Unusual Presentation of Heart Failure Secondary to Ruptured Aneurysmal Sinus of Valsalva: A Case Report

Sinus of Valsalva Aneurysm (SOVA) arises from an abnormal dilation of the aortic root, leading to enlargement between the aortic annulus and the sinotubular junction. Although often presenting without symptoms, these aneurysms bear the potential for life-threatening complications, primarily from the looming risk of rupture. We present the case of a 42-year-old Malay gentleman with a history of bilateral pedal edema and dyspnea on exertion who was diagnosed with a ruptured sinus of Valsalva aneurysm. The patient underwent successful surgical repair of the aneurysm, leading to symptomatic improvement and favorable outcomes. This case highlights the importance of early diagnosis and prompt surgical intervention in managing this uncommon condition.

Giant cavernous aneurysms occluded by aneurysmal thrombosis,calcification,parent artery occlusion:A case report and review of literature

BACKGROUND Patients with giant intracranial aneurysms(GIAs)are at a high risk of rupture,morbidity,and mortality even after surgical or endovascular treatment.We described a case of a spontaneously occluded GIA secondary to gradual growth of the GIA,continuously progressed aneurysmal thrombosis,complete aneurysmal calcification and complete occlusion of the parent artery-the right internal carotid artery(RICA).CASE SUMMARY A 72-year-old female patient complained of sudden pain in her right eye upon admission to our hospital.She had been diagnosed with a GIA[30 mm(axial)×38 mm(coronal)×28 mm(sagittal)]containing an aneurysmal thrombus located in the cavernous sinus segment of RICA diagnosed by magnetic resonance imaging(MRI),enhanced MRI,and magnetic resonance angiography more than 14 years ago.Later,with slow growth of the cavernous carotid GIA,aneurysmal thrombosis progressed continuously,spontaneous occlusion of the RICA,complete aneurysmal calcification,and occlusion of the GIA occurred gradually.She had no history of subarachnoid hemorrhage but missed the chance for endovascular therapy at an early stage.As a result,she was left with severe permanent sequelae from the injuries to the right cranial nerves Ⅱ,Ⅲ,Ⅳ,V1/V2,and Ⅴ.CONCLUSION The risk of rupture of the cavernous carotid GIAs was relatively low and possibly further be reduced by the stasis flow and spontaneous occlusion of the parent artery internal carotid artery(ICA)induced by the mass effect of the cavernous carotid GIAs and the extremely rare aneurysmal calcification.However,nowadays,it is advisable to recommend early endovascular treatment for the cavernous carotid GIAs to prevent injuries to the surrounding intracranial nerves and occlusion of the ICA,mainly caused by the mass effect of the cavernous carotid GIAs.

Noncoronary sinus of Valsalva rupture into the right atrium with a coexisting perimembranous ventricular septal defect

Ruptured sinus of Valsalva is very uncommon,and is < 1% of all congenital defects.The incidence ranges from 0.1%-3.5%.There is a male to female predominance of 4:1,with the highest incidence in the Asian population.Higher incidence is also seen in patients with Marfan's syndrome and Ehlers Danlos syndrome.There is a higher association of ruptured sinus of Valsalva with ventricular septal defect(VSD),aortic stenosis,and bicuspid valve defect.While most patients with VSD often have rupture of their right coronary sinus of Valsalva into the right ventricle due to poor structural integrity,we present a rare case of a patient with VSD who had rupture of his noncoronary sinus of Valsalva into the right atrium.

Unruptured Right Sinus of Valsalva Aneurysm Dissecting into Interventricular Septum Causing Complete Heart Block: Can Early Surgical Correction Revert Rhythm Disturbances?

A sinus of Valsalva aneurysm (SOVA) is abnormal dilatation of the either aortic sinuses, area of the aortic root between the aortic valve annulus and the sinotubular junction. Their clinical presentation may range from being asymptomatic as an incidental finding on cardiac imaging to symptomatic presentations related to the compression of adjoining structures or intracardiac shunting caused by rupture of the SOVA mostly into the right side of the heart. The compression leads to findings of tricuspid valve regurgitation, right ventricular outflow tract (RVOT) obstruction and rarely complete heart block (CHB). Dissection or erosion into interventricular septum is one of the rarest complications of SOVA. The symptomatic presentation is almost always a surgical emergency. Here we present a case report of a patient with unruptured sinus of valsalva originating from right sinus dissecting into interventricular septum causing complete heart bock. In this case after surgical correction the complete heart block reverted to sinus rhythm.

