Caseous calcification of the mitral annulus - the complementary role of computed tomography and transthoracic echocardiogram.

PURPOSE: Caseous calcification is a relatively uncommon variant of calcification of the mitral annulus. The purpose of the study was to assess characteristic radiological features of caseous calcification of the mitral annulus (CCMA) using computed tomography (CT) and compare the usefulness of CT and transthoracic echocardiogram (TTE) in a diagnosis of CCMA. MATERIAL AND METHODS: Seventeen patients with CCMA, who underwent TTE and CT, were analysed. The following features of CCMA were evaluated: location, size, attenuation, enhancement after contrast administration, and margins. RESULTS: In all cases TTE visualised an echo-dense structure with an irregular appearance involving the mitral valve annulus. In five cases the acoustic shadowing artefact was visible, and in four cases the mass contained central areas of echolucency. Eleven patients had valve disease.On CT CCMA appeared as a round mass in one case, in 10 cases as an oval mass, and in six patients it had a semilunar shape.In all cases on unenhanced CT, CCMA appeared as a hyperdense mass. On enhanced CT, CCMA in 10 cases (58.8%) had a hypodense centre, and in 7seven (41.2%) it had a hyperdense centre without enhancement after contrast administration. A hyperdense rim was observed in all cases except one patient. CONCLUSIONS: In cases of the atypical appearance of CCMA on TTE, CT can lead to a definitive diagnosis. The combination of unenhanced CT and after IV contrast administration scans allows for recognition and distinction of CCMA from other pathologies, while TTE allows for assessment of additional valve dysfunction.

Clinical settings leading to presystolic tricuspid regurgitation.

PURPOSE: Few studies describe diastolic (presystolic) tricuspid regurgitation (DTR) mainly in the context of atrioventricular conduction abnormalities. Little is known about its occurrence in the other clinical settings. METHODS: We identified patients with DTR recorded during routine echocardiographic examinations. DTR was defined as low velocity backward flow through the tricuspid valve orifice during end-diastole recorded with a continuous and/or pulsed-wave Doppler and/or color-coded M-mode Doppler echocardiography. RESULTS: Diastolic tricuspid regurgitation was present in a wide variety of clinical entities. Of the 23 patients with DTR 6 patients had ischemic and 10 dilated cardiomyopathy. Others had clinical conditions including: inappropriate pacemaker settings, decompensated hypertrophic cardiomyopathy, biventricular dysfunction following orthotropic heart transplantation, torrential aortic regurgitation, low ejection fraction aortic stenosis, advanced endocardial fibroelastosis, and complex congenital heart disease. Twenty of 23 patients had significantly impaired right ventricle (RV) systolic function. Systolic tricuspid regurgitation was estimated as moderate or severe in 13 cases and mild in the remaining 10 cases. RV systolic pressure was significantly elevated in all but 2 cases. In all but 4 cases DTR was transient. The persistence of DTR was associated with severe pulmonary hypertension, severe biventricular failure, and persistent severe pulmonary regurgitation. CONCLUSIONS: Diastolic tricuspid regurgitation may be encountered in a variety of clinical settings and should be sought for especially in patients with advanced RV systolic dysfunction, pulmonary hypertension, pulmonary regurgitation, or conduction abnormalities. Significant systolic regurgitation is not prerequisite for the development of DTR.

Long-term myocardial performance of the systemic right ventricle during exercise in patients with transposition of the great arteries and atrial switch operation.

The most common clinical scenario in which RV is systemic, is dextroposition transposition of the great arteries (dTGA). This is the second most common cyanotic congenital heart disease [2]. Before the arterial switch operation was introduced in 1975 [3], patients with dTGA were treated with the Mustard or Senning procedure, also referred to as the atrial switch operation, where the systemic veins are routed to the subpulmonary morphological left ventricle and the pulmonary veins are routed to the systemic right ventricle [4].The atrial switch operation leaves the RV in a systemic position, leading to gradual RV dysfunction and RV failure [5-8]. In this context, sRV functional reserve and its ability to augment performance with exercise performance is not well studied.

