Sinus Of Valsalva Aneurysm Research Articles

New-onset aortic dilatation in the population: a quarter-century follow-up.

Aortic size tends to increase with aging but the extent of this dynamic process has not been evaluated in long-term longitudinal population-based studies. We investigated the incidence of new-onset aortic root (AR) dilatation and its principal correlates among middle-aged adults over a 25-year time period. A total of 471 participants with measurable echocardiographic parameters at baseline and after a 25-year follow-up were included in the analysis. Sex-specific upper limits of normality for absolute AR diameter, AR diameter indexed to body surface area (BSA) and to height were derived from healthy normotensive PAMELA participants. New AR dilatation occurred in 7.4% (AR/BSA), 9.1% (AR/height) and 14.6% (absolute AR), respectively. According to the AR/height index, the risk of new dilation was similar in men and women. As for echocardiographic parameters, baseline AR diameter emerged as a key predictor of AR dilation, regardless of the diagnostic criteria and the 10-year change in LVMI was positively associated to new AR/height dilatation. No significant relationship was observed between baseline office and ambulatory systolic/diastolic blood pressure or their changes over time with incident AR dilatation. Baseline and the 25-year change in 24-h pulse pressure were negatively related to new AR dilatation. The incidence of AR dilatation from mid to late adulthood occurs in a small but clinically relevant fraction of participants and is unaffected by both office and out-office BP. It is significant related to baseline AR diameter and to the 25-year change in LVMI. Our data suggest that echocardiography performed in middle-aged individuals of both sexes may identify those at increased risk of future AR dilatation; moreover, preventing LVH may reduce the risk of progressive AR enlargement.

Imaging Evaluation of Kawasaki Disease.

This review provides the summary of the appropriate use of these modalities when caring for patients with Kawasaki disease at diagnosis and for long-term management. Kawasaki disease is an inflammatory syndrome of unknown etiology that can result in coronary artery dilations or aneurysms if left untreated in 25% of the patients and 3-5% in treated patients. In addition to coronary artery aneurysms, patients can have ventricular dysfunction, valvular regurgitation, aortic root dilation, and pericardial effusion due to inflammation of the myocardium. Noninvasive imaging modalities are important to these assessments. Echocardiography is the first-line noninvasive evaluation of coronaries and function. Cardiac magnetic resonance imaging is useful for functional assessment in long-term follow-up. Distal coronaries, thromboses, and stenoses are best evaluated by cardiac computed tomography. Future research should demonstrate the effectiveness of advanced functional imaging in patients with Kawasaki disease and decreased radiation from cardiac computed tomography.

Predictors and Rate of Progression of Aortic Root and Ascending Aorta Dilatation

In the absence of risk factors like bicuspid aortic valve, connective tissue disorder, or family history of aortic dissections, degenerative thoracic aortic aneurysm appears to be an indolent disease. Most American and European societies recommend yearly or biannual imaging of the thoracic aorta with computed tomographic (CT) imaging, magnetic resonance (MRI) imaging, and transthoracic echocardiographic (TTE) examination. We aimed to identify the rate of progression and predictors of early degenerative aortic root dilatation (ARD) and ascending aortic dilatation (AAD) over a period of 10 years on the basis of echocardiographic measurements. A retrospective chart analysis was performed on 340 patients (mean age 67.4 ± 11.6 years; 85.6% men; 83.8% White) with known ARD and AAD. Aortic root and ascending aorta measurements were followed by serial echocardiograms from the time of the first diagnosis for a total of 10 years. During this time, the mean change in ARD was 0.28 ± 0.71 mm and AAD was 0.15 ± 0.18 mm. On multivariate regression after adjusting for baseline demographics, risk factors, and medication use, there was no statistically significant increase in their unit change in mean ARD or AAD. In conclusion, mild to moderate degenerative thoracic aortic aneurysm has a minimal change in dimensions over time, and current guidelines recommending yearly surveillance imaging of ARD and AAD need to be revisited to allow a more liberal follow-up interval.