Aortic Sinus of Valsalva Aneurysm Isolation by Radiofrequency Ablation in Outflow Tract Ventricular Tachycardia

Idiopathic monomorphic ventricular tachycardia and premature ventricular complexes (PVCs) commonly arise from the right and left ventricular outflow tracts (VOT). Their mechanism is most commonly triggered activity from delayed after-depolarizations and successful ablation is performed at the site of earliest endocardial activation. Re-entrant mechanisms have been rarely described. We report a case of an otherwise healthy patient who ultimately underwent six electro-physiology studies (EPS) and suffered numerous implantable cardiac defibrillator (ICD) discharges prior to the successful radiofrequency ablation (RFA) of two idiopathic VOT tachycardias. During the sixth EPS, a proximal aortogram demonstrated a left aortic sinus of valsalva (LASV) aneurysm. Subsequntly, a novel and successful RFA strategy of aneurysm isolation was undertaken. The presence of multiple clinical or inducible VT morphologies and the characterization of a VT as re-entrant should raise concerns that a complex arrhythmogenic substrate is present and defining the anatomy with angiography or an alternative imaging modality is essential in achieving a successful ablation strategy.

Anatomical relevance of ablation to the pulmonary artery root:clinical implications for characterizing the pulmonary sinus of Valsalva and coronary artery

Background and Objective Ablation within the pulmonary sinus of Valsalva (PSV) becomes increasingly common in certain ventricular outflow arrhythmia. Understanding the regional anatomy is intensively concerned to avoid procedure complications. The purpose of this study is to describe the anatomic relationships of PSV to its adjacent structures using computed tomographic coronary angiograms (CTCA).

A rare case of single right coronary artery arising from the right sinus of Valsalva with severe three-vessel disease

A 63-year-old man was admitted to our observation unit with atypical angina found to be non-adherent with his an- ti-hypertensive medication regimen for one month. His medical history was notable for hypertension for ten years and current tobacco smoking. He was hypertensive with otherwise, stable vital signs. Cardiac auscultation revealed normal S1 and $2 without a murmur.

Investigation of the direct microsurgery of ICCA aneurysms via the carotid artery-cavernous sinus space approach

Objective To discuss the approach and technique of the direct microsurgery of intracavemous sinus carotid artery aneurysms (ICCAAns). Methods All the 15 cases of ICCAAns underwent the direct microsurgery via the carotid artery-cavernous sinus space approach. Results Immediate carotid arteriography after the surgery showed that the aneurysms disappeared and the carotid artery could be showed clearly and normall. In a follow up period ranged frcm 1 months to 9 years,it was showed that,among 5 cases with 333,IV,V a,VI nerve paralysis before the surgery, three completely recovered,one recovered incompletely. All patients regained the capability of undertaking mormal cativities. None experienced rebleeding or neuroparalysis. Conclusion Direct microsurgery via this is approach is an ideal treatment of ICCAAns. 6 refs.

Primitive trigeminal artery-cavernous sinus fistula and ICA-primitive trigeminal artery aneurysm: report of two cases and review of literatures

The authors described two cases with primitive trigeminal artery. Case 1 was a 32-year-old woman who suffered dizziness and a serious pulsatile intracranial bruit on the left ear, and sometimes associated with pulsatile intracranial bearing-pain on the left temporal side six months before she was admitted to the hospital. She also suffered from obvious diplopia on left lateral gaze for the last 5 months. She had suffered no recent trauma. Magnetic resonance imaging(MRI) demonstrated a suspected intracranial aneurysm located in left cavernous sinus. Digital subtraction angiography (DSA) was performed and a primitive trigeminal artery-cavernous sinus fistula in left side was found. Intraluminal occlusion of the fistula was successfully performed immediately after angiography using 6 Guglielmi detachable coils (GDC), and the patient was cured finally. Case 2 was a 28-year-old woman who suffered a serious intermittent cephalodynia associated with soreness on the left body two years before she was admitted to the hospital. She had suffered no recent trauma. Magnetic resonance angiography(MRA) demonstrated a suspected intracavernous aneurysm of the right internal carotid artery, Digital subtraction angiography (DSA) was performed. Right internal carotid angiography showed a primitive trigeminal artery (PTA) run between the cavernous segment of the internal carotid artery and the distal portion of the basilar artery. On initiation of PTA of R-ICA a small wide-necked saccular aneurysm was incidentally visualized. The aneurysm was successfully embolized after angiography using 2 Stent (Neuroform, 4.5mmm × 20mmm)-assisted detachable coils (Matrix), the ICA and PTA were preserved, and the patient was cured finally.

