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.

[Selective Sinus Replacement for Aortic Root Disease].

BACKGROUND: Selective sinus replacement seems a reasonable option in cases requiring replacement of one or two sinuses of Valsalva, especially with acute aortic dissection and high bleeding risk. METHODS: Six patients (average age 58±17 years;five males) underwent selective replacement of the right sinus of Valsalva with right coronary artery bypass grafting (n=5) in 2015-2023. Five patients developed acute aortic dissection and one developed aneurysm of the right sinus of Valsalva. RESULTS: All patients survived the operation, and there were no cases requiring re-exploration for bleeding. Intraoperative transesophageal echocardiography showed trivial or less aortic regurgitation (AR) in all patients. Cardiopulmonary bypass time, aortic cross-clamping time, and lower body circulatory arrest time were 214±28 min, 159±22 min, and 31±6 min (n=5), respectively. During follow-up of 55±44 (4-104) months, all patients were asymptomatic. AR was mild or less in four patients, mild-moderate in one patient, and severe in one patient. All patients had normal cardiac function without left ventricular enlargement, and so no reoperation was required. CONCLUSIONS: Although this method appears to be relatively safe and effective, some patients developed late AR. Long-term follow-up of larger numbers of patients will be necessary to confirm its effectiveness.

[Standardized Valve Sparing Aortic Root Repair with Remodeling of Sinus of Valsalva].

The remodeling of sinus of Valsalva( Yacoub operation) is being standardized by adding annular fixation and technique for cusp prolapse as a refined method of valve sparing aortic root repair. It is important to design whole configurations of Valsalva sinus( diameters of aortic annulus and sino-tubular junction) according to the patient's geometric height (geometric height-oriented strategy). In this paper, we summarized our tips of remodeling of sinus of Valsalva operation.

An unusual cause of cardiac arrest in a young infant.

BACKGROUND: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age. CASE PRESENTATION: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm. CONCLUSIONS: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.

Right coronary sinus aneurysm repair.

In this case report, we describe the surgical treatment of a right coronary sinus aneurysm. A 69-year-old male patient was screened because of palpitations. He was finally diagnosed with an aneurysm of the sinus of Valsalva of the right coronary cusp. According to current aortic guidelines, surgical reconstruction was proposed. The patient underwent a cardiac operation through a median sternotomy under routine cardiopulmonary bypass. After aortic cross-clamping, the aorta was opened and the connection between the aorta and the aneurysm was clearly visualized, underneath the ostium of the right coronary artery. After excision of the right coronary button and the remaining right coronary sinus wall, this sinus was reconstructed with a Dacron graft, with subsequent coronary reimplantation. The postoperative course was uneventful. The patient was discharged on postoperative day 7. A complete sinus reconstruction was preferred over local patching of the defect because of the proximity of the aneurysm sac to the right coronary artery and the fragile, thin aortic tissue just underneath the coronary ostium.

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 and Left Coronary and Conus Arteries Originating from Three Separate Ostia in the Right Valsalva Sinus in a Japanese Cadaver: A Case Study with Literature Review.

A rare case of an anomalous location of the orifice of the coronary artery was found in a 99-year-old male cadaver undergoing routine dissection. The presence of the right coronary artery (RCA), left coronary artery (LCA), and conus artery (conus branch) originating from the right Valsalva sinus are the characteristic findings of this case. Then, the LCA passed through the aorta and the pulmonary artery. The LCA and RCA branches were normal. These findings are useful for future surgical procedures, including cardiac catheterization.

Effect of a Hypertensive Response During Exercise on Growth Rates of Aortic Diameters.

BACKGROUND: Aortic diameters are related to age, sex, and body size. There is a scarcity of data on the long-term sequelae of a hypertensive response to exercise (HRE) on aortic diameters. In this retrospective cohort study, we aimed to evaluate the relationship between the growth rates of the aorta in individuals with a HRE. METHODS: Our analysis included follow-up data of 649 patients recruited between January 2009 and December 2014 with a HRE. Participants with known connective tissue disease or a history of acute aortic syndrome were excluded. Sinus of Valsalva (SoV) and ascending aorta (AscAo) diameters were measured by transthoracic echocardiography using leading edge to leading edge convention at end-diastole. RESULTS: At baseline, median age, maximum systolic blood pressure (BP), body mass index (BMI), diameter of the SoV, and AscAo were 62 years, 208 mm Hg, 26.9 kg/m2, 35 mm, and 35 mm respectively. 32% of patients were female and 67% had hypertension. After a median follow-up of 7.1 years, mean yearly growth rates (±SD) of the SoV and AscAo were 0.09 (0.41) mm and 0.13 (0.56) mm, respectively. No significant associations were observed between growth rates of aortic diameters and maximum systolic and diastolic BP or when considering only individuals with a baseline diameter >40 mm. CONCLUSIONS: In this large cohort study, maximum systolic and diastolic BP during exercise showed no association with growth rates of aortic diameters. Furthermore, the mean growth rates of aortic diameters in this population were in line with growth rates in a normal population.

Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review.

Aneurysm of Aortic sinus of Valsalva (ASOV) dissecting into the interventricular septum (IVS) and rupturing into the left ventricle (LV) is a rare clinical diagnosis. Systemic inflammatory diseases like tuberculosis can aggravate this condition. We describe three cases of ASOV dissecting into the IVS and rupturing into the LV. All three patients underwent surgical intervention; two had a successful outcome. A literature review was conducted and19 previously reported cases were studied. The extent and direction of septal dissection determined the associated cardiac valvular and rhythm problems. Patch closure of the mouth of the aneurysm is the surgical method of choice. In the presence of multiple sinus tracts or if there is recurrence after surgical closure, aortic sinus or root replacement techniques have better outcomes.

Heparanase promotes the onset and progression of atherosclerosis in apolipoprotein E gene knockout mice.

BACKGROUND AND AIMS: Atherosclerosis is the primary underlying cause of myocardial infarction and stroke, which are the major causes of death globally. Heparanase (Hpse) is a pro-inflammatory extracellular matrix degrading enzyme that has been implicated in atherogenesis. However, to date the precise roles of Hpse in atherosclerosis and its mechanisms of action are not well defined. This study aims to provide new insights into the contribution of Hpse in different stages of atherosclerosis in vivo. METHODS: We generated Hpse gene-deficient mice on the atherosclerosis-prone apolipoprotein E gene knockout (ApoE-/-) background to investigate the impact of Hpse gene deficiency on the initiation and progression of atherosclerosis after 6 and 14 weeks high-fat diet feeding, respectively. Atherosclerotic lesion development, blood serum profiles, lesion composition and aortic immune cell populations were evaluated. RESULTS: Hpse-deficient mice exhibited significantly reduced atherosclerotic lesion burden in the aortic sinus and aorta at both time-points, independent of changes in plasma cholesterol levels. A significant reduction in the necrotic core size and an increase in smooth muscle cell content were also observed in advanced atherosclerotic plaques of Hpse-deficient mice. Additionally, Hpse deficiency reduced circulating and aortic levels of VCAM-1 at the initiation and progression stages of disease and circulating MCP-1 levels in the initiation but not progression stage. Moreover, the aortic levels of total leukocytes and dendritic cells in Hpse-deficient ApoE-/- mice were significantly decreased compared to control ApoE-/-mice at both disease stages. CONCLUSIONS: This study identifies Hpse as a key pro-inflammatory enzyme driving the initiation and progression of atherosclerosis and highlighting the potential of Hpse inhibitors as novel anti-inflammatory treatments for cardiovascular disease.

Giant unruptured sinus of Valsalva aneurysms causing angina pectoris.

BACKGROUND: Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly which can be congenital or acquired. Patients with SVA are commonly asymptomatic when the occupying effect of SVA is insignificant, while ruptured SVA usually causes severe symptoms including heart failure and myocardial ischemia. CASE PRESENTATION: We present an unusual case of a 64-year-old female manifesting with exertional dyspnea as well as angina pectoris for three months. Echocardiography and cardiac computed tomographic angiography confirmed unruptured left-coronary and non-coronary SVAs. The left anterior descending artery and left circumflex artery were stretched and compressed by the SVA which causing myocardial ischemia. The patient finally received aortic root replacement (Bentall procedure) and got symptom relieved. CONCLUSIONS: Giant unruptured SVA originating from left coronary sinus is extremely rare. Our case highlights that giant SVA should be considered in cases with angina pectoris. Echocardiography and coronary computed tomographic angiography are useful and important for diagnosis. Surgery is highly recommended in patients with SVA.

A report of fifty cases with incidental diagnosis of anomalous origin of the right coronary artery from the left sinus of Valsalva.