Traboulsi syndrome caused by mutations in ASPH: An autosomal recessive disorder with overlapping features of Marfan syndrome

Traboulsi syndrome, otherwise known as facial dysmorphism, lens dislocation, anterior-segment abnormalities and spontaneous filtering blebs, is an autosomal recessive condition associated with characteristic ocular features including dislocated crystalline lenses, anterior segment abnormalities and in some individuals, non-traumatic conjunctival cysts. There is a distinctive facial appearance which includes flattened malar region with convex nasal ridge. Alterations in the aspartate beta-hydroxylase (ASPH) gene are known to be the cause of the condition.We report seven further individuals from six unrelated families with characteristic ocular and facial features. Five individuals had aortic root dilatation, with childhood onset in some, and one undergoing aortic root repair aged 47 years for severe aortic regurgitation and aortic root dilatation. Interestingly, inguinal hernias were commonly reported. Although some skeletal features were seen, these were not consistent. One of the patients had mild deficiency of factor VII on clotting studies. The ASPH protein hydroxylates specific asparagine- and aspartate-residues in epidermal growth factor (EGF)-domain containing proteins including coagulation factors and associated genes including FBN1. We propose this as an explanation for the overlap in clinical features with Marfan syndrome and conclude that Traboulsi syndrome is an important differential diagnosis. We strongly recommend echocardiography surveillance for patients with Traboulsi syndrome.

Pediatric Heart Network Trial of Losartan vs. Atenolol in Children and Young Adults with Marfan Syndrome: Impact on Prescription Practices.

The Pediatric Heart Network (PHN) trial showed similar efficacy of β-blockers (BB) and angiotensin receptor blockers (ARB) for aortic root dilation in Marfan syndrome, but the impact on prescription practices is unknown. We hypothesized BB and ARB prescriptions would increase after the trial results were published (2014). Prescription data (2007-2016) were obtained from outpatient encounters (IBM Marketscan) for Marfan syndrome patients (6months-25years old). Excluding 2014 as a washout period, we analyzed two intervals: 2007-2013 and 2015-2016. Medication categories included BB, ARB, angiotensin converting enzyme inhibitors (ACEI), combination (BB/ARB and/or BB/ACEI), and no drug. Interrupted time-series analysis assessed immediate level change after publication and change in slope for the trend pre- and post-publication. Odds ratios (OR) and 95% confidence intervals from logistic regressions and generalized estimating equation methods accounted for correlation of prescriptions within patients. In 1499 patients (age 14.1 ± 6.1years, 59% female) seen 2007-2013, BB trended lower [OR 0.91 (0.89, 0.93), p < 0.001] and ARB trended higher [OR 1.12 (1.07, 1.18), p < 0.001], while combination, ACEI, and no drug remained stable. This trend persisted, but was not significant, for BB [OR 0.54 (0.27, 1.08), p = 0.37] and ARB [OR 1.91 (0.55, 6.69), p = 0.31] in 2015-2016. Combination, ACEI, and no drug remained similar. In short term follow-up, changes in prescription practices following publication of the PHN trial were not statistically significant. This may be due to a change seen prior to publication with early adoption of ARBs that was maintained after confirmation of their effectiveness.

Platypnea-Orthodeoxia Syndrome in the Setting of Patent Foramen Ovale Without Pulmonary Hypertension or Major Lung Disease.

BackgroundPatent foramen ovale (PFO)‐associated platypnea‐orthodeoxia syndrome is characterized by dyspnea and hypoxemia when upright. The pathogenesis is thought to involve an increase in right atrial pressure or change in degree of right to left shunting with upright posture.Methods and ResultsWe sought to characterize patients with platypnea‐orthodeoxia syndrome related to PFO without pulmonary hypertension. We retrospectively reviewed databases at 3 tertiary referral hospitals in New South Wales, Australia from 2000 to 2019. Fourteen patients with a mean age of 69±14 years had a PFO with wide tunnel separation. Mean New York Heart Association Classification was II (±0.9) and 7 inpatients had been confined to bed (from postural symptoms). Baseline oxygen saturations supine were 93%±5% and 84%±6% upright. Two patients had a minor congenital heart defect and 4 had mild parenchymal lung disease with preserved lung function. The mean aortic root diameter was 37±6 mm and distance between aortic root and posterior atrial wall was 16±2 mm. Platypnea‐orthodeoxia syndrome was preceded by surgery in 5 patients and 1 patient had mild pneumonia. Successful closure of the PFO using an Amplatzer device was performed in 11 of 14 patients. Post‐closure, all patients had New York Heart Association Classification I (improvement 1.6±0.9, P<0.003) and semi‐recumbent oxygen saturations increased by 13%±8% (P<0.001, n=10).ConclusionsPlatypnea‐orthodeoxia syndrome is a debilitating condition, curable by PFO closure. Anatomical distortion of the atrial septum related to a dilated aortic root or shortening of the distance between the aortic root and posterior atrial wall may contribute to the syndrome.

