Sinus Of Valsalva Aneurysm Research Articles

Coronary embolism due to probable clinical bioprosthetic aortic valve thrombosis: a case report

BackgroundCoronary embolism is a relatively rare but important non-atherosclerotic cause of acute coronary syndrome, mainly caused by atrial fibrillation and mechanical heart valve thrombosis due to subtherapeutic anticoagulation. There have been increasing reports of bioprosthetic valve thrombosis (BPVT), but thromboembolic events are rare and mainly affect the cerebrovascular system. Coronary embolism is an extremely rare complication of BPVT.Case presentationA 64-year-old male presented with non-ST-Elevation myocardial infarction (NSTEMI) to an Australian regional health service. Three years ago, he had undergone Bentall procedure with bioprosthetic aortic valve replacement for severe aortic regurgitation and significant aortic root dilatation. Diagnostic coronary angiography revealed embolic occlusion of first diagonal branch in the absence of underlying atherosclerosis. Prior to NSTEMI presentation, the patient was clinically asymptomatic apart from the progressive increase in transaortic mean pressure gradient on transthoracic echocardiography which was first detected seven months after surgical aortic valve replacement. Transoesophageal echocardiography showed restrictions of the aortic leaflet opening but no evidence of mass or vegetation. After eight weeks of warfarin therapy, the raised aortic valve gradient returned to normal. Lifelong warfarin was prescribed, and patient remained clinically well at 39-month follow-up.ConclusionWe experienced a case of coronary embolism in a patient with probable BPVT. Reversible bioprosthetic valve hemodynamic deterioration after anticoagulation strongly supports the diagnosis in the absence of histopathology. Early moderate-to-severe hemodynamic valve deterioration warrants further investigations, including cardiac computed tomography and sequential echocardiography, to investigate for probable BPVT and consideration of timely anticoagulation initiation to prevent thromboembolic events.

Aortic Root Dilatation Measured by Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot

Background: Aortic root dilatation (AoD) frequently occurs following repaired tetralogy of Fallot (rTOF). The objective of this study was to assess aortic dimensions, investigate the prevalence of AoD, and identify predictors of AoD in rTOF patients. Methods: A cross-sectional retrospective study was conducted in repaired TOF patients from 2009 to 2020. Aortic root diameters were measured by cardiac magnetic resonance (CMR). Severe AoD of the aortic sinus (AoS) was defined as a Z-score (z) of >4, reflecting a mean percentile ≥99.99%. Results: Two hundred forty-eight patients, with a median age of 28.2 years (10.2–65.3 years), were included in the study. The median age at the time of repair was 6.6 years (0.8–40.5 years) and the median interval between the repair and CMR study was 18.9 years (2.0–54.8 years). The prevalence of severe AoD was found to be 35.2% when defined by an AoS z greater than 4 and 27.6% when defined by a AoS diameter ≥40 mm, respectively. A total of 101 patients (40.7%) had aortic regurgitation (AR), with 7 patients (2.8%) having moderate AR. Multivariate analysis revealed that severe AoD was only associated with the left ventricular end diastolic volume index (LVEDVi) and a longer duration after repair. The age at the time of repair for TOF was found not to be correlated with the development of AoD. Conclusions: After repair of TOF, severe AoD was found to be prevalent, but no fatal complications were observed in our study. Mild AR was also commonly observed. Larger LVEDVi and a longer duration after repair were identified as factors associated with the development of severe AoD. Therefore, routine monitoring of AoD is recommended.

