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Background Cardiovascular magnetic resonance (CMR) is considered the method of choice for evaluation of aortic root dilatation in congenital heart disease. Usually, a cross-sectional 2D cine stack is acquired perpendicular to the vessel’s axis. However, this method requires a considerable patient collaboration and precise planning of image planes. The present study compares a recently introduced 3D self-navigated free-breathing high-resolution whole heart CMR sequence (3D self nav) allowing a multiplanar retrospective reconstruction of the aortic root as an alternative to the 2D cine technique for determination of aortic root diameters. Methods A total of 6 cusp-commissure (CuCo) and cusp-cusp (CuCu) enddiastolic diameters were measured by two observers on 2D cine and 3D self nav cross-sectional planes of the aortic root acquired on a 1.5 T CMR scanner. Asymmetry of the aortic root was evaluated by the ratio of the minimal to the maximum 3D self nav CuCu diameter. CuCu diameters were compared to standard transthoracic echocardiographic (TTE) aortic root diameters. Results Sixty-five exams in 58 patients (32 ± 15 years) were included. Typically, 2D cine and 3D self nav spatial resolution was 1.1–1.52 × 4.5-7 mm and 0.9–1.153 mm, respectively. 3D self nav yielded larger maximum diameters than 2D cine: CuCo 37.2 ± 6.4 vs. 36.2 ± 7.0 mm (p = 0.006), CuCu 39.7 ± 6.3 vs. 38.5 ± 6.5 mm (p Conclusion Both, the 3D self nav and 2D cine CMR techniques allow reliable determination of aortic root diameters. However, we propose to privilege the 3D self nav technique and measurement of CuCu diameters to avoid underestimation of the maximum diameter, particularly in patients with asymmetric aortic roots and/or BAV.

Proximal aortic enlargement is associated with an increased risk of heart failure and all-cause mortality. Recently, aortic root dilatation (ARD) was reported in postoperative patients with ventricular septal defects (VSDs). However, the impact of ARD on left ventricular (LV) function in postoperative VSD patients remains unclear. Thus, the aim of this study was to investigate the effect of ARD on LV function in patients with postoperative VSD. One hundred and thirty-five patients (> 15 years of age) with surgically repaired isolated ventricular defects and who underwent transthoracic echocardiography in our institution between 2009 and 2013 were identified. ARD was defined as an observed aortic root diameter/body surface area > 2.1 cm/m2. The propensity score estimating the probability of having ARD adjusted for anatomical and clinical characteristics was calculated. Forty-four patients (32.6%) had ARD. In unadjusted analyses, right ventricular systolic pressure, Tei index, and E/e’ were significantly (p

The pathophysiology of aortic diseases in Tetralogy of Fallot has been analyzed in various studies, focusing on the histopathology of the aortic root, altered hemodynamics, and effect of early corrective repair on the incidence of aortic root dilatation. In this review article, we sought to integrate these studies for a better understanding of the natural history and practical implications of aortic root dilatation in Tetralogy of Fallot. This better equips us to appropriately implement potentially effective interventions.

Marfan syndrome (MFS) is a connective tissue disorder characterized by a broad range of clinical manifestations. Cardiovascular involvement is the most life-threatening aspect of the syndrome. Although abnormalities within the cardiovascular system in adults are well documented, there is still a paucity of data regarding manifestation of MFS in childhood. The aim of the study was to compare cardiovascular manifestation of MFS between children and adults. The study population consisted of 236 patients (144 children and 92 adults), who were referred to our department with suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 101 (44 children and 57 adults) out of the 236 patients. The other patients were diagnosed with Ehlers-Danlos syndrome, Loeys-Dietz syndrome, MASS phenotype, ectopia lentis syndrome, marfanoid habitus and other rare syndromes. The most common cardiovascular abnormality was aortic root dilatation (81.19% of patients). It was found that both adults and children had similar high rates of aortic root dilatation. Similarly, there was no significant difference with regard to the prevalence of aortic valve regurgitation and mitral valve prolapse among children and adults. These findings equivocally indicate that the aforementioned abnormalities develop in early childhood, therefore, they may be used in the early identification of patients with MFS. Other assessed abnormalities, which included mitral valve regurgitation, pulmonary artery dilation, aneurysms of aortic arch, descending thoracic aorta and abdominal aorta were found mostly in adults, and thus, are of less use in the early detection of MFS.

Mucopolysaccharidoses (MPS) are disorders characterized by impaired glycosaminoglycan (GAG) catabolism as a consequence of a deficiency or the absence of lysosomal enzymes directly involved in their degradation. Multiple organ systems are involved in MPS, including the cardiovascular system. Recently, aortic root dilatation (ARD) has been described in these patients. Thus, we reviewed aortic root diameter measurements in 69 MPS patients from a single center from 2000 to 2016. Aortic root diameter z scores were calculated based on data published by Colan et al. according to the body surface area (BSA) determined using the Haycock formula. The overall incidence of ARD in MPS patients was 39.1%. Higher mean z scores were present in patients with MPS IVA and VI when compared to MPS I and II. Aortic root z scores were higher in older MPS IVA patients, which may suggest a progressive ARD change in this MPS type. No significant differences were found before and after enzyme replacement therapy (ERT) in 11 patients with available data (2 with MPS I; 4 with MPS II; 2 with MPS IVA, and 3 with MPS VI). This work provides further evidence that ARD is common in different types of MPS, being especially evident in MPS IVA, but with a significant occurrence also in MPS VI.

