Sinus Of Valsalva Aneurysm Research Articles

Clinical and echocardiographic features of platypnea-orthodeoxia syndrome: a single-centre experience

Abstract Funding Acknowledgements Type of funding sources: None. Background Platypnea-orthodeoxia syndrome (POS) is an uncommon condition characterized by dyspnoea and hypoxemia in the upright position that improves with recumbency.1 Possible causes are intracardiac shunt, pulmonary arteriovenous shunt, and V/Q mismatch.1,2 Echocardiography is the cornerstone of POS diagnosis, with special focus on atrial septal defect (ASD) morphology and right-to-left shunt confirmation.3 Purpose To evaluate the clinical and echocardiographic features of patients presenting with POS due to a cardiac cause. Methods We performed a retrospective analysis of patients diagnosed with POS in our centre between 2015 January and 2021 August. Routine blood tests and transthoracic echocardiogram (TTE) were performed in all patients. Demographics, clinical presentation, blood test results, TTE information, and PFO closure procedure details were recorded. Results Seven patients were included, 85.7% female (n = 6). The median (IQR) age was 78 (72-85) years. The most prevalent cardiovascular risk factors were hypertension (100%; n = 7) and overweight/obesity (85.7%; n = 6). Two patients (28.6%) had chronic pulmonary disease. The most common symptoms were fatigue and exercise intolerance (n= 5; 71.4%) and the most frequent sign was persistent hypoxemia (n = 7; 100%), although 28.6% (n = 2) patients did not present the typical positional changes in peripheral oxygen saturation. Haemoglobin levels [14.1 (13.3-15.2)] were within the normal range and serum NTproBNP levels [656 (287-1196)] were slightly elevated. Left ventricle function was preserved in all patients; right ventricle morphology and function were normal in 85.7% (n = 6) patients, low probability of pulmonary hypertension in TTE was found in 85.7% (n = 6), and exuberant Eustachian valve was observed in 14.3% (n = 1). All patients presented atrial septal hypermobility, 87.5% (n = 6) meeting atrial septal aneurysm criteria. Patent foramen ovale was found in 85.7% of patients (n = 6) and ostium secundum ASD in 14.3% (n = 1). POS precipitating factors were aortic root dilation (28.6%; n = 2), chest trauma (14.3%; n = 1), right hip arthroplasty (14.3%; n = 1), atrial septal stretching regarding right volume overload (14.3%; n = 1). The underlying mechanism was unknown in 28.6% (n = 2) of patients. ASD closure was performed in 57.1% (n = 4) of patients: 75% (n = 3) showed residual shunt, but clinical improvement was reported by all. No acute complications were described, except for paroxysmal atrial fibrillation (14.3%; n = 1). Conclusion POS diagnosis depends on high clinical suspicion: the most common manifestations are fatigue and persistent hypoxemia. Typical positional changes in oxygen saturation are not present in all patients. Polycythaemia, right chambers dilation, and pulmonary hypertension are not common. Echocardiography is fundamental for diagnosis, allowing right-to-left shunt confirmation and ASD morphology evaluation to outline a successful closure procedure.

Magnetic resonance angiography derived predictors of progressive dilatation and surgery of the aortic root in Marfan syndrome.