Aortic regurgitation in bicuspid aortic valve: know to decide

Abstract Funding Acknowledgements Type of funding sources: None. Chronic aortic regurgitation (AR) is the second cause of valve surgery in patients with bicuspid aortic valve (BAV) and has distinctive characteristics of trileaflet regurgitation. The quantification variables of aortic regurgitation do not take into account the unique characteristics of bicuspid regurgitation. Recognition of the mechanisms that lead to bicuspid AR is relevant as it allows correct treatment planning and valve repair in candidate patients. Objective a prospective study was designed to identify the mechanisms of AR and the factors (clinical and anatomical) associated with the development of significant AR (moderate/severe) in adult patients with BAV. Materials and methods We included consecutive adult patients with a confirmed diagnosis of BAV evaluated between 2014 and 2021 with at least 3 years of follow-up by the Structural Heart Disease group of our institution. Valve disease was evaluated by echocardiography and complementary methods. It was classified as mild, moderate (Mod), and severe (Sev) aortic regurgitation, according to current criteria (integral approach with quantitative and semi-quantitative criteria). The prevalence of regurgitation mechanisms, the progression of aortic regurgitation, and predictive variables of significant AR and major cardiovascular events (death, surgery) were analyzed using univariate and multivariate analyses. A p<0.05 was considered significant. Surgery was indicated following international recommendations (ESC guidelines): symptoms development or ventricular dysfunction. Results We included 300 patients (39.7 ± 14.9 years, 72.6% men). Regarding valvular phenotype 84% (257p) presented fused BAV (coronary leaflets) and the majority with raphe (87.5%). 71.7% of the patients had some degree of aortic regurgitation at the start of follow-up (24.1% Mod/Sev AR). Among the patients with significant AR, 50% had cusp prolapse (86% prolapse of the fused leaflet), 39.18% dilatation of the aortic root, 26.6% significant calcification of the valve, 1.3% valve perforation and 29.4% restriction of valve movement. 43% with Mod/Sev AR had mixed mechanisms of regurgitation and the combination, half represented by the combination of aortic root dilatation and cusp prolapse (Fig 2). During follow-up 18–6% of patients with Mod AR showed progression to Sev AR and 4.7% underwent surgery. On the other hand, 34.1% of patients with baseline Sev AR required aortic valve surgery (p<0.01). Conclusions In our cohort, AR was highly prevalent: 1 out of 4 patients had significant AR at the start of follow-up. Eccentric jets and the presence of valve prolapse were frequent in patients with significant AR. 43% of BAV patients with Mod/Sev AR exhibited mixed regurgitating mechanisms. Because mechanism affects the choice of surgical technique, comprehensive mechanism description is critical in patients with BAV and AR.

Ascending aorta dilatation is associated to hard cardiovascular events, follow-up from multicentric ARGO-Perspective project.

Aortic root dilatation has been proposed as hypertension-mediated organ damage (HMOD). Nevertheless, the role of the aortic root dilatation as a possible additional HMOD is still unclear since studies conducted so far are quite heterogeneous regarding the type of population analyzed, the aortic tract considered, and the type of outcomes accounted for. The aim of the present study is to assess whether the presence of aortic dilatation is associated with strong cardiovascular (CV) events (MACE: heart failure, CV death, stroke, acute coronary syndrome, myocardial revascularization) in a population of patients affected by essential hypertension. Four hundred forty-five hypertensive patients from six Italian hospitals were recruited as part of ARGO-SIIA study1. For all centers, follow-up was obtained by re-contacting all patients by telephone and through the hospital's computer system. Aortic dilatation (AAD) was defined through absolute sex-specific thresholds as in previous studies (41 mm for males, 36 mm for females). Median follow-up was 60 months. AAD was found to be associated with the occurrence of MACE (HR = 4.07 [1.81-9.17], p < 0.001). This result was confirmed after correction for main demographic characteristics such as age, sex and BSA (HR = 2.91 [1.18-7.17], p = 0.020). At penalized Cox regression, age, left atrial dilatation, left ventricular hypertrophy and AAD were identified as best predictor of MACEs and AAD resulted a significant predictor of MACEs even after correction for these confounders (HR = 2.43 [1.02-5.78], p = 0.045). The presence of AAD was found to be associated with an increased risk of MACE independently of for major confounders, including established HMODs. AAD ascending aorta dilatation, LAe left atrial enlargement, LVH left ventricular hypertrophy, MACEs major adverse cardiovascular events, SIIA Società Italiana dell'Ipertensione Arteriosa (Italian Society for Arterial Hypertension).

Longitudinal Aortic Root Dilatation in Collegiate American-Style Football Athletes.

