Diabetic dyslipidemia is due to a multiple array of metabolic abnormalities determining a typical phenotype characterized by increased plasma triglycerides, reduced HDL and a preponderance of small, dense LDL. This dyslipidemia, defined as atherogenic dyslipidemia, is thought to be highly responsible for the increased cardiovascular risk in diabetes mellitus. Several lines of evidence indicate that the increased liver production of VLDL is the main underlying defect in atherogenic dyslipidemia. This review will recapitulate the pathophysiological aspects of diabetic dyslipidemia with special focus on the molecular mechanism causing increased liver production of VLDL in diabetic patients. The consequences of atherogenic dyslipidemia on mechanisms of atherogenesis will be also reviewed.

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Aims In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. Methods and results Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th–75th centiles). Seven CCTGA patients aged 15 (9–17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). Conclusion Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.

Intra-cardiac thrombi can be incidentally found in recurrent melanoma and need careful assessment. An 81-year-old woman, with a history of malignant nasopharyngeal melanoma, was evaluated by echocardiography and cardiac magnetic resonance due to the detection of undefined masses localized both in right atrium and ventricle during contrast-enhanced thoraco-abdominal computed tomography.

Pulmonary hypertension is not uncommon in adult patients with congenital heart disease and can significantly affect their exercise capacity, quality of life and prognosis. Timely identification and management of pulmonary hypertension in these patients is important. Patients must be allocated to the correct diagnostic group and treatment decided upon, including offering pulmonary arterial hypertension therapies to those who are most likely to benefit.In this paper, we provide an overview of the diagnostic modalities that are used to investigate and diagnose pulmonary hypertension in modern adult congenital heart disease practice. Emphasis is placed on echocardiography, which is the modality of choice for raising the suspicion of PH, and cardiac catheterisation, which is fundamental in establishing the diagnosis. Other modalities, such as cardiac magnetic resonance imaging and computed tomography can provide complementary information on anatomy and physiology.Combining all this information into a clinical diagnosis and management plan requires clinical expertise and a multidisciplinary approach, especially when managing this rare and complex group of patients.

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