ACE Inhibitor and Angiotensin Receptor Blocker use during Pregnancy: data from the ESC Registry Of Pregnancy And Cardiac disease (ROPAC).

Angiotensin-converting enzyme inhibitors (ACE-Is) and angiotensin receptor blockers (ARBs) are not recommended during the second and third trimester due to the significant risk of congenital anomalies associated with their use. However, data are scarce, especially regarding their use in the first trimester and about the impact of stopping just before pregnancy. Our study illustrates the profile of the women who used ACE-Is or ARBs during pregnancy and evaluates the impact on perinatal outcomes. The Registry of Pregnancy and Cardiac disease (ROPAC) is a prospective, global registry of pregnancies in women with structural heart disease. Outcomes were compared between women who used ACE-Is or ARBs and those who did not. Multivariable regression analysis was performed to assess the effect of ACE-I or ARB use on the occurrence of congenital anomalies. ACE-I (n=35) and/or ARB (n=8) were used in 42 (0.7%) of the 5739 ROPAC pregnancies. Women who used ACE-Is or ARBs more often came from a low-or-middle-income country (57% vs 40%, p=0.021), had chronic hypertension (31% vs 6%, p<0.001), or a left ventricular ejection fraction <40% (33% vs 4%, p<0.001). In the multivariable analysis, ACE-I use during the first trimester was associated with an increased risk of congenital anomaly (OR 3.2; 95% CI 1.0-9.6). Therefore, ACE-Is should be avoided during pregnancy, also in the first trimester, due to a higher risk of congenital anomalies. However, there is no need to stop long before pregnancy. Preconception counseling is crucial to discuss the potential risks of these medications, to evaluate the clinical condition and, if possible, change or stop the medication.

Tetralogy of Fallot Associated with Aberrant Right Subclavian Artery. Clinical Implications. / Tetralogia de Fallot Associada a Artéria Subclávia Direita Aberrante. Implicações Clínicas.

Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).

Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).

Predictors of COVID-19 outcomes in adult congenital heart disease patients - anatomy versus function.

BACKGROUND: It is unclear whether patients with adult congenital heart disease (ACHD) should be considered as an increased risk population with poor outcomes when suffering from COVID-19. AIMS: This study aimed to collect clinical outcome data and to identify risk factors of a complicated course of COVID-19 among ACHD patients. METHODS: Among all outpatients who came to medical attention via telemedicine or direct physician contact at our institution between September 1, 2020 and March 31, 2021, we included all with a COVID-19 diagnosis. The incidence of COVID-19, a clinical course of the disease, and outcome were determined. RESULTS: One hundred and four (8.7%) out of 1 197 patients who were seen at our outpatient clinic for ACHD patients met the definition of COVID-19. Most of them reported a mild course of COVID-19 (99 [95.5%]). Five patients (4.5%) experienced severe symptoms and needed hospitalization. Two patients (1.9% of all with a confirmed diagnosis, 40% with severe infection) died. In the multivariable analysis, decreased systemic ventricular systolic function and any significant valve stenosis were predictors of a complicated disease course. CONCLUSIONS: Our study confirmed previous results showing that a physiology-based model, rather than an anatomy-based model, better predicted COVID-19 outcomes among ACHD patients, which is of importance for patients and healthcare providers during the COVID-19 pandemic.

Cardiac Lipomatosis: A Rare Form of Left Ventricular Hypertrophy.

A 30-year-old female patient was referred to our centre due to an abnormal electrocardiography showing left ventricular hypertrophy. The physical examination was normal, and the patient was asymptomatic. Echocardiography was performed, revealing an atypical pattern of left ventricular hypertrophy with heterogeneous echogenicity of the left ventricular walls. A cardiac magnetic resonance examination revealed marked thickening of the interventricular septum and left and right ventricular walls. The signal intensity of the thickened ventricular walls was increased and was similar to that of subcutaneous fat. Follow-up echocardiography and cardiac magnetic resonance showed no progression of left ventricular hypertrophy.

Pregnancy-related cardiac non-elective hospitalizations and pregnancy outcomes. A tertiary referral cardiac center experience.