Background Clinically relevant aortic dilatation (>40 mm) and increased cardiovascular risk are common among retired professional American-style football athletes. Among younger athletes, the effect of American-style football participation on aortic size is incompletely understood. We sought to determine changes in aortic root (AR) size and associated cardiovascular phenotypes across the collegiate career. Methods and Results This was a multicenter, longitudinal repeated-measures observational cohort study of athletes across 3 years of elite collegiate American-style football participation. A total of 247 athletes (119 [48%] Black, 126 [51%] White, 2 [1%] Latino; 91 [37%] linemen, 156 [63%] non-linemen) were enrolled as freshmen and studied at pre- and postseason year 1, postseason year2 (N=140 athletes), and postseason year 3 (N=82 athletes). AR size was measured with transthoracic echocardiography. AR diameter increased over the study period from 31.7 (95% CI, 31.4-32.0) to 33.5 mm (95% CI, 33.1-33.8; P<0.001). No athlete developed an AR ≥40 mm. Athletes also demonstrated increased weight (cumulative mean Δ, 5.0 [95% CI, 4.1-6.0] kg, P<0.001), systolic blood pressure (cumulative mean Δ, 10.6 [95% CI, 8.0-13.2] mm Hg, P<0.001), pulse wave velocity (cumulative mean Δ, 0.43 [95% CI, 0.31-0.56] m/s, P<0.001), and left ventricular mass index (cumulative mean Δ, 21.2 [95% CI, 19.2-23.3] g/m2, P<0.001), and decreased E' velocity (cumulative mean Δ, -2.4 [95%CI, -2.9 to -1.9] cm/s, P<0.001). Adjusting for height, player position, systolic blood pressure, and diastolic blood pressure, higher weight (β=0.030, P=0.003), pulse wave velocity (β=0.215, P=0.02), and left ventricular mass index (β=0.032, P<0.001) and lower E' (β=-0.082, P=0.001) were associated with increased AR diameter. Conclusions Over the collegiate American-style footballcareer, athletes demonstrate progressive AR dilatation associated with cardiac and vascular functional impairment. Future studies delineating aortic outcomes are necessary to determine whether AR dilation is indicative of maladaptive vascular remodeling in this population.

Staged definitive repair for pulmonary atresia and ventricular septal defect 40 years after palliative surgery: a case report

We present a very rare case of pulmonary atresia and ventricular septal defect with staged definitive repair more than 40 years after palliative surgery. The patient, a 43-year-old male, had undergone a Waterston operation at the age of one and had been untreated since then. Two years ago, he underwent an urgent surgery for impending rupture of a huge pulmonary artery aneurysm. Then, after evaluation of cardiac and pulmonary functions, a definitive repair was performed concomitantly with aortic root replacement for the dilated aortic root. He was discharged uneventfully and received ambulatory care 5 years after surgery.

Localized dissection of the sinus of Valsalva mimicking spontaneous coronary artery dissection.

A 56-year-old man, suspected of having ST-segment elevation myocardial infarction due to spontaneous coronary artery dissection, underwent emergency percutaneous coronary intervention. Although he had moderate aortic regurgitation with aortic root dilation and mild heart failure, it was controlled with medications. Two weeks after discharge, he was readmitted with severe heart failure due to severe aortic regurgitation and underwent an aortic root replacement. Intraoperative findings revealed that localized dissection of the sinus of Valsalva involved the right coronary artery, resulting in coronary artery dissection. In cases of spontaneous coronary artery dissection, attention should be paid to coronary artery dissection caused by localized aortic root dissection.

