CME INDIA Case Presentation by Dr. Raj Ashwini Dwivedi, GMC & SSH Nagpur; SR-2 Academic Cardiology.
CME INDIA Case Study
- 35 yr.-old-female presented to us with c/o breathlessness, insidious onset, progressive NYHA 2-4 for 3 months.
- O/E Pedal oedema, dyspnic at rest.
- CVS examination – Continuous murmur grade -4 near aortic area most prominent.
See the Echo Clip and diagnose it
- Adding TEE
CME INDIA Discussion
Dr. Varun Kumar, DM Card DM, Sante vita Hosp, Ranchi: Ruptured sinus of Valsalva to RV.
Dr. Venkatesh Molio, Maregoan, Goa: Ruptured sinus of Valsalva (RSOV).
Dr. Raka Sheohare, Diabetologist, Raipur: RSOV, Ruptured sinus of Valsalva to RV.
Dr. Raj Awashthi: Rsov to Rvot. So, murmur and history s/o rsov.
Differential Diagnosis
- VSD, PDA, AP window, DCMP.
How case was picked up?
- 2 D echo done s/o RSOV to RVOT.
- As we can see in echo clip there is continuous flow from Aortic RCC SINUS to RVOT above pulmonary valve.
- Difference from VSD – defect is after aortic valve at sinus not in IVS.
- TEE: Defect is shown in the TEE. Just to rule out VSD, TEE was done.
- Many times, we get patient with both the lesions VSD+ RSOV.
- In PLAX defect is after aortic valve leaflet at sinus. Valve leaflet is marked with white arrow. In this way we can differentiate it from VSD & clinically murmur and history is unique in RSOV.
Echo after Repair
- Successfully closed with ADO1 device like PDA device closure.
CME INDIA Learning Points
- Sinus of Valsalva aneurysms (SVA) are very uncommon, have a marked male preponderance (4:1), and their incidence is highest in Asian populations.
- A rupture of a sinus of Valsalva is a rare cardiac anomaly.
- A continuous murmur may be the first clinical sign.
- Transoesophageal echocardiography is the preferred diagnostic tool.
Sinus of Valsalva aneurysm (SVA)
| SVA ruptures most commonly into |
| Right Ventricle (60%) |
| At the Right Atrium (29%) |
| Left Atrium (6%) |
| Left Ventricle (4%) |
| Pericardium (1%) |
- Right coronary cusp is the most commonly involved whereas non-coronary cusp involvement is relatively uncommon.
- It is well known that Sinus of Valsalva aneurysm is usually due to a defect in the aortic media. A VSD or aortic regurgitation may be found as associated abnormalities.
- Approximately 70% of aneurysms originate from the right coronary sinus.
- The unruptured aneurysm may be incidentally detected or may present with outflow tract obstruction or coronary insufficiency or tricuspid regurgitation or cardiac conduction abnormality. SVA occurs because of mal-fusion between the aortic media and annulus fibrosus of aortic valve. Affected sinus wall is often relatively elastin deficient.
Rupture of sinus of Valsalva aneurysm (RSOV) to right atrium
What aneurysm can do?
- May compress an adjacent chamber, coronary artery or conduction system. These can cause myocardial ischemia or conduction disturbances.
- About one third of aneurysms rupture and produce acute symptoms in a quarter of patients.
Important symptoms following rupture
- Dyspnea.
- Chest pain.
- Fatigue.
- If it ruptures into the right sided chambers, it produces a left to right shunt.
- The severity of shunt and associated lesions determine the severity of symptoms.
- Rupture (RSOV) usually occurs into the right heart chambers.
- Rarely rupture can occur into left heart chambers, pericardium, pulmonary artery or superior vena cava.
- Rupture into the pericardium can cause cardiac tamponade.
The classical clinical finding in RSOV to right ventricle
- Continuous murmur with diastolic augmentation.
- Why Diastolic Augmentation: The aneurysm track through the right ventricular wall gets compressed during systole and flow is better in diastole, causing the diastolic augmentation of murmur.
- Rupture into the left ventricle will cause only a diastolic murmur.
This cardiac lesion can be congenital or acquired.
Congenital
- A congenital lack of continuity in the media, between the aorta and annulus fibrosus of the aortic valve, may initiate aneurysm formation or, less frequently, infection or degeneration processes may affect an aortic sinus.
- Congenital aneurysms are often associated with other cardiac anomalies, including VSD and aortic valve dysfunction, pulmonary stenosis, a bicuspid aortic valve, mitral insufficiency with or without prolapse, a coarctation of the aorta, a patent ductus arteriosus and tricuspid insufficiency.
Acquired aneurysms
- Associated with trauma, atherosclerosis, infective endocarditis, syphilis, Marfan syndrome and collagen vascular disorders.
Unruptured sinus of Valsalva aneurysms are frequently asymptomatic
- The most frequent complication of an aneurysm of the sinus of Valsalva is rupture.
- A rupture can be immediately fatal when going to the pericardium or if a complete heart block develops.
- A large, acute intracardiac rupture leads to a sudden volume overload with subsegment development of frank pulmonary oedema.
- Subacute perforations are often better tolerated.
- Most patients have a gradual onset of symptoms, including shortness of breath, fatigue, palpitations, chest pain and syncope.
