A 29-year-old male came with history of breathlessness

Known case of decompensated heart failure precipitated by pneumonia

FINDINGS:

A: Ostium secondum ASD with defect measuring ~3.8cm in axial diameter and 3.9cm in length.
B: Moderate dilatation of right and left atrium.
C: Right ventricle is dilated with right ventricular hypertrophy.
D & E: Dilated IVC and hepatic veins.
F: Pulmonary arteries are confluent and dilated, MPA-34mm, right PA is 21 mm and left PA is 26mm.
G: Diffuse ground glass opacities noted in bilateral lungs with smooth interlobular septal thickening.
H: Suggestive of pulmonary edema.
I, J & K: The aortic root is prominent measuring ~3.8cm at the level of sinus of Valsalva, there is defect noted at the level of posterior coronary sinus with communication seen with the right atrium.

DIAGNOSIS:

Large Ostium Secundum ASD with moderate biatrial dilatation.
Dilated Pulmonary Artery (likely PAH), with measurements indicating increased pressure.
Diffuse Ground Glass Opacities in bilateral lungs, with smooth interlobular septal thickening—likely due to pulmonary edema.
Prominent Aortic Root (~3.8 cm at the sinus of Valsalva), with a defect at the posterior coronary sinus and communication with the right atrium, suggestive of a Ruptured Sinus of Valsalva.
CADRADs 0.

DISCUSSION:

A ruptured aneurysm of the aortic sinus is a major cardiovascular event that requires prompt diagnosis and intervention.
The right sinus of Valsalva is the most common site for aneurysmal dilatation, followed by the non-coronary sinus.
After rupture, a fistulous tract forms—typically to the right ventricle in the former and to the right atrium in the latter.
A radiologist's comprehensive evaluation is essential for early recognition of these signs.

Etiology:

Congenital Causes (most common):

Associated with connective tissue disorders, including Marfan syndrome, Loeys-Dietz syndrome, and Ehlers-Danlos syndrome.
Found in defects of the aortic media or a lack of elastic tissue.

Acquired Causes:

Infective endocarditis, which weakens the sinus.
Syphilitic aortitis, trauma, degenerative changes, or post-surgical/catheter complications.

Ruptured Sinus of Valsalva Aneurysm:

Caused by congenital defects or infection (endocarditis) leading to weakening of the aortic wall, or trauma.

Signs of Pulmonary Hypertension:

Main Pulmonary Artery Dilation (>33 mm)
Right Ventricular Enlargement/Hypertrophy
Right ventricle diameter > left ventricle diameter.
Bowing of the interventricular septum toward the left ventricle.
Lung Parenchymal Changes:
Scarring from prior pulmonary infarctions (e.g., wedge-shaped opacities, pleural thickening).
Mosaic lung attenuation.

Main pulmonary artery dilation (> 33 mm)

Right ventricular signs:

Lung parenchymal signs:

Scars from prior pulmonary infarctions

Mosaic lung attenuation

Treatment and Management:

Surgical intervention is the traditional treatment for rupture of the Sinus of Valsalva aneurysm:

Direct suture or patch closure depending on the rupture size.
Percutaneous closure can be performed using devices like umbrella occluders or duct occluders.

Immediate treatment is critical to prevent further complications, such as heart failure or arrhythmias.

DIFFERENTIAL DIAGNOSIS:

REFRENCES:

Doost A, Craig JA, Soh SY. Acute rupture of a sinus of Valsalva aneurysm into the right atrium: a case report and a narrative review.
Marfan Syndrome and other connective tissue disorders—Journal of Cardiovascular Genetics, 2022.
Aneurysms of the Sinus of Valsalva—European Journal of Cardiology, 2023.
Pulmonary Hypertension and its Diagnosis—American Journal of Respiratory Medicine, 2021.

Dr. A Faizel
Consultant Radiologist
Manipal Hospital, Varthur road, Bengaluru.

Dr. Pooja Sethi
Junior Resident
Manipal Hospital, Varthur road, Bengaluru.

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