A rare case of giant left sinus of Valsalva aneurysm complicated by thrombus formation.

A rare transthoracic echocardiographic image of left sinus of Valsalva aneurysm complicated by thrombus formation.

Right Sinus of Valsalva Aneurysm Rupturing into the Right Atrium and Dissecting into the Interventricular Septum.

The right sinus of the Valsalva aneurysm (SVA) rupturing into the right atrium (RA) and dissecting into the interventricular septum (IVS) is rare. The disease can be definitively diagnosed using two-dimensional (2D) echocardiography and color Doppler ultrasonography. Real-time biplane imaging and three-dimensional (3D) echocardiography offer new perspectives for viewing and diagnosing this disease.

Aortic root morphometry revisited-Clinical implications for aortic valve interventions.

The complex anatomy of the aortic root is of great importance for many surgical and transcatheter cardiac procedures. Therefore, the aim of this study was to provide a comprehensive morphological description of the nondiseased aortic root. We morphometrically examined 200 autopsied human adult hearts (22.0% females, 47.9 ± 17.7 years). A meticulous macroscopic analysis of aortic root anatomy was performed. The largest cross-section area of the aortic root was observed in coaptation center plane (653.9 ± 196.5 mm2), followed by tubular plane (427.7 ± 168.0 mm2) and basal ring (362.7 ± 159.1 mm2) (p < 0.001). The right coronary sinus was the largest (area: 234.3 ± 85.0 mm2), followed by noncoronary sinus (218.7 ± 74.8 mm2) and left coronary sinus (201.2 ± 78.08 mm2). The noncoronary sinus was the deepest, followed by right and left coronary sinus (16.4 ± 3.2 vs. 15.9 ± 3.1 vs. 14.9 ± 2.9 mm, p < 0.001). In 68.5% of hearts, the coaptation center was located near the aortic geometric center. The left coronary ostium was located 15.6 ± 3.8 mm above sinus bottom (within the sinus in 91.5% and above sinutubular junction in 8.5%), while for right coronary ostium, it was 16.2 ± 3.5 mm above (83.5% within sinus and 16.5% above). In general, males exhibited larger aortic valve dimensions than females. A multiple forward stepwise regression model showed that anthropometric variables might predict the size of coaptation center plane (age, sex, and heart weight; R2 = 31.8%), tubular plane (age and sex; R2 = 25.6%), and basal ring (age and sex; R2 = 16.9%). In conclusion, this study presents a comprehensive analysis of aortic-root morphometry and provides a platform for further research into the intricate interplay between structure and function of the aortic root.

Retractable congestive heart failure due to sinus valsalva rupture with coexisting gerbode defect: a rare case report.

Sinus of Valsalva aneurysm (SoVA) is a rare disease with less than 1% prevalence in the population. Most cases are asymptomatic, however, significant clinical manifestations are possible due to fistula formation and sudden rupture resulting in cardiac shunt. Eventually it may develop into progressive heart failure with high morbidity. We report the case of a 33 year old female patient who presented with shortness of breath, ascites, and recurring hospitalisation. The cardiac examination revealed sinus tachycardia along with loud and continuous murmurs on the left parasternal border. Several standard diagnostic procedures could not be performed due to malignant arrhythmia in supine position. Echocardiography examination revealed SoV rupture with a gerbode defect, which was the underlying cause of severe retractable heart failure.

Aneurysms involving the coronary arteries in a neonate with neurofibromatosis 1.

Aneurysmal coronary artery disease (ACAD) has been reported rarely in patients with neurofibromatosis type 1 (NF1), mostly in adults. We report on a female newborn affected by NF1 with ACAD disclosed during investigation for an abnormal prenatal ultrasound along with a review of the previously reported cases. The proposita had multiple café-au-lait spots and had no cardiac symptoms. Echocardiography, and cardiac computed tomography angiography confirmed aneurysms on the left coronary artery, left anterior descending coronary artery, and of the sinus of Valsalva. Molecular analysis detected the pathogenic variant NM_001042492.3(NF1):c.3943C>T (p.Gln1315*). Literature findings on ACAD in NF1 indicated that this mostly occurs in males, showing predilection for the development of aneurysms at the left anterior descending coronary artery, and manifesting predominantly as acute myocardial infarction, inclusively in teenagers, though it may be also asymptomatic as in our case. This report documents the first case of ACAD in a patient with NF1 diagnosed at birth, emphasizing that its early diagnosis is essential to prevent potential life-threatening events attributable directly to coronary lesions.

