Incorrect. Measurements after bedside manoeuvres alone are insufficient in a symptomatic patient. It is recommended to perform further exercise stress echocardiography (Class I)

Incorrect. Pharmacological provocation with dobutamine is not advised, as it is not physiological and can be poorly tolerated

Correct. Exercise stress echocardiography is recommended in symptomatic patients if bedside manoeuvres fail to induce LVOT gradient ≥50 mmHg (Class I)

Incorrect. The use of catheterization to assess the severity of LVOT obstruction may be considered in HCM patients with inconclusive non-invasive cardiac imaging. However, this is not recommended yet, as the complete set of recommended non-invasive examinations has not been performed (Class IIb).

Incorrect. Depolarization abnormalities such as low QRS voltage are a common finding in NDLVC (non-dilated left ventricular cardiomyopathy) caused by DSP, PLN variants and laminopathies.

Correct. Prolonged PR interval or AV block is frequent in neuromuscular causes of NDLVC (non-dilated left ventricular cardiomyopathy) and in sarcoidosis.

Incorrect. Prolonged PR interval or AV block is frequent in neuromuscular causes of NDLVC and in sarcoidosis.

Correct. Depolarization abnormalities such as low QRS voltage are a common finding in NDLVC caused by DSP, PLN variants and laminopathies.

Correct. There are very few data to guide risk stratification in patients with NDLVC without a known causative gene variant, but on the basis of the existing literature, the Task Force suggests that it may be reasonable to consider primary prevention ICD implantation in patients with NSVT, a family history of SCD, or significant LGE.

Correct. There are very few data to guide risk stratification in patients with NDLVC without a known causative gene variant, but on the basis of the existing literature, the Task Force suggests that it may be reasonable to consider primary prevention ICD implantation in patients with NSVT, a family history of SCD, or significant LGE.

Correct. There are very few data to guide risk stratification in patients with NDLVC without a known causative gene variant, but on the basis of the existing literature, the Task Force suggests that it may be reasonable to consider primary prevention ICD implantation in patients with NSVT, a family history of SCD, or significant LGE.

Incorrect. As per guidelines, significant LGE on MRI is required.

Correct. Cardiac magnetic resonance with LGE in NDLVC confirms the presence of non-ischaemic myocardial fibrosis that is essential for the diagnosis in most cases. It can also show the extent and pattern of fibrosis distribution. This can provide clues to the underlying aetiology: subepicardial distribution in post-myocarditis forms, patchy in sarcoidosis, extensive inferolateral in dystrophinopathies, septal mid-wall in LMNA carriers, and ring-like in DSP and FLNC variant carriers.

Correct. Cardiac magnetic resonance with LGE in NDLVC confirms the presence of non-ischaemic myocardial fibrosis that is essential for the diagnosis in most cases. It can also show the extent and pattern of fibrosis distribution. This can provide clues to the underlying aetiology: subepicardial distribution in post-myocarditis forms, patchy in sarcoidosis, extensive inferolateral in dystrophinopathies, septal mid-wall in LMNA carriers, and ring-like in DSP and FLNC variant carriers.

Correct. Cardiac magnetic resonance with LGE in NDLVC confirms the presence of non-ischaemic myocardial fibrosis that is essential for the diagnosis in most cases. It can also show the extent and pattern of fibrosis distribution. This can provide clues to the underlying aetiology: subepicardial distribution in post-myocarditis forms, patchy in sarcoidosis, extensive inferolateral in dystrophinopathies, septal mid-wall in LMNA carriers, and ring-like in DSP and FLNC variant carriers.

Correct. Cardiac magnetic resonance with LGE in NDLVC confirms the presence of non-ischaemic myocardial fibrosis that is essential for the diagnosis in most cases. It can also show the extent and pattern of fibrosis distribution. This can provide clues to the underlying aetiology: subepicardial distribution in post-myocarditis forms, patchy in sarcoidosis, extensive inferolateral in dystrophinopathies, septal mid-wall in LMNA carriers, and ring-like in DSP and FLNC variant carriers.

Incorrect. Men are affected more frequently than women and an age-related penetrance has been demonstrated, with high clinical and genetic variability.

Incorrect. Arrhythmogenic right ventricular cardiomyopathy usually manifests in the second to fourth decade of life.

Correct. Men are affected more frequently than women and an age-related penetrance has been demonstrated, with high clinical and genetic variability.

Correct. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized structurally by a progressive myocardial atrophy with fibro-fatty replacement of the RV myocardium, but lesions can also be present in the LV myocardium.

Correct. Abnormalities of the repolarization and depolarization as well as arrhythmias are key to the diagnosis of ARVC.

Correct. However, it has been noted that epsilon waves are frequently overdiagnosed and that there is poor agreement even between experts regarding their presence. Furthermore, it has been demonstrated that they occur in the presence of severe structural disease and thus add little to the diagnosis. Therefore, epsilon waves should be utilized for diagnostic purposes with caution.

Correct. However, the diagnostic utility of late potentials on signal-averaged electrocardiogram (SAECG) has been challenged in patients with ARVC for showing poor sensitivity and specificity. Therefore SAECG should be utilized for diagnostic purposes with caution.

Correct. Abnormalities of the repolarization and depolarization as well as arrhythmias are key to the diagnosis of ARVC.

