A challenging case report of acute sinus of Valsalva aneurysm rupture in cardiogenic shock and multi-organ failure with an emphasis on rapid recognition and ‘never giving up’ in face of futility

Abstract

Abstract Background  Sinus of Valsalva aneurysm (SoVA) is a rare anomaly and can be divided into acquired and congenital forms, the latter being commonly associated with ventricular septal defects (VSDs). Rupture is a catastrophic complication with high mortality without urgent surgical intervention. We would like to highlight the use of echocardiography in an emergency setting for diagnosis and surgical intervention in a critically ill patient. Case summary  We report a 27-year-old female with history of conservatively managed VSD known since childhood. She presented with acute decompensated cardiac failure requiring intubation and inotropic support. Bedside echocardiography performed in the emergency department suggested a ruptured SoVA at the right coronary cusp with underlying supracristal VSD. Despite the patient being critically ill with multi-organ failure, surgery was performed as it was the patient’s best chance for survival. Intraoperative findings tallied with the early echocardiographic results. She recovered gradually and was eventually discharged despite a stormy post-operative period. Discussion  This case report highlights the importance of prompt recognition of SoVA rupture by using bedside echocardiography. Surgical intervention needs to be early despite ongoing sepsis in view of acute mechanical failure. This case was unique as it illustrates a successful management of an acutely ill patient with multi-organ failure through early diagnosis, intensive perioperative stabilization, and surgical intervention.