Abstract
Background
Acute type A aortic syndrome involving a nonaneurysmal sinus of Valsalva (SOV) without predisposing factors is exceptionally rare. The contribution of remote blunt chest trauma (BCT) in delayed aortic root pathology and aortic regurgitation remains uncertain.
Case
A 71-year-old patient presented with acute chest tightness and a large nontamponading pericardial effusion. Computed tomography angiography was inconclusive, but transesophageal echocardiography (TOE) identified rupture of a nonaneurysmal left SOV and left aortic cusp causing severe aortic regurgitation. Urgent surgical repair was successful. No clear etiology was identified, though a bicycle-related BCT 6 years earlier was considered contributory, albeit unproven.
Discussion
This case highlights the rarity and diagnostic challenge of nonaneurysmal left SOV rupture without clear predisposing conditions.
Take-Home Messages
Transesophageal echocardiography is essential when computed tomography angiography is inconclusive for acute type A aortic syndrome. After BCT, comprehensive cardiac evaluation and long-term follow-up may identify delayed cardiac complications, even in patients without prior cardiac disease.
Key words: aorta, aortic valve, dissection, pericardial effusion, tamponade, valve replacement
Visual Summary
History of Presentation
A 71-year-old man presented to his general practitioner with a first episode of sudden, severe chest tightness lasting approximately 3 hours, worsened by inspiration and unresponsive to nitroglycerin or morphine.
A 12-lead electrocardiogram (ECG) showed sinus rhythm 77 beats/min, a vertical heart axis, normal PQ and QRS intervals, discrete, horizontal ST-segment depressions in II, aVF, V4, V5, and preterminal T-wave inversions in leads I and aVL (Figure 1A). Suspecting acute coronary syndrome (ACS), the patient was referred to the emergency department for further evaluation.
Figure 1.
12-Lead ECG on Presentation and Follow-Up
12-lead electrocardiogram recorded in the general practitioner's office (A) and on arrival at the emergency department (B).
During ambulance transport, the patient experienced hypotension (73/45 mm Hg), treated with a single dose of norepinephrine.
On arrival at the emergency department, the patient was alert, afebrile, and breathing comfortably. Vital signs revealed blood pressure 98/54 mm Hg, symmetric radial and femoral pulses, heart rate 76 beats/min, and oxygen saturation 96% on room air. Follow-up ECG demonstrated left axis deviation, considered positional, with repolarization abnormalities similar to the prior tracing ECG (Figure 1B).
Laboratory tests demonstrated increasing high-sensitivity troponin I (115-150 ng/L at 1 hour, normal <34 ng/L), normal myoglobin, creatine kinase, D-dimer, and hemoglobin. Mild leukocytosis (14 × 109/L, 12% neutrophils) was noted; the C-reactive protein level was normal.
Point-of-care ultrasonography revealed a circular, isoechoic pericardial effusion without tamponade (Video 1). Severe aortic regurgitation (AR) was also noted.
Past Medical History
The patient's medical history was notable for bilateral carpal tunnel syndrome, bilateral knee and right hip arthroplasty, and a hepatic cyst with normal liver function.
He had no prior cardiovascular disease, recent infectious history, or recent chest trauma at presentation.
The patient was a lifelong nonsmoker, denied illicit drug use, took pantoprazole occasionally, and had no family history of cardiovascular disease, aortopathies, or sudden cardiac death.
Differential Diagnosis
After reconsidering the presumptive diagnosis of ACS, the differential diagnoses included acute type A aortic syndrome (ATAAS), left ventricular (LV) wall or coronary artery rupture, preexisting AR with LV dilation and pericardial effusion/hematoma (acute, subacute, or chronic), occult aortic dissection, or a combination thereof.
