Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series.

Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co-morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.

Left Atrial Anomalous Muscular Band: Case Report, Literature Review, and Role of Three-Dimensional Echocardiography.

Anomalous fibromuscular bands in the left atrium were already described in the 19th century. Recently, the greater attention to the anatomy of the left atrium and the technological improvement have made their finding more frequent. Here, we present six cases, out of approximately 30,000 unselected echocardiograms, in which the use of the three-dimensional echo allowed a better definition of their anatomy, course, and motility.

[The importance of intensive lipid-lowering therapy after acute coronary syndrome: changing the paradigm to improve the achievement of targets]. / L'importanza di una terapia ipolipemizzante intensiva dopo sindrome coronarica acuta: cambiare il paradigma per migliorare il raggiungimento dei target.

BACKGROUND: Despite the availability of effective lipid-lowering drugs, only few high-risk patients attain their LDL cholesterol (LDL-C) guideline-recommended risk-based goal because of underprescription of combination therapy. We present an 18-month experience with variation of prescription protocols after publication of the 2019 ESC/EAS guidelines for the management of dyslipidemias. METHODS: Overall, 621 consecutive patients hospitalized for acute coronary syndrome at Mauriziano Hospital in Turin, Italy, between January 2020 and June 2021 were enrolled. Lipid-lowering therapy recommended at discharge was registered to evaluate how many patients received statin monotherapy, statin plus ezetimibe combination or triple therapy with high-intensity statin plus ezetimibe and proprotein convertase subtilisin/kexin type 9 inhibitor (PCSK9i). At 6-month follow-up, the reduction in LDL-C, adverse events, compliance and cardiovascular recurrences was analyzed. RESULTS: Of 621 patients enrolled, 7 died during hospitalization. During the entire study period, 33% of patients received statin monotherapy, 50% were discharged on statin-ezetimibe combination, and PCSK9i (evolocumab) was prescribed to 17% of patients. Between April 2020 and June 2021, when new recommendations were introduced into clinical practice, 20% of patients received evolocumab, 56% combination therapy and only 24% were discharged on statin monotherapy. At the beginning of observation, evolocumab was prescribed to 3% of patients hospitalized for acute coronary syndrome, while at the end of the study period 27% of patients were discharged on PCSK9i, with an increase of the prescription rate by 759%; in the same period, prescription of statin monotherapy decreased by 75%. At 6-month follow-up, LDL-C reduction was 77% in patients treated with PCSK9i vs 48% in patients taking statin-ezetimibe combination therapy (p<0.001). All patients on evolocumab reached the guideline-directed goals and a low rate of adverse events was reported, mainly represented by local injection site reactions. Six patients experienced acute coronary syndrome recurrence; only one of them was treated with evolocumab. CONCLUSION: Prescription of intensive lipid-lowering therapy after acute coronary syndrome, eventually with introduction of PCSK9i during hospitalization or at discharge, leads to attainment of guideline-recommended goals for all patients, with a low incidence of adverse events and optimal compliance.

Absence of left atrial stunning after cardioversion of recent-onset atrial fibrillation in patients at low-stroke risk.

OBJECTIVE: The aim of the present study was to evaluate the presence and degree of spontaneous echo contrast (SEC) in the left atrium and of left atrial appendage (LAA) contractility before and after cardioversion (CV) in patients with recent-onset atrial fibrillation (AF). METHODS: Our study included 56 patients divided into two groups: group 1, comprising 32 clinically stable patients who were admitted to the Emergency Department with less than or equal to 48 h duration AF, and who underwent transoesophageal echocardiography (TEE)-guided CV; and the control group (group 2), comprising 24 patients admitted to the Cardiological Department for elective TEE-guided CV of greater than 48 h AF. All patients underwent repeat TEE within 1 h after successful CV. RESULTS: Patients with recent-onset AF (group 1) showed no thrombogenic milieu at baseline without any evidence of atrial stunning after successful CV. SEC mean grade (0-3 grading) was 0.09±0.3 versus 0.12±0.4 after CV (P=0.98), and LAA flow velocity was 60.7±19.4 versus 56.7±20.5 cm/s after CV (P=0.07). Group 2 patients showed a significantly higher degree of SEC compared with those in group 1 (0.09±0.3 vs. 0.66±0.7, P=0.0093) and significantly lower LAA flow velocities (60.7±19.4 vs. 32.5±12.4, P<0.0001), with significant worsening after successful CV (SEC degree: 0.66±0.9 vs. 1.37±0.9, P=0.0093; LAA flow velocity: 32.5±12.4 vs. 20.4±12.7 cm/s, P<0.0001). CONCLUSION: The absence of thrombogenic milieu and of left atrial stunning after CV in patients with recent-onset AF favours early CV without anticoagulation, at least in patients with a low thromboembolic risk profile. These patients could be discharged earlier from urgent care.

