It's a long way to the top! Congenital venous anomalies and left isomerism limiting atrial fibrillation transcatheter ablation.

BACKGROUND: Atrial fibrillation is the most common sustained cardiac arrhythmia in adults and it is associated with a high burden of mortality and morbidity worldwide. Catheter ablation is increasingly used to improve symptoms and prognosis in selected patients. Lower limb venous access with subsequent transseptal approach to the left atrium is the standard procedure for atrial fibrillation catheter ablation. CASE PRESENTATION: We report an unusual case of complex venous anomaly with a left-sided inferior vena cava with hemiazygos continuation to a persistent left superior vena cava draining in an enlarged coronary sinus in a patient with persistent atrial fibrillation scheduled for transcatheter ablation. DISCUSSION: Lower limb venous anomalies may limit a standard transseptal approach to the left atrium thus precluding an effective catheter ablation procedure for atrial fibrillation. Alternative interventions, such as unconventional percutaneous access, thoracoscopic approach and "ablate and pace" procedures, may be necessary in patients with symptomatic atrial fibrillation and complex venous anomalies.

Saphenous vein to the right coronary system from the right thoracic artery or the aorta. Long-term propensity-matched results of 2 groups.

OBJECTIVES: Since 2000, we anastomosed the saphenous vein graft to the right coronary artery system using the stump of the right internal thoracic artery as inflow. The long-term results of patients where the right coronary artery was grafted with the right internal thoracic artery or the ascending aorta as saphenous vein inflow has not been reported. METHODS: From 2000 to 2018, 699 consecutive patients had right internal thoracic artery elongated with saphenous vein (I-graft group, n = 358, 51.2%) or saphenous vein from the aorta (Ao-graft group, n = 341, 48.8%) on right coronary artery system. Inclusion criteria were age ≤75 years, bilateral internal thoracic arteries as a Y graft on the left system (three-vessel disease, n = 603, 86.3%) or as a left internal thoracic artery on left anterior descending and right internal thoracic artery elongated with saphenous vein on the right coronary artery system (two-vessel disease, n = 96, 13.7%), only 1 saphenous vein per patient. Propensity-matching identified 272 patients per group. One-hundred and twenty-two patients underwent coronary computed tomographic angiography to asses grafts patency after a median follow-up of 88 (65-93) months. RESULTS: In the paired samples, there was no difference in the early outcome. Ten-year survival and freedom from death, non-fatal acute myocardial infarction and repeat revascularization were higher in I-graft group: 90.6 [standard error (SE): 2.0] vs 78.2 (SE: 5.3), P = 0.0266, and 85.2 (SE: 2.4) vs 69.9 (SE: 5.3), P = 0.0179. Saphenous vein graft, at a long-time follow-up, showed a higher patency rate (81.6% (SE: 7.0) vs 50.7% (SE: 7.9), P < 0.0001) and a smaller internal lumen diameter (2.7, standard deviation: 0.4 vs 3.4, standard deviation: 0.6 mm, P < 0.0001) when right internal thoracic artery was the inflow. CONCLUSIONS: Grafting the right coronary artery with saphenous vein may entail higher patency rate and better outcome when the inflow is the right internal thoracic artery than when is the ascending aorta. Prospective randomized data are needed to test this hypothesis.

Coronary artery evaluation during ECG-gated CT of the thoracic aorta: Do not consider it a negligible clinical information.

A CT angiography, performed for ascending aorta study, revealed, incidentally, an anomalous origin and course of right coronary artery. Anomalies in the origins and course of the coronary arteries are critical information that should not be missed, because they can worsen the patient's prognosis.

Interrupted inferior vena cava draining into the coronary sinus associated with circumflex artery to coronary sinus fistula.

In an adult patient, coronary artery fistula involving the circumflex artery (CX) connected to the coronary sinus caused aneurysm of the left main and CX associated with ectasia of the intermediate branch. The patient had posterolateral infarction with severe ischemic mitral regurgitation and moderate tricuspid regurgitation. A rare venous return anomaly was also present. The inferior vena cava, which was interrupted at the level of the liver, continued with the hemiazygos to drain into a persistent left superior vena cava, which in turn drained into the coronary sinus. Surgery included CX closure and mitral and tricuspid repair. The strategy had to be adapted to the anatomy. The fistula was dissected and snared for cardioplegia delivery, and venous return was achieved by cannulation of the superior vena cava and femoral vein. The procedure was uneventful, and 4 years later the patient is asymptomatic.

Increase of Epicardial Adipose Tissue (EAT) With Fat Infiltration of Right Ventricular Myocardium: Is it Always a High-Risk Stigmata or a Worrisome Feature?