Surgical Repair of a Sinus of Valsalva Aneurysm.

A surgically challenging case of an unruptured Sinus of Valsalva aneurysm (SoVA) with severe aortic regurgitation (AR) due to cusp prolapse is presented. Sinus reconstruction with a patch cut out from the sinus portion of a Gelweave Valsalva graft (Terumo Vascutek) was performed. Intraoperative measurements showed insufficient effective height of the right coronary cusp; therefore, cusp plication and pericardial patch augmentation of the right coronary cusp were performed with satisfying result.

Incidence and risk factors for late complications after the arterial switch operation.

The purpose of this study was to evaluate our institution's 16-year arterial switch operation (ASO)experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention. The clinical data of 185 transposition of the great arteries (TGA)patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study. There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR;in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristicsanalysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation. Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures.

Outcomes of one-staged procedures to treat aortic coarctation complicated by cardiac anomalies

ObjectiveOne-staged surgical treatment of aortic coarctation combined with cardiac anomalies is challenging. We aim to evaluate the feasibility of bilateral aortofemoral bypass technique in one-staged surgery treating coractation by comparing surgical outcomes with catheter intervention plus stent (hybrid).MethodsBetween January 2012 and December 2017, 50 patients underwent one-staged surgical procedures to treat coarctation and repair concomitant cardiac anomalies, like aortic root dilatation, cardiac valvular disease and so on. Among them, 30 patients underwent bilateral aortofemoral bypass and 20 patients underwent hybrid procedure to treat coarctation. We retrospectively analyzed the data of these patients and compared the early and late results.ResultsAll the baseline clinical characteristics were comparable between groups except that the mean age of bypass group was 39.5 ± 14.0 years which was older than hybrid group (27.9 ± 8.5 years, P = 0.002). Technical success was achieved in all patients, with no hospital death or other severe complications. Immediately after surgery, in bypass and hybrid group, the mean upper-limb systolic blood pressure decreased from 159.4 to 119.7 mmHg and 148.4 to 111.6 mmHg, the median peak systolic gradient decreased from 68.0 to 10 mmHg and 46.5 to 10 mmHg respectively (P = 0.09). And the mean upper-lower limbs gradient decreased from 21.7 to 5.9 mmHg and 21.0 to 2.7 mmHg respectively (P = 0.104). The mean follow-up time was 76.92 ± 18.7 in bypass group and 85.4 ± 20.6 months in hybrid group. There were 4 late deaths in bypass group (one died of gastrointestinal bleeding, one died of pulmonary embolism and the other two died of heart failure caused by mechanical prosthetic valve dysfunction). The follow-up peak systolic gradient and other blood pressure parameters showed stable and no differences between two groups.ConclusionsThe bilateral aortofemoral bypass surgery is a safe and effective method which can be used in one-staged surgical strategy to treat coarctation complicated by cardiac anomalies and can be an alternative to the hybrid method.

Collaborative advanced nurse practitioner (ANP) and senior cardiac physiologist (SCP) ECHO use in a community chest pain clinic: an audit of case management and clinical outcomes.