Chest Pain on Exertion: Origin of the Right Coronary Artery from the Left Coronary Sinus, through the Aortic Pulmonary Sulcus with Squeezed, or Anterior Descending Branch Borderline Coronary Lesion?

The prognosis of patients in whom the right coronary artery (RCA) arises from the left coronary sinus (LCS) is unequal. An initial intramural course of the coronary artery within the aortic media is considered to cause myocardial ischemia in cases of coronary anomalies. In this case report, we present one patient admitted due to chest pain on exertion that suggested angina. Her only risk factor for coronary artery disease was hypertension. Clinical examination findings that the origin of the right coronary artery from the LCS, through the aortic pulmonary sulcus with atherosclerotic narrowing and squeezed of the RCA critical ostial, stenosis at the ostium of the RCA and the middle of left anterior descending (LAD) coronary, intravascular ultrasonography (IVUS) showed LAD and RCA minimal lumen area were 3.9 mm2, 5.9 mm2;plaque burden 66%, 65% respectively. We inserted EXCEL 3.0 × 18 mm stent in LAD stenosis. No complaints and adverse events were noticed during a 1-year follow-up.

Aneurysmal dilatation of the aortic sinuses of Valsalva -- beyond Marfan syndrome： a single centre experience and review of the literature

Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsaiva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less weil-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.

Complication of interventional treatment:noncoronary sinus of Valsalva aneurysm ruptured into the right atrium

Sinus of Valsalva aneurysm is a rare cardiac anomaly thought to be caused by a deficiency of fusion of the aorta＇s media with the aortic valve＇s annulus fibrosis, or by actual detachment. It is usually congenital but less commonly associated with endocarditis, atherosclerosis, trauma, syphilis, or aortic dissection. Since the first report in the mid-eighteenth century, sinus of Valsalva aneurysm has been described increasingly. The estimated prevalence of the disease is about 1% in patients undergoing open-heart surgery, and the morbidity is higher in the oriental than in Western population.

UNRUPTURED ANEURYSM OF THE LEFT CORONARY SINUS OF VALSALVA ASSOCIATED WITH ANEURYSM OF THE INTERVENTRICULAR SEPTUM

Aneurysms of the coronary sinus of Valsalva are commonly seen in the oriental people.Their clinical courses and prognosis depend on the location,size and propensity to rupture.They often occur in the right coronary sinus and tend to rupture to the

Diagnosis and surgical treatment of ruptured aneurysm in sinus of Valsalva

Objective To evaluate the methods used to diagnose and surgically treat ruptured aneurysm in sinus of Valsalva (RASV) Methods Thirty seven hospitalized patients with ruptured aneurysms in the sinus of Valsalva from September 1981 to April 2001, including 21 cases (56 7%) of RASV associated with ventricular septal defects (VSD) and 11 (29 7%) with aortic valvular prolapse were given surgical interventions Under hypothermia and extracorporeal circulation, we successfully performed the surgical correction of RASV for all 37 patients VSD repair in 21 patients, aortic valvuloplasty in 6 and aortic valvular replacement in 2 Results There was no hospital deaths among these patients, although residual shunting occurred in two patients and acute renal failure was found one Follow up study of one month to 20 years in the patients undergoing repair of RASV revealed that the mostly individuals treated with operation obtained satisfactory cardiac function Conclusion Correct diagnosis of ruptured aneurysm in sinus of Valsalva should be confirmed immediately and surgical correction should be carried out as soon as possible