INTRODUCTION: Anomalous origin of the right coronary artery from the left sinus of Valsalva (R-ACAOS) is a relatively rare condition that can potentially lead to devastating outcomes. The current study aims to investigate the cardiac-related disorders among patients with incidental R-ACAOS diagnosis through computed tomography angiography (CTA). METHODS: The current cross-sectional study has been conducted on 50 patients diagnosed with R-ACAOS who underwent CTA. Based on CTA, the patients' were categorized as R-ACAOS with interarterial course and non-interarterial course. The demographic and medical characteristics, any history of cardiac intervention and New York Heart Association (NYHA) Functional Classification at the time of diagnosis were recruited. Patients were revisited to assess cardiac-associated variables, including symptoms, the presence of heart failure and current NYHA function class. RESULTS: The variables including the history of cardiac intervention (P-value<0.001), the presence of heart failure (P-value = 0.010) and NYHA function class at the time of diagnosis (P-value = 0.006) were remarkably higher among those with interarterial course of R-ACAOS; while, the other variables including chest pain at rest (P-value = 0.55) or on exertion (P-value = 0.12), current NYHA function class, current cardiac-associated symptoms except for dyspnea at rest (P-value = 0.012), mortality and coronary calium score did not differ (P-value>0.05). coronary interventions led to significantly improved NYHA function class (P-value<0.05). CONCLUSION: Based on the findings of the current study, R-ACAOS with interarterial course leads to significantly higher rates of atherosclerotic-related symptoms and events compared with the other types of RCA anomalies. Moreover, coronary interventions led to significantly improved NYHA functional class regardless of R-ACAOS category.

Aortic Root Dimension Using Transthoracic Echocardiography: Results from the Copenhagen City Heart Study.

Findings regarding the relation between aortic size and risk factors are heterogeneous. This study aimed to generate new insights from a population-based adult cohort on aortic root dimensions and their association with age, anthropometric measures, and cardiac risk factors and evaluate the incidence of acute aortic events. Participants from the fifth examination round of the Copenhagen City Heart study (aged 20 to 98 years) with applicable echocardiograms and no history of aortic disease or valve surgery were included. Aorta diameter was assessed at the annulus, sinus of Valsalva, sinotubular junction, and the tubular part of the ascending aorta. The study population comprised 1,796 men and 2,316 women; mean age: 56.4 ± 17.0 and 56.9 ± 18.1 years, respectively. Men had larger aortic root diameters than women regardless of height indexing (p <0.01). Age, height, weight, systolic and diastolic blood pressure, mean arterial pressure, pulse pressure, hypertension, diabetes, ischemic heart disease, and smoking were positively correlated with aortic sinus diameter in the crude and gender-adjusted analyses. However, after full adjustment, only height, weight, and diastolic blood pressure remained significantly positively correlated with aortic sinus diameter (p <0.001). For systolic blood pressure and pulse pressure, the correlation was inverse (p <0.001). During follow-up (median 5.4 [quartile 1 to quartile 3 4.5 to 6.3] years), the incidence rate of first-time acute aortic events was 13.6 (confidence interval 4.4 to 42.2) per 100,000 person-years. In conclusion, beyond anthropometric measures, age, and gender, diastolic blood pressure was the only cardiac risk factor that was independently correlated with aortic root dimensions. The number of aortic events during follow-up was low.

RV1+RV3 Index to Differentiate Idiopathic Ventricular Arrhythmias Arising From Right Ventricular Outflow Tract and Aortic Sinus of Valsalva: A Multicenter Study.

BACKGROUND: This study aimed to investigate the predictive value of parameters of every precordial lead and their combinations in differentiating between idiopathic ventricular arrhythmias (IVAs) from the right ventricular outflow tract and aortic sinus of Valsalva (ASV). METHODS AND RESULTS: Between March 1, 2018, and December 1, 2021, consecutive patients receiving successful ablation of right ventricular outflow tract or ASV IVAs were enrolled. The amplitude and duration of the R wave and S wave were measured in every precordial lead during IVAs. These parameters were either summed, subtracted, multiplied, or divided to create different indexes. The index with the highest area under the curve to predict ASV IVAs was developed, compared with established indexes, and validated in an independent prospective multicenter cohort. A total of 150 patients (60 men; mean age, 45.3±16.4 years) were included in the derivation cohort. The RV1+RV3 index (summed R-wave amplitude in leads V1 and V3) had the highest area under the curve (0.942) among the established indexes. An RV1+RV3 index >1.3 mV could predict ASV IVAs with a sensitivity of 95% and a specificity of 83%. Its predictive performance was maintained in the validation cohort (N=109). In patients with V3 R/S transition, an RV1+RV3 index >1.3 mV could predict ASV IVAs, with an area under the curve of 0.892, 93% sensitivity, and 75% specificity. CONCLUSIONS: The RV1+RV3 index is a simple and novel criterion that accurately differentiates between right ventricular outflow tract and ASV IVAs. Its performance outperformed established indexes, making it a valuable tool in clinical practice.