Abstract Funding Acknowledgements Type of funding sources: None. Background In the primary care setting chest pain is a common presenting complaint with an over diagnosis/suspicion of stable angina, however General Practitioners (GPs) lack access to a cardiac specialist in a timely fashion, as a result 31% of all ED chest pain patients are referred to the emergency department. The Integrated community chest pain clinic (ICCPC) provides an alternative pathway for GPs to refer patients with non-acute chest pain. This clinic is advanced nurse practitioner (ANP) led in partnership with a senior cardiac physiologist (SCP) to provide cardiac diagnostics. ESC guidelines recommend echocardiography (ECHO) as a level 1b recommendation for patients with suspected coronary artery disease for the assessment of chest pain. Purpose The aim of this study is to assess referral to and impact of SCP provided ECHO in an integrated community chest pain clinic. Methods A retrospective clinical audit of all patients who were referred for ECHO over a 15-month period was performed. Clinical indication for referral, case management and final diagnosis were evaluated, approved by the hospital audit department. Results A total of 89 patients were referred by the ANP to the SCP for ECHO, (18%) of total patient cohort. All patients presented with chest pain and the clinical reason for referral included abnormal ECG n=25 (28%), hypertension n=24 (26%), dyspnoea n=19 (21%) or new murmur n=6 (7%). Other referral rational n= 15 (18%) included elevated NT-proBNP and long covid. Seventy percent of all cases were managed by collaborative autonomy between the ANP and SCP and discharged to the general practitioner. Collaboration with the Consultant Cardiologist was required for n=26 (30%) of cases. Of these n=4 were discharged. Twenty-two or 24% of the total number of ECHOs performed showed notable abnormalities requiring further management. This included dilated aortic root n= 6, valve disease n=8, one of which was severe requiring surgical valve replacement and regional wall motion abnormalities n=5. Three patients exhibited significant myocardial disease including Hypertrophic cardiomyopathy, Hypertrophic obstructive cardiomyopathy and Hypertrophic Cardiomyopathy with Severe Asymmetrical Hypertrophy. Discussion The integrated community chest pain clinic provides the patient with non-acute chest pain access to and advanced nurse practitioner and senior cardiac physiologist outside the acute setting. The provision of SCP led ECHO provides a timely and useful diagnostic tool as per ESC guidelines. Most patients post ECHO were managed by the ANP in collaboration with the cardiac physiologist. Chest pain is not always due to coronary heart disease and patients with significant myocardial and valve disease were diagnosed and promptly managed in the community setting. Advanced practice nursing and cardiac physiologist led care with access to ECHO has proven vital for this patient cohort.

Evaluation of Aortic Root Dilation in Adult Patients after Repair of Tetralogy of Fallot

Background and Aim: Aortic root dilation is one of the common complications in patients with a history of tetralogy of Fallot total correction (TFTC). We evaluate the frequency of aortic root dilation in adult TFTC patients and analyze probable risk factors related to the dilated aortic root. Materials and Methods and Results: We reviewed echocardiography images of 146 adult TFTC patients who admitted at Rajaie Cardiovascular Medical and Research Center from 1383 to 1399 in an observational retrospective cohort study. Sinus of Valsalva (SOV) was measured in parasternal long-axis view. Aortic root dilation was determined by two definitions. When we adjusted SOV diameter for body surface area and sex, the frequency of aortic root dilation was 68%, with a mean SOV diameter of 3.5 ± 0.7 cm and when we used absolute diameter ≥4 cm for dilated SOV, 22% showed aortic root dilation. By multivariate logistic regression analysis, male sex (odds ratio (OR) = 3.47, P = 0.003), age at the time of TFTC (OR = 1.06, P = 0.009), and aortic regurgitation (OR = 3.97, P = 0.003) were associated with increased adds of dilated aortic root. Three patients underwent aortic surgery, including one case of type A aortic dissection. Conclusion: Although aortic root dilation was common, aneurysmal dilation and adverse events were not so frequent. Serial evaluation of all segments of the ascending aorta, including the aortic root, is important but not more frequent than previously suggested.

