Acute Stanford type A aortic dissection associated with aortic coarctation repaired by Tirone E. David & debranching techniques with combined axillar and femoral perfusion: a case report

We present the case of a 38-year-old male with a diagnosis of Stanford A aortic dissection and associated coarctation of the thoracic aorta. Acute dissection associated with coarctation of the aorta is a rare problem and difficult to manage surgically. Establishing a cardiopulmonary bypass (CPB) with adequate flows is the main objective of the procedure; optimal cannulation ensures the protection of cerebral and visceral organs. We successfully performed aortic valve re-implantation surgery (T. David Surgery), replacement of the ascending aorta and aortic arch, as well as debranching of the supra-aortic trunks. The cannulation technique was axillary and femoral to guarantee flows through the coarctation area.

Acute Aortic Syndrome in Marfan Syndrome with Chest Pain and Negative Initial Imaging

A 25-year-old male with Marfan Syndrome and previous aortic root surgery presented with severe chest and upper back pain. Initial CT angiogram showed mild aortic dilatation but no acute dissection. Anti-impulse therapy was initiated. Five days after presentation, follow-up CT angiography revealed a new pseudoaneurysm of the descending thoracic aorta. The patient underwent replacement of the mid-descending thoracic aorta with a collagen-impregnated woven polyester tube graft without complication. Despite negative initial imaging and relatively small aortic size, patients with Marfan Syndrome with acute chest pain should be treated with high suspicion and may benefit from admission and serial imaging.

Ascending Aortic Length and Its Association With Type A Aortic Dissection

Background The aim of this study was to determine the role of ascending aortic length and diameter in type A aortic dissection. Methods and Results Computed tomography scans from patients with acute type A dissections (n=51), patients with proximal thoracic aortic aneurysms (n=121), and controls with normal aortas (n=200) were analyzed from aortic annulus to the innominate artery using multiplanar reconstruction. In the control group, ascending aortic length correlated with diameter ( r 2 =0.35, P <0.001), age ( r 2 =0.17, P <0.001), and sex ( P <0.001). As a result of immediate changes in aortic morphology at the time of acute dissection, predissection lengths and diameters were estimated based on models from published literature. Ascending aortic length was longer in patients immediately following acute dissection (median, 109.7 mm; interquartile range [IQR], 101.0–115.1 mm), patients in the estimated predissection group (median, 104.2 mm; IQR, 96.0–109.3 mm), and patients in the aneurysm group (median, 107.0 mm; IQR, 99.6–118.7 mm) in comparison to controls (median, 83.2 mm; IQR, 74.5–90.7 mm) ( P <0.001 all comparisons). The diameter of the ascending aorta was largest in the aneurysm group (median, 52.0 mm; IQR, 45.9–58.0 mm), followed by the dissection group (median, 50.3 mm; IQR, 46.6–57.5 mm), and not significantly different between controls and the estimated predissection group (median, 33.4 mm [IQR, 30.7–36.7 mm] versus 35.2 mm [IQR, 32.6–40.3 mm], P =0.09). After adjustment for diameter, age, and sex, the estimated predissection aortic lengths were 16 mm longer than those in the controls and 12 mm longer than in patients with nondissected thoracic aneurysms. Conclusions The length of the ascending aorta, after adjustment for age, sex, and aortic diameter, may be useful in discriminating patients with type A dissection from normal controls and patients with nondissected thoracic aneurysms.

Sudden unexpected non-cardiac death in the Genoa Medico-Legal District: A six year study (2014–2019) and literature review

Sudden death is described as a natural but unexpected death occurring within one hour from the onset of the patient’s final symptoms. Despite cardiac disease being recognised as the cause of death in most people, sudden and unexpected death can also be non-cardiac related. Often a natural but sudden death is not subject to an autopsy, but only to an external examination, and this runs the risk of wrongly attributing the death to a cardiac cause. The present review is a retrospective-observational study which looks into the cases of sudden non-cardiac death recorded in the Genoa District (Italy) from 2014 to 2019 and investigated through complete autopsy examinations. Amongst these cases, 39 (31.5%) were attributable to gastrointestinal diseases, mostly due to the rupture of oesophageal varices; 39 (31.5%) to respiratory diseases, especially pulmonary infections; 31 (25%) to peripheral vascular disease, mostly attributable to pulmonary thromboembolisms or the acute dissection of aneurysms whilst the remaining 15 cases (12%) were attributable to intracranial haemorrhages.

