The Role of T2 Mapping in Cardiac Amyloidosis.

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy divided into two types: light-chain (LA) and transthyretin (ATTR) CA. Cardiac magnetic resonance (CMR) has emerged as an important diagnostic tool in CA. While late gadolinium enhancement (LGE), T1 mapping and extracellular volume (ECV) have a consolidate role in the assessment of CA, T2 mapping has been less often evaluated. We aimed to test the value of T2 mapping in the evaluation of CA. This study recruited 70 patients with CA (51 ATTR, 19 AL). All the subjects underwent 1.5 T CMR with T1 and T2 mapping and cine and LGE imaging. Their QALE scores were evaluated. The myocardial T2 values were significantly (p < 0.001) increased in both types of CA compared to the controls. In the AL-CA group, increased T2 values were associated with a higher QALE score. The myocardial native T1 values and ECV were significantly (p < 0.001) higher in the CA patients than in the healthy subjects. Left ventricular (LV) mass, QALE score and ECV were higher in ATTR amyloidosis compared with AL amyloidosis, while the LV ejection fraction was lower (p < 0.001). These results support the concept of the presence of myocardial edema in CA. Therefore, a CMR evaluation including not only myocardial T1 imaging but also myocardial T2 imaging allows for more comprehensive tissue characterization in CA.

Cardiac Magnetic Resonance Feature-Tracking Identifies Preclinical Abnormalities in Hypertrophic Cardiomyopathy Sarcomere Gene Mutation Carriers.

BACKGROUND: Assessing myocardial strain by cardiac magnetic resonance feature tracking (FT) has been found to be useful in patients with overt hypertrophic cardiomyopathy (HCM). Little is known, however, of its role in sarcomere gene mutation carriers without overt left ventricular hypertrophy (subclinical HCM). METHODS: Thirty-eight subclinical HCM subjects and 42 healthy volunteers were enrolled in this multicenter case-control study. They underwent a comprehensive cardiac magnetic resonance study. Two-dimensional global radial, circumferential, and longitudinal strain of the left ventricle (LV) were evaluated by FT analysis. RESULTS: The subclinical HCM sample was 41 (22-51) years old and 32% were men. FT analysis revealed a reduction in global radial strain (29±7.2 versus 47.9±7.4; P<0.0001), global circumferential strain (-17.3±2.6 -versus -20.8±7.4; P<0.0001) and global longitudinal strain (-16.9±2.4 versus -20.5±2.6; P<0.0001) in subclinical HCM compared with control subjects. The significant differences persisted when considering the 23 individuals free of all the structural and functional ECG and cardiac magnetic resonance abnormalities previously described. Receiver operating characteristic curve analyses showed that the differential diagnostic performances of FT in discriminating subclinical HCM from normal subjects were good to excellent (global radial strain with optimal cut-off value of 40.43%: AUC, 0.946 [95% CI, 0.93-1.00]; sensitivity 90.48%, specificity 94.44%; global circumferential strain with cut-off, -18.54%: AUC, 0.849 [95% CI, 0.76-0.94]; sensitivity, 88.10%; specificity, 72.22%; global longitudinal strain with cut-off, -19.06%: AUC, 0.843 [95% CI, 0.76-0.93]; sensitivity, 78.57%; specificity, 78.95%). Similar values were found for discriminating those subclinical HCM subjects without other phenotypic abnormalities from healthy volunteers (global radial strain with optimal cut-off 40.43%: AUC, 0.966 [95% CI, 0.92-1.00]; sensitivity, 90.48%; specificity, 95.45%; global circumferential strain with cut-off, -18.44%: AUC, 0.866 [95% CI, 0.76-0.96]; sensitivity, 92.86%; specificity, 77.27%; global longitudinal strain with cut-off, -17.32%: AUC, 0.838 [95% CI, 0.73-0.94]; sensitivity, 90.48%; specificity, 65.22%). CONCLUSIONS: Cardiac magnetic resonance FT-derived parameters are consistently lower in subclinical patients with HCM, and they could emerge as a good tool for discovering the disease during a preclinical phase.

The Value of a Coronary Computed Tomography Angiography plus Stress Cardiac Magnetic Resonance Imaging Strategy for the Evaluation of Patients with Chronic Coronary Syndrome.

