Trans-catheter Bioprosthetic Heart Valve Implantation in Iran (Tehran Heart Centre Experiance)

Introduction: With the development of interventional cardiology in the world, in addition to coronary and aortic diseases, the treatment of heart valve diseases through catheters has recently begun. The treatment of aortic stenosis (which was only possible with open surgery and valve replacement) was first performed in the world in 2002 by Alain Cribier in France with catheter insertion of the valve and was called Trans catheter Aortic valve implantation TAVI. Trans catheter Aortic valve implantation (TAVI) was performed in Iran in 2009 in the Heart Centre of Tehran and now is routinely performed by both Balloon Expanding and self-expanding valves. In addition, we do valve in valve implantation for degenarative biopresthetic valve, in all Heart valve positions too. The short and medium term results of this treatment were reviewed in the form of research projects and published in several articles. In this review, we have explained the initiating of this new procedure in our country with the following results.

Case Report: Occurrence of Severe Thoracic Aortic Aneurysms (Involving the Ascending, Arch, and Descending Segments) as a Result of Fibulin-4 Deficiency: A Rare Pathology With Successful Management

Aortic diseases requiring surgery in childhood are distinctive and rare. Very few reports in the literature account for the occurrence of multiple thoracic aortic aneurysms in the same pediatric patient because of a genetic cause. We report a rare occurrence of severe thoracic aortic aneurysms (involving the ascending, arch and descending aortic segments) with severe aortic insufficiency in a 7-year-old female child secondary to the extremely rare and often lethal genetic disorder, cutis laxa. She was eventually identified as a carrier of a homozygous EFEMP2 (alias FBLN4) mutation. This gene encodes the extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B that leads to severe aortopathy with aneurysm formation and vascular tortuosity. Parents of the child were not known to be consanguineous. Significant symptomatic improvement in the patient could be discerned after timely intervention with the valve-sparing aortic root replacement (David V procedure) and a concomitant aortic arch replacement. This is a unique report with a successful outcome that highlights the occurrence of a rare hereditary aortopathy associated with a high morbidity and mortality, and the importance of an early diagnosis and timely management. It also offers insight to physicians in having a very broad differential and multimodal approach in handling rare pediatric cardio-pathologies with a genetic predisposition.

Are Aortic Root and Ascending Aorta Diameters Measured by the Pediatric Versus the Adult American Society of Echocardiography Guidelines Interchangeable?

Ascending aorta diameters have important clinical value in the diagnosis, follow-up, and surgical indication of many aortic diseases. However, there is no uniformity among experts regarding ascending aorta diameter quantification by echocardiography. The aim of this study was to compare maximum aortic root and ascending aorta diameters determined by the diastolic leading edge (DLE) and the systolic inner edge (SIE) conventions in adult and pediatric patients with inherited cardiovascular diseases. Transthoracic echocardiograms were performed in 328 consecutive patients (260 adults and 68 children). Aorta diameters were measured twice at the root and ascending aorta by the DLE convention following the 2015 American Society of Echocardiography (ASE) adult guidelines and the SIE convention following the 2010 ASE pediatric guidelines. Comparison of the diameters measured by the two conventions in the overall population showed a non-significant underestimation of the diameter measured by the SIE convention at root level of 0.28 mm (CI -1.36; 1.93) and at tubular ascending aorta level of 0.17 mm (CI-1.69; 2.03). Intraobserver and interobserver variability were excellent. Maximum aorta diameter measured by the leading edge convention in end-diastole and the inner edge convention in mid-systole had similar values to a mild non-significant underestimation of the inner-to-inner method that permits them to be interchangeable when used in clinical practice.

Multidetector computed tomography study to measure thoracic aorta diameters in Egyptian population