P406 LAUBRY–PEZZI SYNDROME: EVERYTHING IS NOT AS IT SEEMS

Abstract Introduction Laubry–Pezzi syndrome is a congenital heart disease characterized by the prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD), mostly supracristal, leading to severe aortic regurgitation (AR). It has been reported nearly always in childhood, the Venturi effect is the predominant factor and onset symptoms may be variable. Case Report A 61–years–old male was admitted to our intensive case unit for chest pain radiated to interscapular region and exertional dyspnea. Hemodynamic parameters were normal. At ECG sinus rytm with heart rate 92 bpm and non–specific repolarization abnomalities were reported. Hs troponin I was 276 ng/L, myoglobin 30.2 ng/mL and BNP 325 pg/mL. Transthoracic echocardiography showed a dilated left ventricle (LV EDV 107 mL/m2) with normal ejection fraction (55%), severe AR without aortic root dilation and a suspicious shunt left to right at level of interventricular septum. Therefore, transesophageal echocardiography was performed and confirmed severe AR by probable bicuspid valve and right cusp prolapse, in absence of calcifications and/or vegetations by endocarditis, restrictive perimembranous VSD (maximum diameter 0.6 cm) and ostium secundum ASD with shunt L–R. Right chambers were not dilated and non invasive systolic pulmonary artery pressure was 43 mmHg. Coronary angiography was performed and showed a significant stenosis of distal left anterior descending (LAD) artery and of proximal circumflex artery. Right heart catetherization excluded significant high values of pulmonary pressures (mPAP=18 mmHg), of pulmonary capillary wedge pressure (9 mmHg), with a mild increase of pulmonary vascular resistances (1.16 HRU; nv &amp;lt; 96). Coronary artery bypass grafting (left internal mammary artery on LAD and Y–graft radial artery on obtuse marginal artery), aortic valve replacement with bioprothesis (Inspiris Resilia 23 mm), ASD closure by direct suture and VSD closure with pericardial patch were performed. No post–surgical complications were reported. Interestingly, during the replacement of aortic valve, it resulted tricuspid, confirming the suspicion of Laubry Pezzi syndrome. Conclusions our case was object of great debate because restrictive VSD and severe AR were not previously known, were diagnosed in middle age and until then paucisymptomatic. Laubry–Pezzi syndrome is a very rare disease that may explain controversial cases of severe AR.

Childhood rheumatic diseases: bites not only the joint, but also the heart.

Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective tissue diseases. Though coronary vasculitis is the prototypical manifestation of Kawasaki disease, it can also be seen in patients with polyarteritis nodosa. Pericarditis is the most common manifestation seen in juvenile rheumatic diseases like systemic onset JIA, and lupus. Cardiac tamponade, valvular insufficiency, aortic root dilatation and arrhythmias are seen rarely. Cardiac involvement is often recognized late in children. The development of cardiac disease in juvenile systemic sclerosis is associated with a poor outcome. In long term, childhood onset of rheumatic diseases predisposes to diastolic dysfunction and premature atherosclerosis during adulthood. Key Points • Pericarditis is the most common cardiac manifestation in SLE and can lead to tamponade. • Conduction defects are common in juvenile mixed connective tissue disease and systemic sclerosis. • Pulmonary hypertension is a significant contributor to mortality in juvenile systemic sclerosis. • In Kawasaki disease, early treatment can reduce risk of coronary artery aneurysms.

Prophylactic effect of angiotensin receptor blockers in children with genetic aortopathies: the early bird catches the worm

AimsIn genetic aortopathies (GA) particular attention is paid to aortic root dilatation which has an impact on morbidity and mortality. This study focuses on the effects of therapy with angiotensin-II-receptor-blockers (ARB) or beta-blockers (BB) on aortic root growth and the question which therapy should be initiated at which dosage and at what age.MethodsSince 1998 we diagnosed 208 patients with GA (170 FBN-1). 81 patients between 5 months and 18 years receiving either ARB or BB therapy were included. We retrospectively analyzed the progression of the dilatation of Sinus Valsalva aortae (SV) using calculated z-scores before and after therapy initiation and compared BB and ARB treatment.ResultsBoth ARB and BB (p < 0.05) therapy showed significant improvement in aortic root growth, while the effect is significantly more pronounced in ARB (p < 0.01) independent of age and genetic cause. A detailed comparison of the two drug groups showed a more sustained effect in limiting the progression of the dilatation of the aortic root in patients treated with ARB. Progression of dilatation of the SV was significantly lower in children treated with ARBs compared to BB (delta z-score, p < 0.05). In addition, ARBs were better tolerated and had a significantly lower discontinuation rate (3%) compared to BB (50%) (p < 0.01). Independently of age at initiation all children and adolescents were able to reach the target dose under ARB.ConclusionWe demonstrated a significant change in both treatment options, with the effect of ARB being more pronounced while being better tolerated throughout the treatment period.

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants

ObjectiveWe sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). MethodsA single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. ResultsOf 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045). ConclusionsPatients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.