Diagnostic Tools
Echocardiography
- The classical echocardiographic appearance in RSOV to RV is a ‘windsock appearance’, of the aneurysm in the right ventricular outflow tract. Windsock appearance is better seen on transesophageal echocardiography.
- TTE with colour Doppler can correctly establish the diagnosis of a ruptured aneurysm in 75 % of patients.
Multislice computed tomography (MSCT)
MRI.
Cardiac catheterisation with coronary and aortic angiography can play a role in providing exact haemodynamic data and identifying associated conditions
Management
- Little is known about the natural history of an unruptured, asymptomatic sinus of Valsalva aneurysm, therefore the optimal management is controversial.
- When an unruptured aneurysm becomes symptomatic, surgical intervention is needed.
- Ruptured aneurysms, both symptomatic and asymptomatic, require an early, corrective procedure.
- A mean survival period of 3,9 years in patients with untreated ruptured sinus of Valsalva aneurysms has been reported.
- An early, aggressive approach is recommended to prevent clinical deterioration.
- Operative procedures include simple plication, patch repair, aortic root replacement and aortic valve replacement/repair.
- Recently, the percutaneous transcatheter closure of ruptured sinus of Valsalva aneuysms using an Amplatzer™ duct occluder has been reported, which allows to obviate open heart surgery in some carefully selected cases, but experience and follow-up are very limited.
Quick Takeaways
- A rupture of a sinus of Valsalva has to be taken into account in the differential diagnosis of a continuous heart murmur, even though the patient looks healthy and is asymptomatic.
- Clinical suspicion has to be confirmed by imaging investigations.
- Repair, mostly by surgery, is indicated.
CME INDIA Tail Piece
Story of the first successful surgical closure of RSOV was reported by Lillehei et al in 1957(Courtesy Reference 1)
- A 37-year-old man had led a vigorous and healthy existence up until the time of his sudden disability on June 6, 1956. During World War II he had served as a pilot officer in the R.C.A.F.
- When demobilized in 1946, he was told that he had a systolic heart murmur (Grade II along the right stemal border).
- This surprised both him and his physicians since he had had many previous physical examinations in which no murmur had been detected. However, he remained completely free of any cardiac disability until June 6, 1956.
- That day he was stricken suddenly by a severe upper abdominal pain attributed to indigestion, but which on consultation proved due to an enlarged liver caused by acute heart failure.
- His blood pressure was 180/20, and his pulses were of “pistol shot” quality. A continuous thrill was palpated, and a continuous murmur was now heard over the
entire precordium but maximally over the xyphoid. The neck veins were distended and the liver was down some 10 centimeters. - Chest x-ray and heart fluoroscopy revealed advanced cardiomegaly involving both ventricles and the left atrium. The pulmonary artery was enlarged and the aorta, al-
though very active, was less enlarged. The lungs were congested and pleural effusion was present in the right chest the electrocardiogram was interpreted as compatible with left heart strain or digitalis effect. - Recovery from this acute episode of decompensation was only partial and limited. Heart catheterization revealed a 5 volume per cent increase in the oxygen concentration between the cavae and the right atrium.
- Despite continuous hospitalization for the next six months, he remained in intractable failure. This was his condition upon transfer to the Heart Hospital. Despite vigorous efforts to eradicate his obvious edema by medical treatment before reparative surgery, he was little improved after sometime, and it was therefore decided to proceed with the operation despite the presence of persistent cardiac decompensation including pleural effusions.
- At operation, which was undertaken on December 20, 1956, the heart was markedly enlarged in all its chambers and an intense continuous thrill was palpated over the right atrium with transmission from the base of the aorta. The required cannulations were completed, the patient linked to the pump-oxygenator, and then the aortic arch was cross clamped while cardiac arrest was induced through the injection of 50 cc. of 2.5% solution of potassium citrate in oxygenated blood into the
coronary circulation. - A right atriotomy revealed the aneurysmal sac which measured six centimeters in
length and 11,2 centimeters in diameter tricuspid valve and had two perforations, one at the apex of 15 millimeters and a second along the side with an opening only a few millimeters wide This sac was amputated at its base and the fistulous tract closed with interrupted transfixing sutures. These were carefully placed as to obliterate the site of origin of the defect in the aortic wall without injury to the aortic leaflets.
References:
- LILLEHEI, C W et al. “Surgical treatment of ruptured aneurysms of the sinus of Valsalva.” Annals of surgery vol. 146,3 (1957): 459-72. doi:10.1097/00000658-195709000-00014.
- Mahimarangaiah J, Chandra S, Subramanian A, Srinivasa KH, Usha MK, Manjunath CN. Transcatheter closure of ruptured sinus of Valsalva: Different techniques and mid-term follow-up. Catheter Cardiovasc Interv. 2016 Feb 15;87(3):516-22. doi: 10.1002/ccd.26107. Epub 2015 Aug 10. PMID: 26255646.
- Rupture of sinus of Valsalva aneurysm (RSOV) – All About Cardiovascular System and Disorders (johnsonfrancis.org)
- Vautrin E, Barone-Rochette G, Philippe J. Rupture of right sinus of valsalva into right atrium: Ultrasound, magnetic resonance, angiography and surgical imaging. Arch Cardiovasc Dis. 2008;101:501–502.
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