Aortic Root Dilatation in Children and Adolescents At Al-Hawary General Hospital, & National Benghazi Cardiac Center -Libya

Isolated dilatation of the aortic root and/or ascending aorta is a rare but well-known cardiovascular manifestation, can be caused by a variety of congenital or acquired conditions; that lead to the weakening of the aortic wall. The study aimed to detect the cause and the rate of the aortic root dilatation in children and adolescents, and to assess the effect of the Beta-adrenergic blockers in preventing further dilatation in the aortic root. A case series study was perform with five years of follow-up at Al-Hawary General Hospital, National Benghazi Cardiac Center. A total of 91 patients were seen with ascending aortic dilatation and/or root dilatation during the period from 6/2016 - 6/2021 included in the study diagnosed by clinical examination, chest x-ray, and echocardiogram. The diagnosis in 34/91(37%) was Tetralogy of fallout (TOF) and truncus arteriosus, 57/91 (63%) was dilated aortic root, 25/57 (44%) bicuspid aortic valve (BAV), 22/57 (38.5%) Marfan syndrome, 4/57(7%) Noonan syndrome, 2/57(3.5%) Turner syndrome, 3/57(5%) Ehlers-Danlos syndrome, 1/57(2%) idiopathic. Follow-up results of three months – five years: 57/91 patients with aortic root dilatation were followed up, none of the Marfan syndrome and Ehlers-Danlos syndrome patients who received beta-blockers had shown progression in the dilatation of the aortic root, and all patients who had bicuspid aortic valve did not show any progression in the dilatation without using medication. Conclusions: Dilated aortic root is a common finding in Marfan syndrome, bicuspid aortic root, and Ehlers-Danlos syndrome, and its progress could be decreased by using beta-adrenergic blockers in rapidly progressing dilation.

Aortic Root Dimensions and Pulse Wave Velocity in Young Competitive Athletes

The assessment of aortic root dimensions is a cornerstone in cardiac pre-participation screening as dilation can result in severe cardiac events. Moreover, it can be a hint for an underlying connective tissue disease, which needs individualized sports counseling. This study examines the prevalence of aortic root dilatation in a cohort and its relationship to arterial stiffness as an early marker of cardiovascular risk due to vascular aging. From May 2012 to March 2018, we examined 281 young male athletes (14.7 ± 2.1 years) for their aortic root dimension. Moreover, we noninvasively assessed arterial stiffness parameter during pre-participation screening. Mean aortic diameter was 25.9 ± 3.1 mm and 18 of the 281 (6.4%) athletes had aortic root dilation without other clinical evidence of connective tissue disease. After adjusting for BSA, there was no association of aortic root diameter to pulse wave velocity (p = −0.054 r = 0.368) nor to central blood pressure (p = −0.029 r = 0.634). Thus, although a significant proportion of young athletes had aortic root dilatation, which certainly needs regular follow up, no correlation with arterial stiffness was found. It could be suggested that a dilated aortic root in young athletes does not alter pulse waveform and pulse reflection, and thus there is no increased cardiovascular risk in those subjects.

Quadricuspid aortic valve and a large patent ductus arteriosus treated with device closure

Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Prevalence and outcome of extra-cardiac findings in patients referred for cardiac computed tomography angiography

Background Cardiac computed tomography angiography (CCTA) includes several extra-cardiac structures. A variety of abnormalities may be clinically important and, in some cases, explain the patient's symptoms. The incidence of extra cardiac findings (ECFs) on CCTA examination ranges between 7% and 56%, with the true prevalence likely affected by patient selection. Methods Cardiac CT scans with suspected coronary disease were prospectively and independently reviewed by a radiologist for the presence of ECFs. Data was collected from a prospectively collated database of all patients who underwent a CCTA scans between June 2011 and October 2015. Patients were followed up for one year after incidental findings were made. Results A total of 635 patients had a CCTA scan during the study period. Of these 325 (51.2%) were male, mean age was 66 [22–88] years, with a mean BMI of 27 [17–56]. Extra-cardiac findings were reported in 126 (19.8%) of these 62 (49%) were classified as clinically important and had further imaging and follow-up. The most common abnormalities were pulmonary nodules 61 (46.8%), Hiatus hernia 15 (12%), lymphadenopathy 12 (10%) and liver cysts 10 (8%), Figure 1a. Malignant tumours were identified in 2 (1.6%) patients and one (0.8%) had both aortic root dilatation and type B aortic dissection. Conclusion Non-cardiac abnormalities in patients undergoing CCTA provides useful information e.g. early diagnosis of lung malignancies and other life threatening conditions in up to 40% of patients. FUNDunding Acknowledgement Type of funding sources: None.

Aortic Root Dilatation Is Attenuated with Diabetes but Is Not Associated with Renal Progression in Chronic Kidney Disease