Calcium promotes vascular smooth muscle cell phenotypic switching in Marfan syndrome

Fibrillin 1 (Fbn1) mutations cause Marfan syndrome (MFS), with aortic root dilatation, dissection, and rupture. Few studies reported the blood calcium and lipid profile of MFS, and the effect of vascular smooth muscle cell (VSMC) phenotypic switching on MFS aortic aneurysm is unclear. Here, we aimed to investigate the role of calcium-related VSMC phenotypic switching in MFS. We retrospectively collected MFS patients' clinical data, performed bioinformatics analysis to screen the enriched biological process in MFS patients and mice, and detected markers of VSMC phenotypic switching on Fbn1C1039G/+ mice and primary aortic vascular smooth muscle cells. We found that patients with MFS have elevated blood calcium levels and dyslipidemia. Furthermore, the calcium concentration levels were increased with age in MFS mice, accompanied by the promoted VSMC phenotypic switching, and SERCA2 contributed to maintaining the contractile phenotype of VSMCs. This study provides the first evidence that the increased calcium is associated with the promoted VSMC phenotype switching in MFS. SERCA may become a novel therapeutic target for suppressing aneurysm progression in MFS.

Abstract 520: Stethoscope Still Saving Lives: Catching A Ruptured Sinus Of Valsalva Aneurysm

Introduction: Sinus of Valsalva aneurysm (SVA) is a rare cardiac abnormality that can occur congenitally or as a result of acquired conditions. It is characterized by a weakness in the elastic lamina at the junction of the aortic media and annulus fibrosis, leading to the formation of an SVA. We present an interesting case of a 54-year-old man, initially diagnosed with infective endocarditis and later on found to have a severe acute rupture of an SVA. Case Presentation: A 54-year-old man with a past medical history of raynaud's disease and rheumatoid arthritis presented with shortness of breath and low-grade fever for two weeks. On further evaluations, a diligent finding of a continuous cardiac murmur and a palpable thrill across the precordium led to a transthoracic echocardiogram (TTE). The TTE revealed a 1.3 x 1.3 cm, nonmobile, ball of material near the base of the septal leaflet of the tricuspid valve, raising concerns about infective endocarditis and thus initiation of broad-spectrum intravenous antibiotics. Subsequent transesophageal echocardiogram, however, revealed an outpouching from the right coronary sinus suggestive of the right sinus of Valsalva which was ruptured with jet hitting the tricuspid valve continuously during diastole and systole, mimicking falsely as vegetation on TTE. Emergency interventions including CT angiogram, catheterization, and surgical repair resulted in a favorable outcome for the patient. Discussion: SVA has a higher prevalence among males and individuals of Asian ethnicity. Its presentation is variable and can include chest discomfort, shortness of breath, pulmonary edema, or even rupture. It can also be an incidental finding on an echocardiogram. The preferred initial diagnostic approach is echocardiography, and surgical repair is often required for patients, especially if the aneurysm has ruptured. Upon diagnosis, prompt evaluation by a cardiothoracic surgeon is crucial as delay in treatment may lead to fatal consequences. Conclusion: The prognosis for ruptured SVAs is poor, which further emphasizes the significance of early diagnosis and prompt treatment. A diligent physical examination is very crucial and can be lifesaving, especially in such circumstances.

H Syndrome Presenting with Sinus of Valsalva Aneurysm and Possible Founder Mutation (p.Arg134cys) in Indian Patients.

H Syndrome Presenting with Sinus of Valsalva Aneurysm and Possible Founder Mutation (p.Arg134cys) in Indian Patients.