Background: Noninvasive imaging methods, either anatomical or functional tests, serve as essential instruments for the appropriate management of patients with established or suspected coronary artery disease (CAD). We sought to evaluate the safety and efficacy of a coronary computed tomography angiography (CCTA) plus stress cardiac magnetic resonance imaging (S-CMR) strategy in patients with chronic coronary syndrome (CCS). Methods: Patients with suspected CCS showing intermediate coronary plaques (stenosis 30-70%) at CCTA underwent S-CMR. Patients with a positive S-CMR were referred to invasive coronary angiography (ICA) plus instantaneous wave-free ratio (iFR), and myocardial revascularization if recommended. All patients received guideline-directed medical therapy (GDMT), including high-dose statins, regardless of myocardial revascularization. The primary endpoint was a composite of death from cardiovascular causes, non-fatal myocardial infarction, and unplanned revascularization. Results: According to the results of CCTA, 62 patients showing intermediate coronary plaques underwent S-CMR, which was positive for a myocardial perfusion deficit in n = 17 (27%) and negative in n = 45 (73%) patients. According to the results of ICA plus iFR, revascularization was performed in 13 patients. No differences in the primary endpoint between the positive and negative S-CMR groups were observed at 1 year (1 [5.9%] vs. 1 [2.2%], p = 0.485) and after a median of 33.4 months (2 [11.8%] vs. 3 [6.7%]; p = 0.605). Conclusions: Our study suggests that a CCTA plus S-CMR strategy is effective for the evaluation of patients with suspicion of CCS at low-intermediate risk, and it may help to refine the selection of patients with intermediate coronary plaques at CCTA needing coronary revascularization.

When it rains, it pours: Early treatment with tecovirimat of cardiac complications associated with monkeypox infection in a person with HIV and previously undiagnosed Lyme disease. A case report.

Cardiac involvement, such as myocarditis and pericarditis, can be a severe complication of monkeypox virus (mpox) infection and could be related to other co-infections with cardiac involvement. Tecovirimat is an antiviral specifically designed to inhibit smallpox infection diffusion and approved by the FDA for other Orthopoxvirus infections; its efficacy in mpox-infected patients is not well established. We present the case of a cardiac complication during mpox infection in a previously undiagnosed Lyme disease in a 42-year-old man living with HIV. Two days after the typical maculopapular rash, the patient reported a rise in body temperature up to 39 °C, chest pain without irradiation, and shortness of breath. We found an increase in troponin level, a slight reduction in ejection fraction, and grade 2 AV block (Mobitz 1 and 2) with frequent sinus pauses (the longest of 10.1 s). Given the suspicion of myopericarditis with cardiac conduction system involvement, the patient was admitted to the Intermediate Care Unit for continuous monitoring and further evaluation. Treatment included Ibuprofen 600 mg every 12 hours (bid) and colchicine 1 mg once daily for anti-inflammatory purposes. Concomitantly, treatment with tecovirimat was started at 600 mg bid for a total of 14 days. Cardiac MRI with gadolinium showed mild interstitial edema and pericardial enhancement. However, despite the clinical and laboratory resolution of the acute phase, bradycardia with episodes of AV block persisted at follow-up, suggesting the possibility of an additional etiology. Thus, the patient was investigated for Lyme disease because high-degree AV block is the most common presentation of Lyme carditis. Serological results evidenced a previous Borrelia burgdorferi senso latu. We decided to start treatment with doxycycline 100 mg every 12h, even pending the uncertainty of the role of a previous Lyme disease in determining the cardiac rhythm disturbances. At the evaluation on day 44, the patient was systemically well, and after cardiologist consultation, pace-maker implantation was not deemed indicated. This case underscores the importance of considering alternative causes of carditis when the clinical picture remains unclear or persists after the acute phase.

Sunitinib in the therapy of malignant paragangliomas: report on the efficacy in a SDHB mutation carrier and review of the literature

SUMMARY Metastatic pheochromocytomas (PHEOs) and paragangliomas (sPGLs) are rare neural crest-derived tumors with a poor prognosis. About 50% of them are due to germ-line mutations of the SDHB gene. At present, there is no cure for these tumors. Their therapy is palliative and represented by different options among which antiangiogenic drugs, like sunitinib, have been hypothesized to be effective especially in malignant SDHB mutated tumors. We report the effects of sunitinib therapy in a SDHB mutation carrier affected by a malignant sPGL. During 101 weeks of therapy at different doses, sunitinib was able to cause a partial response and then a stable disease for a total of 78 weeks. This favorable response is the longest, out of the 35 so far reported in the literature, registered in a patient treated exclusively with sunitinib but, similarly to the other responses, the effect was limited in time. From our analysis of the scanty data present in the literature, the effect of sunitinib does not seem to be different among wild-type patients and those carrying a cluster 1 germ-line mutation. Sunitinib seems able to slow the disease progression in some patients with malignant PHEO/PGL and therefore may represent a therapeutic option, although randomized controlled studies are needed to assess its efficacy definitively in the treatment of these aggressive tumors.