Correct. Differential diagnosis in patients with suspected ARVC includes myocarditis, sarcoidosis, RV infarction, DCM (dilated cardiomyopathy), Chagas disease, pulmonary hypertension, and CHD with volume overload (such as Ebstein anomaly or atrial septal defect).

Correct. Differential diagnosis in patients with suspected ARVC includes myocarditis, sarcoidosis, RV infarction, DCM (dilated cardiomyopathy), Chagas disease, pulmonary hypertension, and CHD with volume overload (such as Ebstein anomaly or atrial septal defect).

Correct. Differential diagnosis in patients with suspected ARVC includes myocarditis, sarcoidosis, RV infarction, DCM (dilated cardiomyopathy), Chagas disease, pulmonary hypertension, and CHD with volume overload (such as Ebstein anomaly or atrial septal defect).

Correct. Differential diagnosis in patients with suspected ARVC includes myocarditis, sarcoidosis, RV infarction, DCM (dilated cardiomyopathy), Chagas disease, pulmonary hypertension, and congential heart disease with volume overload (such as Ebstein anomaly or atrial septal defect).

Incorrect. GLA is a gene associated with Anderson–Fabry disease, which is a specific type of HCM. Anderson–Fabry disease is an inborn error of metabolism where a deficient or absent enzyme, alpha-galactosidase A (α-Gal A), causes the storage of some degradation cell products, mainly globotriaosylceramide in patient’s lysosomes. This storage causes cell dysfunction and activates cellular hypertrophy pathways, common to other causes of HCM, as well as inflammation and immune activation.

Correct. The genes underlying ARVC mainly encode proteins of the cardiac desmosome: plakophilin-2 (PKP2), desmoplakin (DSP), desmoglein-2 (DSG2), desmocollin-2 (DSC2), and plakoglobin (JUP). In addition to desmosomal genes, pathological variants have also been described in other genes, including DES, TMEM43, and PLN.

Correct. The genes underlying ARVC mainly encode proteins of the cardiac desmosome: plakophilin-2 (PKP2), desmoplakin (DSP), desmoglein-2 (DSG2), desmocollin-2 (DSC2), and plakoglobin (JUP). In addition to desmosomal genes, pathological variants have also been described in other genes, including DES, TMEM43, and PLN.

Correct. The genes underlying ARVC mainly encode proteins of the cardiac desmosome: plakophilin-2 (PKP2), desmoplakin (DSP), desmoglein-2 (DSG2), desmocollin-2 (DSC2), and plakoglobin (JUP). In addition to desmosomal genes, pathological variants have also been described in other genes, including DES, TMEM43, and PLN.

Incorrect. Experience with antiarrhythmics dofetilide and ranolazine is limited to very small case series, therefore no recommendation for its use can be reliably stated.

Correct. Beta-blocker therapy is recommended in ARVC patients with VE, NSVT, and VT (Class I, Level C).

Correct. Flecainide should be considered when single agent treatment has failed to control arrhythmia-related symptoms in patients with ARVC or when autonomic side effects limit the use of beta-blockers (Class IIa)

Correct. Amiodarone should be considered when regular beta-blocker therapy fails to control arrhythmia-related symptoms in patients with ARVC (Class IIa)

Incorrect. Patients with overt RCM manifest signs and symptoms typical of HFpEF. In the advanced phases, the stroke volume declines, and the heart rate may increase. Hepatomegaly, ascites, and peripheral edema are common in decompensated patients.

Correct. Patients with overt RCM manifest signs and symptoms typical of HFpEF. In the advanced phases, the pulse volume is low, the stroke volume declines, and the heart rate may increase. Hepatomegaly, ascites, and peripheral edema are common in decompensated patients.

Correct. Patients with overt RCM manifest signs and symptoms typical of HFpEF. In the advanced phases, the pulse volume is low, the stroke volume declines, and the heart rate may increase. Hepatomegaly, ascites, and peripheral edema are common in decompensated patients.

Correct. Patients with overt RCM manifest signs and symptoms typical of HFpEF. In the advanced phases, the pulse volume is low, the stroke volume declines, and the heart rate may increase. Hepatomegaly, ascites, and peripheral edema are common in decompensated patients.

Correct. The diagnosis of restrictive cardio-desminopathy is confirmed by accumulations of desmin-immunoreactive material in cardiac muscle, which must be confirmed ultrastructurally, and in skeletal muscle, which may be seen on light microscopic immunohistochemical analysis.

Correct. Imununohistochemical detection of ferritin is an early marker of iron overload in the heart. The switch from the acidic to the basic ferritin in the heart is strictly associated with hereditary hemochromatosis and with secondary iron overload with a positive human leukocyte antigen typing for A3 hemochromatosis allele.

Correct. Endomyocardial biopsy is a precision diagnostic tool in restrictive cardio-desminopathies; iron myocardial overload, both intramyocyte in haemochromatosis and mitochondrial in Friedreich ataxia cardiomyopathy; cystinosis; generalized arterial calcification of infancy; and lysosomal storage diseases (LSDs)

Correct. Endomyocardial biopsy is a precision diagnostic tool in restrictive cardio-desminopathies; iron myocardial overload, both intramyocyte in haemochromatosis and mitochondrial in Friedreich ataxia cardiomyopathy; cystinosis; generalized arterial calcification of infancy; and lysosomal storage diseases (LSDs)

Incorrect. Mavacamten is a cardiac myosin ATPase inhibitor that reduces the LVOT gradient and improves exercise capacity. This drug should be considered as monotherapy in symptomatic adult HCM patients with LVOTO who are intolerant or have contraindications to beta-blockers, verapamil/diltiazem, or disopyramide (Class IIa recommendation).