BACKGROUND: Pregnant women with cardiovascular diseases (CVD) and their offspring are at higher risk of morbidity and mortality. AIMS: To provide data on pregnancy outcomes among women with different types of CVD requiring non-elective cardiac hospitalization in a tertiary referral cardiac center. METHODS: We identified all records of non-elective hospitalizations of pregnant women hospitalized between January 2009 through March 2018, at our institution - a tertiary referral cardiac center. The incidence and types of cardiac complications during pregnancy, as well as the pregnancy and offspring outcomes, were determined. RESULTS: One hundred and sixty-one out of 328 pregnancy-related hospitalizations in 140 pregnancies were non-elective. Cardiac complications occurred in 62 (44%) pregnancies, with the most frequent being episodes of arrhythmia (22.1% pregnancies), followed by heart failure exacerbations (6.4% pregnancies). Maternal mortality reached 2.1% and affected only women with primary cardiomyopathies (CMP). Offspring mortality was 2.8%. Newborns of mothers with cardiac complications had significantly lower Apgar scores and gestational age at delivery, compared to mothers without cardiac complications. CONCLUSIONS: In our series mortality and morbidity among pregnant women with CVD hospitalizations were high. An unfavorable maternal outcome mainly affected women with CMP. Offspring of mothers with cardiovascular complications are prone to have a lower gestational age and Apgar score.

Echocardiography during the coronavirus disease 2019 pandemic - the impact of the vaccination program. A 2021 update of the expert opinion of the Working Group on Echocardiography of the Polish Cardiac Society.

The coronavirus disease 2019 (COVID-19) pandemic resulted in an urgent need to reorganize the work of echocardiography laboratories in order to ensure the safety of patients and the protection of physicians, technicians, and other staff members. In the previous Expert Opinion of the Working Group on Echocardiography of Polish Cardiac Society we provided recommendations for the echocardiographic services, in order to ensure maximum possible safety and efficiency of imagers facing epidemic threat. Now, with much better knowledge and larger experience in treating COVID-19 patients and with introduction of vaccination programs, we present updated recommendations for performing transthoracic and transesophageal examinations, including information on the potential impact of personnel and the patient vaccination program, and growing numbers of convalescents on performance of echocardiographic laboratories, with the goal of their ultimate reopening.

Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry.

BACKGROUND: Patients with systemic right ventricle (sRV), including transposition of great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of great arteries (ccTGA), may require anticoagulation for thromboembolism (TE) prevention. In the absence of data on non-vitamin K antagonist oral anticoagulants (NOACs), vitamin K antagonists (VKAs) remain the agent of choice. We investigated the safety, efficacy and feasibility of NOACs treatment in adults with sRV in a worldwide study. METHODS: This is an international multicentre prospective study, using data from the NOTE registry on adults with sRV taking NOACs between 2014 and 2019. The primary endpoints were TE and major bleeding (MB). The secondary endpoint was minor bleeding. RESULTS: A total of 76 patients (42.5 ±â€¯10.0 years, 76% male) with sRV (74% TGA, 26% ccTGA) on NOACs were included in the study. During a median follow-up of 2.5 years (IQR1.5-3.9), TE events occurred in 3 patients (4%), while no MB episodes were reported. Minor bleeding occurred in 9 patients (12%). NOAC treatment cessation rate was 1.4% (95%CI:0.3-4%) during the first year of follow-up. All the patients with TE events had a CHA2DS2-VASc score ≥ 2 and impaired sRV systolic function at baseline. The total incidence of major events during follow-up was significantly lower compared to historical use of VKAs or aspirin before study inclusion (1.4% (95%CI:0.29-4%) vs 6,9% (95%CI:2.5-15.2%); p = .01). CONCLUSIONS: In this prospective study, NOACs appear to be well-tolerated, with excellent efficacy and safety at mid-term in patients with sRV.

[Not only after myocardial infarction - left intraventricular thrombus in the Churg-Strauss syndrome]. / Nie tylko po zawale serca- skrzeplina w lewej komorzew przebiegu zespolu Churga i Strauss.

Churg-Strauss syndrome is a rare systemic disease characterized by necrotising vasculitis and peripheral eosinophilia. Cardiac involvement is found in up to 64% of patients and is given a high rank among the causes of morbidity and mortality. We presented a case of 26 year-old male with Churg-Strauss syndrome and left intraventricular thrombus.