BackgroundTo identify magnetic resonance (MR) angiography derived predictors of progressive dilatation and surgery of the aortic root in Marfan syndrome.Material and methodsWe retrospectively included 111 patients (32.7±16.5 years, range: 7–75 years) with a total of 446 MR angiographies. Aortic diameter growth rates of the entire thoracic aorta and Z-scores were estimated from annual diameter measurements. Aortic root shape was subdivided into three different types: (T0) normal; (T1) localized dilatation; (T2) generalized aortic root dilatation. Aortic diameter, Z-score, age, and aortic root shape at baseline were tested as predictors of aortic root dilatation using a multivariate logistic regression model.ResultsThe highest aortic growth rate was observed at the level of the sinuses of Valsalva. Higher aortic root diameters and Z-scores at baseline predicted an increased growth of the aortic root (p = 0.003 and p<0.001). Young age (<30 years) was a predictor for the increase of Z-scores when compared to patients ≥30 years (p = 0.019). 25/111 patients (22.5%) had a T0 aortic root shape, 59/111 patients (53.2%) had a T1 aortic root shape, and 27/111 patients (24.3%) had a T2 aortic root shape. Aortic root shape did not predict further aortic growth (p>0.05). However, significantly more patients undergoing surgery had a generalized aortic dilatation (19/28, 76.9%) than a localized aortic root dilatation (9/28, 32.1%) (p = 0.001).ConclusionLarger baseline aortic root diameter and Z-score as well as young age predict solely progressive aortic root dilatation in Marfan patients. MR angiography derived type of aortic root shape does not predict aortic growth, but patients with generalized aortic root dilatation are referred more frequently for aortic surgery.

Engineering analysis of aortic wall stress and root dilatation in the V-shape surgery for treatment of ascending aortic aneurysms.

OBJECTIVESThe study objective was to evaluate the aortic wall stress and root dilatation before and after the novel V-shape surgery for the treatment of ascending aortic aneurysms and root ectasia.METHODSClinical cardiac computed tomography images were obtained for 14 patients [median age, 65 years (range, 33–78); 10 (71%) males] who underwent the V-shape surgery. For 10 of the 14 patients, the computed tomography images of the whole aorta pre- and post-surgery were available, and finite element simulations were performed to obtain the stress distributions of the aortic wall at pre- and post-surgery states. For 6 of the 14 patients, the computed tomography images of the aortic root were available at 2 follow-up time points post-surgery (Post 1, within 4 months after surgery and Post 2, about 20–52 months from Post 1). We analysed the root dilatation post-surgery using change of the effective diameter of the root at the two time points and investigated the relationship between root wall stress and root dilatation.RESULTSThe mean and peak max-principal stresses of the aortic root exhibit a significant reduction, between pre- and post-surgery for both root mean stress (median among the 10 patients presurgery, 285.46 kPa; post-surgery, 199.46 kPa) and root peak stress (median presurgery, 466.66 kPa; post-surgery, 342.40 kPa). The mean and peak max-principal stresses of the ascending aorta also decrease significantly from pre- to post-surgery, with for the mean value (median presurgery, 296.48 kPa; post-surgery, 183.87 kPa), and for the peak value (median presurgery, 449.73 kPa; post-surgery, 282.89 kPa), respectively. The aortic root diameter after the surgery has an average dilatation of 5.01% in total and 2.15%/year. Larger root stress results in larger root dilatation.CONCLUSIONSThis study marks the first biomechanical analysis of the novel V-shape surgery. The study has demonstrated significant reduction in wall stress of the aortic root repaired by the surgery. The root was able to dilate mildly post-surgery. Wall stress could be a critical factor for the dilatation since larger root stress results in larger root dilatation. The dilated aortic root within 4 years after surgery is still much smaller than that of presurgery.

Mid-term outcomes of stentless Bio-Bentall vs. David Reimplantation for aortic root replacement.