Incidental epicardial adipose tissue (EAT) and subepicardial fat infiltration on CT scans are not uncommon and the differential diagnosis can be a challenge. Considering the vastness of the possible disorders, it is important to differentiate physiologic age-related condition from pathologic disease. We present a case of an asymptomatic 81-year-old woman in which according to ECG and CMR findings we considered as possible differential diagnoses arrhythmogenic cardiomyopathy (ACM) dominant-right variant, lipomatosis and physiological growth of epicardial fat. We focus on patient characteristics, location of the fat replacement, heart morphovolumetry, wall motion of the ventricles and absence of late gadolinium enhancement to diagnose pericardial fat hypertrophy and physiological fatty infiltration. The role of EAT is unclear and it could play a part in the development of atherosclerosis and atrial fibrillation. Therefore, the clinicians should not underestimate this condition even if it would be an incidental finding in asymptomatic patients.

Constrictive Pericarditis With Massive Calcification in Systemic Sclerosis Treated With Pericardiectomy: A Case Report.

Calcific constrictive pericarditis is a very rare complication of systemic sclerosis. This is the first report of surgically treated calcific constrictive pericarditis in systemic sclerosis. A 53 years-old woman, affected by limited systemic sclerosis, had a diagnosis of calcific constrictive pericarditis. She had a medical history of congestive heart failure since 2022. The patient was treated with pericardiectomy. Via a median sternotomy, the pericardium was dissected and removed from the midline to the left phrenic nerve, thus freeing the heart. Three months after the pericardiectomy, there was a significant clinical improvement. The calcific evolution of chronic pericarditis is a rare complication of systemic sclerosis. This case represents, at best of our knowledge, the first report of calcific constrictive pericarditis, in systemic sclerosis, treated with pericardiectomy.

Cutting the second order chords during mitral valve repair.

The chordae tendinae connect the papillary muscles (PMs) to the mitral valve. While the first-order chordae serve to secure the leaflets to maintain valve closure and prevent mitral valve prolapse, the second-order chordae are believed to play a role in maintaining normal left ventricle size and geometry. The PMs, from where the chordae tendinae originate, function as shock absorbers that compensate for the geometric changes of the left ventricular wall. The second-order chordae connect the PMs to both trigons under tension. The tension distributed towards the second-order chordae has been demonstrate to be more than threefold that in their first-order counterpart. Cutting the second-order chordae puts all the tension on the first-order chordae, which are then closer to their rupture point. However, it has been experimentally demonstrated that the tension at which the first-order chordae break is 6.8 newtons (N), by far higher than the maximal tension reached, that is 0.4 N. Even if the clinical reports have been favorable, the importance of cutting the second-order chordae to recover curvature of the anterior leaflet and increase the coaptation length between the mitral valve leaflets has been slowly absorbed by the surgical world. Nevertheless, there are progressive demonstrations that chordal tethering affects the anterior leaflet not only in secondary, but also in primary mitral regurgitation, having a not negligible role in the long-term outcome of mitral repair.

Neutrophil to lymphocyte ratio predicts permanent pacemaker implantation in TAVR patients.

INTRODUCTION: In this prospective multicenter analysis, we aimed to investigate the predictive role of neutrophil/lymphocyte ratio (NLR) in permanent pacemaker implantation (PPI) in patients undergoing transcatheter aortic valve replacement (TAVR). MATERIALS AND METHODS: One hundred and seventy-nine consecutive patients without previous PPI underwent TAVR from February 2017 to September 2021. Patients were further divided based on presence (n = 48) and absence of conduction abnormalities (CAs) at hospital admission (n = 131). RESULTS: In patients with previous CAs, NLR values did not differ significantly between patients requiring PPI (n = 16, 33%) and those not requiring it. In contrast, in patients with no CAs at hospital admission, NLR values measured at admission and on TAVR day were significantly higher in patients requiring PPI (n = 17, 13%) (4.07 ± 3.22 vs. 3.01 ± 1.47, p = .025, and 10.81 ± 7.81 vs. 5.84 ± 3.78, p = .000, respectively). Multivariable analysis showed that NLR at TAVR day was an independent predictor of PPI in patients without CAs (OR 1.294; 95% CI 1.028-1.630; p = .028), but not in those with previous CAs. ROC curve analysis showed that the cut point was a NLR value of >7.25. Time to PPI was delayed till 21 days in patients without CAs. CONCLUSIONS: In this prospective study, higher NLR values on the day of TAVR day were associated with an increased PPI rate in patients undergoing TAVR with no previous CAs. It is advisable, being inflammation part of the process, to prolong the time of observation for all patients without CAs till at least 21 days not to miss any new CA necessitating PPI.

The appearance of phagocytic microglia in the postnatal brain of Niemann Pick type C mice is developmentally regulated and underscores shortfalls in fine odor discrimination.

