Rupture of sinus of Valsalva aneurysm: a rare cause of acute ischaemic chest pain in the emergency department

Abstract

Acute chest pain is a leading cause of emergency department (ED) visits in the adult population. However, patients with a life-threatening cause of chest pain comprise only a small fraction, hence identification of those who need immediate intervention is very important. One rare cause is rupture of the aneurysm, which may present with a wide variety of symptomatology, including anginal chest pain. In a busy ED, evaluation of chest pain suggestive of myocardial ischaemia needs to cater to early identification of acute coronary syndrome (ACS), limit over investigating patients for low or intermediate probability of ACS and appropriate decision making for discharge, to reduce ED over boarding. Validated scores for evaluating the low or intermediate probability chest pain like the HEART pathway may miss such rare diagnoses in the absence of ECG changes, requiring high degree of suspicion and individualisation of patient care.

Background

Acute chest pain is one of the leading causes of emergency department (ED) visits in the adult population.^{1 2} However, the patients with a life-threatening cause of chest pain comprise only a small fraction of the patients, hence identification of those who need immediate intervention is very important. According to the 2017 Heart Disease and Stroke Statistics update of the American Heart Association, 16.5 million people aged 20 and above in the USA had coronary heart disease, thereby making the prevalence of angina pectoris quite high.³ The most common pathophysiology relates angina pectoris and myocardial ischaemia to coronary artery stenosis or atherosclerosis. There also exist other causes which cause an imbalance between demand and supply causing myocardial ischaemia and hence symptoms of angina.⁴ One such cause is the aneurysm of sinus of Valsalva, which can be defined as an enlargement of the aortic root area between the aortic valve annulus and the sinotubular ridge.⁵ With an approximate prevalence of only 0.09% in the general population, aneurysm of sinus of Valsalva becomes a very rare disease in itself (usually detected incidentally on imaging). A serious life-threatening complication is the rupture of the aneurysm, usually seen in the age group of 20–40 years with significant outliers in infancy and late adulthood which may present with a wide variety of symptomatology, one of which is anginal chest pain. The consequence of which is missed diagnosis in the ED, hence increased risk of mortality depending on the location, size, position and function of the shunt of a potentially repairable cause.⁶

Case presentation

A 30-year-old Asian man presented to our ED of an urban tertiary care hospital with the complaint of acute onset retrosternal chest pain, dyspnoea and profuse sweating of 1-day duration. On primary survey, the patient was conscious, oriented and had a patent airway. His respiratory rate was 20 per minute, an oxygen saturation of 99% on room air with bilateral normal vesicular breath sounds on auscultation. Assessment of circulation showed a heart rate of 90 per minute and blood pressure of 98/50 mm Hg. The patient was shifted to a monitored bed and an electrocardiograph and cardiac biomarkers were immediately obtained. The ECG showed no dynamic ST-T changes (figure 1) and the cardiac biomarkers were also well within the normal limit. Further cardiovascular examination revealed the presence of a grade 5/6 harsh, high pitched continuous murmur best heard in fourth left intercostal space radiating to right parasternal border. There were no signs suggestive of Marfan’s syndrome or any connective tissue disorders. The patient Bedside point of care transthoracic echocardiography was done, parasternal short axis view showed a defect in the non-coronary sinus of aorta with colour flow across it from the non-coronary sinus to right atrium (RA) just above the insertion of septal tricuspid leaflet suggestive of Ruptured sinus of Valsalva into RA (figures 2–3). The echocardiography showed no other valvular abnormality. The patient was taken for emergency transoesophageal echocardiography (TEE) to better delineate the lesion, number and size of defect(s) and margins across the defect for possible transcatheter repair, and to look for associated cardiac anomalies associated with it like ventricular septal defect (VSD). On TEE, there was a single 6–8 mm defect in the non-coronary sinus of aorta with colour flow into RA. There was no associated aortic regurgitation and VSD.

Figure 1.

A12-lead ECG of the patient at time of arrival.

Figure 2.

Tranthoracic echocardiography in parasternal short axis view showing aortic valve and its sinuses with a defect in non coronary sinus opening into the right atrium just above the tricuspid valve. 1- defect, 2-non-coronary sinus, 3-right coronary sinus, 4-left coronary sinus.

Figure 3.

Transthoracic echocardiography in parasternal short axis view (colour compare image) showing colour flow across the defect from non coronary sinus into right atrium.

Treatment

The patient was found to be a suitable candidate for transcatheter device closure and underwent cath study with aortogram in right anterior oblique view (30°) for precise sizing of the defect. It was followed by device closure using Amplatzer duct occlude device-1 of 16×18 mm. Successful device closure was done with no periprocedural complications.

Outcome and follow-up

The patient was managed in the cardiac care unit for 48 hours, during which he had no complications. He was eventually discharged after 7 days of hospital stay with stable vitals. He was followed up in the cardiology out patient department (OPD) after 2-week duration. The patient was doing well at the time of follow-up with no new complaints.

Discussion

Sinus of Valsalva aneurysm is a rare congenital abnormality which is usually asymptomatic and detected either incidentally on imaging or when it presents with one of its complications. Rupture of the same is one such complication. This too can present asymptomatic to a myriad of symptoms like chest pain, abdominal pain, dyspnoea, acute heart failure, cardiovascular collapse or even cardiac death. The rarity of the disease, complication along with variable symptomatology makes this a diagnostic challenge, especially in the ED. The first-line diagnostic modality includes transthoracic and TEE. Transthoracic echocardiography can detect >89.2% of the ruptured sinus of Valsalva aneurysms while TEE is required in 25% of the cases for better visualisation, both of which were done in our case.⁴

In the busy setting of the ED, evaluation of chest pain suggestive of myocardial ischaemia needs to cater to early identification of acute coronary syndrome, limit over investigating patients for low or intermediate probability of acute coronary syndrome and appropriate decision making for discharge patients to reduce over boarding of the ED. One of the validated decision-making tools is the HEART score, which is also followed in our institute for evaluating chest pain.⁷ However, adhering to the same may miss out rare cases like the one discussed above. Our patient had a highly suspicious history, normal ECG, age <45 years, no risk factors and normal troponin levels at 0 and third hour, (HEART score=2) making the patient suitable for early discharge from the ED. The highly suspicious history, presence of a continuous murmur, non-resolving nature of the patient’s chest pain after initial pain management prompted us to evaluate the patient beyond the protocol by doing an emergent bedside echocardiography which revealed the rare diagnosis of ruptured sinus of Valsalva in the RA. Ruptured sinus of Valsalva, if remained undiagnosed and untreated has a poor prognosis with life expectancy of only 1 year.⁸

Traditionally ruptured sinus of Valsalva was surgically repaired, however, the last two decades have seen a rise in the use of transcatheter device closure with good clinical outcome, limiting surgery to only select patients.⁹ As our candidate was suitable for device closure, he underwent the same on an emergent basis. The high mortality in untreated patients coupled with the good clinical outcome on prompt diagnosis and management requires a high degree of suspicion in evaluation of such patients, especially in the ED.

Learning points.

• The above clinical scenario cautions the emergency physician to look out for rare causes of life-threatening chest pain.

• The presentation of rupture of sinus of Valsalva can be among a myriad of symptoms hence difficult to be identified in the emergency settings.

• Rupture of sinus of Valsalva though rare, if identified and managed promptly can improve the mortality and morbidity of the patient.

Ethics statements

Patient consent for publication

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