To compare the early and midterm clinical outcomes of patients undergoing two different surgical procedures (stentless biological Bentall [SBB] vs. valve-sparing David Reimplantation [VSDR]) for aortic root dilatation, with or without concomitant aortic valve pathology. A population of 106 patients underwent aortic root replacement from 2004 to 2015 at our unit. Mean age at operation was 63.1 ± 10 years. The patients were retrospectively assigned to 2 groups according to surgical procedure. David operation was carried out in 52 patients (group A-VSDR) and stentless biological Bentall in 54 patients (group B-SBB). Preoperative characteristics were similar in the 2 cohorts, except for age and EuroSCORE. Mean follow-up time was 7.09 years (0.26-14.50 years), and 96% complete. Patients were evaluated and analyzed for intra-operative results and long-term clinical outcomes. Intraoperative mortality was 0% in both groups. Overall survival probability at 11 years was 91.8% in group A and 73.8% in group B (p = .004). Cardiac mortality at 11 years was similar in the 2 groups (p = .116). Freedom from structural valve deterioration at 11 years was 88.8% (VSDR) and 90.6% (SBB) [p = .689]. Freedom from reoperation at 11 years did not differ between the groups (83.6% VSDR vs. 98.5% SBB, p = .574). Freedom from major adverse cardiac and cerebrovascular events at 11 years was 76.7% (VSDR) versus 80.1% (SBB) (p = .542). Mean gradient at last follow-up was lower in VSDR group (8.13 vs. 11.70 mmHg, p < .001). David reimplantation and stentless biological Bentall procedures achieve excellent and comparable results at midterm follow-up. David operations provide superior hemodynamic performance preserved over time and may be preferred in younger, active patients.

4D-flow cardiac magnetic resonance imaging after aortic root replacement with long-valved decellularized aortic homografts: comparison to valve-sparing aortic root replacement and healthy controls.

OBJECTIVESLong-valved decellularized aortic homografts (DAH) may be used in young patients to treat aortic valve disease associated with aortic root dilatation, thereby eliminating the need for prosthetic material and anticoagulation.METHODSThirty-three male subjects in 3 equally sized cohorts were compared: patients following DAH implantation with a median age of 29 years [interquartile range (IQR) 27.5–37.5], patients post-valve-sparing aortic root replacement (VSARR), median 44 years (IQR 31.5–49) and healthy controls, median 33 years (IQR 28–40, P = 0.228). Time-resolved three-dimensional phase-contrast cardiac magnetic resonance imaging was performed to assess maximum blood flow velocity, pulse wave velocity, mechanical energy loss (EL), wall shear stress and flow patterns (vorticity, eccentricity, helicity) in 5 different planes of the aorta.RESULTSThe mean time between surgery and cardiovascular magnetic resonance was 2.56 ± 2.0 years in DAH vs 2.67 ± 2.1 in VSARR, P = 0.500. No significant differences in maximum velocity and pulse wave velocity were found between healthy controls and DAH across all planes. Velocity in the proximal aorta was significantly higher in VSARR (182.91 ± 53.91 cm/s, P = 0.032) compared with healthy controls. EL was significantly higher in VSARR in the proximal aorta with 1.85 mW (IQR 1.39–2.95) compared with healthy controls, 1.06 mW (0.91–1.22, P = 0.016), as well as in the entire thoracic aorta. In contrast, there was no significant EL in DAH in the proximal, 1.27 m/W (0.92–1.53, P = 0.296), as well as in the thoracic aorta, 7.7 m/W (5.25–9.90, P = 0.114), compared with healthy controls. There were no significant differences in wall shear stress parameters for all 5 regions of the thoracic aorta between the 3 groups. DAH patients, however, showed more vorticity, helicity and eccentricity in the ascending aorta compared with healthy controls (P < 0.019).CONCLUSIONSDecellularized long aortic homografts exhibit near to normal haemodynamic parameters 2.5 years postoperatively compared with healthy controls and VSARR.

Cardiovascular manifestations of hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders.