Aortic Dissection Presenting as Shortness of Breath from Diffuse Alveolar Hemorrhage

BackgroundAortic dissection is a rare but well-known life-threatening disease that classically presents with tearing chest pain radiating to the back yet can have deceiving clinical presentations. Case ReportA 54-year-old man with a history of hypertension presented to the emergency department with mild shortness of breath without chest pain. Point-of-care ultrasound (POCUS) detected diffuse B-lines, a dilated aortic root, aortic regurgitation, and pericardial effusion. A computed tomography angiogram confirmed a Stanford type A aortic dissection with diffuse alveolar hemorrhage (DAH), a rare complication of type A aortic dissection involving the posterior aortic wall with extension into the main pulmonary artery. Why Should an Emergency Physician Be Aware of This?Acute aortic dissection can present with a wide range of clinical manifestations with a high mortality rate for patients with an untimely diagnosis. Although an intimal flap within the aortic lumen is the characteristic finding on ultrasound, additional POCUS findings of a pericardial effusion, aortic regurgitation, and a dilated aortic root may be seen with proximal dissections. Diffuse B-lines on thoracic POCUS, although commonly associated with pulmonary edema in decompensated heart failure, can be seen in patients with DAH which has a multitude of etiologies, including aortic dissection.

Gender differences in bicuspid aortic valve Sievers types, valvulopathy, aortopathy, and outcome of aortic valve replacement.

The gender difference of the bicuspid aortic valve (BAV) is not well understood. We evaluated the impact of gender on the Sievers types, valvulopathy, aortopathy, and outcomes of aortic valve replacement (AVR) of BAV patients in a cohort of Chinese patients. Among 992 BAV patients without aortic dissection nor congenital heart disease, 658 underwent AVR. The demography, Sievers types, valvulopathy, aortopathy, and outcomes of AVR were compared between genders. Aortic regurgitation (AR ≥ 2+) (39.0%vs. 12.8%, p<.001), aortic root dilation only (3.8%vs. .8%, p=.014), and diffuse dilation (25.3%vs. 4.3%, p<.001) were more common in men, while moderate to severe aortic stenosis (AS) (21.3% vs. 45.7%, p<.001) and ascending dilation only (46.2%vs. 61.2%, p<.001) were more common in women. Men were more prone to develop preoperative AR ≥ 2+ (OR=5.15, p<.001), moderate to severe AS + AR ≥ 2 + (OR=2.95, p=.001), and Diffuse aortic dilation (OR=3.91, p<.001). Sievers types did not have a significant effect on valvular dysfunction. Gender didn't predict early adverse events after AVR (n=90) (HR=1.21, p=.44), but male gender predicted a left ventricular ejection fraction <50% after AVR (OR=3.07, p=.03). In this BAV series of Chinese patients, gender didn't differ significantly in Sievers types of BAV but showed significant differences in valvulopathy, aortopathy, and LV function after AVR. In addition, the male patients developed more severe conditions at a younger age.

Distinct Contribution of Global and Regional Angiotensin II Type 1a Receptor Inactivation to Amelioration of Aortopathy in Tgfbr1 M318R/+ Mice.

Angiotensin II (Ang II) type 1 receptor (AT1R) signaling controls both physiological and pathogenetic responses in the vasculature. In mouse models of Loeys-Dietz syndrome (LDS), a hereditary disorder characterized by aggressive aortic aneurysms, treatment with angiotensin receptor blockers (ARBs) prevents aortic root dilation and associated histological alterations. In this study we use germline and conditional genetic inactivation of Agtr1a (coding for the AT1a receptor) to assess the effect of systemic and localized AT1R signaling attenuation on aortic disease in a mouse model of LDS (Tgfbr1M318R/+). Aortic diameters and histological features were examined in control and Tgfbr1M318R/+ mice with either germline or Mef2CSHF-Cre mediated genetic inactivation of Agtr1a, the latter resulting in deletion in second heart field (SHF)-derived lineages in the aortic root and proximal aorta. Both systemic and regional AT1R signaling attenuation resulted in reduction of diameters and improvement of tissue morphology in the aortic root of LDS mice; these outcomes were associated with reduced levels of Smad2/3 and ERK phosphorylation, signaling events previously linked to aortic disease in LDS. However, regional AT1a inactivation in SHF-derived lineages resulted in a more modest reduction in aortic diameters relative to the more complete effect of germline Agtr1a deletion, which was also associated with lower blood pressure. Our findings suggest that the therapeutic effects of AT1R antagonisms in preclinical models of aortic disease depend on both regional and systemic factors and suggest that combinatorial approaches targeting both processes may prove beneficial for aneurysm mitigation.

