Rupture of aneurysm of sinus of Valsalva into the right atrium mimicking tricuspid valve endocarditis is a rare presentation. We review a case of spontaneous rupture of aneurysm of sinus of Valsalva into the right atrium presenting as a murmur. [ncbi.nlm.nih.gov]

Clinical complications from SVA are often the initial presentation of SVA. [emedicine.com]

Pounding pulses may be present suggesting hyper-dynamic circulation from large left to right shunt or aortic insufficiency. Diastolic murmur may occur due to aortic incompetence. Bi-basillar rales may be present due to congestive heart failure. [heartviews.org]

When present, it is usually in either the right (65–85%) or in the noncoronary (10–30%) sinus, rarely in the left (< 5%) sinus. [en.wikipedia.org]

Aortogram perfomed in the same patient 3 years before presentation demonstrating no major change in the findings. [circ.ahajournals.org]

• Developmental Delay

  delay and mild mixed aminoaciduria. [ncbi.nlm.nih.gov]

• Arachnodactyly

  CONGENITAL Connective tissue disordersRheumatoid arthritis, Ehlers-Danhlos syndrome, Marfan’s syndrome, Klippel Feil syndrome, Turner’s syndrome, Trisomies 13 and 15, Loeys-Dietz syndrome, Arachnodactyly, Osteogenesis imperfecta. [slideshare.net]

• Upper Abdominal Pain

  An large acute rupture is announced by the dramatic onset of severe retrosternal or upper abdominal pain and intractable dyspnea. 31, 36 The rupture often but not necessarily follows physical stress. [clinicalgate.com]

• Epigastric Pain

  Symptoms typically present in young adulthood (usually in patients Sudden massive rupture may occur after strenuous exertion and may be signaled by acute chest or epigastric pain with dyspnea. [chdbabies.com]

• Suggestibility

  Transthoracic echocardiogram showed a mobile mass that appeared to be attached to the tricuspid valve leaflet with moderate tricuspid regurgitation suggestive of tricuspid valve endocarditis. [ncbi.nlm.nih.gov]

  CT coronary angiography axial, coronal and sagittal images showed a contrast filled out-pouching arising from the left coronary sinus suggestive of aneurysm. [elynsgroup.com]

  These findings suggested an unusual rupture (communication) between left coronary sinus and pulmonary artery. Coronaries were normal on angiography. [annalspc.com]

Unruptured SOV aneurysms Overall, unruptured aneurysms are asymptomatic and are incidentally detected during imaging workup of heart murmurs or abnormal cardiomediastinal silhouette on radiograph ( 16 ). [ncbi.nlm.nih.gov]

The patient had a successful postsurgical recovery and was discharged home with anticoagulation treatment. [ncbi.nlm.nih.gov]

The surgery is considered to the treatment of choice in most of the cases where as trans-catheter treatment by the vascular occluder devices is an effective alternative method of treatment. [elynsgroup.com]

Treatment Therapy for Aneurysm of sinus Valsalva includes controlling blood pressure by use of medicines, such as beta-blockers. However, the long-term treatment is surgical repair. [medigest.uk]

Surgery is definitive treatment Urgent surgical repair for ruptures aneurysms Many recommend surgical repair of non-ruptures aneurysms as well Complications Rupture (see above) Myocardial infarction Heart block Right ventricular outflow tract obstruction [learningradiology.com]

Prognosis in patients with a ruptured aneurysm who have not undergone surgical repair may be poor, with survival beyond 1 year uncommon. [chdbabies.com]

Treatment and prognosis Surgical repair with a Bentall procedure could be performed. [radiopaedia.org]

Urgent surgical repair for ruptures aneurysms Many recommend surgical repair of non-ruptures aneurysms as well Complications Rupture (see above) Myocardial infarction Heart block Right ventricular outflow tract obstruction Cardiac tamponade Sudden death Prognosis [learningradiology.com]

[…] echocardiography (TEE) provides conclusive information regarding the origin and size of the aneurysm and presence of thrombotic material and allows precise identification of structural anomalies and shunt locations for perioperative assessment. [1], [4], [5] Prognosis [annals.in]

Rose V, Izukawa T, et al: Syndromes of asplenia and polysplenia: A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J, 37: 840-852, 1975. [kotobank.jp]

An anatomic etiology of symptoms may be suspected with heart auscultation (murmur), and diagnosis is made by a routine echocardiogram or, if the patient is suspected of having coronary disease as the etiology of symptoms, an angiogram. [hqmeded-ecg.blogspot.com]

Root replacement or Ross procedure maybe used if severe local septic etiology. Complications Rupture into RV > RA > LV > IVS/PA leads to formation intracardiac fistulae or tamponade. [pie.med.utoronto.ca]

For etiology, SOV aneurysms are commonly congenital and represent 0.1% to 3.5% of congenital heart defects ( 6, 7 ). [ncbi.nlm.nih.gov]

The etiology of the aneurysm seems atherosclerotic as suggested by age of the patient, atherosclerotic changes visualized on computed tomography imaging of the aorta and histopathology of the surgically excised aneurysm. [annalspc.com]

Cardiac tamponade may occur if the rupture involves the pericardial space. [1] Epidemiology Frequency United States SVA was present in 0.09% of cadavers in a large autopsy series and ranged to 0.14-0.23% in a Western surgical series. [4] Two-dimensional [emedicine.com]

Chest trauma, vasculitic diseases, and iatrogenic injury during aortic valve replacement have all been reported as causes of acquired SOVA.[ 1, 6-8 ] Epidemiology Although the true prevalence of SOVAs is unknown, the estimated rate is approximately 0.09% [onlinelibrary.wiley.com]

Associated conditions include: Syphilis Trauma Endocarditis Atherosclerosis Marfans (diffuse dilatation) Uncommon – Ehlers Danlos (other connective tissue disorders), Takayasu arteritis (other vasculitides), post AV replacement Pathophysiology Congenital [pie.med.utoronto.ca]

[…] intracardiac shunting caused by rupture of the SVA into the right side of the heart. [1] Approximately 65-85% of SVAs originate from the right sinus of Valsalva, while SVAs originating from noncoronary (10-30%) and left sinuses (< 5%) are exceedingly rare. [2] Pathophysiology [emedicine.com]

Pathophysiology Aneurysmal dilatation of the sinuses of Valsalva occurs when the aortic media is defective, allowing separation of the media from the aortic annulus fibrosus. [chdbabies.com]

Deterrence/Prevention Because the genetic mutation that causes the sinus of Valsalva aneurysm is presumed to be spontaneous, no preventive measures are available. [chdbabies.com]

Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. [nemours.org]

Such a consideration may prevent some instances of congenital aneurysms from being missed simply because they have not been reported. [pediatrics.aappublications.org]

Specific inhibitors and promoters of calcium action in the excitation-contraction coupling of heart muscle and their role in the prevention or production of myocardial lesions. In Calcium and the Heart, ed. ‎ [books.google.es]

A mean survival period of 3,9 years in patients with untreated ruptured sinus of Valsalva aneurysms has been reported. 1 An early, aggressive approach is recommended to prevent clinical deterioration. 1,2,4,6 Operative procedures include simple plication [ecrjournal.com]