Correct. Dehydration can lead to hypovolemia, resulting in inadequate diastolic filling pressure. Consequently, it can exacerbate the reduction in cardiac output caused by LVOTO, leading to a worsening of symptoms.

Correct. Arterial dilators cause a reduction in afterload therefore can lead to increased LVOT gradient and could exacerbate LVOT obstruction.

Correct. One of the primary effects of digoxin is its positive inotropic effect, meaning it increases the contractility of the heart. In HCM, the left ventricular wall is especially muscular and prone to self-obstruction during contraction. That is why an increase in contractility caused by digoxin can worsen the already present obstruction

Incorrect. Danon disease is part of a heterogeneous group of metabolic diseases called glycogen storage disorders (GSDs). To date, there are no approved aetiological therapies for Danon disease. Only symptomatic treatment such as the treatment of potential heart failure and arrhythmias is used.

Correct. Iron overload cardiomyopathy can be easily treated using chelation therapy or therapeutic phlebotomy. Iron chelators enter cells, bind free iron, and remove it from the body. Phlebotomy is an easily applicable, safe, and inexpensive procedure that prevents the development of iron-induced organ damage.

Correct. Pompe disease is part of a heterogeneous group of metabolic diseases called glycogen storage disorders (GSDs). The disease occurs in enzyme deficiency of acid alpha-glucosidase (GAA). Enzyme replacement therapy is recommended in patients diagnosed with Pompe disease, because in the absence of therapeutic intervention, Pompe disease has a poor prognosis, mainly due to end-stage heart failure.

Incorrect. PRKAG2 syndrome is part of a heterogeneous group of metabolic diseases called glycogen storage disorders (GSDs). To date, there are no approved aetiological therapies for PRKAG2 syndrome. Only symptomatic treatment like the treatment of heart failure and arrhythmias is used.

Correct. More than 50% of children with RCM are at risk of death (including SCD) or transplantation shortly after diagnosis; clinical features associated with increased risk of death or transplantation include: heart failure symptoms; reduced LV systolic function; increased left atrial size; syncope; ischaemia; and impaired LV diastolic function on echocardiography.

Correct. More than 50% of children with RCM are at risk of death (including SCD) or transplantation shortly after diagnosis; clinical features associated with increased risk of death or transplantation include: heart failure symptoms; reduced LV systolic function; increased left atrial size; syncope; ischaemia; and impaired LV diastolic function on echocardiography.

Correct. More than 50% of children with RCM are at risk of death (including SCD) or transplantation shortly after diagnosis; clinical features associated with increased risk of death or transplantation include: heart failure symptoms; reduced LV systolic function; increased left atrial size; syncope; ischaemia; and impaired LV diastolic function on echocardiography.

Correct. More than 50% of children with RCM are at risk of death (including SCD) or transplantation shortly after diagnosis; clinical features associated with increased risk of death or transplantation include: heart failure symptoms; reduced LV systolic function; increased left atrial size; syncope; ischaemia; and impaired LV diastolic function on echocardiography.

Incorrect. For sarcoidosis the correct treatment are Steroids and Steroid-sparing immunosuppressant drugs. Selective transthyretin stabilizer and polyneuropathy treatment is used in the management of ATTR cardiac amyloidosis

Incorrect. For Danon disease there is no available therapy. Enzyme replacement therapy is available for example for Anderson–Fabry disease or Pompe disease

Incorrect. In the management of ATTR cardiac amyloidosis selective transthyretin stabilizer and polyneuropathy treatment is used. Steroids and Steroid-sparing immunosuppressant drugs are the correct treatment for sarcoidosis.

Correct. Anderson–Fabry disease is an inborn error of metabolism where a deficient or absent enzyme, alpha-galactosidase A (α-Gal A), due to a pathogenic genetic variant in the GLA gene, causes the storage of some degradation cell products, mainly globotriaosylceramide (Gb3) in a patient’s lysosomes. So the treatment of this disease is based on enzyme replacement therapy, which replaces the deficient or absent enzyme.

Correct. Glucocorticoids, more precisely prednisone and deflazacort, are the main drug treatment for DMD.

Correct. Iron overload cardiomyopathy can be easily treated using chelation therapy or therapeutic phlebotomy. Iron chelators enter cells, bind free iron, and remove it from the body. Phlebotomy is an easily applicable, safe, and inexpensive procedure that prevents the development of iron-induced organ damage.

Incorrect. PRKAG2 syndrome is part of a heterogeneous group of metabolic diseases called glycogen storage disorders (GSDs). To date, there are no approved aetiological therapies for PRKAG2 syndrome. Only symptomatic treatment like the treatment of heart failure and arrhythmias is used.

Incorrect. Danon disease is part of a heterogeneous group of metabolic diseases called glycogen storage disorders (GSDs). To date, there are no approved aetiological therapies for Danon disease. Only symptomatic treatment such as the treatment of heart failure and arrhythmias is used.