17 Beta-estradiol impedes aortic root dilation and rupture in male Marfan mice

Background: Marfan syndrome causes a hereditary form of TAA with dilation of the aortic root. Male patients with Marfan syndrome are more likely than women to have aortic dilation and dissection and mouse models of Marfan syndrome demonstrate larger aortic roots in males compared to females even after adjustment for body size. In this study we examine the effects of estrogen17 β-estradiol on aortic dilation and rupture in a Marfan mouse model. Methods: Marfan male mice were administered estrogen17 β-estradiol pellets with 60 day release and the growth in aortic root size was measured every 2 weeks for 8 weeks. Aortic root tissue from vehicle and 17 b-estradiol mice were subjected to proteomic analysis and bioinformatics was used to ascertain enriched pathways. Marfan mice with and without 17 b-estradiol were also treated with continuous angiotensin II infusion and survival was determined for 30 days. Results: Male Marfan mice with estrogen17 β-estradiol had a significant reduction in the ratio of aortic diameter 8 weeks after pellet implantation to baseline aortic diameter compared to the same ratio in Marfan mice with sham surgery. Proteomic analysis demonstrated complement C3 and C4 protein upregulation in Marfan mice compared to wild type and Marfan mice treated with 17 b-estradiol. Aortic smooth muscle cells express complement components in vivo and in vitro and expression of these factors are regulated by cytokines and estrogen17 β-estradiol in vitro. Additionally, 17 b-estradiol and female sex protect against aortic rupture when Marfan mice are treated with continuous infusion of angiotensin II and this protection correlates with lower protein levels of C3. Conclusions: Our findings support a protective role of 17 b-estradiol in preventing the dilation and rupture of aortic roots in Marfan male mice and suggest that these therapeutic actions may develop through anti-inflammatory properties. FAER Foundation for Anesthesia Education and Research This is the full abstract presented at the American Physiology Summit 2023 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Abstract #1395486: Uncontrolled Acromegaly Resulting in the Need for LVAD as Bridge to Heart Transplant

Acromegaly is an uncommon pituitary disorder with an incidence of six per million persons. While hypertension is often encountered in these patients, heart failure rarely is seen with an incidence rate under 10%. We describe a case of an individual who was diagnosed with acromegaly after an acute exacerbation of heart failure with subsequent management requiring an LVAD to perform Transsphenoidal Surgery (TSS). 37-year-old male otherwise healthy initially presented to an emergency room and was found to be in acute heart failure exacerbation. Concerning acromegaly features included macrognathia, enlarged hands and feet, swollen phalanges, widened spacing of teeth, and frontal bossing. IGF-1 level was found 455 ng/mL. MRI showed a 10mm macroadenoma. A right heart catheterization showed elevated filling pressures. Cardiac MRI showed no defects or enhancement. Endomyocardial biopsy showed no inflammatory infiltrates or evidence of infiltrative diseases. Patient had an ejection fraction of 15% corroborated by cardiac MRI along with the presence of aortic root dilatation and mitral regurgitation. The patient started on 0.5mg of Cabergoline twice weekly and 120mg weekly Lanreotide injections. Patient stabilized with plans for further close monitoring and outpatient neurosurgical evaluation. The COVID-19 pandemic and insurance gaps led the patient to spend two years off his medicines and he was unable to be seen by his medical team. Patient was seen by our system after recurrent hospitalizations for heart failure at our sister hospital, AICD was unable to be placed due to the patient’s anatomy, he was placed on wearable cardiac defibrillator and required milrinone infusion for progression to end-stage heart failure with cardiac cachexia. At our institution, the patient was evaluated for Orthotopic Heart Transplant (OHT) but due to active GH secreting macroadenoma there was concern for OHT failure without TSS. Decision was made to utilize LVAD as Bridge-to-Transplant for OHT so the patient could be stabilized and safely undergo TSS. The patient tolerated surgeries well and is currently on the active transplant list. Heart failure is an uncommon presentation of severe acromegaly requiring multidisciplinary management. We describe a case of a patient who initially presented with heart failure too unstable for surgery. Due to the COVID-19 pandemic the patient’s disease progressed resulting in end-stage heart failure requiring LVAD placement for further treatment. We would like to draw attention to the use of LVAD placement in acromegalic patients who develop severe cardiovascular disease who are not candidates for OHT.

Smooth Muscle Cell Klf4 Expression Is Not Required for Phenotype Modulation or Aneurysm Formation in Marfan Syndrome Mice-Brief Report.