Investigations
Transthoracic echocardiography demonstrated an eccentric hypertrophic, severely dilated LV (indexed end-diastolic volume 120 mL/m2) with mildly reduced LV ejection fraction (LVEF 45%) and diffuse hypokinesia. The degenerative, tricuspid aortic valve demonstrated severe, holodiastolic, eccentric regurgitation of unclear mechanism. The sinus of Valsalva (SOV) was mildly dilated (41.8-mm leading-to-leading edge). The mitral valve showed mild-to-moderate regurgitation due to geometric distortion and posterior leaflet tethering. LV perforation was excluded using contrast enhancement.
Initial, non–ECG-gated computed tomography angiography (CTA) only partially visualized the SOV but showed no dissection. Pulmonary embolism and coronary artery dissection were excluded and nonobstructive coronary atheromatosis was noted within the constraints of a non–ECG-gated study. Ongoing analgesia-refractory chest pain in the setting of biomarker-confirmed myocardial injury and borderline ECG abnormalities, consistent with an ACS, prompted coronary angiography, which definitively ruled out coronary artery dissection and obstructive coronary disease (Video 2, Video 3, Video 4). Supra-aortic injection (Video 5) confirmed the severe AR.
With the working diagnosis refocused on ATAAS, CTA was repeated using retrospective ECG gating at a heart rate of approximately 70 beats/min, but the diagnosis remained uncertain. Subsequent review by a cardiovascular radiologist identified subtle irregularities of the aortic root suggestive of intramural hematoma particularly involving the left SOV (Figure 2) and rupture of the left coronary cusp (Figure 3).
Figure 2.
ECG-Gated Cardiac CT Angiography, Short-Axis View at the Aortic Valve Level
Computed tomography angiography of the aortic root short-axis view through the sinus portion showing a hyperdense, well-demarcated hematoma (yellow arrows) in the region of the left sinus of Valsalva on the noncontrast series (A), barely visible on the contrast-enhanced series (B).
Figure 3.
ECG-Gated Cardiac CT Angiography, Zoomed View of the Aortic Valve and Sinus of Valsalva
Computed tomography angiography of the aortic root short-axis (A) and 3-chamber views (B). During diastole, the left coronary cusp exhibits an atypical configuration with a membranous structure protruding subvalvularly, causing a pronounced coaptation defect (yellow arrows).
While awaiting this review, transesophageal echocardiography (TOE) was performed under anesthesia standby. The procedure was initiated with caution to prevent worsening of the hemodynamics in the setting of already progressive hypotension and gastric content aspiration after recent oral intake and persistent nausea despite antiemetic therapy. TOE clarified the mechanism of the severe AR, revealing rupture and prolapse of the left coronary cusp and a left SOV wall hematoma with a partially visualized discontinuity plane (Video 6, Video 7, Video 8, Figure 4). However, image quality was limited by early termination due to aspiration and hemodynamic instability (systolic blood pressure <60 mm Hg; oxygen saturation <60%).
Figure 4.
Transesophageal Echocardiography, Long-Axis View With M-Mode and Color Doppler Suggesting Holodiastolic Aortic Regurgitation
Management
The patient was transferred for urgent surgical intervention, arriving in cardiac tamponade.
Intraoperatively, the pericardial effusion was evacuated. The aortic valve was thinned and demonstrated multiple fenestrations and a 5 × 8 mm thrombosed rupture of the left SOV with a hematoma just beneath the left main stem. The tear was sutured, and a 27-mm biological prosthetic aortic valve was implanted (Figure 5).
Figure 5.
Schematic Intraoperative Findings
Upper panel: wall hematoma and thrombosed rupture of the left coronary sinus of Valsalva beneath the left main coronary ostium, extending toward the pericardial space and the aortic valve. Lower panel: suture repair of the sinus of Valsalva rupture and bioprosthetic aortic valve replacement. BioAVR = bioprosthetic aortic valve replacement; LCA = left coronary artery; LCC = left coronary cusp.
Outcome and Follow-Up
Postoperatively, the patient developed hemorrhagic and cardiogenic shock from rapid pericardial bleeding and transient severe LV dysfunction with worsened mitral regurgitation; the prosthetic aortic valve remained functional. After coagulopathy correction and transfusion, he stabilized.