Incorrect. It is inherited in an X-linked manner. Males are therefore always affected, while females’ organ involvement usually develops later in life but can become similar to males due to the lyonization phenomenon.

Incorrect. Anderson–Fabry disease is an inborn error of metabolism where a deficient or absent enzyme, alpha-galactosidase A (α-Gal A), due to a pathogenic genetic variant in the GLA gene, causes the storage of some degradation cell products, mainly globotriaosylceramide (Gb3) in a patient’s lysosomes.

Correct. Anderson–Fabry disease is an inborn error of metabolism where a deficient or absent enzyme, alpha-galactosidase A (α-Gal A), due to a pathogenic genetic variant in the GLA gene, causes the storage of some degradation cell products, mainly globotriaosylceramide (Gb3) in a patient’s lysosomes.

Correct. Anderson-Fabry disease causes globotriaosylceramide (Gb3) to accumulate in the vascular endothelium of the skin, kidneys, nervous system, and heart, thereby triggering inflammation and fibrosis. These processes generally result in organ dysfunction. Patients with classic Anderson-Fabry disease have various symptoms, e.g. acroparesthesias, hypohidrosis, angiokeratomas, corneal opacities, cerebrovascular lesions, cardiac disorders, andrenal dysfunction.

Correct. The diagnosis is established by assessment of α-GalA activity and lyso-Gb3 measurement in male patients; in females, genetic testing is usually required to confirm the diagnosis.

Incorrect. The diagnosis is established by assessment of α-GalA activity and lyso-Gb3 measurement in male patients; in females, genetic testing is usually required to confirm the diagnosis.

Incorrect. The diagnosis is established by assessment of α-GalA activity and lyso-Gb3 measurement in male patients; in females, genetic testing is usually required to confirm the diagnosis.

Incorrect. The diagnosis is established by assessment of α-GalA activity and lyso-Gb3 measurement in male patients; in females, genetic testing is usually required to confirm the diagnosis.

Correct. Extracardiac Anderson–Fabry disease red flags include: Gastrointestinal symptoms, Angiokeratoma, Cornea verticillata, Chronic kidney disease, Proteinuria, Sensorineural hypoacusia, Stroke/TIA, Neuropathic pain, X-linked inheritance pattern, Short PR interval, Low native T1 at cardiac, CMR.

Incorrect. Hepatomegaly is not commonly associated with Anderson–Fabry disease, however it can be associated with other cardiomyopathy causing diseases like the Pompe disease.

Correct. Extracardiac Anderson–Fabry disease red flags include: Gastrointestinal symptoms, Angiokeratoma, Cornea verticillata, Chronic kidney disease, Proteinuria, Sensorineural hypoacusia, Stroke/TIA, Neuropathic pain, X-linked inheritance pattern, Short PR interval, Low native T1 at cardiac, CMR.

Correct. Extracardiac Anderson–Fabry disease red flags include: Gastrointestinal symptoms, Angiokeratoma, Cornea verticillata, Chronic kidney disease, Proteinuria, Sensorineural hypoacusia, Stroke/TIA, Neuropathic pain, X-linked inheritance pattern, Short PR interval, Low native T1 at cardiac, CMR.

Incorrect. Extracardiac RASopathy red flags include: Abnormal facial features, Cryptorchidism, Pulmonary valve stenosis, Congenital heart disease, Extreme right-axis deviation at ECG, Lymphangectasis, Bleeding diathesis, Café au lait spots, Lentigines, Growth retardation, Sensorineural deafness.

Correct. Extracardiac RASopathy red flags include: Abnormal facial features, Cryptorchidism, Pulmonary valve stenosis, Congenital heart disease, Extreme right-axis deviation at ECG, Lymphangectasis, Bleeding diathesis, Café au lait spots, Lentigines, Growth retardation, Sensorineural deafness.

Correct.Extracardiac RASopathy red flags include: Abnormal facial features, Cryptorchidism, Pulmonary valve stenosis, Congenital heart disease, Extreme right-axis deviation at ECG, Lymphangectasis, Bleeding diathesis, Café au lait spots, Lentigines, Growth retardation, Sensorineural deafness.

Correct. Extracardiac RASopathy red flags include: Abnormal facial features, Cryptorchidism, Pulmonary valve stenosis, Congenital heart disease, Extreme right-axis deviation at ECG, Lymphangectasis, Bleeding diathesis, Café au lait spots, Lentigines, Growth retardation, Sensorineural deafness.

Incorrect. Compared with sarcomeric HCM, RAS-HCM shows earlier age at diagnosis.

Correct. Compared with sarcomeric HCM, RAS-HCM shows increased prevalence and severity of left or biventricular obstruction.

Incorrect. Compared with sarcomeric HCM, (RAS-HCM) shows higher rates of early hospitalizations for heart failure or need for interventional procedures or surgery.

Correct. Compared with sarcomeric HCM, RAS-HCM shows earlier age at diagnosis.

Incorrect. Bilateral hilar lymphadenopathy is not associated with Amyloidosis but Sarcoidosis.