Resolution of aortic regurgitation due to Venturi effect after device closure of ruptured sinus of Valsalva aneurysm.

We describe a case of a ruptured sinus of Valsalva with severe aortic regurgitation treated by transcatheter device closure. The regurgitation was purely due to leaflet entrapment because of the hemodynamic Venturi effect which was effectively treated.

Ruptured sinus of Valsalva aneurysm with dynamic aortic regurgitation.

The ruptured sinus of Valsalva aneurysm (SVA) can present with dynamic aortic regurgitation (AR). Hemodynamic changes elicited by induction of general anesthesia can lead to dynamic AR in setting of ruptured SVA. Perioperative echocardiography is critical in understanding the etiology of AR and in guiding surgical decision-making. If the aortic valve is structurally normal, AR may resolve following patch repair of the SVA rupture defect. Conventional measures of assessing AR severity are not accurate with continuous left-to-right flow across a ruptured SVA.

Unruptured sinus of valsalva aneurysm diagnosed by multimodal imaging.

We describe a rare case of unruptured right SOVA in a 52-year-old man who was successfully treated with right SOVA repair and right coronary artery reconstruction. Our case demonstrates the usefulness of transthoracic echocardiography, contrastive echocardiography, and transesophageal echocardiography in diagnosing SOVA.

Aortic valve fenestrations: Macroscopic assessment and functional anatomy study.

Aortic valve fenestrations are defined as a loss of aortic valve leaflet tissue. They are a common but overlooked finding with unclear significance. The aim of this study was to investigate the varied functional anatomies of aortic valve fenestrations. A total of 400 formalin-fixed autopsied human hearts were macroscopically assessed and the function of the aortic valve of 16 reanimated human hearts were imaged using Visible Heart® methodologies. Aortic valve leaflet fenestrations were present in 43.0% of autopsied hearts (in one leaflet in 24.0%, in two leaflets 16.0%, in all leaflets 3.0%). Fenestrations were mostly present in left (25.5%) followed by right (23.3%) and noncoronary leaflet (16.3%). In 93.8% of cases, the fenestrations form clusters and were mainly located at the free edge of the leaflet in the commissural area (95.4%). Hearts with aortic valve fenestrations had significantly larger aortic valve diameters and aortic valve areas (p < 0.001). The average surface area sizes of fenestrations were 23.8 ± 16.6 mm2 , and the areas were largest for left followed by right and noncoronary leaflet fenestrations (p < 0.001). The fenestration areas positively correlated with donor age (r = 0.31; p = 0.02). Significant hypermobility and subjective weakening of the leaflet adhesion levels of the fenestrated regions were observed. In conclusion, fenestrations of the aortic leaflets are frequent, and their sizes may be significant. They occur in all age groups, yet their size increase with aging. Fragments of leaflets with fenestrations show different behaviors during the cardiac cycle versus unchanged areas.

Rupture of a non sinus of Valsalva aneurysm during pregnancy: Case report and review of literatures.

Ruptured Sinus of Valsalva aneurysms during pregnancy is rare and presents a threat both to the mother and the fetus. We report a case of ruptured nonsinus of Valsalva aneurysms in a 26-year-old woman diagnosed at 32+4 weeks of gestation. A successful elective lower-segment cesarean section was conducted under general anesthesia. A successful surgical correction of the ruptured aneurysm under cardiopulmonary bypass (CPB) was performed with patch repair after 13 days. A multidisciplinary approach with respect to the pregnant patient's diagnosis, indications, and timing of surgery is necessary in ensuring the best possible outcomes for both the mother and the child.

Sinus of Valsalva Dimension and Clinical Outcomes in Patients Undergoing Transcatheter Aortic Valve Implantation.

