Case report: desmoplakin cardiomyopathy presenting as an inflammatory cardiomyopathy with repeated sudden cardiac arrests.

Background: Desmoplakin cardiomyopathy has been recently classified as a non-dilated left ventricular cardiomyopathy, which is characterized by inflammatory-like episodes followed by left ventricular fibrosis/dysfunction and ventricular arrhythmias. Specific management is unclear. Case summary: We report a detailed case of a 46-year-old Caucasian woman presenting with repeated sudden cardiac arrests who was diagnosed with a new variant in the desmoplakin gene. Because the initial 18F-fluorodeoxyglucose positron emission tomography scan showed significant hypermetabolism, she was treated with immunosuppressors, with only minimal improvement on imaging. Discussion: Desmoplakin cardiomyopathy should be considered in the differential diagnosis of inflammatory cardiomyopathies. Little is known about the use of immunosuppressive treatments, but it could be reasonable for some selected patients.

Combined lactic acidosis and ketoacidosis in a female diabetic patient with severe heart failure.

SGLT2i are now recommended in a wide spectrum of indications including type 2 diabetes (T2DM), heart failure, and chronic kidney disease. This medication class is now available in combination with metformin, which is still a fundamental treatment in patients with T2DM. Despite excellent proven safety profile for both drugs, the expanding use of these agents in clinical practice may lead to an increased incidence of rare side effects, like metformin-associated lactic acidosis (MALA) and euglycemic diabetic ketoacidosis (EDKA), which can be life-threatening. A 58-year-old woman with T2DM and severe heart failure treated by metformin and empagliflozin developed progressive EDKA triggered by fasting that was also complicated by severe acute renal failure and MALA. She was successfully treated with intermittent hemodialysis. This case report highlights the importance of the recognition of rare, but very serious adverse effects due to combined metformin and SGLT2i therapy.

Palpitations Revealing a Primary Pulmonary Artery Sarcoma.

Primary pulmonary artery sarcoma is an exceedingly rare and aggressive malignancy that carries poor prognosis. Clinical manifestations are nonspecific and include chest pain, dyspnea, syncope, palpitations, and asthenia, among others. Delay to diagnosis is common and compromises the prognosis. Here, we report an interesting case of primary pulmonary artery sarcoma presenting with frequent monomorphic premature ventricular contractions arising from the right/left ventricle outflow tract. Cardiac imaging is key in the evaluation of patients with frequent premature ventricular contractions to rule out rare pathologies such as tumour compression.

Le sarcome primaire de l'artère pulmonaire est une tumeur maligne extrêmement rare et agressive de mauvais pronostic. Les manifestations cliniques sont non spécifiques et peuvent inclure de la douleur thoracique, de la dyspnée, des syncopes, des palpitations et de l'asthénie. Le retard diagnostic est fréquent et compromet le pronostic. Nous rapportons ici un cas intéressant de sarcome primaire de l'artère pulmonaire pour lequel le patient présentait des extrasystoles ventriculaires prématurées monomorphes qui provenaient des chambres de chasse des ventricules gauche et droit. L'imagerie est essentielle à l'évaluation des patients présentant de fréquentes extrasystoles ventriculaires afin d'écarter des pathologies rares comme la compression tumorale d'une chambre cardiaque.

Heart Failure With Midrange Ejection Fraction-What Is It, If Anything?

The patient cohort with left ventricular ejection fractions (LVEFs) of 41%-49%, which has been defined as heart failure with midrange ejection fraction (HFmrEF), represent a significant proportion of the heart failure (HF) population. Despite the clear cutoffs established by different society guidelines, confusion remains regarding the exact significance of midrange LVEF within the HF syndrome. Patients with LVEF 41%-49% represent a heterogeneous group of patients sharing pathophysiologic mechanisms, biomarker profiles, comorbidities, and clinical characteristics with patients with preserved and reduced LVEF. In this clinical review, we discuss the underlying pathophysiologic mechanisms that culminate in the clinical syndrome of HF and contribute to the disparities observed between HFpEF, HFrEF, and HFmrEF. We highlight differences and similarities in clinical characteristics and imaging features between HFpEF and HFrEF in an effort to disentangle the heterogeneous group of patients with midrange LVEF, but ultimately we conclude that LVEF should be seen as simply one important element of a continuum throughout the HF syndrome, and that although is useful, it is an oversimplification, because HF syndrome is more of a continuum. The underlying pathophysiology, etiology, and comorbidities of patients presenting with HF is becoming ever more important as the limitations of a classification solely based on LVEF are being better recognised, and as patient-specific personalisation of care is becoming ever more important.

Current Review of Implantable Cardioverter Defibrillator Use in Patients With Left Ventricular Assist Device.

PURPOSE OF REVIEW: While use of implantable cardioverter defibrillator (ICD) in patients with heart failure (HF) and reduced ejection fraction is recommended, their role in patients with left ventricular assist device (LVAD) remains controversial, especially with continuous flow (CF) devices. RECENT FINDINGS: Ventricular arrhythmias (VA) in LVAD patients are frequent and associated with worse outcomes, especially postoperatively. Data on the role of ICDs in LVAD patients are scarce, based on case reports or small retrospective series. While the majority of LVAD patients currently have an ICD, it seems that some might not derive any benefit, with additional risks in terms of inappropriate shocks, psychological distress, and infections. Some CF-LVAD patients are at high risk of VA and hemodynamic collapse; under those circumstances, an ICD might provide benefits. A randomized-controlled trial of routine ICD implantation in CF-LVAD would be needed to clarify their impact on survival in low risk patients.