Correct. Red flags associated with amyloidosis include: Bilateral carpal tunnel syndrome, Lumbar spinal stenosis, Autonomic dysfunction, Peripheral neuropathy, Relative apical sparing pattern, Ejection fraction/strain ratio >5, Pseudonecrosis Q waves, Low ECG voltages, Positive serum or urine monoclonal chain at immunofixation

Correct. Red flags associated with amyloidosis include: Bilateral carpal tunnel syndrome, Lumbar spinal stenosis, Autonomic dysfunction, Peripheral neuropathy, Relative apical sparing pattern, Ejection fraction/strain ratio >5, Pseudonecrosis Q waves, Low ECG voltages, Positive serum or urine monoclonal chain at immunofixation

Incorrect. Thinned basal interventricular septum is not associated with Amyloidosis but Sarcoidosis.

Correct. Verapamil or diltiazem, titrated to maximum tolerated dose, are recommended to improve symptoms in symptomatic patients with resting or provoked LVOT gradient ≥50 mmHg who are intolerant or have contraindications to beta-blockers (Class I)

Correct. Disopyramide, titrated to maximum tolerated dose, is recommended in addition to a beta-blocker (or, if this is not possible, with verapamil or diltiazem) to improve symptoms in patients with resting or provoked LVOT gradient ≥50 mmHg (Class I)

Incorrect. Captopril is commonly used in heart failure, which can develop in HCM patients. However, most experts do not endorse the use of ACE inhibitors for patients with HCM and an elevated outflow gradient at rest. The additional reduction in afterload may serve to augment the outflow gradient and worsen symptoms or may lead to syncope.

Correct. Non-vasodilating beta-blockers, titrated to maximum tolerated dose, are recommended as first-line therapy to improve symptoms in patients with resting or provoked LVOT gradient ≥50 mmHg (Class I)

Incorrect. Invasive diagnostic criteria apply to all forms of cardiac amyloidosis, whereas non-invasive criteria are accepted only for ATTR.

Correct. Invasive diagnostic criteria apply to all forms of cardiac amyloidosis, whereas non-invasive criteria are accepted only for ATTR.

Incorrect. Invasive diagnostic criteria apply to all forms of cardiac amyloidosis, whereas non-invasive criteria are accepted only for ATTR.

Incorrect. ECG evaluation is not included in cardiac amyloidosis non-invasive diagnostic criteria. They include typical echocardiographic/CMR findings combined with SPECT, myocardial radiotracer uptake scintigraphy and exclusion of a clonal dyscrasia by the following tests: serum free light chain assay, serum and urine protein electrophoresis with immunofixation.

Incorrect. Mild-to-moderate physical activity for up to 150 min per week is considered safe and is recommended.

Incorrect. Mild-to-moderate physical activity for up to 150 min per week is considered safe and is recommended.

Incorrect. Mild-to-moderate physical activity for up to 150 min per week is considered safe and is recommended.

Correct. Mild-to-moderate physical activity for up to 150 min per week is considered safe and is recommended.

Incorrect. High-intensity exercise, including competitive sport, is not recommended in symptomatic individuals, those with a left ventricular ejection fraction ≤40%, exercise-induced arrhythmias or pathogenic variants in LMNA or TMEM43. (Class III)

Correct. High-intensity exercise and competitive sport may be considered in a select group of asymptomatic and optimally treated individuals with a left ventricular ejection fraction ≥50% in the absence of exercise-induced complex arrhythmias. (Class IIb)

Incorrect. High-intensity exercise, including competitive sport, is not recommended in symptomatic individuals, those with a left ventricular ejection fraction ≤40%, exercise-induced arrhythmias or pathogenic variants in LMNA or TMEM43. (Class III)

Incorrect. High-intensity exercise, including competitive sport, is not recommended in symptomatic individuals, those with a left ventricular ejection fraction ≤40%, exercise-induced arrhythmias or pathogenic variants in LMNA or TMEM43. (Class III)

Correct. Pregnancy is contraindicated in women with mWHO class IV, including patients with EF <30% or NYHA class III–IV or previous PPCM (peripartum cardiomyopathy) with persisting impairment of the LV function.

Incorrect. Pregnancy is contraindicated in women with mWHO class IV, including patients with EF <30% or NYHA class III–IV or previous PPCM (peripartum cardiomyopathy) with persisting impairment of the LV function.

Correct. Pregnancy is contraindicated in women with mWHO class IV, including patients with EF <30% or NYHA class III–IV or previous PPCM (peripartum cardiomyopathy) with persisting impairment of the LV function.

Incorrect. Pregnancy is contraindicated in women with mWHO class IV, including patients with EF <30% or NYHA class III–IV or previous PPCM (peripartum cardiomyopathy) with persisting impairment of the LV function.

Correct. Progestin-only contraceptives are an alternative, as they have little or no effect on coagulation factors, blood pressure, and lipid levels.

Correct. Levonorgestrel-based long-acting reversible contraception implants or intrauterine devices are the safest and most effective contraceptives and have few side effects affecting cardiomyopathies.

Correct. Levonorgestrel-based long-acting reversible contraception implants or intrauterine devices are the safest and most effective contraceptives and have few side effects affecting cardiomyopathies.

Incorrect. Ethinylestradiol-containing contraceptives have the highest risk of thrombosis and are not recommended for women with a high risk of thromboembolic disease.

Incorrect. PLN risk score is used for evaluation of SCD risk in in patients positive for PLN gene.

Incorrect. NYHA Classification and LVEF evaluation are only a part of the mWHO classification used in this scenario.