The article Correlation Between Total Repair Timing and Late Aortic Root Dilatation in Repaired Tetralogy of Fallot, written by Hyung Tae Sim, Jeong-Won Kim, Seong Ho Kim, Su-Jin Park, So-Ick Jang, and Chang-Ha Lee, was originally published electronically on the publisher’s internet portal (currently SpringerLink) on July 08, 2020 with open access. With the author(s)’ decision to step back from Open Choice, the copyright of the article was changed on August 2020 to © Springer Science+Business Media, LLC, part of Springer Nature 2020 and the article is forthwith distributed under the terms of copyright.

Annuloplasty is considered to play a key role to control aortic valve regurgitation (AR) and prevent recurrence of AR after aortic valve repair, because aortic root dilatation has emerged as a risk factor for recurrence of AR. Various modifications of annuloplasty have been advocated, however, none of them has become standardized. Thus in this review they are outlined and classified (internal/external, with/without ring, rigid/flexible ring), and their advantages and disadvantages are clarified. Their clinical outcomes seem currently acceptable in general, and external flexible annuloplasty has been performed more frequently with favorable outcomes. However, they are still performed for a minority of patients by special experienced teams with limited follow-up periods. Therefore, it seems too early to determine the superiority or inferiority of each approach. We must carefully conduct evaluation to clarify which approach will become reproducible, effective, and standardized.

Accumulating data suggest that activity of matrix metalloproteinases (MMPs) is increased in aortic dissection, and in thoracic and abdominal aneurysms. In the present study we sought to determine circulating stromelysin (MMP-3) concentration and its relationship with hypertension-induced aortic root dilatation. The study population included 42 patients with essential hypertension. The subjects were divided into two groups according to echocardiographically measured aortic diameter as those with aortic dilatation (n = 22) and without aortic root dilatation (n = 20). Plasma concentration of MMP-3 was determined by one-step sandwich enzyme immunoassay (EIA) method and compared in both groups. Baseline demographic properties were similar in both groups. Plasma stromelysin (MMP-3) level was significantly higher in patients with aortic dilatation than those without aortic dilatation (5.2 +/- 2.3 vs 3.3 +/- 1.9 ng/ml; P = 0.007). In conclusion, we found that circulating stromelysin (MMP-3) concentration was elevated in hypertension-induced aortic root dilatation.

Due to cardiovascular and skeletal abnormalities, anesthetic management of parturients with Marfan's syndrome can be particularly challenging. Parturients with aortic root dilatation are at risk for aortic dissection. We describe the anesthetic management of a parturient with Marfan's syndrome and aortic root dilatation, who required general anesthesia for Cesarean delivery.At 26 weeks gestation, a nulliparous woman with Marfan's syndrome presented to the Anesthesia Clinic. Her history revealed asymptomatic aortic root dilatation of 41 mm, and partial correction of scoliosis with Harrington rods. Her cardiologist advised metoprolol, serial echocardiograms, and Cesarean delivery to decrease the risk of aortic dissection. At a multidisciplinary conference, a decision was made to proceed with Cesarean delivery, at term, at the cardiac surgery centre. After placement of arterial and central lines, general anesthesia was induced with remifentanil, propofol, and succinylcholine. Anesthesia was maintained with N(2)O, sevoflurane, and remifentanil (0.02-0.08 microg x kg(-1) x min(-1)). Transesophageal echocardiography examination confirmed stable aortic root dilatation. The patient remained hemodynamically stable. The baby's Apgars were 4 and 8, at one and five minutes, respectively. At the end of the procedure, the patient's trachea was extubated when she was awake. Initial postoperative care was in the intensive care unit. Both mother and baby recovered uneventfully.Peripartum hemodynamic changes can be life-threatening to the parturient with Marfan's syndrome and aortic dilatation. Anesthetic goals for delivery included preparation for possible aortic dissection, and avoidance of increased aortic root shear stress, through careful hemodynamic monitoring, and general anesthesia using remifentanil.

The objectives of this study were to examine the relative contributions of development and hemodynamics in aortic root dilatation of tetralogy of Fallot, to assess the impact of systemic to pulmonary artery shunt on aortic annular size, and to seek any relationship between the timing of corrective surgery and subsequent aortic root size. We performed a retrospective analytical study at a tertiary referral center of M-mode and two-dimensionol aortic root measurements in children with tetralogy of Fallot prior to any surgical or palliative intervention, after insertion of a surgical shunt, and on intermediate and long-term follow-up post-repair. The main outcome measures were aortic root diameter prior to correction, after palliative shunt insertion, and after definitive repair. The study found that the aortic root is enlarged in unrepaired tetralogy of Fallot irrespective of age, increased in the first 3-6 months after palliative shunt surgery, and persisted into adulthood in those repaired after 1 year of age. Age-corrected aortic root dimensions normalized by mid-childhood in those who were repaired in infancy. Preexisting aortic root dilatation normalizes by 7 years of age in patients with tetralogy of Fallot who have been repaired in infancy, whereas it persists into adulthood in the group repaired post-infancy.

The objective of this study was to determine if calculation of the sinus of Valsalva to annulus ratio (SOV/Ann) effectively screens for root dilatation (ARD) during two-dimensional (2D) echocardiography in young patients (1 month-15 years) with suspected or confirmed Marfan syndrome. The aortic root was imaged using 2D echocardiography (parasternal long-axis view) and the SOV/Ann ratio calculated/analyzed in 27 Marfan patients and 45 age-matched controls. All controls had a SOV/Ann ratio

Cardiovascular involvement occurring in 20-30% patients is the second most common cause of mortality in patients with relapsing polychondritis. Aortic insufficiency occurs as a result of aortic root dilatation rather than primary valvular involvement. We are reporting a patient of relapsing polychondritis with aortic root dilatation, in whom institution of early and aggressive therapy successfully prevented the progression of aortic insufficiency.