Introduction: Mitral valve prolapse and aortic root dilatation are reported in association with hypermobile Ehlers-Danlos syndrome (hEDS), but the full phenotypic spectrum of cardiovascular complications in this condition has not been studied in the aftermath of updated nosology and diagnostic criteria. Methods: We performed a retrospective review of 258 patients (> 94% adults) referred to a multidisciplinary clinic for evaluation of joint hypermobility between January 2017 and December 2020 and diagnosed with hEDS or a hypermobility spectrum disorder (HSD) to determine the incidence and spectrum of cardiovascular involvement. Results: Mitral valve prolapse was present in 7.5% and thoracic aortic dilatation in 15.2%. Aortic dilatation was more frequent in individuals with hEDS (20.7%) than with HSD (7.7%) and similarly prevalent between males and females, although was mild in > 90% of females and moderate-to-severe in 50% of males. Five individuals (1.9%) with hEDS/HSD had extra-aortic arterial involvement, including cervical artery dissection (CeAD, n = 2), spontaneous coronary artery dissection (SCAD, n = 2), and SCAD plus celiac artery pseudoaneurysm (n = 1). This is the first series to report the prevalence of CeAD and SCAD in hEDS/HSD. Conclusions: Cardiovascular manifestations in adults with hEDS/HSD, especially females, are typically mild and readily assessed by echocardiography. Since the risk of progression has not yet been defined, adults with hEDS/HSD who are found to have aortic dilatation at baseline should continue ongoing surveillance to monitor for progressive dilatation. Cardiovascular medicine specialists, neurologists, and neurosurgeons should consider hEDS/HSD on the differential for patients with CeAD or SCAD who also have joint hypermobility.

Outcomes Following Supracoronary Ascending Aortic Replacement with Aortic Valve Resuspension versus Modified Bentall's Operation for Acute Type A Aortic Dissection.

IntroductionType A acute aortic dissection (AAD) remains a challenging cardiac emergency despite the availability of various management strategies. This study compared the outcomes of supracoronary ascending aortic replacement (SCAAR) with aortic valve (AV) resuspension with those of modified Bentall's operation for type A AAD and the progression of aortic regurgitation (AR), long-term dilatation of aortic root and proximal arch, and long-term mortality in SCAAR patients.MethodsSixty patients underwent surgery for type A AAD (January 2005 to December 2015). Forty-three patients underwent SCAAR with AV resuspension and 17 underwent modified Bentall's operation. All patients were followed up.ResultsUpon follow-up of SCAAR patients (n=40), there was significant reduction in aortic root size (preoperative 39.3 mm [9.4] vs. postoperative 33.1 mm [9.1]; P<0.001). Three of these patients worsened to severe AR while others had similar or lesser degree of AR. On comparison between preoperative and postoperative dimensions of all patients (n=53), there was no significant difference in distal ascending aorta size (35.7 mm [8.1] vs. 34.4 mm [8.9]; P=0.52). However, an increase in descending thoracic aorta size (28.8 mm [7.8] vs. 33.7 mm [9.9]; P<0.001) was observed. In-hospital and late mortalities for SCAAR vs. modified Bentall's procedure were 11.7% (seven patients) (7% [3] vs. 23.5% [4]) and 28% (15 patients) (15% [6] vs. 69% [9]), respectively.ConclusionSCAAR with AV resuspension is a safe surgical option for type A AAD. Preservation of AV is associated with better long-term outcomes and reduced mortality. Modified Bentall's operation may be associated with long-term mortality.

Diagnostic Challenge of a Ruptured Sinus of Valsalva Aneurysm (RSOVA) in the Presence of Concurrent Valvular Pathology: The Utility of Multimodality Imaging

Diagnostic Challenge of a Ruptured Sinus of Valsalva Aneurysm (RSOVA) in the Presence of Concurrent Valvular Pathology: The Utility of Multimodality Imaging

Marfan Syndrome Complicated by Aortic Arch Aneurysm and Aortic Dissection: A Case Report from Congo