Background The aorta is the largest and strongest artery in the body that plays an important role in the control of systemic vascular resistance and heart rate. Aortic diseases contribute to the wide spectrum of arterial diseases that may be diagnosed after a long period of subclinical development. Multidetector computed tomographic scanners (≥ 64 detector rows) for aortic imaging remain one of the most preferred imaging techniques for diagnosis and follow-up of aortic conditions in acute as well as chronic presentations. The aim of this study is to establish a normal reference values for aortic diameters among Egyptian population and to find which of the cardiovascular risk factors could be an independent determinant of the aortic diameters. Results Five hundred and sixteen Egyptian individuals were enrolled in our study, the mean age was 53.5 ± 10.9, and males comprised 61.4% of the study population. Aortic root diameters measured at the annulus, sinus and STJ were 23.09 ± 2.55 mm, 33.75 ± 3.93 mm and 26.13 ± 3.05 mm, respectively. The BSA-indexed diameters were 11.70 ± 1.39, 17.10 ± 2.10 and 13.25 ± 1.65, respectively. The diameter of the tubular part of ascending aorta was 30.97 ± 4.16 mm, and the BSA-indexed diameter was 15.71 ± 2.28. The aortic diameters measured at the level of the pulmonary bifurcation were 24.56 ± 2.95 mm and 23.79 ± 2.96 mm at systolic and diastolic phases, respectively. The BSA-indexed diameters were 12.44 ± 1.52 and 12.05 ± 1.52 at systolic and diastolic phases, respectively. At the diaphragmatic level, the mean diameters were 22.39 ± 2.72 mm and 21.49 ± 2.79 mm at systolic and diastolic phases, respectively. The BSA-indexed diameters were 11.34 ± 1.43 and 10.98 ± 1.48 at systolic and diastolic phases, respectively. Age, gender, BSA, BMI and hypertension were statistically significant independent predictors of ascending and descending aortic diameters. Conclusions Our study established a normal reference value for thoracic aortic diameters among Egyptians using contrast enhanced MSCT aortography. Age, Gender, BSA, BMI and hypertension are the major determinants of aortic diameters.

Arrhythmia and cardiomyopathy in Heritable Thoracic Aortic Disease: an international retrospective cohort study

Background Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS) and related heritable thoracic aortic diseases (HTAD) are well-known for their aortic complications. Myocardial dysfunction and arrhythmia are less known in this setting but have been increasingly reported as additional causes of morbidity and mortality. Related to the rarity of the disorders, data on the prevalence of these features and clinical characteristics of the patients are difficult to obtain, calling for a multicentre initiative. Purpose To study the prevalence of myocardial dysfunction and arrhythmia in patients with HTAD and describe their clinical and genetic profile. Methods Nine centres from seven countries participated in this multicentre retrospective study. Medical records of patients 12 years or older carrying a (likely) pathogenic variant in the FBN1 gene, LDS genes (TGFBR1, TGFBR2, TGFB2, TGFB3 and SMAD3) or ACTA2 gene were screened. Patients presenting myocardial dysfunction and/or arrhythmia were identified, and clinical and genetic data were collected. Myocardial dysfunction included (a)symptomatic reduced ejection fraction (EF <50% – HFrEF) or symptomatic preserved EF (HFpEF), as documented in the patient charts. Arrhythmias included atrial fibrillation or flutter (AF/AFL), ventricular tachycardia (VT), ventricular fibrillation (VF) and (aborted) sudden cardiac death (SCD) (presumed arrhythmogenic). Results In total, 3219 patients with HTAD were screened: 2761 with a variant in FBN1, 385 with a variant in one of the LDS genes (TGFBR1, TGFBR2, TGFB2, TGFB3 and SMAD3) and 73 carrying a variant in ACTA2. Myocardial dysfunction and arrhythmia were not reported in patients carrying an ACTA2 variant. Myocardial dysfunction was observed in patients with a variant in FBN1 and the LDS genes, without significant differences in prevalence (2.3% vs. 1.8%, p=0.563). Patients with a variant in the LDS genes presenting myocardial dysfunction were younger than patients carrying a variant in FBN1 (25±11 years vs. 39±17 years, p=0.034). The prevalence of VT/VF/SCD was similar in patients with a variant in one of the LDS genes compared to those with a variant in FBN1 (1.6% vs. 0.8%, p=0.132) and there was no difference in age at time of event (26±13 years vs. 33±14 years, p=0.289). Among patients with a variant in the LDS genes, the prevalence of VT/VF/SCD was highest in patients carrying a variant in the TGFBR2 gene and was significantly higher compared to patients with a variant in FBN1 (3.4% vs. 0.8%, p=0.017). In contrast, AF/AFL was significantly more often reported in patients with a variant in FBN1 compared to those with a variant in one of the LDS genes (1.7% vs. 0.3%, p=0.033). Conclusions Myocardial dysfunction and arrhythmia are rare features in patients with HTAD. They occur predominantly in patients with a variant in FBN1 and LDS genes, but were not reported in patients carrying a variant in the ACTA2 gene. Further analysis to identify other contributing factors is necessary. FUNDunding Acknowledgement Type of funding sources: None. Figure 1

Changes in health perception of COVID-19 among patients with aortic diseases: a longitudinal study between the first and second wave of the COVID-19 pandemic