Marfan Syndrome in Two Sibs: A Case Report

Marfan syndrome (MFS) is a rare inherited disorder of connective tissue characterized by various phenotypical and genetic manifestations. It involves mutation in FBN1 gene which encodes for microfibrillin glycoprotein fibrillin-1. Predominant involvement occurs in eyes, skeleton and cardiovascular system. Cardiovascular abnormalities such as aortic root dilatation and mitral valve prolapse are the two main life-threatening complications associated with MFS. The diagnosis is based upon clinical findings, some of which are age dependent. If not properly treated, premature death may be caused by the severe cardiovascular and pulmonary complications associated with Marfan syndrome. Therefore, it is important to identify this potentially life-threatening condition in general practice. We are presenting two cases of Marfan syndrome from same family due to its rarity.&#x0D; BANGLADESH J CHILD HEALTH 2021; VOL 45 (2) : 108-110

Neonatal presentation of Loeys-Dietz syndrome: two case reports and review of the literature

BackgroundLoeys-Dietz syndrome (LDS) is a rare connective tissue disorder characterized by cardiovascular manifestations, especially aortic dilatations and arterial tortuosity, craniofacial and skeletal features, joint laxity or contractures, skin abnormalities, hypotonia and motor delay. Its diagnosis is established by the identification of a pathogenic variant in TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2 or TGFB3 genes. In newborns and toddlers, vascular complications such as aneurism rupture, aortic dissection, and intracerebral incidents, can occur already in the weeks of life. To avoid these events, it is crucial to precociously identify this condition and to start an apunderwent a surgical procedurepropriate treatment which, depending on the severity of the vascular involvement, might be medical or surgical.Case presentationWe report two cases of Loeys-Dietz syndrome precociously diagnosed. The first describes a male, born at 38 + 1 weeks of gestation, with hypotonia, joint hypermobility, arachnodactyly, and fingers joint contractures, as well as senile appearance and facial dysmorphisms. In the suspect of a connective tissue disorder, an echocardiography was performed and revealed an aortic root dilatation of 13 mm (Z score + 3). A trio based Whole Exome Sequencing found a novel de novo variant in the TGFBR2 gene. Despite the onset of a low-dose angiotensin receptor blocker therapy, the aneurysm progressed. The second case describes a female, born at 41 + 3 weeks of gestation. During the neonatal examination a cleft palate was noticed, as well as minor dysmorphisms. Since the family history was suspicious for connective tissue disorders, a genetic panel was performed and identified a pathogenetic variant in TGFB3 gene. In this case, the echocardiography revealed no abnormalities.ConclusionsIn addition to our cases, we identified 14 subjects with neonatal LDS in the medical literature. All of them had aortic involvement. Skeletal and face abnormalities, including eyes and palate malformations, were also highly frequent. Overall, 10 subjects required medical therapy to avoid aneurysm progression, and 8 patients underwent surgical procedures.Benefits of an early diagnosis of LDS are various and imply a potential modification of the natural history of the disease with early interventions on its complications.

Aortic root replacement in the setting of a mildly dilated nonsyndromic ascending aorta

ObjectiveThere is controversy on how to address mild aortic root dilation during concomitant aortic valve replacement: composite aortic valve conduit replacement or separate ascending aorta and aortic valve replacement. We reviewed our experience to address the issue. MethodsWe retrospectively reviewed 778 adult nonsyndromic patients with aortic root diameter 55 mm or less who received replacement of the ascending aorta and aortic valve from January 1994 to June 2017. Patients were divided into 2 groups based on the type of aortic root intervention: composite aortic valve conduit replacement in 406 patients (52%) and separate ascending aorta and aortic valve replacement in 372 patients (48%). Propensity matching was used to mitigate differences in baseline patient characteristics and produced 188 matched pairs. ResultsSinus of Valsalva diameter was 43 mm (39-47). Operative mortality occurred in 3 patients (2%) in the composite aortic valve conduit replacement group and in 5 patients (3%) in the separate ascending aorta and aortic valve replacement group (P = .470). Median follow-up was 9.6 years (8.4-10.1). Long-term mortality was similar in the 2 groups (P = .083). Repeat operation was performed in 13 patients (7%) in the composite aortic valve conduit replacement group and in 19 patients (10%) in the separate ascending aorta and aortic valve replacement group (P = .365). Sinus of Valsalva diameter decreased 2 mm (−4-0; median follow-up 41 months) in the propensity-matched separate ascending aorta and aortic valve replacement group. ConclusionsIn patients with mild aortic root dilation, separate ascending aorta and aortic valve replacement results in a similar risk of repeat operation and mortality in comparison with composite aortic valve replacement. Separate ascending aorta and aortic valve replacement is not associated with subsequent aortic root dilation on medium-term echocardiography follow-up.