Context and Aim: Marfan syndrome is a transmissible genetic disease of the connective tissue that is rarely encountered in Congo and in sub-Saharan African countries. Its cardiovascular complications can be life frightening. The management of that disease is still limited in our country because of a lack of technical capacity in cardiovascular surgery. The aim of this clinical report is to show the interest of echocardiography and especially angioscanner as the main technique in the diagnosis of the severity of this disease, elaborate a literature review, but also to highlight the difficulties encountered in the management of that affection in our countries. Observation: The authors report the medical observation of a 48-year-old adult with a history of cataract of the left eye and a subluxation of the lens for which he underwent surgery in 2016, without any etiology being found. He is a smoker at a rate of 6 packs yearly. He consulted for progressively worsening dyspnea and constrictive mediosternal pain. The clinical examination revealed a moderate alteration of the general state, apyrexia, a blood pressure of 140/90 mmHg, a SPO2 of 97% in ambient air, a respiratory frequency of 32 cycles/min, signs of left ventricular insufficiency, a diastolic murmur of aortic insufficiency of intensity 4/6th, a long-limbed morphotype with a wingspan superior to the height and a kyphoscoliosis. Chest X-ray showed cardiomegaly with a cardiothoracic ratio of 58%, a highly dilated and uncoiled aorta, convexity of the left inferior arch, and venocapillary hypertension and a quiet alveolar-interstitial pulmonary oedema. The ECG was in sinus rhythm and showed a poor R-wave progression in anteroseptal leads. Echocardiography showed significant aortic root dilatation up to 72.6 mm and aortic regurgitation grade IV. Angioscanner showed a dissected aortic aneurysm and areas of emphysema located in the lungs. The medical treatment was palliative with beta blocker and angiotensin II receptor antagonists, diuretics and analgesics. The patient is awaiting surgery. Conclusion: Marfan syndrome is a genetic disease of the connective tissue that can manifest itself by cardiovascular, pulmonary, orthopedic, ophthalmological and cutaneous signs. Echocardiography and especially angioscanner are the tools of choice for the diagnosis and follow-up of this condition. Surgery is reserved for serious complications of this condition.

Aortic Root Dilatation in Children and Adolescents At Al-Hawary General Hospital, &amp; 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.

Man With New-Onset Seizure

A 39-year-old man presented to the emergency department with a new-onset generalized tonic-clonic seizure. On assessment, he was lucid and complained of mild head fullness and transient numbness of both lower extremities. His blood pressure was 108/66 mm Hg, and the physical examination, blood work, chest radiography, and brain computed tomography (CT) were normal. After some time, he developed differential blood pressures in his arms (98/52 mm Hg versus 76/40 mm Hg) without any other symptoms. The emergency physician performed bedside ultrasonography to rule out vascular injury and observed an intimal flap with thrombus formation in the false lumen of the left common carotid artery (Figure 1, Video E1, Video E2; available at http://www.annemergmed.com) as well as aortic root dilation of 4.8 cm.

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.

Short- and Mid-Term Autograft Outcomes Following the Reinforced Ross Operation

The Ross operation for aortic valve replacement continues to be a controversial option because of concerns related to late autograft dilation and progressive neo-aortic insufficiency. In 2005, the reinforced Ross procedure was described at our institution to address this problem. We aim to analyze the short and mid-term outcomes following this procedure. This is a retrospective study of patients who underwent the reinforced Ross operation between 2004 and 2019. A comprehensive chart review was performed. Echocardiograms were independently reviewed by an adult congenital cardiologist. The time to reintervention was evaluated with a Kaplan-Meier curve. Analysis was conducted in JMP 15.1 (SAS Inc., Cary, NC). Twenty-five patients underwent the reinforced Ross operation. Twenty-three patients (92%) had bicuspid aortic valve and the most common indication for surgery was a combination of aortic insufficiency and stenosis (n = 18, 72%). The mean follow-up was 6.1 ± 5.0 years. All patients were alive at the time of follow-up. Six patients (24%), from early in our experience, required subsequent aortic reintervention. Median time to reintervention was 41.8 months (0-81.5 months). Sixteen (64%) patients had less than moderate aortic insufficiency at last follow-up. Additionally, average aortic root measurements remained unchanged. The reinforced Ross technique was initially proposed as a way to mitigate aortic root dilation seen in the traditional Ross procedure. Our experience suggests an associated learning curve with the majority of aortic reinterventions occurring within the first few years following surgery. Continued follow-up is warranted to assess its long-term durability and functionality.