His subsequent course was notable for transient delirium, positioning-related neuropathies, improving with treatment and persistent retrograde amnesia.
A 4-pillared heart failure regimen, including an angiotensin-converting enzyme inhibitor, β-blocker, spironolactone, and dapagliflozin, was started alongside diuretics. Aspirin 100 mg/d was initiated for the bioprosthetic aortic valve and rosuvastatin 20 mg/d for its potential aortic wall-stabilizing effects and secondary prevention of coronary atheromatosis.
Follow-up CTA showed expected postoperative findings.
Histology revealed fibrotic tissue with minimal mucoid deposits and no inflammation. Results of infectious work-up, including blood and urine cultures and HIV and Treponema pallidum serology, were negative. Thyroid function, erythrocyte sedimentation rate, and immunoelectrophoresis results were within normal limits. Genetic testing was not conducted.
On postoperative day 10, the patient began inpatient cardiac rehabilitation.
At 3- and 8-month follow-up, the patient reported good overall well-being and improved exercise tolerance. Echocardiography showed LVEF recovery to 54%, a reduction in LV indexed end-diastolic volume to 68 mL/m2, preserved prosthetic aortic valve function (Videos 9 and 10), and improvement of the mitral regurgitation (Video 11).
Discussion
ATAAS is a rare condition associated with high mortality, especially in case of hemodynamic instability.1 Most SOV ruptures result from SOV aneurysms, defined as irreversible, usually asymmetric dilation exceeding 50% of normal diameter.2 SOV aneurysms are rare (global prevalence approximately 0.09%) and occur more frequently in men and individuals of Asian ancestry.3,4 Retrospective data (1964-1987) show that most ruptures originate from the right coronary sinus (77%), followed by the noncoronary (20%) and left coronary sinuses (3%), typically extending into the right ventricle (72%) or right atrium (23%) with exceptionally rare involvement of left heart chambers, pericardium, or septum (each <1%).5 Similar distributions, frequently coexisting with AR or ventricular septal defects, have been repeatedly reported.4 However, myocardial ischemia is an uncommon complication of left SOV aneurysm, occurring with large aneurysms rather than mild dilation, compressing the left coronary system.6 It remains uncertain to what extent the intramural hematoma located caudally to the left main contributed to the myocardial ischemia, or whether the ACS-like presentation instead reflected ischemia secondary to reduced coronary perfusion pressure in the setting of decompensated AR, LV dysfunction, and pericardial effusion. A small, localized thrombus with embolization is theoretically possible but speculative. Bacterial and nonbacterial, thrombotic endocarditis with coronary embolization was excluded as a cause of myocardial ischemia, supported by negative clinical, imaging, microbiological, serologic, and histopathologic findings.
This case is notable for several reasons. Rupture of a nonaneurysmal left SOV into the pericardium is exceptionally rare.3, 4, 5 Our patient's aortic root was symmetric and mildly dilated, with a leading-edge-to-leading-edge diameter of 41.8 mm on echocardiography (indexed diameter 22.8 mm/m2, z-score 2.12) and an inner-edge-to-inner-edge diameter of 39 mm on cardiac CTA (Figure 6).
Figure 6.