Incorrect. The Milan criteria are a set of criteria applied in consideration of patients with cirrhosis and hepatocellular carcinoma (HCC) for liver transplant.

Correct. The risk associated with pregnancy in a patient with a cardiomyopathy is estimated using the modified World Health Organization (mWHO) classification. (Class I)

Correct. LVOT gradient of 55 mmHg means that a significant LVOT obstruction is present. Caesarean section should be considered for obstetric indications, patients with severe outflow tract obstruction, or in cases of severe acute/intractable heart failure, or in cases at high risk of threatening arrhythmia and for patients presenting in labour on oral anticoagulants.

Correct. NYHA class III in cardiomyopathy patients indicates that a significant heart failure is probably present. Caesarean section should be considered for obstetric indications, patients with severe outflow tract obstruction, or in cases of severe acute/intractable heart failure, or in cases at high risk of threatening arrhythmia and for patients presenting in labour on oral anticoagulants.

Correct. Caesarean section should be considered for obstetric indications, patients with severe outflow tract obstruction, or in cases of severe acute/intractable heart failure, or in cases at high risk of threatening arrhythmia and for patients presenting in labour on oral anticoagulants.

Incorrect. Caesarean section should be considered for obstetric indications, patients with severe outflow tract obstruction, or in cases of severe acute/intractable heart failure, or in cases at high risk of threatening arrhythmia and for patients presenting in labour on oral anticoagulants.

Incorrect. The post-partum period is associated with significant haemodynamic changes and fluid shifts, particularly in the first 24–48 h after delivery, which may precipitate heart failure. Haemodynamic monitoring should therefore be continued for at least 24–48 h in patients at risk.

Incorrect. The post-partum period is associated with significant haemodynamic changes and fluid shifts, particularly in the first 24–48 h after delivery, which may precipitate heart failure. Haemodynamic monitoring should therefore be continued for at least 24–48 h in patients at risk.

Correct. The post-partum period is associated with significant haemodynamic changes and fluid shifts, particularly in the first 24–48 h after delivery, which may precipitate heart failure. Haemodynamic monitoring should therefore be continued for at least 24–48 h in patients at risk.

Incorrect. The post-partum period is associated with significant haemodynamic changes and fluid shifts, particularly in the first 24–48 h after delivery, which may precipitate heart failure. Haemodynamic monitoring should therefore be continued for at least 24–48 h in patients at risk.

Incorrect. Atenolol has been linked with reduced growth of the fetus (smaller in size and/or low birth weight). Amiodarone, atenolol, dronedarone, ACE-Is, ARBs, renin inhibitors, and spironolactone are contraindicated in pregnancy

Correct. Sotalol together with oral verapamil are in the well tolerated group of antiarrhythmics in pregnancy.

Incorrect. The use of an ACE inhibitor during the second and third trimesters of pregnancy has been associated with a number of serious foetal malformations including oligohydramnion, foetal and neonatal renal failure, bone malformations, limb contractures, pulmonary hypoplasia, prolonged hypotension and neonatal death. ACE inhibitors are contraindicated after week 12 of pregnancy. Amiodarone, atenolol, dronedarone, ACE-Is, ARBs, renin inhibitors, and spironolactone are contraindicated in pregnancy

Incorrect. Amiodarone, its main metabolite (desethylamiodarone) and iodine are transferred, albeit incompletely, through the placenta, resulting in a relevant fetal exposure to the drug and iodine overload. Since the fetus acquires the capacity to escape from the acute Wolff-Chaikoff effect only late in gestation, the iodine overload may cause fetal/neonatal hypothyroidism and goiter.

Correct. Continuation of VKAs throughout pregnancy should be considered when the dose is low. When a higher dose of VKAs is required, discontinuation of VKAs between weeks 6 and 12 and replacement with i.v. UFH or LMWH should be considered.

Incorrect. VKA should be continued until pregnancy is achieved. There's no evidence to suggest that taking warfarin reduces fertility in either men or women.

Incorrect. Continuation of VKAs throughout pregnancy should be considered when the dose is low. When a higher dose of VKAs is required, discontinuation of VKAs between weeks 6 and 12 and replacement with i.v. UFH or LMWH should be considered.

Incorrect. Vitamin K antagonists are anticoagulant drugs that provide efficient thromboprophylaxis, however they are teratogenic and can cross the placenta. 5-12 % of infants exposed to warfarin between 6-9 weeks gestation present nasal hypoplasia and skeletal abnormalities, including short limbs and digits (brachydactyly), and stippled epiphyses. Therefore when a higher dose of VKAs is required, discontinuation of VKAs between weeks 6 and 12 and replacement with i.v. UFH or LMWH should be considered.

Correct. Non-neurocardiogenic syncope for which there is no explanation after investigation is associated with an increased risk of SCD.

Correct. The severity and extent of LVH measured by TTE are associated with the risk of SCD. Several studies have shown the greatest risk of SCD in patients with a maximum wall thickness of ≥30 mm.

Correct. The effect of age on SCD has been examined in a number of studies and two have shown a significant association, with an increased risk of SCD in younger patients.

Correct. NSVT (defined as ≥3 consecutive ventricular beats at ≥120 b.p.m. lasting <30 s) occurs in 20–30% of patients during ambulatory ECG monitoring and is an independent predictor of SCD.