BACKGROUND: Ascending aortic root anatomy is routinely evaluated on pre-procedural multi-detector computed tomography (MDCT). However, its clinical significance has not been adequately studied. We aimed to investigate the impact of the sinus of Valsalva (SOV) dimension on clinical outcomes in patients undergoing transcatheter aortic valve implantation (TAVI). METHODS: In a prospective TAVI registry, we retrospectively assessed SOV dimensions by pre-procedural MDCT. Patients were stratified according to tertiles of SOV diameter indexed to body surface area (SOVi). The primary endpoint was all-cause mortality at 1 year. RESULTS: Among 2066 consecutive patients undergoing TAVI between August 2007 and June 2018, 1554 patients were eligible for the present analysis. Patients in the large SOVi group were older (83 ± 6 vs 82 ± 6 vs 81 ± 6; P < .001) and had a higher Society of Thoracic Surgeons Predicted Risk of Mortality (6.3 ± 3.8 vs 5.1 ± 3.1 vs 4.9 ± 3.5; P < .001) than those in the other groups. Patients in the large SOVi group had a higher incidence of moderate or severe paravalvular regurgitation (11.9% vs 4.5% vs 3.5%; P < .001). At 1 year, a large SOVi was independently associated with an increased risk of mortality (HR: 1.62; 95% CI: 1.19-2.21; P = .002) and major or life-threatening bleeding (HR: 1.30; 95% CI: 1.02-1.65; P = .035). CONCLUSIONS: Dilatation of the aortic root at the SOV was associated with adverse outcomes after TAVI. The assessment of the aortic root should be integrated into the risk stratification system in patients undergoing TAVI.

Ventricular tachycardia targeted in the aortic sinuses of Valsalva in patients with prior myocardial infarction.

INTRODUCTION: Ventricular tachycardia (VT) in structurally normal hearts or nonischemic cardiomyopathy can originate from the aortic sinuses of Valsalva (SoV). It is unknown whether VT can originate from the SoVs in patients with prior myocardial infarction (MI). OBJECTIVE: To evaluate the prevalence, arrhythmogenic substrate, and ablation outcomes of postinfarction VT originating from the SoVs. METHODS: Among 217 consecutive patients with postinfarction VT undergoing ablation, we identified 13 (6%) patients who had ≥1 VT mapped in a SoV. Control groups of 13 patients with idiopathic SoV VT and 13 postinfarction patients without SoV VT were included. RESULTS: In the study group, 17 VTs were mapped in a SoV (right n = 5, left-right commissure n = 6, left n = 6). SoV VT target sites had low bipolar voltage during sinus rhythm [median 0.42 (IQR: 0.16-0.53) mV] which was significantly lower than target sites in patients with idiopathic SoV VTs [median 1.02 (IQR: 0.89-1.52) mV; p < .001]. An area of endocardial low voltage was found below the aortic valve in all patients with postinfarction SoV VTs compared to 9 (69%) of the patients in the postinfarction control group without SoV VT (p = .02). Morphology characteristics of postinfarction SoV VTs differed from idiopathic SoV VTs. None of the postinfarction SoV VTs were inducible after ablation and none recurred after a median follow-up of 14 months. CONCLUSION: In patients with prior MI, VT can be targeted in an aortic SoV. The SoVs should be routinely investigated in postinfarction patients with inferior axis VT and an area of low voltage below the aortic valve.

Rupture of sinus of Valsalva aneurysm: a case report in a child.

BACKGROUND: Sinus of Valsalva aneurysm (SVA) is a rare congenital disease that can cause severe clinical presentations when the aneurysm ruptures. Here, we report a rare case of a noncoronary sinus of Valsalva aneurysm with rupture into the right atrium. CASE PRESENTATION: A 14-year-old Chinese female patient presented viral myocarditis with acute heart failure at the local hospital, and she was finally diagnosed with a noncoronary sinus Valsalva aneurysm with rupture into the right atrium by digital subtraction angiography with cardiac catheterization angiography and echocardiography at our hospital (Children's Hospital of Chongqing Medical University). Percutaneous closure intervention was performed shortly after her diagnosis, and the patient showed good functional recovery. CONCLUSIONS: We report a case of ruptured sinus of Valsalva aneurysm successfully treated by percutaneous closure, which is an excellent alternative treatment.

Incidental detection of ruptured sinus of Valsalva aneurysm and coronary abnormality in a patient with penetrating chest trauma.

Aortic sinus of Valsalva aneurysm is a rare congenital or acquired anomaly with a potential for rupture into adjacent cardiac chambers. Ruptured aneurysm of the sinus of Valsalva leads to volume overload of the receiving chamber and should be repaired as soon as the diagnosis is made. Here, we present a young patient with history of chest trauma leading to discovery of an asymptomatic ruptured sinus of Valsalva aneurysm.

Percutaneous closure of a ruptured right coronary sinus of Valsalva aneurysm that mimics severe aortic regurgitation.