Análisis preliminar del cuestionario Potenziani para la validación diagnóstica del hipogonadismo de comienzo tardío y su concordancia con los cuestionarios de Heinemann (AMS) y de Morley (ADAM) / Preliminary analysis of the Potenziani questionnaire for the late onset hypogonadism diagnostic validation and its concordance with the Heinemann (AMS) and Morley (ADAM) questionnaires

Hipogonadismo de comienzo tardío, es una condición que afecta 6% al 12% de hombres entre 40 y 70 años y aun así, está subdiagnosticada, por lo que se propone un cuestionario de validación diagnóstica, con el objetivo de lograr mayor sensibilidad, especifidad y predictividad que los cuestionarios ya existentes. Se analizaron 107 hombres entre 45 y 70 años, con disminución del entusiasmo en actividad diaria, cansancio fácil, menor productividad en su trabajo, cambios del humor con propensión a la irritabilidad, disminución de su masa magra muscular, con tendencia al sobrepeso y afectación en actividades recreativas y deportivas. Se hizo interrogatorio exhaustivo, examen físico y pruebas de laboratorio (perfil 20, perfil hormonal urológico masculino, antígeno prostático específico total, libre y relación libre/total, examen de orina y urocultivo). Se solicitó contestar al paciente tres cuestionarios de validación diagnóstica del hipogonadismo de comienzo tardío: Heinemann AMS (Ageing Males Survey-1999, St. Louis University, Androgen Deficiency in Aging Male), Morley ADAM-2000 y el cuestionario de validadción diagnóstica del hipogonadismo de comienzo tardío-Potenziani-2007, para ser comparados y demostrar su validez con pruebas de especificidad y sensibilidad, índice de Youden, pruebas de concordancia con intervalos de confianza del 95%, en relación al diagnóstico bioquímico del hipogonadismo de comienzo tardío. Los resultados arrojaron que el cuestionario "Potenziani" fue más sensible (88,57%), fue más específico (41,67%), tuvo el índice de validez más alto (57,01%) y el valor predictivo positivo más alto de los tres cuestionarios con el 42,5%. Por tal motivo se ha demostrado que el cuestionario propuesto es más adecuado que Heineman-AMS y el Morley-ADAM en la aproximación diagnóstica del síndrome de hipogonadismo de comienzo tardio

Late onset hypogonadism a condition which affect 6%-12% of men between 40-70 years old, and still it is subdiagnosed for which we did a validation questionnaire with the objetive to be more sensitive, especific and predictive that old questionnaires. We analized 107 men with ages between 45-70 years old whom consulted for libido deterioration, erectile dysfunction, less enthusiasm of daily life, less work-productivity, easy tiredness, humor changes with irritability, less muscle mass, overweight, and deterioration of sexual life in general. We performed exhaustive interrogatory, physical examination, and laboratoty test (20 profile, hormonal-urologic profile, prostatic specific antigen, urine and urocultive). We ask them to complete three questionnaires of late onset hypogonadism diagnostic validation: Heinemann AMS, Morley ADAM, Potenziani 2007, to be compared and show its validity with specificity and sensibility tests, Youden Index, test of concordance with confidence interval of 95%, in relation to biochemical diagnosis of deficiency testosterone syndrome. The results were that the Potenziani`s cuestionary was more sensible (88.57%), more specific (41.67%), with the validation index more high (57.01%) and with the positive predictive value more high too (42.5%). For that reason we show that Potenziani`s validation questionnaire of late onset hypogonadism, is more adecuate in the diagnostic aproximation of this condition

Varicocele en población juvenil parte I: revisión de un tema controversial / Varicolcele in juvenile population part I: theme controversy of a review

El presente artículo de revisión tiene como objetivo mencionar aspectos importantes del tema del varicocele juvenil, pasando por su definición, clasificación, historia, temas controversiales, fisiopatología, factores pronósticos, diagnóstico, biopsia testicular, indicaciones de cuando estudiar a éstos pacientes juveniles, terapias actuales con un extensa bibliografía