Electrocardiogram-Gated Computed Tomography Angiography of the Aortic Root Showing a Sinus of Valsalva Diameter of 39 mm, Measured Using the Inner-to-Inner Edge Method
Diagnostic challenges from cardiac motion artifacts on CTA underscore the value of ECG-gated imaging and expert cardiovascular radiology review, while TOE remains essential when uncertainty persists.2
The etiology of the SOV rupture and AR remains unclear. Given preexisting severe left ventricular dilation and reduced LVEF, in the absence of other certain causes, the regurgitation is considered chronic with acute worsening secondary to rupture of the left SOV and associated left coronary cusp rupture. Whether the tear began in the left aortic cusp and extended into the left SOV, or originated in the SOV with retrograde cusp involvement, cannot be definitively determined. In either case, ATAAS is exceedingly rare in the absence of heritable thoracic aortic disease, arterial hypertension, bicuspid aortic valve, endocarditis, and rheumatic disease.7
Although extremely rare, a potential but unproven association may exist with remote blunt chest trauma (BCT). Six years earlier, the patient sustained left dorsolateral rib fractures (ribs 6-8) in a bicycle accident. Imaging also revealed a previously healed fracture of rib 9, suggesting at least one additional prior BCT (Figure 7). This could represent a contributing factor, although imaging at the time was limited to a left hemithorax radiograph without cardiac evaluation.
Figure 7.
Limited Left Hemithorax Radiograph Obtained After a Bicycle Accident
Nondisplaced posterolateral fractures of the left ribs 6-8 are visible, with clearly defined posterior fracture lines suggesting acute fractures (yellow arrows). Rib 9 demonstrates lateral callus formation (red arrow), consistent with a healed fracture. Costodiaphragmatic angle obliteration is noted, suggestive of pleurisy. Image quality was suboptimal due to antalgic positioning, limiting full assessment of all ribs and cardiothoracic structures.
Delayed aortic lesions after BCT are very rare, usually appearing days to years later as pseudoaneurysms or aneurysms, often involving the sinotubular junction.8,9 Delayed posttraumatic AR is also rare, with 96 cases reported worldwide, showing onset from immediately to over 10 years after injury.10
Despite initial hemodynamic instability, the patient underwent successful surgical repair and achieved functional recovery.
Conclusions
This case underscores the importance of suspecting ATAAS in patients presenting with hemorrhagic pericardial effusion and relevant AR, accompanied by intense retrosternal pain. If CTA imaging remains inconclusive, TOE is crucial for diagnosis confirmation.2 After BCT, delayed cardiac complications may occur, even in patients who remain asymptomatic, highlighting the potential for late sequelae of prior injuries.8, 9, 10
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Take-Home Messages
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Transesophageal echocardiography is essential when computed tomography angiography is inconclusive for acute type A aortic syndrome.
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•
After blunt chest trauma, comprehensive cardiac evaluation and long-term follow-up may identify delayed cardiac complications, even in patients without prior cardiac disease.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
Visual Summary.
| Day 1 presentation | Acute chest tightness, large nontamponading pericardial effusion, severe AR, and suspected ATAAS. CTA inconclusive. TOE reveals rupture of nonaneurysmal left SOV and left coronary cusp. LV severely dilated, LVEF 45%. Coronary angiography: no dissection or significant stenosis. |
| Day 1 operation | Urgent evacuation of pericardial blood, suture repair of ruptured SOV, and bioAVR. Postoperative course complicated by combined hemorrhagic and cardiogenic shock, LVEF 25%, worsened MR, and well-functioning bioAVR. Stabilized after coagulation correction. |
| POD 4 | Heart failure therapy: angiotensin-converting enzyme inhibitor, β-blocker, spironolactone, dapagliflozin. Aspirin 100 mg/d for bioprosthetic valve and rosuvastatin 20 mg/d for coronary and aortic wall protection. |
| POD 8 | LVEF 36%. Follow-up CTA shows no residual dissection or hematoma. The etiology of the SOV rupture and AR is unclear but likely represents chronic AR with superimposed acute cusp rupture. A history of blunt chest trauma 6 years ago is noted as a possible, though unproven, contributing factor. |
| POD 10 | Transferred to inpatient cardiac rehabilitation. |
| POD 90 (3 mo) | LVEF improved to 50%, partial recovery of wall motion abnormalities. |
| POD 237 (8 mo) | NYHA functional class I, improved exercise tolerance. LVEF 54%, LVEDVi 68 mL/m2, prosthetic aortic valve normal (DVI 0.41). Mild-to-moderate MR persisted (posterior leaflet tethering). |
Equipment List.
