmonary valve was evaluated by X-plane, and a bicuspid pulmonary valve was represented. This patient underwent pulmonary valve percutaneous valvuloplasty. Case 2: Case 2 was a 48-year-old male patient who was admitted to our hospital with a history of cerebrovascular event. His initial transthoracic and transesophageal echocardiography showed a hyperechoic mobile mass on the non-coronary cusp. However, after X-plane was used to detect the aortic valve, it was the prolapsed part of the non-coronary cusp adjacent to the commissure. These 2 case reports demonstrated the efficacy of the X-plane method in the diagnosis of semilunar valve pathologies in daily practice.

The Frequency of Fabry Disease in Patients with Cardiac Hypertrophy of Various Phenotypes Including Prominent Papillary Muscle: The TUCARFAB Study in Turkey.

BACKGROUND: The present study aimed to identify the frequency of Fabry disease in patients with cardiac hypertrophy of unknown etiology and to evaluate demographic and clinical characteristics, enzyme activity levels, and genetic mutations at the time of diagnosis. METHODS: This national, multicenter, cross-sectional, single-arm, observational registry study was conducted in adult patients with a clinical echocardiographic diagnosis of left ventricular hypertrophy and/or the presence of prominent papillary muscle. In both genders, genetic analysis was performed by DNA Sanger sequence analysis. RESULTS: A total of 406 patients with left ventricular hypertrophy of unknown origin were included. Of the patients, 19.5% had decreased enzyme activity (≤2.5 nmol/mL/h). Although genetic analysis revealed GLA (galactosidase alpha) gene mutation in only 2  patients (0.5%), these patients were considered to have probable but not 'definite Fabry disease' due to normal lyso Gb3 levels and gene mutations categorized as variants of unknown significance. CONCLUSION: The prevalence of Fabry disease varies according to the characteristics of the population screened and the definition of the disease used in these trials. From cardiology perspective, left ventricular hypertrophy is the major reason to consider screening for Fabry disease. Enzyme testing, genetic analysis, substrate analysis, histopathological examination, and family screening should be performed, when necessary, for a definite diagnosis of Fabry disease. The results of this study underline the importance of the comprehensive use of these diagnostic tools to reach a definite diagnosis. The diagnosis and management of Fabry disease should not be based solely on the results of the screening tests.

Assessment of papillary muscle free strain in hypertrophic cardiomyopathy and hypertension-induced left ventricular hypertrophy.

OBJECTIVES: We aimed to evaluate and compare papillary muscle free strain in hypertrophic cardiomyopathy (HCMP) and hypertensive (HT) patients. METHODS: Global longitudinal strain (GLS), and longitudinal myocardial strain of the anterolateral (ALPM) and posteromedial papillary muscles (PMPM) were obtained in 46 HCMP and 50 HT patients. RESULTS: Interventricular septum (IVS)/posterior wall (PW) thickness ratio, left ventricular mass index (LVMI), left atrial anteroposterior diameter (LAAP) and mitral E/E' were found to be increased in patients with HCMP compared to HT patients. Left ventricular cavity dimensions were smaller in HCMP patients. GLS of HCMP and HT patients were - 14.52 ± 3.01 and -16.85 ± 1.36%, respectively (p < 0.001). Likewise, ALPM and PMPM free strain values were significantly reduced in HCMP patients over HT patients [-14.00% (-22 to -11%) and -15.5% (-24.02 to -10.16%) vs -23.00% (-24.99 to -19.01%) and -22.30% (-26.48 to -15.95%) (p = 0.016 and p = 0.010)], respectively. ALPM free strain showed a statistically significant correlation with GLS, maximal wall thickness, IVS thickness and LVMI. PMPM free strain showed a significant correlation with GLS, IVS thickness and LAAP. The GLS value of - 13.05 had a sensitivity of 61.9% and a specificity of 97.4% for predicting HCMP. ALPM and PMPM free strain values of -15.31 and -17.17% had 63 and 76.9% sensitivity and 85.7 and 76.9% specificity for prediction of HCMP. CONCLUSIONS: Besides other echocardiographic variables, which were investigated in earlier studies, papillary muscle free strain also could be used in HCMP to distinguish HCMP- from HT-associated hypertrophy.