Acute Myocardial Infarction in a Patient with Two-Vessel Occlusion and a Large Lambl's Excrescence.

A 59-year-old man underwent an echocardiography study after myocardial infarction and it showed a thin, mobile mass attached to the aortic valve. A diagnosis of Lambl's excrescence (LE) was suspected. Coronary occlusion as a consequence of embolism of LE's material could not be excluded and the patient underwent surgical excision. Histology confirmed the diagnosis; however a differential diagnosis with papillary fibroelastoma could not be established because both of these structures are histologically indistinguishable. A brief survey of the literature is presented. Evidence-based recommendations for treatment have not been established yet.

Late gadolinium enhancement on cardiac magnetic resonance and phenotypic expression in hypertrophic cardiomyopathy.

BACKGROUND: Cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) can identify areas of myocardial fibrosis in vivo in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to examine the association between clinical-morphological variables, risk factor for sudden death, and LGE findings in a consecutive, unselected population of HCM patients. METHODS: From January 2005 to August 2009, 124 HCM patients (53 ± 17 years, 86 men) were prospectively evaluated with CMR examination, assessing left ventricular (LV) hypertrophy, function, and LGE. RESULTS: In univariate analysis, patients were divided into tertiles according to the number of segments positive for LGE (first tertile, 0.3 ± 0.4; second tertile, 2.2 ± 0.4; third tertile, 5.2 ± 1.9 segments). Male gender (P = .05), maximum LV wall thickness (P = .002), nonsustained ventricular tachycardia (P = .001), ejection fraction <50% (P = .02), LV mass (P = .02), left atrium dilation (P = .04), perfusion defects (P ≤ .001), and telesystolic volume (P = .04) were all positively related with the number of segments of LGE. In multivariable analysis, male gender (P = .007), maximum LV wall thickness (P = .006), LV mass (P = .031), and perfusion alterations (P = .017) were independent predictors of LGE extent. CONCLUSIONS: Our study shows an independent association, even at multivariate analysis, between the entity of LGE and maximum LV wall thickness, mass, and perfusion defects in patients with HCM. Whether the presence and the extent of LGE translates into clinical events later on awaits further long-term follow-up studies.

Cardiac tamponade and successful pericardiocentesis in an extremely low birth weight neonate with percutaneously inserted central venous line: a case report.

BACKGROUND: Pericardial effusion and cardiac tamponade are rare but life-threatening complications of percutaneosuly inserted central line (PICL) use in extremely low birth weight (ELBW) neonates, with an incidence reported between 0.07% and 2% of PICLs placement. Timely diagnosis and pericardiocentesis has been proven to be life-saving. CASE PRESENTATION: The patient was a 620 g birth weight neonate who presented with sudden cardiac instability 18 days after the insertion of a PICL and in spite of a presumed satisfactory position of the catheter tip. The transthoracic echocardiography demonstrated severe pericardial effusion with evidence of cardiac tamponade. Successful urgent subxiphoid pericardiocentesis was performed; totally 2 ml of whitish fluid was collected, which resulted consistent to the composition of the hyperosmolar TPN solution infused. CONCLUSION: Cardiac tamponade should be considered in any newborn with a peripherally inserted central catheter who presents with cardiorespiratory instability (bradycardia, cyanosis and metabolic acidosis), even when lines are believed to be placed correctly.

