Publications by authors named "G Tini"

For years, the treatment of many cardiomyopathies has been solely focused on symptom management. However, cardiomyopathies have a genetic substrate, and directing therapy towards the pathophysiology rather than the epiphenomenon of the disease may be a winning strategy. Gene therapy involves the insertion of genes or the modification of existing ones and their regulatory elements through strategies like gene replacement and gene editing.

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Article Synopsis
  • The study analyzed changes in electrocardiographic (ECG) characteristics in patients with different types of cardiac amyloidosis (AL, ATTRv, and ATTRwt) and looked at their predictive value for patient outcomes.
  • In a large sample of 356 patients, it was found that those with ATTRwt had more conduction issues, while AL patients exhibited more signs of low QRS voltage and T wave inversion.
  • Overall, the ECG abnormalities progressed similarly in all subtypes, but a longer QRS duration at baseline was associated with worse survival, indicating it could be a potential marker for advanced disease.
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The knowledge of pericardial diseases has now improved, including prospective and retrospective cohort studies focusing on the pathogenesis, diagnosis, treatment, and outcomes. The complex interplay between genetic predisposition (especially for autoinflammatory conditions), inflammation, and autoimmunity is now known to trigger recurrences of pericarditis. Moreover, diagnostic capabilities have improved with the implementation of multimodality imaging, particularly cardiac magnetic resonance (CMR), to detect and monitor pericardial inflammation, to allow diagnosis in more complicated cases, and tailor the duration of therapy based on objective parameters.

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Aims: Amyloid cardiomyopathy is caused by the deposition of light chain (AL) or transthyretin amyloid (ATTR) fibrils, that leads to a restrictive cardiomyopathy, often resulting in heart failure (HF) with preserved or reduced ejection fraction. This study aimed to determine whether cardiac output reduction or ventilation inefficiency plays a predominant role in limiting exercise in patients with amyloid cardiomyopathy.

Methods: We conducted a multicentre prospective study in patients with AL or ATTR cardiomyopathy who underwent cardiopulmonary exercise testing across four centres.

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