Surgery for sinus of Valsalva aneurysm： 33-year of a single center experience

Background Sinus of Valsalva aneurysm （SVA） is a rare anomaly and few large or long-term series are well established.This study was designed to review 33-year surgical experience of SVA in one center.Methods From August 1980 to December 2013,patients with SVA underwent surgical repair were retrospectively studied.Results A total of 160 patients were identified with mean age of （30±12） years and 112 （70％） of them were males.The right coronary sinus origin of SVA was found in 108 patients （67.5％）,the non-coronary sinus in 51 patients （31.9％）,and the left coronary sinus in one patient （0.6％）.The rupture of 3VA into the right ventricle was identified in 89 （55.6％） cases,the right atrium in 61 （38.1％）,the left ventricle in 2 （1.3％） and no rupture in 8 （5.0％）.Ventricular septal defect （VSD） and aortic regurgitation （AR） were found in 59 （37％） and 45 （28％） patients respectively.An approach via the involved chamber was used in 86 patients （54％）,aortotomy in 8 （5％）,and a combined approach in 66 （41％）.Either direct suture （56,35％） or patch （104,65％） closure were used to repair the SVA.The VSD was closed with a patch （44/59,75％） or direct suture （15/59,25％）.aortic valve replacement （AVR） was performed in 23/45 （51％） and aortic valvuloplasty （AVP） in 9/45 （20％） patients combined with AR.There were 3 hospital deaths （1.9％） and 2 late deaths and 84％ of the patients were followed up for （17.6±4.2） years.New York Heart Association functional class improved significantly after surgery （P＜0.01）.Actuarial survival was 94％ at 10 years,and 88％ at 20 years.Conclusions Surgical treatment of SVA is safe and effective,ruptured SVA or unruptured SVA with VSD and/or AR should be repaired surgically as early as possible.However,late progressive AR is still a risk during long-term follow-up,and early aggressive measures are recommended.These include more use of a combined approach to achieve optimal evaluation of lesions,more patch repairs to reduce the chance of recurrence,and more AVR in patients with moderate to severe AR.

Outcome of surgery for sinus of Valsalva aneurysm with discrete membranous subaortic stenosis

Background Sinus of Valsalva aneurysm （SVA） is a rare cardiac anomaly, and SVA with discrete membranous subaortic stenosis is even rarer. The aim of the study was to make sure the incidence of SVA with discrete membraneous subaortic stenosis in SVA and their surgical results. We retrospectively analyzed 234 patients receiving surgical repair of SVA and reported the incidence of ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis. We also reported seven cases of SVA combined with discrete membranous subaortic stenosis and their surgical results.Methods Between January 1999 and December 2009, seven patients of SVA with discrete membranous subaortic stenosis underwent surgical repair of SVA and resection of subaortic discrete membrane. There were six male and one female patients. The mean age was （33.71±13.25） years （range 16–52 years）. Associated cardiovascular lesions were aortic regurgitation （n=7）, ventricular septal defect （n=5）, coarctation of aorta （n=1）, bicuspid aortic valve （n=1）, patent ductus arteriosus （n=1）, and aortic valve stenosis （n=1）. The aortic valve was replaced in four patients and valvuloplasty was done in three. The other co-existing anomalies were corrected at the same time. All the seven patients were followed up from 18 to 125 months （mean （63.14±39.54） months）. Among 234 SVA patients who underwent surgical repair, the number of cases with coexisting ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis was 129, 108, and 7, respectively.Results There was neither early death after operation nor late death during the follow-up period. All the seven patients were in the New York Heart Association （NYHA） functional classes I and II. There was no recurrence of discrete subaortic membrane during the follow-up period. The incidence of ventricular septal defect, aortic valve incompetence, and discrete membranous subaortic stenosis among 234 SVA patients was 55.13%, 46.15%, and 2.99%, respectively.Conclusions Surgical repair of SVA with discrete membranous subaortic stenosis showed good mid-term results. Resection of discrete subaortic membrane should be done actively while repairing SVAs. Long-term results need to be followed up.

Preliminary experience using transthoracic echocardiography guiding percutaneous closure of ruptured right sinus of Valsalva aneurysm

Background In the 21st century, minimally invasive treatment is one of the main developmental directions of medical sciences. It is well known that the echocardiography plays an important role during interventional treatments of some structural heart diseases. Because the ruptured right sinus of the Valsalva aneurysm （RRSVA） is a rare disease, there were few reports about percutaneous catheter closure of RRSVA. This study aimed to sum up our experience with transthoracic echocardiography （TTE） during percutaneous catheter closure of RRSVA.Methods Five RRSVA cases were treated with percutaneous catheter closure. The whole procedure was guided and monitored by TTE and fluoroscopy. The maximum diameter of the RRSVA was measured by TTE before and after the catheter passed through the rupture site. A duct occluder 2 mm larger than the maximum diameter was chosen. The closure effects were evaluated with TTE and fluoroscopy immediately after the occluding device was deployed. All patients were followed up by TTE for 8 to 30 months.Results Before the catheter passed through the rupture site the maximum diameter of the RRSVA measured with TTE and aortography were （7.9 ±2.1） mm and （7.8 ± 1.8） mm. After the catheter passed through the rupture site the maximum diameter measured with TTE was （11.2 ± 3.2） mm, which was significantly larger than before the procedure （P 〈0.05）. The percutaneous catheter closure was successful in four cases and failed in one. Compared to the aortography the TTE was better at distinguishing residual shunts from aortic valve regurgitation immediately after the occluding device was deployed. There were no complications during 8 to 30 months of follow-up.Conclusion Transthoracic echocardiography can play an important role during percutaneous catheter closure of RRSVA,especially for estimating the size of the RRSVA after the catheter passes through the rupture site, and differentiating residual shunt from aortic valve regurgitation immediately after the occluding device is deployed