INCIDENT AORTIC ROOT DILATATION IN THE GENERAL POPULATION: FINDINGS FROM THE PAMELA STUDY

Objective: We sought to assess the long-term changes in aortic root (AR) diameter in a population-based sample, focusing on new onset AR dilatation, as well as on the demographic and clinical variables independently related to this dynamic process. Design and method: A total of 1,122 participants with measurable echocardiographic parameters at baseline and after a ten-year follow-up were included in the analysis. Sex-specific upper limits of normality for absolute AR diameter, AR diameter indexed to body surface area (BSA) and to height were derived from 712 healthy normotensive PAMELA participants. Results: Over the ten-year follow-up new AR dilatation occurred in 3.4% (AR/BSA),4.4% (AR/height) and 7.3% (absolute AR), respectively. No substantial relationship was observed between baseline office and ambulatory BP or their changes over time and incident AR/BSA and AR/height dilatation. Baseline AR diameter and left ventricular mass index (LVMI) emerged as important predictors of AR dilation, regardless of the diagnostic criteria used. This was also the case for the 10-year change in LVMI. The strength of association between non-hemodynamic variables and new onset AR dilatation was variable, depending on the definition of the aortic phenotype. Conclusions: The incidence of AR dilatation in a general middle-aged population is a relatively infrequent but not so rare event and scarcely influenced by both office and out-office BP. On the contrary, it is strongly related to LVMI (and its variations over time). From a clinical perspective, this underlines that LVH prevention and regression can reduce the risk of AR dilatation in the community.

Dysphagia Caused by the Unruptured Aneurysm in the Left Sinus of Valsalva.

Dysphagia Caused by the Unruptured Aneurysm in the Left Sinus of Valsalva.

Repair of Ruptured Sinus of Valsalva Aneurysm: 15 years of Single Center experience

Background and Aims: Ruptured sinus of Valsalva aneurysm is an uncommon cardiac anomaly, fatal if not treated in time. This study was designed to retrospectively analyze our 15‐year experience of the surgical repair for ruptured sinus of Valsalva aneurysm and to study the optimal surgical strategy, morbidity, mortality, and long-term surgical outcome.&#x0D; Methods: This study was conducted on 48 (36 Male, and 12 Female) patients of ruptured sinus of Valsalva aneurysm, operated at department of cardiac surgery, Shahid Gangalal National Heart Centre, Nepal, from January 2006 to December 2020 and followed up till March 2021. Follow‐up data were obtained from the outpatient department records and telephone calls.&#x0D; Results: Mean age was 30.17±11.5 (12-63) years. Rupture of the right coronary sinus into the right atrium was the most common anatomic type (52%). Preoperative aortic regurgitation equal to or greater than grade II were seen in 9 patients (19%) and ventricular septal defects in 6 cases (13%). Two patients had preoperative renal failure. One patient had associated aortic root dilatation and underwent modified Bentall’s procedure. Ruptured sinus of Valsalva aneurysm was repaired from single chamber approach in 9 patients, and dual chamber approach in 39. There was a single mortality (2.08%), two patients required permanent pacemaker placement for complete heart block, and two had wound infection. Follow‐up data were available for 36 patients (75%). With the mean follow up of 7.07±3.93 (range, 0.83-15) years, there was no recurrence present. All survivors were in New York Heart Association functional Class I or II. There was one late death, due to non-cardiac cause.&#x0D; Conclusion: Ruptured sinus of Valsalva aneurysm is rare, yet prompt diagnosis and optimal surgical management is crucial in reducing the deleterious effects. Surgical repair of ruptured sinus of Valsalva carries an acceptable low operative risk and can be performed with laudable long-term outcome, with low incidence of recurrence.