The formation mechanism of acute dissection of blood blister-like aneurysm and its implication of endovascular treatment

Background Acute mural dissection of the anterior wall of the internal carotid artery which may contribute to the development of blood blister-like aneurysms (BBLAs) was postulated, and stenting or flow diversion treatment across the soi-disant aneurysm was reported in this study. Methods From December 2016 to December 2018, 8 patients presenting with subarachnoid hemorrhage (SAH) due to BBLA were subjected to endovascular treatment with stent-assisted coiling. Clinical outcomes were evaluated using a clinical outcome score scale. Results Based on angiograms, pathologic change involving the supraclinoid segments of the internal carotid artery (ICA) adjacent to BBLA was found in all patients. This pathologic change meant a focal dissection of the supraclinoid segment of the ICA which constituted the pathogenesis of BBLAs. Closed-cell, open-cell, and braided stents were used in 1, 1, and 6 patients, respectively. Complete obliteration was achieved following endovascular treatment among all 8 patients harboring BBLA. One re-bleeding successive to a closed-cell stent across the aneurysmal neck was observed. Follow-up angiograms revealed stable complete exclusion of all BBLAs from the parent vessel at 3 to 8 months. All patients had a favorable clinical outcome score of 0–1. Conclusions Acute dissection of a focal point of the intracranial vessels underlies the development of BBLAs. Open-cell and braided-cell stent-assisted coiling may constitute appropriate treatment due to good apposition against the vascular walls. Adjunctive coils may facilitate immediate complete occlusion of BBLAs.

Outcomes of post mortem genetic diagnosis in SCD victims and primary prevention of cardiac arrest in relatives: a nationwide multidisciplinary and multicentric collaboration in the Czechia

Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Supported by Ministry of Health of the Czech Republic Introduction Post mortem genetic analysis in sudden cardiac death (SCD) represents an important diagnostic tool for the primary prevention of cardiac arrest in victim´s relatives. Purpose To assess the underlying molecular pathogenesis of SCD in a representative Czech cohort and to evaluate the effects of primary prevention of SCD in genetic relatives. Patients and Methods Between 2016 and 2020 we have ascertained 100 SCD cases (29 females/71 males; age range 0-52 years). According to autopsy protocols, cases with SCD were divided into categories of sudden arrhythmic death (SADS), sudden unexplained death (in infants; SUD/SUDI), thoracic aortic aneurysm/dissection and cardiomyopathy hypertrophic, arrhythmic, dilated (HCM, ACM, DCM) and sudden infant death syndrome (SIDS). DNA was isolated from post mortem biopsies / relatives blood and subjected to massively parallel sequencing (Illumina, USA) comprising custom-made candidate gene panel (100 genes). Genetic counselling and cardiological examinations were carried out in 245 family members. Results According to post mortem-established diagnosis, we identified 20 victims with SADS and SUD/SUDI, 11 with HCM and DCM, 19 with ACMG, 8 SIDS cases and 9 acute dissection cases. Most of victims died at sleep or at rest, while only 10/100 victims died during strenuous sport activities. About 50% of SCD victims did not report any apparent cardiac complaints. Highly likely or certain molecular etiology (i.e. based on presence of ACMG.net Class 4 to 5 variants) was disclosed in 19/100 (19%) in RYR2, KCNH2, SCN5A, FLNC (stop), TTN, RBM 20, LMNA/C, PRKAG2, MYBPC3, DSC2, FHL1, TGFBR1 and Col3A1 genes (see Tab). Finally, we identified 52/241 phenotype/genotype positive family members who are at risk of cardiac arrest and were offered corresponding cardiological care. Conclusion Multidisciplinary cooperation, together with centralized and standardized molecular genetic testing, enables the primary prevention of cardiac arrest in relatives of SCD victims. Results of post mortem genetic analysis Post mortem diagnosis Nr. Gender Age (years) Nr. of positive cases (DNA variant class IV or V) Gene Nr. examined relatives/phenotype or genotype positive cases SADS 20 8 females12 males 3-52 5/20 (25%) KCNH2 3x RYR2RANGFR 56/11 SUD/SUDI 20 5 females, 15 males 0-50 1/18 (5%, 2 non informative cases) RYR2 45/9 HCM 11 0 females11 males 14-52 3/11 (27%) MYBPC3FHL1PRKAG2 26/9 DCM 11 3 females8 males 8-48 4/11 (36%) TTN (3x)RBM 20FLNC (stop) 24/7 ACM 19 9 females10 males 17 - 49 4/19 (21%) SCN5AFLNC (stop)DSC2LMNA/C 58/9 SIDS 8 3 females5 males &lt; 1 0/8 - 12/0 Acute dissection 9 1 female8 males 16-49 2/9 (22%) TGFBR1Col3A1 24/7