鸡胚半月瓣Valsalva窦的形态发生

用光镜和扫描电镜方法,观察鸡胚半月瓣Valsalva窦的形态发生过程。按鸡胚发育的H-H分期法,窦发生开始于第30期,至第34期已基本形成。在形成过程中,瓣膜心室面和动脉面的内皮细胞有明显差异;瓣膜内的间充质在不同的部位疏密分布不均,与内皮细胞的形态有对应关系。本研究表明,Valsalva窦的形成,不仅与瓣膜动脉面的内皮细胞向深部的间充质生长有关,而且与间充质疏松并形成一些腔隙有密切联系。

Myocardial infarction in non-dissecting aortic root aneurysm

We describe a case of a 49-year-old man who presented with an uncomplicted aortic root aneurysm, aortic insufficiency, and ST-elevation myocardial infarction (STEMI) without obstructive coronary artery disease on angiography. The computed tomo- graphy angiogram (CTA) of the thorax, performed without cardiac gating, was misinterpreted as normal. In retrospect, an overlooked extravasation of contrast material lateral to the aortic root was detected on non-gating magnetic resonance angiography (MRA). Exploration of the aortic root revealed an unsuspected horizontal intimal tear of the left sinus of Valsalva with limited extramural hematoma. The presence of an otherwise silent intimal tear on preoperative imaging studies makes the overall management more problematic. For example, initiating early broad empirical anticoagulants or fibrinolytics therapy to treat the accompanied myocardial infarction may extend the tear into a full life-threatening aortic dissection, tamponade or rupture. We highlight many of the difficulties associated with the diagnosis and treatment of limited sinus tear when aortic root aneurysm is presenting with cryptogenic STEMI. Accurate morphologic characterization of intimal tear would be best defined with either an electrocardiogram-gating CTA or MRA imagings. These non-invasive tests are needed to make appropriate management decisions. Depending on other pathologic components of aortic root, cusps and the commissural geometry, sinus tear is a critical component for the overall treatment plan and it shifts the surgical intervenetion from valve-sparing operation, commissural resuspension and leaflet repair to composite aortic root replacement (modified version of the Bentall procedure).

主动脉窦瘤的超声特点与漏诊分析

【目的】总结主动脉窦瘤(SVA)的超声特点,分析漏诊原因,探讨SVA的超声诊断技巧,提高诊断率。【方法】回顾性分析2014年1月至2022年3月期间在中山大学附属第一医院行外科手术的52例SVA患者的超声心动图特征及手术资料,按改良Sakakibara分型分为5型。【结果】52例SVA患者中,男性32例,女性20例,年龄18~66(36.1±11.6)岁。其中44例窦瘤起自主动脉右冠窦,8例起自无冠窦,未见起自左冠窦的病例。Ⅰ型、Ⅱ型和Ⅲv型为膨入右心室的窦瘤共35例,32例(91.4%)合并室间隔缺损(VSD),Ⅲa型、Ⅳ型和Ⅴ型为膨入右心房或其他部位的窦瘤共17例,仅2例(17.6%)合并VSD。各型SVA均常合并主动脉瓣病变,因主动脉瓣病变情况较严重需要外科手术换瓣或成形者共27例(51.9%)。52例患者共有4例漏诊SVA,漏诊率为7.7%;8例漏诊VSD,漏诊率达23.5%,漏诊者多为I型SVA合并干下型VSD;各型SVA中均有合并感染性心内膜炎(IE)患者,共19例,其中有2例漏诊,漏诊率为10.5%。【结论】SVA声像图具有多样性和复杂性。膨入右心房的SVA较少合并VSD,而膨入右心室的SVA绝大多数合并VSD,但诊断具有挑战性,且SVA还易合并主动脉瓣病变和IE,进一步加大诊断难度。超声检查过程中必须提高警惕,灵活运用多个标准和非标准切面,减少漏诊,提高诊断率。