310 Ruptured sinus of Valsalva aneurysm as a rare cause of sisto-diastolic murmur: role of multimodality imaging in diagnosis and surgical planning

Abstract A 27-year-old man had an incidental finding of sisto-diastolic murmur during a screening medical evaluation. Transthoracic and subsequent transesophageal echocardiography revealed a saccular enlargement of non-coronary (NC) sinus of Valsalva. The aneurysm extended into the right atrium (RA) and presented a large wall discontinuity, with continuous sisto-diastolic flow from the aorta to the right atrium. Computed tomography confirmed the findings and excluded other associated abnormalities. The patient underwent urgent surgical intervention. Intraoperatively, findings reported a floppy and cribrose saccular aneurysm sprouting from the NC sinus inside the RA. The correction turned out to be very challenging due to the close connection between the aneurysmal wall and the tricuspid valve (TV) and aortic valve (AV) annuli. The aortic NC sinus was excised and replaced with a Dacron patch. Damaging of the TV septal leaflet required repair through a consolidating suture involving the septal leaflet of TV and the corresponding annular insertion. AV replacement was also needed due to damage of the AV NC cusp. No complications occurred during the postoperative stay. Histopathology revealed severe atrophy of muscular and elastic fibres of the aortic wall, elastic fiber fragmentation, replacement fibrosis and extensive deposition of mucopolysaccharides. Sinus of Valsalva aneurysm (SoVA) is a rare condition characterized by an enlargement of the aortic root between the aortic valve and the sinotubular junction. SoVA can be either congenital, as a consequence of weakness of the elastic lamina, or acquired, due to infective, degenerative, or traumatic conditions. A prevalence of 0.09% was described in autopsy series and males are more frequently affected. Acute rupture of SoVA requires emergent surgery because of acute life-threatening haemodynamic instability. Congenital and chronic ruptured SoVA could be asymptomatic. Nevertheless, such incidental finding requires urgent surgical correction due to the possibility of unexpected further rupture, generating massive left-right shunt. The close relationship with nearby anatomical structures is a major issue which should be taken into account. In this perspective multimodality imaging is of paramount importance, allowing for a fine surgical planning and avoidance of complications.

Personalized external aortic root support – how to implant it

Personalized External Aortic Root Support (PEARS) is an evolving method of treatment for patients with a dilated aortic root or ascending aorta. This treatment is being adopted in an increasing number of centres. For the sake of the safety of the procedure a standardized surgical technique is necessary. The authors describe a surgical technique of implantation that is derived from their extensive experience.

C59. Management of Subacute Painless Aortic Dissection Stanford A Debakey II in Patient With Undiagnosed Marfan Syndrome During Pregnancy

Abstract Background Pregnant women with Marfan’s syndrome needed multidisciplinary approach to evaluate pregnancy status, fetal and maternal outcome. Marfan’s syndrome was an inherited autosomal dominant with mutation of fibrillin-I (FBN1) gene. Pregnant woman with MFS can lead to aortic dissection. It’s increase 23% of maternal mortality and 35% of fetal death. Case Summary a 30 year-old pregnant woman referred from secondary hospital care with valvular heart disease (severe aortic regurgitation due to dilatation of the aortic root and severe mitral regurgitation). Due to her severe myopia, history of aortic dissection in her family, enlarged of aortic root, pulnus excavatum, scoliosis and arachnodactyly, de novo MFS was suspected. She was diagnosed with marfan’s syndrome according her systemic score was 10 (posive &amp;gt;7), and calculated aortic z score was 6.2 (positive &amp;gt;2). Intimal tears and dilatation of the aortic root were considered to terminate the pregnancy. It’s can result bad neonatal outcome. For the management of a potential vascular catastrophe , monitoring of delivery and repairing the aortic root, we needed to multidisciplinary approach. Discussion Patient with marfan’s syndrome during pregnancy, may develop painless aortic dissection because massive dilatation of aortic arch prevent compression of pain sensitive structure. Less evidence to manage pregnant woman with MFS with aortic root dilatation more than 45 mm and or progressively dilatation of aortic root ( more than 0.5 mm/year). This patient was categorized as class IV modified WHO classification with high risk maternal mortality and severe morbidity.

