Unicuspid aortic valve: a rare congenital anomaly

Unicuspid aortic valve (UAV) is a rare congenital aortic valve anomaly. It has two subtypes and often is associated with aortic valve dysfunction and corresponding clinical presentations. Echocardiography is the first choice of diagnostic method for UAV. Three-dimensional echocardiography has played an increasingly important role in diagnosis, intraprocedural guidance, and post-procedural assessment in recent years. There remain challenges in distinguishing UAV from bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV). Misdiagnosis is most resulted from raphes or leaflet calcifications. Multi-modality imaging has obvious advantages over single-modality imaging and is crucial for UAV diagnosis. Accurate identification of aortic valve morphology is important to choose the best treatment. Aortic balloon valvoplasty, surgical valvotomy, commissurotomy, Ross procedure, surgical valve repair and surgical replacement are most common treatment modalities of UAV. In this review, we aim to discuss UAV including epidemiology, definition, classification, diagnostic importance and necessity, valvular function, clinical presentation, associated disorders, non-invasive diagnostic modalities and therapeutic approaches.

Coronary Computer Tomography Angiography in 2021—Acquisition Protocols, Tips and Tricks and Heading beyond the Possible

Recent technological advances, together with an increasing body of evidence from randomized trials, have placed coronary computer tomography angiography (CCTA) in the center of the diagnostic workup of patients with coronary artery disease. The method was proven reliable in the diagnosis of relevant coronary artery stenosis. Furthermore, it can identify different stages of the atherosclerotic process, including early atherosclerotic changes of the coronary vessel wall, a quality not met by other non-invasive tests. In addition, newer computational software can measure the hemodynamic relevance (fractional flow reserve) of a certain stenosis. In addition, if required, information related to cardiac and valvular function can be provided with specific protocols. Importantly, recent trials have highlighted the prognostic relevance of CCTA in patients with coronary artery disease, which helped establishing CCTA as the first-line method for the diagnostic work-up of such patients in current guidelines. All this can be gathered in one relatively fast examination with minimal discomfort for the patient and, with newer machines, with very low radiation exposure. Herein, we provide an overview of the current technical aspects, indications, pitfalls, and new horizons with CCTA, providing examples from our own clinical practice.

The VALVE REGEN project. Developing an innovative tissue engineered heart valve, in vitro and in vivo testing – preliminary results

Background Regenerative Medicine and Tissue Engineering are the grounds on which multidisciplinary teams aspire to obtain the perfect valvular substitute, which overcomes shortcomings of the present prostheses. Purpose Was to obtain a tissue engineered heart valve (TEHV) by repopulating with valvular resident cells – endothelial (EC) and fibroblasts (FB) a decellularized heart valve scaffold. Then their functionality and behavior was assessed in vitro and in vivo. Methods This study is part of a research grant approved by the Ethics Committee of the University. Six ovine pulmonary valves underwent a perfusion based decellularization protocol. Using a sequence of chemical and enzymatic treatment under a pressure gradient, cell removal was achieved and attested by histological investigations (DAPI nuclear staining –4',6-diamidino-2-phenylindol and haematoxylin-eosin) and DNA extraction. Ovine sub-dermal adipose tissue was harvested followed by stem cells isolation and culture. Using Endothelial Cell Growth Supplement and mechanical stimuli EC were differentiated and with Transforming Growth Factor-B1, FB were obtained. FB were internally seeded into cuspis bases using a 22 gauge needle and externally on the adventitia by using a rotator allowing a uniform distribution and seeding of cells. EC were seeded into leaflets pockets and intra-luminal also using the rotator. The repopulated valves were preconditionated in a bioreactor by gradually exposing them to the pulmonary hemodynamic regimen. By using a high speed camera, their behavior was examined when exposed to in vivo conditions. The in vivo testing was performed by surgical implantation in the gold model considered animal – the sheep. By transesophageal ultrasound (TEE US) and epicardic US, their intra-operatory function was evaluated. Post-procedure, evaluation was performed by periodic trans-thoracic (TTE US). Results Six TEHV were obtained. The decellularization histology assessment revealed acellular scaffolds and non-detectable nucleic material at the DNA extraction. Six adipose derived stem cells cultures were obtained and subsequently specialized towards EC and FB lines. The repopulation procedures underwent without incidents. During the bioreactor preconditioning, the TEHV showed complete opening and competent central coaptation. Leaflets presented physiological movement and absence of damage of valvular apparatus. The TEE US evaluation in vivo revealed normal valvular function without signs of stenosis or regurgitation. The periodic TTE US showed preserved valvular function. Conclusions Our preliminary results point out a manufactured TEHV with physiological behavior when tested in vitro and in vivo. Their interaction with a living body will be pointed out only in the explant phase, after histology analysis. The present results appear optimistic but only extended studies and follow-ups will certify their superiority in terms of performances and behavior. Funding Acknowledgement Type of funding source: Public grant(s) – EU funding. Main funding source(s): This paper was financed by a grant from the Competitiveness Operational Programme 2014-2020, Tissue engineering technologies for cardiac valve regeneration, valve-regen, id P_37_673, Mysmis code: 103431, contract 50/05.09.2016.

Myocardial disease and ventricular arrhythmia in Marfan syndrome: a prospective study

Background Aortic root dilatation and—dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case–control study including 86 patients with MFS (55.8% women, mean age 36.3 yr—range 13–70 yr–) and 40 age—and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30 ± 7 months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N = 55) and with previous surgery or valvular dysfunction (MFS-2; N = 31). Results Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles [(S)VES]. Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.

Myocardial Disease and Ventricular Arrhythmia in Marfan Syndrome – a prospective study

Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3yr - range 13-70yr-) and 40 age- and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30±7months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N=55) and with previous surgery or valvular dysfunction (MFS-2; N=31).Results: Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles ((S)VES). Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT.Conclusion: Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.

Myocardial Disease and Ventricullar Arrhythmia in Marfan Syndrome – A Prospective Study

Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3yr - range 13-70yr-) and 40 age- and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30±7months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N=55) and with previous surgery or valvular dysfunction (MFS-2; N=31). Results: Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles ((S)VES). Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion: Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Myocardial function should be evaluated in all patients with MFS, especially those with valvular disease and cardiac surgery.

Bicuspid aortic valve disease and competitive sports: key considerations and challenges

Bicuspid aortic valve (BAV) is a common congenital disorder. It could simply be a minor anatomic abnormality or be associated with progressive aortic stenosis, aortic regurgitation, and aortic dilation. If an athlete is recognized to have a BAV, questions arise with regard to whether they can pursue their selected sports, particularly elite athletic activity, and what type of follow-up examinations are necessary and how often should be done. Valvular disorders such as the degree of aortic stenosis and aortic regurgitation, aortic size, and coexisting disorders are also influencing factors. The absence of robust controlled studies, which are difficult to perform, make decision-making difficult, although recommendations by expert panels provide some guidance. The general consensus is that athletes with BAV with normal valvular function and no aortic dilation can participate in all athletic activities. Those with mild aortic dilation should undergo annual screening, some more frequently than others. Those with moderate or severe valvular stenosis or regurgitation should be managed based on the haemodynamic impact of the valve lesion. Athletes with coexisting lesions or syndromes should be evaluated comprehensively. The overall recommendation to an individual athlete should incorporate many factors and employ a multidisciplinary approach.