Patients with chronic kidney disease (CKD) often have cardiac functional and structural abnormalities which can lead to adverse cardiovascular outcomes. In this study, we investigated associations between diabetes mellitus (DM) and cardiac functional and structural parameters in patients with CKD focusing on aortic root diameter (ARD). We also investigated associations of renal outcomes with DM and cardiac functional and structural characteristics. We enrolled 419 patients with CKD stage 3–5 were enrolled. ARD was normalized to body surface area (BSA) (ARD/BSA), and the rate of decline in renal function was assessed by the estimated glomerular filtration rate (eGFR) slope (mL/min/1.73 m2/year). ARD/BSA ≥2.1 cm/m2 in men or ≥2.2 cm/m2 in women was defined as indicating aortic root dilatation. The patients with DM had lower ARD/BSA, higher left atrial dimension (LAD), lower left ventricular ejection fraction, lower ratio of peak early transmitral filling wave velocity to peak late transmitral filling wave velocity, and higher left ventricular relative wall thickness, than those without DM. After multivariable analysis, DM (vs. non-DM; coefficient β, −0.060; p = 0.018) was significantly associated with low ARD/BSA. Significantly fewer patients with DM had aortic root dilatation compared to those without DM (14.3% vs. 23.1%, p = 0.022). In the patients with DM, there were significant associations between a high left ventricular mass index (LVMI) (per 1 g/m2, β, −0.016; p = 0.040) and high LAD (per 1 cm; β, −1.965; p < 0.001) with a low eGFR slope. However, other parameters, including ARD/BSA, were not associated with eGFR slope. Furthermore, there were no associations between eGFR slope and any of the echocardiographic parameters in the patients without DM. Aortic root dilatation was attenuated in the patients with DM, but it was not associated with a decline in renal function. However, high LAD and LVMI were associated with rapid renal function decline in the CKD patients with DM.

Effect of Valve Height on the Opening and Closing Performance of the Aortic Valve Under Aortic Root Dilatation

Patients with aortic valve disease can suffer from valve insufficiency after valve repair surgery due to aortic root dilatation. The paper investigates the effect of valve height (Hv) on the aortic valve opening and closing in order to select the appropriate range of Hv for smoother blood flow through the aortic valve and valve closure completely in the case of continuous aortic root dilatation. A total of 20 parameterized three-dimensional models of the aortic root were constructed following clinical surgical guidance. Aortic annulus diameter (DAA) was separately set to 26, 27, 28, 29, and 30 mm to simulate aortic root dilatation. HV value was separately set to 13.5, 14, 14.5, and 15 mm to simulate aortic valve alterations in surgery. Time-varying pressure loads were applied to the valve, vessel wall of the ascending aorta, and left ventricle. Then, finite element analysis software was employed to simulate the movement and mechanics of the aortic root. The feasible design range of the valve size was evaluated using maximum stress, geometric orifice area (GOA), and leaflet contact force. The results show that the valve was incompletely closed when HV was 13.5 mm and DAA was 29 or 30 mm. The GOA of the valve was small when HV was 15 mm and DAA was 26 or 27 mm. The corresponding values of the other models were within the normal range. Compared with the model with an HV of 14 mm, the model with an HV of 14.5 mm could effectively reduce maximum stress and had relatively larger GOA and less change in contact force. As a result, valve height affects the performance of aortic valve opening and closing. Smaller HV is adapted to smaller DAA and vice versa. When HV is 14.5 mm, the valve is well adapted to the dilatation of the aortic root to enhance repair durability. Therefore, more attention should be paid to HV in surgical planning.

Personalised external aortic root support for elective treatment of aortic root dilation in 200 patients

Background and objectivesIn personalised external aortic root support (PEARS), a custom-made, macroporous mesh is used to stabilise a dilated aortic root and prevent dissection, primarily in patients with genetically driven aortopathies. Data are needed on the safety and postoperative incidence of aortic events.MethodsWe present a multicentre cohort study evaluating the first 200 consecutive patients (median age 33 years) undergoing surgery with an intention to perform PEARS for aortic root dilatation in 23 centres between 2004 and 2019. Perioperative outcomes were collected prospectively while clinical follow-up was retrieved retrospectively. Median follow-up was 21.2 months.ResultsThe main indication was Marfan syndrome (73.5%) and the most frequent concomitant procedure was mitral valve repair (10%). An intervention for myocardial ischaemia or coronary injury was needed in 11 patients, 1 case resulting in perioperative death. No ascending aortic dissections were observed in 596 documented postoperative patient years. Late reoperation was performed in 3 patients for operator failure to achieve complete mesh coverage. Among patients with at least mild aortic regurgitation (AR) preoperatively, 68% had no or trivial AR at follow-up.ConclusionsThis study represents the clinical history of the first 200 patients to undergo PEARS. To date, aortic dissection has not been observed in the restrained part of the aorta, yet long-term follow-up is needed to confirm the potential of PEARS to prevent dissection. While operative mortality is low, the reported coronary complications reflect the learning curve of aortic root surgery in patients with connective tissue disease. PEARS may stabilise or reduce aortic regurgitation.

Root Dilatation Is More Malignant Than Ascending Aortic Dilation

Background Data from the International Registry of Acute Aortic Dissection indicate that the guideline criterion of 5.5 cm for ascending aortic intervention misses many dissections occurring at smaller dimensions. Furthermore, studies of natural behavior have generally treated the aortic root and the ascending aorta as 1 unit despite embryological, anatomical, and functional differences. This study aims to disentangle the natural histories of the aforementioned aortic segments, allowing natural behavior to define specific intervention criteria for root and ascending segments of the aorta. Methods and Results Diameters of the aortic root and mid‐ascending segment were measured separately. Long‐term complications (dissection, rupture, and death) were analyzed retrospectively for 1162 patients with ascending thoracic aortic aneurysm. Cox regression analysis suggested that aortic root dilatation ( P =0.017) is more significant in predicting adverse events than mid‐ascending aortic dilatation ( P =0.087). Short stature posed as a serious risk factor. The dedicated risk curves for the aortic root and the mid‐ascending aorta revealed hinge points at 5.0 and 5.25 cm, respectively. Conclusions The natural histories of the aortic root and mid‐ascending aorta are uniquely different. Dilation of the aortic root imparts a significant higher risk of adverse events. A diameter shift for intervention to 5.0 cm for the aortic root and to 5.25 cm for the mid‐ascending aorta should be considered at expert centers.