Three-dimensional-mapping-guided permanent conduction system pacing in paediatric patients with congenitally corrected transposition of the great arteries.

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.

Long-term follow-up after recanalisation of aortic arch atresia.

AIMS: Aortic arch atresia (AAA) is one of the rarest obstructive defects. The presence of this anomaly in adult age is uncommon. The typical anatomic feature consists of a complete occlusion of the membranous obstruction resulting in an acquired atresia without flow continuity between the proximal and distal segments. This feature is important in determining the feasibility of percutaneous intervention. The aim of the present study was to share long-term follow-up data of adult patients with AAA requiring percutaneous interventions for the management of this rare anomaly involving five different centres. METHODS AND RESULTS: Retrospective data of 19 patients (12 males, 63.2%, mean age 32.2±18.9 years) diagnosed with AAA treated in five different centres between 1999 and 2017 were collected. All patients underwent percutaneous recanalisation by (1) radiofrequency (RF) system (five patients, 26.3%), (2) extra-stiff guidewire (12 patients, 63.2%), and (3) transseptal needle (two patients, 10.5%). All procedures were subsequently followed by covered stent implantation. Two patients developed complications during the procedure and one of them died. Over a median follow-up of 4.94 years, four (21%) patients were able to be weaned from medications for hypertension. All the patients underwent reassessment for recurrence or restenosis during the follow-up. Seven (36.8%) patients underwent successful stent dilatation with a balloon. After the intervention, one patient experienced a late complication; however, one patient died due to an unknown cause believed to be unrelated to the previous recanalisation procedure. CONCLUSIONS: Percutaneous treatment of AAA is feasible with good long-term survival. This study reports the largest case series so far available in the literature.

Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease.

Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. Expert opinion: PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.

Cardiac thrombi mistaken for metastasis in recurrent melanoma.

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.

[Anomalous origin of the right coronary artery from the left sinus of Valsalva: essential role of computed tomography to clarify the coronary artery course and literature review on diagnostic and therapeutic management in 215 cases]. / Origine anomala della coronaria destra dal seno di Valsalva sinistro: ruolo essenziale della tomografia computerizzata nello studio del decorso arterioso coronarico e revisione della letteratura sull'approccio diagnostico-terapeutico in 215 casi.

Coronary artery anomalies are among the most confusing topics in cardiology. Although the medical community is increasingly aware that coronary anomalies can be fatal, the reasons for a sudden fatal event and the frequency with which it occurs are generally unclear. In addition, coronary anomalies are usually compatible with normal prenatal myocardial development and postnatal growth and function, even permitting intense athletic activity. Nevertheless, coronary anomalies may lead to a pathological state, which usually originates suddenly, and the diagnostic and therapeutic management remains controversial, as outlined in our review of 215 cases in the literature. We report the case of a 53-year-old female who presented with effort/stress angina. She underwent coronary angiography, which demonstrated no significant parietal lesions but an anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva: the RCA showed an anomalous course between the aorta and the pulmonary artery with systolic compression and potential myocardial ischemia. The patient was then referred for surgical treatment and, according to the surgeons, she underwent isolated coronary artery bypass with the right internal mammary artery on the RCA and ligature of the native RCA to prevent competitive flow.

Mechanisms of diabetic dyslipidemia: relevance for atherogenesis.

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.