The loss of NPC1 or NPC2 function results in cholesterol and sphingolipid dyshomeostasis that impairs developmental trajectories, predisposing the postnatal brain to the appearance of pathological signs, including progressive and stereotyped Purkinje cell loss and microgliosis. Despite increasing evidence reporting the activation of pro-inflammatory microglia as a cardinal event of NPC1 disease progression at symptomatic stages both in patients and preclinical models, how microglia cells respond to altered neurodevelopmental dynamics remains not completely understood. To gain an insight on this issue, we have characterized patterns of microglia activation in the early postnatal cerebellum and young adult olfactory bulb of the hypomorphic Npc1nmf164 mouse model. Previous evidence has shown that both these areas display a number of anomalies affecting neuron and glial cell proliferation and differentiation, which largely anticipate cellular changes and clinical signs, raising our interest on how microglia interplay to these changes. Even so, to separate the contribution of cues provided by the dysfunctional microenvironment we have also studied microglia isolated from mice of increasing ages and cultured in vitro for 1 week. Our findings show that microglia of both cerebellum and olfactory bulb of Npc1nmf164 mice adopt an activated phenotype, characterized by increased cell proliferation, enlarged soma size and de-ramified processes, as well as a robust phagocytic activity, in a time- and space-specific manner. Enhanced phagocytosis associates with a profound remodeling of gene expression signatures towards gene products involved in chemotaxis, cell recognition and engulfment, including Cd68 and Trem2. These early changes in microglia morphology and activities are induced by region-specific developmental anomalies that likely anticipate alterations in neuronal connectivity. As a proof of concept, we show that microglia activation within the granule cell layer and glomerular layer of the olfactory bulb of Npc1nmf164 mice is associated with shortfalls in fine odor discrimination.

Outcome of permanent pacemaker implantation in transcatheter or surgical aortic valve replacement: A still unsolved problem.

Despite advances in technologies and clinical experience, conduction disorders, after transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR), represent the weak point of these procedures, requiring permanent pacemaker implantation (PPI) till 37.7% of patients in TAVR recipients. The role of PPI in TAVR and SAVR remains controversial in mid- and long-term outcomes. Indeed, many studies have been published with contradictory results, leaving doubts rather than certainties.

A morphofunctional analysis of the regurgitant mitral valve as a guide to repair: Another point of view.

Based on Carpentier's classification and principles, the techniques for mitral valve repair continue to evolve. We herein report our experience with the morphofunctional echocardiographic analysis of single mitral leaflets, as different anatomic features, even if conflicting, may coexist not only in the two leaflets but in the same leaflet as well. A classification is proposed, based on the length (normal, short, or long) and mobility (normal, restricted, or excessive) of mitral leaflets. The surgical techniques adopted for mitral valve repair are the direct consequence of this analysis.

A historical appraisal of the techniques of left ventricular volume reduction in ischemic cardiomyopathy: Who did what?

Resection or exclusion of scars following a myocardial infarction on the left anterior descending artery territory started even before the beginning of the modern era of cardiac surgery. Many techniques were developed, but there is still confusion on who did what. The original techniques underwent modifications that brought to a variety of apparently new procedures that, however, were only a "revisitation" of what described before. In some case, old techniques were reproposed and renamed, without giving credit to the surgeon that was the original designer. Herein we try to describe which are the seminal procedures and some of the most important modifications, respecting however the merit of who first communicated the procedure to the scientific world.

The secret life of the mitral valve.

In secondary mitral regurgitation, the concept that the mitral valve (MV) is an innocent bystander, has been challenged by many studies in the last decades. The MV is a living structure with intrinsic plasticity that reacts to changes in stretch or in mechanical stress activating biohumoral mechanisms that have, as purpose, the adaptation of the valve to the new environment. If the adaptation is balanced, the leaflets increase both surface and length and the chordae tendineae lengthen: the result is a valve with different characteristics, but able to avoid or to limit the regurgitation. However, if the adaptation is unbalanced, the leaflets and the chords do not change their size, but become stiffer and rigid, with moderate or severe regurgitation. These changes are mediated mainly by a cytokine, the transforming growth factor-ß (TGF-ß), which is able to promote the changes that the MV needs to adapt to a new hemodynamic environment. In general, mild TGF-ß activation facilitates leaflet growth, excessive TGF-ß activation, as after myocardial infarction, results in profibrotic changes in the leaflets, with increased thickness and stiffness. The MV is then a plastic organism, that reacts to the external stimuli, trying to maintain its physiologic integrity. This review has the goal to unveil the secret life of the MV, to understand which stimuli can trigger its plasticity, and to explain why the equation "large heart = moderate/severe mitral regurgitation" and "small heart = no/mild mitral regurgitation" does not work into the clinical practice.