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The caseous calcification of the mitral annulus (CCMA) is a rare variant of the mitral annulus calcification (MAC), with prevalence of 0.067% in general population. It is usually an asymptomatic condition but rarely it can cause severe mitral valve dysfunction, embolization, and conduction abnormalities. Transthoracic echocardiography (TTE) is a useful diagnostic tool. However, it can be necessary a multi-modality imaging approach for a differential diagnosis among other intra-cardiac masses. We are presenting a case of CCMA in an 88-year-old woman admitted to our department for acute decompensation of chronic heart failure. TTE has shown pathognomonic findings of CCMA. Tridimensional trans-esophageal echocardiography (3D TEE) gave a better characterization of the CCMA, avoiding further diagnostic procedures.

Aims Systemic amyloidosis is a multisystem disorder caused by the misfolding, aggregation, and deposition of certain proteins in various organs and tissues. Cardiac involvement is common and worsens the prognosis. Atrial fibrillation is the most frequent arrhythmia in cardiac amiloidosis (CA), but many cases of ventricular arrhythmias (VA) and sudden cardiac death (SCD) have been described. Methods and results We present a case of a 64-year-old man admitted following exertional syncope occurred after climbing a flight of stairs. In the previous 2 weeks he had experienced fatigue and poor tolerance to exertion; moreover, 2 days before he had another episode of transient loss of consciousness after exertion. His medical history included left gonarthrosis and surgery for right carpal tunnel syndrome one year before. Physical examination did not revealed signs of heart failure. A 12-lead electrocardiogram (ECG) showed a pseudoinfarction pattern with QS waves in V2 and V3 leads and low voltage in all limb leads (Figure 1). Routine blood tests revealed N-terminal pro-bran natriuretic peptide (NT-ProBNP) of 3436 pg/ml (n.v. 0–125) and high-sensitive troponin T of 67 pg/ml (n.v. < 58). A trans-thoracic echocardiogram showed left ventricle (LV) concentric thickening wall with granular and sparkling pattern, mild reduced ejection fraction, reduced global longitudinal strain with apical sparing, grade 3 diastolic disfunction, biatrial enlargement, mild mitral regurgitation, right ventricle free wall thickening (8 mm), mild reduced TAPSE, and mild pericardial effusion. Cardiac magnetic resonance (CMR) confirmed ventricular wall thickening with evidence at T1 mapping of interstitial infiltration more evident in the septum and inferior-lateral wall with apical savings (Figure 2). A total body 99mTc-HDP scintigraphy showed cardiac uptake with intensity similar to bone signal (Perugini Score 2) suggesting ATTR cardiac amyloidosis (Figure 3). Both kappa and lambda concentrations were normal. The genetic testing did not reveled mutations in the TTR gene. We concluded for a diagnosis of ATTR wild-type CA. The Holter ECG monitoring registered numerous ventricular ectopic beats and an episode of non-sustained ventricular tachycardia. A coronary angiography ruled out coronary artery disease. In consideration of the clinical-instrumental picture an ICD was implanted. Conclusions In conclusion, CA is still underdiagnosed. VA and SCD may further complicate the prognosis. Early diagnosis and adequate stratification of the arrhythmic risk are essential to ensure patients the best therapeutic strategies.

Myocarditis is an inflammatory disease of cardiac muscle with a variable clinical presentation, ranging from asymptomatic cases to different degrees of left ventricular systolic dysfunction up to heart failure and dilated heart disease. Ventricular arrhythmias (VA) can occur in patients with myocarditis and implantable cardioverter defibrillator (ICD) may be indicated in patients with life-threatening VA who are not in the acute phase of myocarditis and who are receiving optimal medical therapy. Reduced left ventricular ejection fraction (LVEF) below 35%, which is used as the main criterion for stratifying the risk of sudden cardiac death (SCD), has low sensitivity and low specificity for arrhythmic risk stratification in patients with myocarditis. Myocardial scar is the main determinant for VA in these patients. Cardiac magnetic resonance imaging (CMR), using late gadolinium enhancement(LGE), has an excellent ability to determinate the extension and characterization of myocardial scar, indeed CMR can potentially improve SCD risk stratification and indication for ICD implantation in patients with myocarditis. We present a case of a 36 years-old male presenting to the Emergency Department with a monomorphic sustained ventricular tachycardia in whom MRI revealed myocardial-pericardial recurrent inflammatory involvement and worsening disease progression. ICD was implanted in consideration of the high risk of life-threatening arrhythmias.