C59. Management of Subacute Painless Aortic Dissection Stanford A Debakey II in Patient With Undiagnosed Marfan Syndrome During Pregnancy

Abstract Background Pregnant women with Marfan’s syndrome needed multidisciplinary approach to evaluate pregnancy status, fetal and maternal outcome. Marfan’s syndrome was an inherited autosomal dominant with mutation of fibrillin-I (FBN1) gene. Pregnant woman with MFS can lead to aortic dissection. It’s increase 23% of maternal mortality and 35% of fetal death. Case Summary a 30 year-old pregnant woman referred from secondary hospital care with valvular heart disease (severe aortic regurgitation due to dilatation of the aortic root and severe mitral regurgitation). Due to her severe myopia, history of aortic dissection in her family, enlarged of aortic root, pulnus excavatum, scoliosis and arachnodactyly, de novo MFS was suspected. She was diagnosed with marfan’s syndrome according her systemic score was 10 (posive &amp;gt;7), and calculated aortic z score was 6.2 (positive &amp;gt;2). Intimal tears and dilatation of the aortic root were considered to terminate the pregnancy. It’s can result bad neonatal outcome. For the management of a potential vascular catastrophe , monitoring of delivery and repairing the aortic root, we needed to multidisciplinary approach. Discussion Patient with marfan’s syndrome during pregnancy, may develop painless aortic dissection because massive dilatation of aortic arch prevent compression of pain sensitive structure. Less evidence to manage pregnant woman with MFS with aortic root dilatation more than 45 mm and or progressively dilatation of aortic root ( more than 0.5 mm/year). This patient was categorized as class IV modified WHO classification with high risk maternal mortality and severe morbidity.

Abstract 12192: Arterial Tortuosity Syndrome: A Longitudinal Assessment Of Cardiovascular Features

Introduction: Arterial tortuosity syndrome (ATS) is an ultra-rare arteriopathy caused by pathogenic biallelic variants in SLC2A10 leading to tortuosity, elongation, and dilation of vessels, and pulmonary artery (PA) stenosis (Fig 1). No studies to date have evaluated the progression of cardiovascular (CV) features, including aortic root (AR) and ascending aorta (AA) dilation. Hypothesis: We hypothesize AR and AA dilation progress similar to other aortopathies with increase dimensions but stable z-scores in childhood. Methods: A retrospective cohort study of patients at our institution with ATS, defined by homozygous or compound heterozygous SLC2A10 mutations with consistent phenotypes, from 2000-2021 was conducted. CV imaging, medications, and interventions were reviewed. AR and AA measurements were collected from echocardiographic reports and z-scores were calculated. Dilation was defined as z-score &gt; +2. Slopes were calculated using mixed linear regression. Results: Six subjects (5 males) aged 1 month-16 years at diagnosis were included. All had AR dilation, 50% had AA dilation, and 50% had main or branch PA stenosis at a given point. CV intervention was only performed in 1 patient who underwent multiple bilateral PA angioplasties and stenting for severe distal branch PA hypoplasia. Three patients were prescribed beta-blockers, of whom one was also prescribed losartan. Figure 2 shows mean slopes for AR and AA changes. No patients had aortic dissection or CV surgery. Conclusions: In conclusion, AR and AA dilation persist in ATS but with stable z-scores similar to other aortopathies. Larger sample size investigations may further elucidate the role of medications in ATS.