Incorrect. Male sex has been reported to be consistently associated with an increased SCD rate in DCM.

Correct. Females with HCM are diagnosed later than males (8–13 years later) and are more severely affected.

Incorrect. Females with HCM more commonly develop advanced heart failure during follow-up, have more severe symptoms at baseline, and more often have LVOTO.

Incorrect. Females and males appear to show similar survival benefit from invasive SRT.

Correct. Individual genetic predisposition favours a dilated phenotype in the presence of trigger factors, such as inflammation, infection, toxic insults from alcohol or drugs, and tachyarrhythmias.

Correct. Individual genetic predisposition favours a dilated phenotype in the presence of trigger factors, such as inflammation, infection, toxic insults from alcohol or drugs, and tachyarrhythmias.

Correct. Individual genetic predisposition favours a dilated phenotype in the presence of trigger factors, such as inflammation, infection, toxic insults from alcohol or drugs, and tachyarrhythmias.

Correct. Individual genetic predisposition favours a dilated phenotype in the presence of trigger factors, such as inflammation, infection, toxic insults from alcohol or drugs, and tachyarrhythmias.

Correct. Routine (first-level) laboratory tests used in HCM include: CK, Liver function, NT-proBNP, Proteinuria, Renal function, Troponin

Correct. Routine (first-level) laboratory tests used in HCM include: CK, Liver function, NT-proBNP, Proteinuria, Renal function, Troponin

Correct. Routine (first-level) laboratory tests used in HCM include: CK, Liver function, NT-proBNP, Proteinuria, Renal function, Troponin

Incorrect. Alpha-galactosidase A levels are not a part of routine (first-level) laboratory tests used in HCM. Alpha-galactosidase A levels are used to identify the presence of Anderson–Fabry disease.

Correct. Cardiac amyloidosis, HCM (hypertrophic cardiomyopathy), and RCM (restrictive cardiomyopathy) are associated with a particularly increased risk of stroke.

Correct. The EORP registry indicated a worse prognosis for the population with cardiomyopathy and concurrent AF with an annual incidence of stroke/transient ischaemic attack (TIA) about three times higher in the cardiomyopathy group with AF.

Correct. Cardiac amyloidosis, HCM (hypertrophic cardiomyopathy), and RCM (restrictive cardiomyopathy) are associated with a particularly increased risk of stroke.

Correct. Cardiac amyloidosis, HCM (hypertrophic cardiomyopathy), and RCM (restrictive cardiomyopathy) are associated with a particularly increased risk of stroke.

Correct. Sotalol should not be used in patients with HFrEF, significant LVH, prolonged QT interval, asthma, hypokalemia, or creatinine clearance (CrCl) less than 30 ml/min.

Correct. Sotalol should not be used in patients with HFrEF, significant LVH, prolonged QT interval, asthma, hypokalemia, or creatinine clearance (CrCl) less than 30 ml/min.

Correct. Sotalol should not be used in patients with HFrEF, significant LVH, prolonged QT interval, asthma, hypokalemia, or creatinine clearance (CrCl) less than 30 ml/min.

Correct. Sotalol should not be used in patients with HFrEF, significant LVH, prolonged QT interval, asthma, hypokalemia, or creatinine clearance (CrCl) less than 30 ml/min.

Incorrect. Catheter ablation of AF is a safe and superior alternative to AAD therapy for maintenance of sinus rhythm, reducing AF-related symptoms, and improving QoL, and can be considered an alternative to AAD therapy in practically any type and context of AF.

Incorrect. Catheter ablation of AF is a safe and superior alternative to AAD therapy for maintenance of sinus rhythm, reducing AF-related symptoms, and improving QoL, and can be considered an alternative to AAD therapy in practically any type and context of AF.

Incorrect. Catheter ablation of AF is a safe and superior alternative to AAD therapy for maintenance of sinus rhythm, reducing AF-related symptoms, and improving QoL, and can be considered an alternative to AAD therapy in practically any type and context of AF.

Correct. Catheter ablation of AF is a safe and superior alternative to AAD therapy for maintenance of sinus rhythm, reducing AF-related symptoms, and improving QoL, and can be considered an alternative to AAD therapy in practically any type and context of AF.

Incorrect. Dilated cardiomyopathy (DCM) is a separate group of cardiomyopathies with systolic dysfunction unexplained solely by abnormal loading conditions (hypertension, valve disease, CHD) or CAD. However NDLVC phenotype does include individuals that up until now may have been described as having DCM but without LV dilatation.

Correct. The NDLVC phenotype includes individuals who may have previously been described as having DCM (but without left ventricular dilatation), arrhythmogenic left ventricular cardiomyopathy (ALVC), left-dominant ARVC, or arrhythmogenic DCM (but often without meeting the diagnostic criteria for ARVC).

Incorrect. Right ventricle dominance is part of the definition of basic ARVC, however the new term NDLVC includes variant with dominant lesion on the left ventricle.

Correct. The NDLVC phenotype includes individuals who may have previously been described as having DCM (but without left ventricular dilatation), arrhythmogenic left ventricular cardiomyopathy (ALVC), left-dominant ARVC, or arrhythmogenic DCM (but often without meeting the diagnostic criteria for ARVC).