Echocardiography during the coronavirus disease 2019 (COVID-19) pandemic: expert opinion of the Working Group on Echocardiography of the Polish Cardiac Society.

Transthoracic and transesophageal echocardiography may be required in patients with coronavirus disease 2019 (COVID­19), resulting in direct contact with the patient and risk of transmitting the infection from patients to medical personnel. Therefore, we recommend to perform problem­oriented time­limited transthoracic examinations. Whenever possible, examinations should be analyzed offline, outside the isolation zones. Transesophageal echocardiography is considered an aerosol­generating procedure and should be performed only as a lifesaving procedure. Personnel should use appropriate personal protection equipment in the immediate vicinity of the patients in accordance with the relevant guidelines.

Galectin-3 plasma levels in adult congenital heart disease and the pressure overloaded right ventricle: reason matters.

Aim: To assess galectin-3 (Gal-3) levels and their relationship with clinical status and right ventricular (RV) performance in adults with RV pressure overload of various mechanisms due to congenital heart disease. Materials & methods: A cross-sectional study was conducted. Patients underwent clinical examination, blood testing and transthoracic echocardiography. Results: The study included 63 patients with congenitally corrected transposition of the great arteries, 41 patients with Eisenmenger syndrome and 20 healthy controls. Gal-3 concentrations were higher in patients compared with controls (7.83 vs 6.11 ng/ml; p = 0.002). Biomarker levels correlated with age, New York Health Association class, N-terminal probrain natriuretic peptide and RV function only in congenitally corrected transposition of the great arteries patients. Conclusion: Gal-3 profile in congenital heart disease patients and pressure-overloaded RV differs according to the cause of pressure overload.

Long-term follow-up and comparison of techniques in radiofrequency ablation of ventricular arrhythmias originating from the aortic cusps (AVATAR Registry).

INTRODUCTION: Radiofrequency ablation (RFA) of outflow tract ventricular arrhythmia (VA) that originates from the aortic cusps can be challenging. Data on long­ term efficacy and safety as well as optimal technique after aortic cusp ablation have not previously been reported. OBJECTIVES: This aim of the study was to determine the short- and long­ term outcomes after RFA of aortic cusp VA, and to evaluate aortic valve injuries according to echocardiographic screening. PATIENTS AND METHODS: This was a prospective multicenter registry (AVATAR, Aortic Cusp Ventricular Arrhythmias: Long Term Safety and Outcome from a Multicenter Prospective Ablation Registry) study. A total of 103 patients at a mean age of 56 years (34-64) from the "Electra" Registry (2005-2017) undergoing RFA of aortic cusps VA were enrolled. The following 3 ablation techniques were used: zero­fluoroscopy (ZF; electroanatomical mapping [EAM] without fluoroscopy), EAM with fluoroscopy, and conventional fluoroscopy­ based RFA. Data on clinical history, complications after RFA, echocardiography, and 24­ hour Holter monitoring were collected. The follow up was 12 months or longer. RESULTS: There were no major acute cardiac complications after RFA. In one case, a vascular access complication required surgery. The median (interquartile range [IQR]) procedure time was 75 minutes (IQR, 58-95), median follow­ up, 32 months (IQR, 12-70). Acute and long term procedural success rates were 93% and 86%, respectively. The long­ term RFA outcomes were observed in ZF technique (88%), EAM with fluoroscopy (86%), and conventional RFA (82%), without differences. During long­ term follow­up, no abnormalities were found within the aortic root. CONCLUSIONS: Ablation of VA within the aortic cusps is safe and effective in long­ term follow up. The ZF approach is feasible, although it requires greater expertise and more imaging modalities.

[Acute aortic dissection type A with concomitant myocardial infarction]. / Ostre rozwarstwienie aorty wstepujacej ze wspólistniejacym rozleglym zawalem serca - trudny problem kliniczny i diagnostyczny.

The authors describe a patient with acute aortic dissection type A complicated by left main coronary artery occlusion and acute myocardial infarction. The patient underwent urgent surgery, however, died 4 hours after operation.