Abstract 10428: Bad Fall, Good Catch: Diagnosing a Rare Condition Presenting as Acute Coronary Syndrome

A 72 year-old mountain bicyclist suffered a biking accident. The following week, he presented to the emergency room with abdominal pain, where he was treated for superior mesenteric artery thrombosis and new-onset atrial fibrillation. Two months later, he presented with intermittent left scapular pain that radiated to the chest. Pharmacologic stress testing revealed a large reversible perfusion defect in the anterior wall with an ejection fraction of 41%. Left heart catheterization showed narrowing of the distal left main coronary artery and the proximal left anterior descending (LAD) and circumflex arteries, by what appeared to be external compression. Follow-up CT angiogram demonstrated two sinus of Valsalva aneurysms (SOVA) originating from the left and right coronary cusps. A transesophageal echocardiogram confirmed these findings and showed tremendous thrombus within the larger aneurysm. The patient was admitted to cardiothoracic surgery. Troponin was elevated to 0.16 μg/L, with EKG showing 1 mm ST-segment depressions in V4-6, prompting treatment for a non-ST segment myocardial infarction. He underwent complex surgery involving thrombus extraction and closure of the SOVA with pericardial patch repair, and coronary artery bypass grafting of the LAD and obtuse marginal arteries. After a prolonged ICU course in which the patient was managed for cardiogenic shock, he was discharged to a hospital equipped with a rehabilitation facility. Unfortunately, he developed profound lactic acidosis from ischemic bowel and passed away. SOVA are rare lesions that arise from congenital or acquired etiologies, such as this patient’s biking accident. Multi-modality imaging is important for quantifying size and anatomy. Unruptured SOVA can present as embolic phenomenon from thrombus, valvular regurgitation, or chest pain from compression of the coronary arteries. Rupture is a highly morbid condition, and so urgent referral to cardiothoracic surgery is recommended.

Abstract 10395: Did Prescription Practice Change After Publication of a Landmark Trial for Children and Young Adults with Marfan Syndrome

Intro: The Pediatric Heart Network (PHN) trial of β-blockers (BB) vs. angiotensin receptor blockers (ARB) for aortic root dilation in Marfan Syndrome showed similar efficacy, but its impact on prescribing practices is unknown. We hypothesized there would be an increase in single and combination use of BB and ARB after publication in 2014. Methods: Prescription data (2007-2016) were derived from outpatient encounters (IBM Marketscan) for children and young adults aged 6 mo to 25 yrs diagnosed with Marfan Syndrome who had ≥4 encounters with an adult or pediatric cardiologist or geneticist. Excluding 2014 as washout, data were divided into 2 time periods: 2007-2013 and 2015-2016. Categories included BB, ARB, angiotensin converting enzyme inhibitors (ACEI), combination (BB/ARB and/or BB/ACEI), and no drug use. We performed interrupted time-series analysis to assess the immediate level change and the change in slope for the prescription trend. We reported odds ratios (OR) and 95% confidence intervals (CI) from logistic regressions with generalized estimating equation methods to account for correlation of prescriptions within subjects. Results: Of 1499 patients (mean age 14.1 yrs, 59% female), BB had a downtrend [OR 0.91 (0.89, 0.93), p&lt;0.001] and ARB trended higher [OR 1.12 (1.07, 1.18), p&lt;0.001] 2007-2013, while combination, ACEI, and no drug use remained stable. There was no immediate effect after publication (2013-2015). Although the trend persisted for BB and ARB use (see Figure), the change in slope was not significant (2015-2016). Combination, ACEI and no drug use remained consistent. Conclusions: In short term follow-up, the impact of the PHN trial on prescription practices for aortic root dilation in Marfan Syndrome in children and young adults did not reach statistical significance. This may be due to early adoption of ARBs with subsequent confirmation of their usefulness. Longer term data are needed to fully explore the trial’s impact on practice change.