Correct. Implantation of an ICD should be considered in patients with an estimated 5-year risk of sudden death of ≥6%. (Class IIa)

Correct. An ICD is recommended to reduce the risk of sudden death and all-cause mortality in patients with DCM who have survived a cardiac arrest or have recovered from a ventricular arrhythmia causing haemodynamic instability. (Class I)

Correct. Implantation of an ICD is recommended in HCM patients who have survived a cardiac arrest due to VT or VF, or who have spontaneous sustained VT with haemodynamic compromise. (Class I)

Correct. An ICD may be considered in selected patients with DCM with a genotype associated with high SCD risk and LVEF >35% without additional risk factors. (Class IIb)

Correct. A clear association between the activity of the thyroid gland and the presence of dilated cardiomyopathy is not well established in the literature. It is thought that the cardiovascular effect in hyperthyroidism specifically is related to the direct effect of thyroid hormones on the cardiac muscle. Other endocrinological non-genetic causes of dilated cardiomyopathy include: hypothyroidism, Cushing's/Addison's disease, pheochromocytoma, acromegaly, and diabetes mellitus.

Correct. Certain viruses, such as enterovirus, adenovirus, coxsackie etc. can lead to myocarditis, which may progress to dilated cardiomyopathy.

Correct. Pregnancy-related factors, such as pre-eclampsia or peripartum cardiomyopathy, can also contribute to the development of dilated cardiomyopathy.

Correct. Alcohol-induced toxicity leads to non-ischemic dilated cardiomyopathy characterized by loss of contractile function and dilation of myocardial ventricles. Other toxic non-genetic causes of dilated cardiomyopathy include: cocaine, amphetamines, ecstasy, cobalt, anabolic/androgenic steroids and iron overload.

Incorrect. No SCD risk-prediction scoring system has been developed for FLNC-truncating (filamin C-truncating) ariants. Therefore the guidelines suggest evaluation of LVEF and LGE (late gadolinium enhancement) on CMR (cardiac magnetic resonance) as predictors of SCD in this high-risk genotype.

Incorrect. No SCD risk-prediction scoring system has been developed for DSP (desmosome-plakin gene). Therefore the guidelines suggest evaluation of LVEF and LGE (late gadolinium enhancement) on CMR (cardiac magnetic resonance) as predictors of SCD in this high-risk genotype.

Correct. For certain high-risk NDLVC (non-dilating left ventricular) genotypes, such as the PLN p.Arg14del variant, gene-specific risk scores incorporating genotype characteristics and additional clinical features have been developed. These dedicated scores, when available, should be used to guide decisions regarding primary prevention ICD implantation.

Correct. For certain high-risk Non-Dilated Left Ventricular Cardiomyopathy (NDLVC) genotypes like Lamin A/C (LMNA) or Phospholamban (PLN) p.Arg14del variant, gene-specific risk-prediction scores have been developed that consider genotype characteristics and additional phenotypic features. Where such scores are available, they should be used to guide primary prevention Implantable Cardioverter-Defibrillator (ICD) implantation.

Incorrect. The PLN risk score applies only to a specific high-risk Non-Dilated Left Ventricular Cardiomyopathy (NDLVC) genotype, the PLN p.Arg14del variant. Therefore, it is not useful in a patient without this specific high-risk genotype.

Correct. In patients without a high-risk genotype and LVEF >35%, the presence and extent of myocardial scarring determined by LGE (late gadolinium enhancement) on CMR imaging can be helpful in risk stratification in patients with DCM. Late gadolinium enhancement is observed in 25–35% of patients with DCM and its presence is a strong risk marker for all-cause mortality and ventricular arrhythmias, both in retrospective and prospective studies.

Incorrect. There is a limited role for radionuclide imaging in DCM (dilated cardiomyopathy). Measurement of 18F-fluorodeoxyglucose (18F-FDG) uptake using PET can be useful in suspected cardiac sarcoidosis, but it won't help with determining SCD risk in this patient.

Incorrect. The data given is not sufficient for SCD risk evaluation and the absence of high-risk genotype doesn't outrule the presence of an increased risk. A further investigation is necessary to establish the real state of this patient's probability of SCD.

Incorrect. Causative gene variants occur in up to 40% of DCM patients in contemporary cohorts, and between 10 and 15% in chemotherapy-induced, alcoholic, or peripartum DCM. Although the prevalence of genetic variants is higher in familial DCM, causative genetic variants are also identified in over 20% of non-familial DCM cases.

Correct. Causative gene variants occur in up to 40% of DCM patients in contemporary cohorts, and between 10 and 15% in chemotherapy-induced, alcoholic, or peripartum DCM. Although the prevalence of genetic variants is higher in familial DCM, causative genetic variants are also identified in over 20% of non-familial DCM cases.

Correct. Causative gene variants occur in up to 40% of DCM patients in contemporary cohorts, and between 10 and 15% in chemotherapy-induced, alcoholic, or peripartum DCM. Although the prevalence of genetic variants is higher in familial DCM, causative genetic variants are also identified in over 20% of non-familial DCM cases.

Incorrect. Causative gene variants occur in up to 40% of DCM patients in contemporary cohorts, and between 10 and 15% in chemotherapy-induced, alcoholic, or peripartum DCM. Although the prevalence of genetic variants is higher in familial DCM, causative genetic variants are also identified in over 20% of non-familial DCM cases.