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.

Right atrial reservoir strain and right ventricular strain improves in patients recovered from hospitalisation for non-severe COVID-19.

PURPOSE: Those hospitalised with coronavirus disease 2019 (COVID-19) have recently been shown to have impaired right ventricular (RV) strain, but data about the course of heart function after discharge are limited. Our aim was to compare right ventricular strain and right atrial reservoir strain (RASr) associated with COVID-19 between acute disease (during hospitalisation) and follow-up (after discharge). METHODS: In this retrospective single-center study, we analysed the echocardiograms of 43 patients hospitalised for non-severe COVID-19 between December 2020 and March 2021, undergoing echocardiography both during and after hospitalisation. In addition to conventional echocardiographic parameters, we applied 2-dimensional speckle tracking to obtain RV global longitudinal strain (RV-GLS), RV free wall strain (RV-FWS), and RASr. RESULTS: Mean (standard deviation) age of the study population was 50 (9) years, and 18 (42%) of the participants were women. Median duration between exams was 6 months (range, 5-7 months). Both mean RV-GLS and mean RV-FWS significantly increased at follow-up (-20.8 [3.8] vs. -23.5 [2.8], p < 0.001 and -23.3 [4.2] vs. -28.2 [2.8], p < 0.001; respectively), and RASr significantly improved as well (-32.3 [6.6] vs. -41.9 [9.8], p < 0.001). CONCLUSION: In patients hospitalised for non-severe COVID-19 pneumonia, RV-GLS, RV-FWS, and RASr improved significantly between acute disease and 6 months after discharge.

Persistent right heart dilatation after percutaneous mitral balloon valvuloplasty: A weird coexistence of iatrogenic and congenital shunts.

We present an interesting case of concomitant congenital anomalies with an iatrogenic defect. The female patient underwent a percutaneous mitral balloon valvuloplasty due to rheumatic mitral stenosis. Unfortunately, an iatrogenic atrial septal defect (ASD) transpired during the procedure. Upon post-procedure examination, partial anomalous pulmonary venous (PAPVR) return was observed. The patient was symptomatic; on imaging, dilatation of the right heart chambers were detected. In addition, another crucial point was that the patient was planning a pregnancy, thus robotic surgery for iatrogenic ASD and PAPVR return was recommended. After a successful operation, the patient was asymptomatic and the size of right heart chambers were normalized.

A novel approach of tricuspid valve repair: mitralization of tricuspid valve.

OBJECTIVES: This study presents the mid-term results of a novel tricuspid valve (TV) repair strategy defined as 'mitralization of TV' (resection and plication of the posterior leaflet, ring implantation, optional leaflet procedures) applied for the correction of tricuspid regurgitation (TR). METHODS: Between 2017 and 2020, a total of 22 patients underwent concomitant TV repair using mitralization of the TV. Fourteen of the patients had functional TR (2 of them had severe tethering), 5 patients had prolapse and 3 patients had rheumatic involvement. RESULTS: There was no in-hospital mortality. Moderate or severe TR was not observed in any patient in echocardiographic evaluations before discharge. The mean follow-up duration was 30.9 + 6.2 months. Moderate-to-severe TR-free survival was 100% in the second year and 94.7% in the third year. CONCLUSIONS: Mitralization of the TV is a safe and effective treatment modality in terms of its mid-term results. This new technique provides an innovative perspective for the treatment of TR, especially in complex TV pathologies.

Cost-Effectiveness Analysis of the Triclip™ Transcatheter Tricuspid Valve Repair System in Patients with Tricuspid Regurgitation.