Morphological and dynamic analysis of the normal aortic valve with 4D computed tomography.

OBJECTIVES: To evaluate the precise dimensions of the normal aortic root, especially the true aortic annulus, during the cardiac cycle using an innovative reconstruction method based on multiphase cardiac computed tomography and to assess the feasibility and the reproducibility of this method for aortic root analysis. METHODS: Between January 2019 and June 2021, 30 optimal consecutive ECG-gated multiphase cardiac computed tomography of patients with normal tricuspid aortic valve were analysed using an in-house software. Aortic annulus border was pinpointed on 9 reconstructed planes and the 3D coordinates of the 18 consecutive points were interpolated into a 3D curve using a cubic spline. Three additional planes were generated at the level of the left ventricular outflow tract, the level of the Valsalva sinus and the level of the sinotubular junction. This procedure was repeated for all the 10 temporal phases of the RR interval. RESULTS: The aortic annulus mean 3D and 2D areas were 7.67 ± 1.51 and 5.16 ± 1.40 cm2, respectively. The mean 2D diameter was 2.51 ± 0.23 cm. The mean global area expansion was 11.8 ± 3.5% and the mean perimeter expansion of 7.1 ± 2.6%. During the cardiac cycle, the left ventricle outflow tract expands, reaching its maximum surface at the end of diastole, followed by the aortic annulus, the Valsalva sinuses and the sinotubular junction. The aorta changes from a clover-shaped cone during diastole to more cylindrical shape during systole. Compared to the 3D measurements, the analysis of the virtual basal ring significantly underestimates the annulus area, perimeter, and mean diameter. CONCLUSIONS: 4D morphometric analysis enables to have a precise and reproducible evaluation of the aortic annulus. The aortic annulus and root are deformable structures that undergo a unique expansion sequence during the cardiac cycle which should be considered for procedural planning.

Spontaneous rupture of the right aortic sinus resulting in a sterile aorto-atrial fistula in a pet rabbit (Oryctolagus cuniculus).

Aortocardiac fistula is a broad term used to describe defects between the aorta and other cardiac chambers that can occur in humans and animals. A 1.5-year-old, 1.7 kg, male castrated Holland lop rabbit (Oryctolagus cuniculus) was presented for a two-week history of a heart murmur with corresponding cardiomegaly on radiographs. Physical examination confirmed a grade-V/VI continuous heart murmur on the right sternal border with a regular rhythm and a gallop sound. Echocardiography revealed an aortic-to-right-atrial fistula causing severe left-sided volume overload. Based on the echocardiographic findings, rupture of the right aortic sinus was suspected. Due to the poor prognosis, euthanasia was elected. On necropsy, a fistula was found connecting the right aortic sinus with the right atrium, without evidence of an inflammatory response nor evidence of an infectious etiology. The sudden onset of a heart murmur supported acquired fistulation from a ruptured aortic sinus (also known as the sinus of Valsalva), though a congenital malformation could not be completely excluded.

Aorto-atrial tunnel arising from sinus of Valsalva aneurysm in a young female.

A young female patient presented with complaints of breathlessness and palpitations since 2 years. On clinical examination, there was a loud continuous murmur at the right sternal border.Transthoracic echocardiography and colour Doppler showed a tunnel-like structure originating from the aneurysmal right coronary sinus and opening into the right atrium with left to right shunt. Coronary angiography revealed a large tunnel beginning in right coronary sinus and terminating in the right atrium and right coronary artery (RCA) was seen originating from the tunnel. Cardiac catheterisation revealed normal pulmonary artery pressure. CT and 3D-reconstructed images delineated the extracardiac course of the tunnel.Various treatment modalities including percutaneous transcatheter approach and surgical treatment were taken into consideration, but because of the close proximity of RCA from the tunnel opening, surgical closure was preferred.