Smooth muscle cell (SMC) phenotypic reprogramming toward a mixed synthetic-proteolytic state is a central feature of aortic root aneurysm in Marfan syndrome (MFS). Previous work identified Klf4 as a potential mediator of SMC plasticity in MFS. MFS (Fbn1C1041G/+) mouse strains with an inducible vascular SMC fluorescent reporter (MFSSMC) with or without SMC-specific deletion of Klf4 exons 2 to 3 (MFSSMC-Klf4Δ) were generated. Simultaneous SMC tracing and Klf4 loss-of-function (Klf4Δ mice) was induced at 6 weeks of age. Aneurysm growth was assessed via serial echocardiography (4-24 weeks). Twenty-four-week-old mice were assessed via histology, RNA in situ hybridization, and aortic single-cell RNA sequencing. MFS mice demonstrated progressive aortic root dilatation compared with control (WTSMC) mice regardless of Klf4 genotype (P<0.001), but there was no difference in aneurysm growth in MFSSMC-Klf4Δ versus MFSSMC (P=0.884). Efficient SMC Klf4 deletion was confirmed via lineage-stratified genotyping, RNA in situ hybridization, and immunohistochemistry. Single-cell RNA sequencing of traced SMCs revealed a highly similar pattern of phenotype modulation marked by loss of contractile markers (eg, Myh11, Cnn1) and heightened expression of matrix genes (eg, Col1a1, Fn1) between Klf4 genotypes. Pseudotemporal quantitation of SMC dedifferentiation confirmed that Klf4 deletion did not alter the global extent of phenotype modulation, but reduced expression of 23 genes during this phenotype transition in MFSSMC-Klf4Δmice, including multiple chondrogenic genes expressed by only the most severely dedifferentiated SMCs (eg, Cytl1, Tnfrsf11b). Klf4 is not required to initiate SMC phenotype modulation in MFS aneurysm but may exert regulatory control over chondrogenic genes expressed in highly dedifferentiated SMCs.

Ruptured sinus of Valsalva aneurysm: A Case Report

One of the rare cardiac anomaly is Sinus of Valsalva aneurysm (SVA) that can rupture spontaneously into other cardiac chambers or the pericardial space.1 Here, we report a rare case of a right coronary sinus of Valsalva aneurysm with rupture into the Left ventricular outflow tract (LVOT). Cardiovasc j 2023; 15(2): 182-184

Morphological analysis of ventricular septal defect by echocardiography for prediction of aortic regurgitation in pediatric population

Ventricular septal defects (VSD) are the most common congenital heart diseases in children. Among them, perimembranous VSD (pm-VSD) have a higher risk of complications, including aortic valve prolapse and aortic regurgitation (AR). The aim of our study was to assess echocardiographic criteria associated with AR during follow-up of pm-VSD. Forty children with restrictive pm-VSD, followed-up in our unit and who underwent a workable echocardiographic evaluation between 2015 and 2019 were included and retrospectively analyzed. The propensity score was used to match 15 patients with AR to 15 patients without AR. Median age was 2.2 year [1.4–5.7]. Median weight was 14 kg [9.9–20.3]. Aortic annulus z-score, Valsalva sinus z-score, sinotubular junction z-score, valve prolapse and commissure commitment were significantly different between the two groups (p = 0.047, p = 0.001, p = 0.010, p = 0.007, p < 0.001 respectively). Aortic root dilatation, aortic valve prolapse and commissure commitment to a perimembranous VSD are associated to aortic regurgitation.

Beta-Blocker use During Pregnancy Correlates with Less Aortic Root Dilatation in Patients with Marfan Syndrome.

Background Pregnant patients with Marfan syndrome (MFS) are at an increased risk for adverse aortic outcomes. While beta-blockers are used to slow aortic root dilatation in non-pregnant MFS patients, the benefit of such therapy in pregnant MFS patients remains controversial. The purpose of this study was to investigate the effect of beta-blockers on aortic root dilatation during pregnancy in MFS patients. Methods This was a longitudinal single-center retrospective cohort study of females with MFS who completed a pregnancy between 2004 and 2020. Clinical, fetal, and echocardiographic data were compared in patients on vs off beta-blockers during pregnancy. Results A total of 20 pregnancies completed by 19 patients were evaluated. Beta-blocker therapy was initiated or continued in 13 (65%) of the 20 pregnancies. Pregnancies on beta-blocker therapy experienced less aortic growth compared to those off beta-blockers (0.10cm [IQR: 0.10-0.20cm] vs 0.30cm [IQR: 0.25-0.35cm]; P=0.03). Using univariate linear regression, maximum systolic blood pressures (SBP), increase in SBP, and absence of beta-blocker use in pregnancy were found to be significantly associated with greater increase in aortic diameter during pregnancy. There were no differences in rates of fetal growth restriction between pregnancies on vs off beta-blockers. Conclusions This is the first study to evaluate changes in aortic dimensions in MFS pregnancies stratified by beta-blocker use. Beta-blocker therapy was found to be associated with less aortic root growth during pregnancy in MFS patients.