BACKGROUND: Tricuspid regurgitation is a condition that affects 1.6 million patients in the United States and is independently associated with morbidity and mortality. The TriClip™ procedure repairs the tricuspid valve without the need for open-heart surgery. The aim of this study is to evaluate the cost-effectiveness of TriClip™ treatment in patients with advanced tricuspid regurgitation from the Turkish reimbursement agency perspective. METHODS: Within the scope of this study, the general literature was searched in order to reach data on tricuspid regurgitation. The utilization of health care services used in the expert panel was re-calculated with the current reimbursement costs to determine the cost of heart failure in Turkey. In this study, Markov analysis, Tornado analysis, cost-effectiveness analysis, and partitioned survival analysis have been performed to determine whether TriClip™ is an effective treatment method compared to medication treatment. RESULTS: In according to calculations, 5-year survival rate was found as 49.91% for medication treatment and 57.64% for TriClip™ treatment. According to the analysis performed, the cost of medication treatment was calculated as €3879.72 and TriClip™ Transcatheter Tricuspid Valve Repair System treatment as €25 661.15 for a 60-month period in patients with tricuspid regurgitation and New York Heart Association III-IV. In the calculation, it was found that TriClip™ treatment gave patients an average of 1.64 life years and it was found to be cost-effective compared to medication treatment. CONCLUSIONS: Considering the positive effect of TriClip™ treatment on patients with tricuspid regurgitation in terms of mortality and regression of the heart failure stage, as recommended in the guidelines, widespread of its use has great importance.

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.

Giant pseudoaneurysm masquerading as large pericardial effusion in a patient with old myocardial infarction.

Pericardial effusions are common in patients with anterior myocardial infarction and resolve slowly after it. The diagnosis is generally established via echocardiography. However, a persistent, large pericardial effusion after the myocardial infarction may mask a more hazardous complication: pseudoaneurysm. Herein, we report a young gentleman presenting with dyspnea who had myocardial infarction and was misdiagnosed with large pericardial effusion.

[Turkish Cardiology Association Consensus Report: COVID-19 Pandemic and Cardiovascular Diseases (May 13, 2020)]. / Türk Kardiyoloji Dernegi Uzlasi Raporu: COVID-19 Pandemisi ve Kardiyovasküler Hastaliklar Konusunda Bilinmesi Gerekenler (13 Mayis 2020).

In December 2019, in the city of Wuhan, in the Hubei province of China, treatment-resistant cases of pneumonia emerged and spread rapidly for reasons unknown. A new strain of coronavirus (severe acute respiratory syndrome coronavirus-2 [SARS-CoV-2]) was identified and caused the first pandemic of the 21st century. The virus was officially detected in our country on March 11, 2020, and the number of cases increased rapidly; the virus was isolated in 670 patients within 10 days. The rapid increase in the number of patients has required our physicians to learn to protect both the public and themselves when treating patients with this highly infectious disease. The group most affected by the outbreak and with the highest mortality rate is elderly patients with known cardiovascular disease. Therefore, it is necessary for cardiology specialists to take an active role in combating the epidemic. The aim of this article is to make a brief assessment of current information regarding the management of cardiovascular patients affected by COVID-19 and to provide practical suggestions to cardiology specialists about problems and questions they have frequently encountered.

[Consensus Report from Turkish Society of Cardiology: COVID-19 and Cardiovascular Diseases. What cardiologists should know. (25th March 2020)]. / Türk Kardiyoloji Dernegi Uzlasi Raporu: COVID-19 Pandemisi ve Kardiyovasküler Hastaliklar Konusunda Bilinmesi Gerekenler (25 Mart 2020).

In December 2019, in the city of Wuhan, in the Hubei province of China, treatment-resistant cases of pneumonia emerged and spread rapidly for reasons unknown. A new strain of coronavirus (severe acute respiratory syndrome coronavirus-2 [SARS-CoV-2]) was identified and caused the first pandemic of the 21st century. The virus was officially detected in our country on March 11, 2020, and the number of cases increased rapidly; the virus was isolated in 670 patients within 10 days. The rapid increase in the number of patients has required our physicians to learn to protect both the public and themselves when treating patients with this highly infectious disease. The group most affected by the outbreak and with the highest mortality rate is elderly patients with known cardiovascular disease. Therefore, it is necessary for cardiology specialists to take an active role in combating the epidemic. The aim of this article is to make a brief assessment of current information regarding the management of cardiovascular patients affected by COVID-19 and to provide practical suggestions to cardiology specialists about problems and questions they have frequently encountered.