Teaching Case

A 14-year-old boy who was previously feeling well but who had a sudden onset of chest pain and tachycardia was referred by the General Practitioner. His transthoracic echocardiogram showed a "windsock" protruding into the right atrium with right atrial dilatation. The boy underwent an emergency cardiac surgery and made a satisfactory recovery.

A 14-year-old boy who was previously fit and feeling well was referred to the Paediatric emergency department. His pulse rate was 120/min and his ECG was found to be normal. Later on in the evening, he developed a central crushing chest pain, which was non-radiating and became worse on inspiration. There was no history of trauma or gastritis. Three years earlier he was referred to a cardiologist because he had an irregular pulse and heart murmur. His echocardiogram was found to be normal at the time, and his ECG showed atrial premature beats, which sometimes had a bigeminal pattern. The murmur was thought to be physiological. Upon presentation, he appeared to be in good health and his pulse rate was 120/min with a good volume. On auscultation, he had normal heart sounds with a continuous murmur and no radiation. His peripheral pulses were palpable, and his blood pressure was 100/48 mm of Hg (sitting) and 100/62 mm of Hg (standing). He had some epigastric tenderness. The rest of the examination proved to be unremarkable. His transthoracic echocardiogram showed the ruptured sinus as a “windsock” protruding into the right atrium with right atrial dilatation.

Sinus of Valsalva aneurysm is believed to be a rare congenital cardiac anomaly, which is usually diagnosed in adulthood. The average age when the rupture of sinus of Valsalva occurs approximately 30 years with a range of 11–67 years. The three sinuses of valsalva are located in the most proximal portion of the aorta, just above the cusps of the aortic valve. Aneurysmal dilatation of these sinuses occurs when the aortic media is defective. The rupture of the aneurysm can be fatal. Although rare, it can present in childhood. Sinus of Valsalva can rupture in different chambers of the heart, including the right ventricle and the pulmonary artery. The patient's echocardiogram (long axis view) shows the flow of blood from the aorta into the right atrium. An emergency cardiac surgery is recommended when the ruptured sinus of Valsalva is diagnosed. Presentation of a child with a sudden onset of chest pain and a continuous murmur suggest the diagnosis to be ruptured sinus of Valsalva. Transoesophageal echocardiography is a very useful investigation that helps define the exact point of rupture. Other useful investigations are chest X-ray examinations, ECG and MRI. Appropriate investigations and early intervention can prove to be life saving.