Imaging
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Appendix
Transthoracic Echocardiography (4-Chamber View) Showing Circular, Isoechoic Pericardial Effusion and Mildly Reduced Left Ventricular Ejection Fraction
Coronary Angiography of the Right Coronary Artery Demonstrating no Relevant Stenosis or Dissection
Coronary Angiography of the Left Coronary Artery System Demonstrating no Relevant Stenosis or Dissection
Coronary Angiography of the Left Coronary Artery System Demonstrating no Relevant Stenosis or Dissection
Sinus of Valsalva Aortography Demonstrating Significant Retrograde, Holodiastolic Contrast Flow From the Aorta Into the Left Ventricle
Transesophageal Echocardiography (Midesophageal Long Axis View 135° Color Doppler) Demonstrating Eccentric Aortic Regurgitation, Significant Mitral Regurgitation, and Reduced Left Ventricular Ejection Fraction
Transesophageal Echocardiography (Midesophageal, 96°) Demonstrates an Intramural Hematoma of the Sinus of Valsalva at the Level of the Left/Noncoronary Sinus
The aortic valve is degenerative, with prolapse of the left coronary cusp into the left ventricular outflow tract, without clear evidence of flail motion.
Transesophageal Echocardiography (Midesophageal Short-Axis View, 30° Color Doppler) of the Aortic Valve Demonstrates Regurgitation Along the Left Coronary Cusp and a Hematoma Involving the Sinus of Valsalva at the Transition Between the Noncoronary and Left Coronary Sinuses
Transthoracic Echocardiography (Parasternal Long-Axis View, Color Doppler) 8 Months Postoperatively, Showing Good Aortic Valve Function and Mild-to-Moderate Mitral Regurgitation
Transthoracic Echocardiography (4-Chamber View) 8 Months Postoperatively, Showing Improved Left Ventricular Function, Dilated Right Ventricle With Reduced Function, and Bilateral Atrial Dilation
Case Video Summary
References
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Associated Data
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Supplementary Materials
Transthoracic Echocardiography (4-Chamber View) Showing Circular, Isoechoic Pericardial Effusion and Mildly Reduced Left Ventricular Ejection Fraction
Coronary Angiography of the Right Coronary Artery Demonstrating no Relevant Stenosis or Dissection
Coronary Angiography of the Left Coronary Artery System Demonstrating no Relevant Stenosis or Dissection
Coronary Angiography of the Left Coronary Artery System Demonstrating no Relevant Stenosis or Dissection
Sinus of Valsalva Aortography Demonstrating Significant Retrograde, Holodiastolic Contrast Flow From the Aorta Into the Left Ventricle
Transesophageal Echocardiography (Midesophageal Long Axis View 135° Color Doppler) Demonstrating Eccentric Aortic Regurgitation, Significant Mitral Regurgitation, and Reduced Left Ventricular Ejection Fraction
Transesophageal Echocardiography (Midesophageal, 96°) Demonstrates an Intramural Hematoma of the Sinus of Valsalva at the Level of the Left/Noncoronary Sinus
The aortic valve is degenerative, with prolapse of the left coronary cusp into the left ventricular outflow tract, without clear evidence of flail motion.
Transesophageal Echocardiography (Midesophageal Short-Axis View, 30° Color Doppler) of the Aortic Valve Demonstrates Regurgitation Along the Left Coronary Cusp and a Hematoma Involving the Sinus of Valsalva at the Transition Between the Noncoronary and Left Coronary Sinuses
Transthoracic Echocardiography (Parasternal Long-Axis View, Color Doppler) 8 Months Postoperatively, Showing Good Aortic Valve Function and Mild-to-Moderate Mitral Regurgitation
Transthoracic Echocardiography (4-Chamber View) 8 Months Postoperatively, Showing Improved Left Ventricular Function, Dilated Right Ventricle With Reduced Function, and Bilateral Atrial Dilation
Case Video Summary