A 34-year-old male was admitted with presumed acute, severe aortic regurgitation. Multimodal imaging was performed and showed a ruptured right coronary sinus of Valsalva aneurysm into the right atrium. He underwent a percutaneous closure of the ruptured sinus of Valsalva aneurysm. The patient had major clinical improvement.

Sinus of valsalva aneurysm: A single institutional experience with 216 patients over 30 years.

BACKGROUND: Sinus of valsalva aneurysm (SVA) with rupture is a rare cardiac anomaly which can be congenital or acquired with reported incidence of 0.46%-3.57% among Asians population. AIM OF THE STUDY: The aim of this study is to analyze 30 years of single institutional surgical experience in management of 216 cases with SVAs from 1992 till date. METHODS: Age group was from 6 to 64 years (mean: 32.5 ± 11 years) with male to female ratio of 2.2:1. The aneurysms originated from right coronary sinus in 181 cases (83.79%), noncoronary sinus in 35 cases (15.74%) and ruptured into the right ventricle in 149 cases (68.98%), right atrium in 59 cases (27.31%). Bicameral approach was used in majority of the cases (n = 213, 98.61%). Aneurysms were repaired using Dacron patch in 173 cases (80.09%) and direct closure in 43 cases (19.9%). Associated ventricular septal defect was closed with Dacron patch in 123 cases (56.94%). Aortic valve was replaced in 21 cases (9.72%) and aortic valve repair was performed in 14 cases (6.48%) for associated Aortic regurgitation. RESULTS: There were no perioperative hospital deaths. Follow-up was available in 204 patients (94.44%) ranging from 2 to 26 years (mean: 10 ± 5.6 years). Two deaths (0.92%) occurred during the postoperative follow-up period. The actual survival was 99.5% at 1 year, 99% at 5 and 10 years. CONCLUSION: Long term results of surgically repaired SVAs are good with low morbidity (3.24%) and mortality (0.92%) even when associated with major cardiac anomalies. Aortic valve repair and replacement both are equally feasible alternatives for management of moderate to severe aortic regurgitation with associated merits and demerits.

Risk Factors for Aortic Regurgitation Progression After Repair of Sinus of Valsalva Aneurysm.

BACKGROUND: The main treatment for a ruptured sinus of Valsalva aneurysm (SVA) is surgical repair. Postoperative progression of aortic regurgitation (AR) following SVA repair increases the risk of reoperation, which decreases the long-term survival. Thus, identifying the risk factors for postoperative AR progression is of great significance. METHODS: Adult patients who were diagnosed with ruptured SVA and underwent surgical repair at the current centre were reviewed. Necessary data in the institutional database were extracted. The perioperative and follow-up assessments of the aortic valve by transthoracic echocardiography were also obtained. The aortic regurgitation progression was grouped into three categories: newly developing, recurrence, and worsening. Sixteen (16) variables were screened to identify potential risk factors by univariate logistic regression analysis or Chi-squared test. Variables with p-values <0.1 were further analysed by multivariate logistic regression models to find independent risk factors. RESULTS: A total of 198 consecutive patients from June 2006 to January 2018 were included. The overall incidence of postoperative AR progression was 19.2% (38 of 198). After the univariate analysis, SVA originating from the right coronary sinus, coexisting with ventricular septal defect, larger diameter of aortic annulus, and larger cardiothoracic ratio were screened as potential risk factors. Multivariate analysis indicated that coexisting with a ventricular septal defect (VSD) (OR, 2.82; 95% CI, 1.217-6.532; p=0.016) and larger cardiothoracic ratio (OR, 1.061; 95% CI, 1.001-1.124; p=0.047) were independent risk factors for postoperative AR progression. CONCLUSIONS: To prevent postoperative AR progression after surgical repair, more careful inspection and appropriate surgical techniques are necessary for patients coexisting with VSD or with a larger cardiothoracic ratio.

Comprehensive Ex Vivo Comparison of 5 Clinically Used Conduit Configurations for Valve-Sparing Aortic Root Replacement Using a 3-Dimensional-Printed Heart Simulator.