Background The rapidly spread of the novel coronavirus disease (COVID-19) worldwide has become the most challenging global health pandemic since the 1918 flu. In Germany, more than 2.5 million cases are confirmed so far, with more than 70,000 deaths. An increased fatality rate was seen among patients with preexisting comorbid conditions, especially with cardiovascular diseases, representing this group at particular risk. Purpose Risk perceptions of public health crises like the COVID-19 pandemic can affect people's mental health, reveal gaps in support, and influences the adherence to regulatory requirements. The aim of this study was to evaluate changes in health perception among patients with aortic diseases during the first and second wave of the COVID-19 pandemic in Germany. Methods Patients (n=262) diagnosed with aortic disease participated in telephone interviews during the first (w1, April 6–April 29, 2020) and second wave (w2, January 11–January 29, 2021) of the pandemic in Germany. The perception of COVID-19 as a threat was examined using relevant items of the Brief Illness Perception (BIP) questionnaire to address four dimensions (consequences, control, personal control, and understanding). Relevant data focusing on different aortic diseases and cardiovascular risk factors were taken from patient records. Results Aortic diseases included mainly aortic aneurysm of the ascending (n=164, 62.6%) and the descending aorta (n=37, 14.1%). Patients with acute or chronic aortic dissection made up a third (n=41, 15.6%, and n=48, 18.3%, respectively). At baseline (w1), none of the participants had neither been quarantined nor were taken ill with COVID-19. At the second survey (w2), 24 participants (n=252, 9.5%) had already been quarantined and five (n=252, 2%) were diagnosed with COVID-19. The BIP score increased 9.18 (SD=7.132) to 14.58 (SD = 6.956) between w1 and w2 (p<.001). At the level of dimensions, that meant a significant difference between w1 and w2 regarding “consequences” (M=−2.821, SD=3.049, 95% CI [−3.200, −2.443], t(251)=−14.691, p<.001, d=0.92), “control” (M=0.908, SD=2.492, 95% CI [0.589, 1.218], t(249)=5.760, p<.001, d=0.36), and “concern” (M=−1.669, SD=3.349, 95% CI [−2.086, −1.253], t(250)=−7.898, p<0.001, d=0.50). Only “understanding” showed no significant difference (M=−0.032, SD=1.520, 95% CI [−0.220, 0.157], t(251)=−0.332, p=0.740). Conclusion Although patients with aortic diseases are highly at risk of having worse outcomes from COVID-19, their overall perception of COVID-19 as a threat was low in the beginning, but rising during the second wave. The main reasons were the increased effects on personal life and elevated concerns about the COVID-19 pandemic, but concerns did not include the educational aspect of COVID-19. Tailored risk communication strengthens the mental health of people in a public health crisis and ensures the success of governmental guidelines and policies. FUNDunding Acknowledgement Type of funding sources: None. Course of COVID-19 pandemic in Germany

Sex Differences in Proximal Thoracic Aortic Disease Pathology: A Call to Action

Introduction/Objective Sex disparity is reported across all forms of cardiovascular diseases. Only few studies have focused on sex differences in thoracic aortic disease pathology. We aim to identify and understand sex differences in this patient group to bridge the knowledge gap and improve clinicopathologic outcomes. Methods/Case Report This is a retrospective analysis of 83 proximal thoracic aortic aneurysm and dissection (TAAD) cases treated at a single quaternary care center in 2019. Chart review was done for demographics. Consensus criteria (Stone JR et al. Cardiovasc Pathol 2015; 24:267-78; Halushka MK et al. Cardiovasc Pathol 2016; 25:247-57) and a scoring system (Waters KM et al. Cardiovasc Pathol 2017; 30:6-11) were used for pathology reporting. Clinical correlation was also made. Pearson’s chi-square test was used for statistical analysis. Results (if a Case Study enter NA) 83 patients (61 male and 22 female) were retrieved. Overall thoracic aortopathy was higher among males, accounting for 73.4% of individuals with TAAD. In a subgroup analysis, there was no sex difference in dissection, aortic root involvement, and bicuspid aortic valve (p>0.05). Genetic aortopathy was more prevalent in females than males (27.2% vs 9.8%, p=0.04) alongside early age at first aortic event (median age: 31y vs 52y). Histopathologically, females had frequent translamellar mucoid extracellular matrix accumulation (45.4% vs 22.9%, p=0.04), extensive (54.5% vs 27.8%, p=0.02) and severe (59% vs 34.4%, p=0.04) elastic fiber fragmentation, higher band like (9% vs 6.5%, p>0.05) plus extensive (13.6% vs 4.9%, p>0.05) smooth muscle nuclei loss, and extensive (13.6% vs 1.6%, p=0.01) plus dense (4.5% vs 1.6%, p>0.05) laminar medial collapse than males. Conclusion In our patient population, females have a lower prevalence of thoracic aortic disease treated with open repair. However, those who develop TAAD harbor a greater burden of wall pathology and probable worse outcomes. We recommend sex-based analysis of all research on thoracic aortic diseases.