Electromechanical coupling in patients with the short QT syndrome: further insights into the mechanoelectrical hypothesis of the U wave.

BACKGROUND: Patients with a short QT syndrome (SQTS) are at risk of sudden cardiac death (SCD). It is not known whether abbreviation of cardiac repolarization alters mechanical function in SQTS. Controversies persist regarding whether the U wave is a purely electrical or mechanoelectrical phenomenon. OBJECTIVE: The present study uses echocardiographic measurements to discriminate between the hypotheses for the origin of the U wave. METHODS: Diagnostic work-up including echocardiography and electrocardiogram was performed in 5 SQTS patients (39 +/- 19 years old) from 2 unrelated families with a history of SCD and 5 age-matched and gender-matched control subjects. RESULTS: QT intervals were 268 +/- 18 ms (QTc 285 +/- 28 ms) in SQTS versus 386 +/- 20 ms (QTc 420 +/- 22 ms) in control subjects (P < .005). In SQTS patients, the end of the T wave preceded aortic valve closure by 111 +/- 30 ms versus -12 +/- 11 ms in control subjects (P < .005). The interval from aortic valve closure to the beginning of the U wave was 8 +/- 4 ms in patients and 15 +/- 11 ms in control subjects (P = .25). Thus, the inscription of the U wave in SQTS patients coincided with aortic valve closure and isovolumic relaxation, supporting the hypothesis that the U wave is related to mechanical stretch. CONCLUSION: Our data show for the first time a significant dissociation between the ventricular repolarization and the end of mechanical systole in SQTS patients. Coincidence of the U wave with termination of mechanical systole provides support for the mechanoelectrical hypothesis for the origin of the U wave.

Clinical value of left atrial appendage flow for prediction of long-term sinus rhythm maintenance in patients with nonvalvular atrial fibrillation.

OBJECTIVES: This study evaluated the role of various clinical and echocardiographic parameters, including the left atrial appendage (LAA) anterograde flow velocity, for prediction of the long-term preservation of sinus rhythm (SR) in patients with successful cardioversion (CV) of nonvalvular atrial fibrillation (AF). BACKGROUND: Echocardiographic parameters for assessing long-term SR maintenance after successful CV of nonvalvular AF are not accurately defined. METHODS: Clinical, transthoracic echocardiographic and transesophageal echocardiographic (TEE) data--measured in AF lasting >48 h--of 186 consecutive patients (116 men, mean age: 65 +/- 9 years) with successful CV (electrical or pharmacologic) were analyzed for assessment of one-year maintenance of SR. RESULTS: At one-year follow-up, 91 of 186 (49%) patients who underwent successful CV continued to have SR. Mean LAA peak emptying flow velocity was higher in patients remaining in SR for one year than in those with AF relapse (41.7 +/- 20.2 cm/s vs. 27.7 +/- 17.0 cm/s; p < 0.001). On multivariate logistic regression analysis, only the mean LAA peak emptying velocity >40 cm/s (p = 0.0001; chi(2): 23.9, odds ratio [OR] = 5.2, confidence interval [CI] 95% = 2.7 to 10.1) and the use of preventive antiarrhythmic drug treatment (p = 0.0398; chi(2): 4.2; OR = 2.0, CI 95% = 1.0 to 3.8) predicted the continuous preservation of SR during one year, outperforming other univariate predictors such as absence of left atrial spontaneous echocardiographic contrast during TEE, the left atrial parasternal diameter <44 mm, left ventricular ejection fraction >46% and AF duration <1 week before CV. The negative and positive predictive values of the mean LAA peak emptying velocity >40 cm/s for assessing preservation of SR were 66% (CI 95% = 56.9 to 74.2) and 73% (CI 95% = 62.4 to 83.3), respectively. CONCLUSIONS: In TEE-guided management of nonvalvular AF, high LAA flow velocity identifies patients with greater likelihood to remain in SR for one year after successful CV. Low LAA velocity is of limited value in identifying patients who will relapse into AF.