BACKGROUND: Many graft configurations are clinically used for valve-sparing aortic root replacement, some specifically focused on recapitulating neosinus geometry. However, the specific impact of such neosinuses on valvular and root biomechanics and the potential influence on long-term durability are unknown. METHODS: Using a custom 3-dimenstional-printed heart simulator with porcine aortic roots (n=5), the anticommissural plication, Stanford modification, straight graft (SG), Uni-Graft, and Valsalva graft configurations were tested in series using an incomplete counterbalanced measures design, with the native root as a control, to mitigate ordering effects. Hemodynamic and videometric data were analyzed using linear models with conduit as the fixed effect of interest and valve as a fixed nuisance effect with post hoc pairwise testing using Tukey's correction. RESULTS: Hemodynamics were clinically similar between grafts and control aortic roots. Regurgitant fraction varied between grafts, with SG and Uni-Graft groups having the lowest regurgitant fractions and anticommissural plication having the highest. Root distensibility was significantly lower in SG versus both control roots and all other grafts aside from the Stanford modification (P≤0.01 for each). All grafts except SG had significantly higher cusp opening velocities versus native roots (P<0.01 for each). Relative cusp opening forces were similar between SG, Uni-Graft, and control groups, whereas anticommissural plication, Stanford modification, and Valsalva grafts had significantly higher opening forces versus controls (P<0.01). Cusp closing velocities were similar between native roots and the SG group, and were significantly lower than observed in the other conduits (P≤0.01 for each). Only SG and Uni-Graft groups experienced relative cusp closing forces approaching that of the native root, whereas relative forces were >5-fold higher in the anticommissural plication, Stanford modification, and Valsalva graft groups. CONCLUSIONS: In this ex vivo modeling system, clinically used valve-sparing aortic root replacement conduit configurations have comparable hemodynamics but differ in biomechanical performance, with the straight graft most closely recapitulating native aortic root biomechanics.

Ruptured sinus of valsalva aneurysm presenting as syncope and hypotension: a case report.

BACKGROUND: Unruptured sinus of valsalva aneurysm (SOVA) are typically asymptomatic, and hence can be easily ignored. Ruptured sinus of valsalva aneurysm (RSOVA) usually protrude into the right atrium or ventricular. However, in this case, the RSOVA protruded into the space between the right atrium and the visceral pericardium leading to compression of the right proximal coronary artery. Very few such cases have been reported till date. CASE PRESENTATION: We describe a case of ruptured right SOVA in a 61-year-old man with syncope and persistent hypotension. At the beginning, considered the markedly elevated troponin, acute myocardial infarction was considered. However, emergency coronary angiography unexpectedly revealed a large external mass compressed right coronary artery (RCA) resulting in severe proximal stenosis. Then, aorta computed tomography angiography (CTA) and urgent surgery confirmed that the ruptured right SOVA led to external compression of the right proximal coronary artery. Finally, ruptured right SOVA repair and RCA reconstruction were successfully performed, and the patient was discharged with no residual symptoms. CONCLUSIONS: It is very important to be vigilant about the existence of SOVA. RSOVA should be suspected in a patient presenting with acute hemodynamic compromise, and echocardiography should be immediately performed. Moreover, it is very important to achieve dynamic monitoring by using cardiac color ultrasound. Definitive diagnosis often requires cardiac catheterization, and an aortogram should be performed unless endocarditis is suspected.

Myocardial dissection complicating left sinus of Valsalva aneurysm in silent takayasu arteritis.

BACKGROUND: Myocardial dissection (MD) in a left sinus of Valsalva aneurysm (LSVA) is a rare condition that may lead to a fatal complication. Determining the MD etiology is challenging because of various possibilities ranging from congenital to acquired diseases. Here, we discuss an approach for determining the etiology of MD complicating LSVA in Takayasu arteritis (TA) and its treatment. CASE PRESENTATION: A 41-year-old man presented with dyspnea on heavy activities and a history of consciousness loss at the age of 24 years. He was diagnosed with dilated cardiomyopathy and MD complicating LSVA in TA based on combined clinical and pathognomonic diagnostic criteria of TA evaluated using vascular Doppler and computed tomography angiography of the aorta. The patient refused to undergo surgery and received an optimal dose of chronic heart failure therapy, a high-dose steroid, and azathioprine. The patient experienced some improvements in clinical condition, functional outcome, and inflammatory markers at 1-year follow-up. CONCLUSIONS: Clinical criteria and various imaging modalities may be used to determine the etiology of MD complicating LSVA in silent TA. As an alternative to surgery, the optimal medical treatment might result in a satisfactory outcome.