In silico thrombin and factor Xa generation profiles in adult patients after Fontan operation.

: Single-ventricle defects are associated with increased risk of thromboembolic events. To analyze the prothrombotic potential in a long-term follow-up on Fontan patients via plasma contribution to thrombin and factor (F)Xa generation profiles. Thrombin and FXa generation was simulated from plasma concentrations of FII, FV, FVII, FVIII, FIX, FX, antithrombin and tissue factor (TF) pathway inhibitor from Fontan patients (n = 48) and healthy controls (n = 34). TF and thrombin-antithrombin complex (TAT) were measured by ELISA. Fontan patients had significantly reduced procoagulant protein concentrations and increased anticoagulant protein concentrations over controls, resulting in a lowered procoagulant potential. However, Fontan patients showed increased hemostatic activation as evidenced by increased TF and TAT. Modeling this increased TF showed a more prothrombotic profile. Observed changes in procoagulant and anticoagulant proteins may be a compensatory mechanism aimed at mitigating the underlying disease effects characterized by elevated TF and TAT.

Cardiac arrest and ventricular arrhythmia in adults with Ebstein anomaly and left ventricular non-compaction.

BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features). We reviewed echocardiograms and CMR scans for concomitant morphological abnormalities (LVNC, PDA - patent ductus arteriosus, VSD - ventricular septal defect, ASD - atrial septal defect, mitral valve prolapse, BAV - bicuspid aortic valve, CoA - coarctation of aorta). RESULTS: The studied group consisted of 84 consecutive patients (mean age 38±15 years, 50 women) with EA. We found four patients (4.8%) with LVNC, two of them had cardiac arrest, one had VT, and one was symptomless, but had QTc prolongation in Holter recordings. Concomitant abnormalities were VSD (4.8%), PDA (1.2%), CoA (1.2%), mitral valve prolapse (1.2%), and BAV (2.4%). The most common anomaly was ASD type II - 23 patients (27.3%) and WPW - Wolff-Parkinson-White's syndrome - 9 patients (10.7%). CONCLUSIONS: Non-compaction is a notable abnormality in adult patients with EA and it may affect their prognosis. Although other concomitant lesions were more common, only patients with LVNC suffered from cardiac arrest or ventricular arrhythmia.

Collagen turnover biomarkers and systemic right ventricle remodeling in adults with previous atrial switch procedure for transposition of the great arteries.

BACKGROUND: Myocardial fibrosis is a potential pathophysiological mechanism leading to systemic right ventricular (SRV) deterioration. We hypothesize that circulating levels of collagen deposition markers are elevated in patients with SRV remodeling and this elevation may have a predictive value. METHODS: We prospectively evaluated 56 patients with D-TGA after the atrial switch procedure (mean age 25.6 ± 4.8, range 18-37 years; 67% males). Serum levels of procollagen type III amino-terminal propeptide (PIIINP), collagen type I carboxy-terminal telopeptide (CITP), procollagen type I N-terminal propeptide (PINP), matrix metalloproteinase (MMP 1, MMP 9) and a tissue inhibitor of matrix metalloproteinase (TIMP 1) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured and compared with healthy controls. The relationship between these serum markers, echocardiographic and cardiac magnetic resonance parameters and the outcome at a follow-up of 61 months (range, 24-85 months) was determined. RESULTS: Compared with the healthy control group, the study group had significantly higher levels of TIMP1, PIIINP, CITP, PINP and NT-pro-BNP (p<0.05, each). The levels of PIIINP and CITP were significantly higher among patients with an SRV mass index above the mean value. The level of PIIINP was significantly higher among patients with an SRV EDV index above the mean value. CITP was significantly elevated in SRV late gadolinium enhanced (LGE) positive patients, compared to patients without SRV LGE. MMP9 and TIMP1 predicted an adverse clinical outcome on univariate Cox proportional hazard survival analysis in addition to well proven predictors of outcome (SRV EF and NYHA). CONCLUSIONS: We demonstrated a pattern of altered collagen turnover adversely related with the indices of SRV remodeling and an adverse clinical outcome in patients with SRV.