Diagnosis of “Bursting Heart Syndrome” Due to an Ilio-Caval Fistula in the Emergency Department: A Case Report

Abstract: “Bursting Heart Syndrome” is a clinical condition resulting from a sudden increase in venous return associated with peripheral arterial insufficiency, caused by an arteriovenous fistula typically located between the caval system and the aorta, most commonly in aorto-caval fistulas. These represent one of the least recognized complications of abdominal aortic aneurysms encountered in the emergency setting. This syndrome may go unrecognized and can rapidly lead to cardiac arrest. Among these fistulas, those involving communication between the inferior vena cava (IVC) and the common iliac artery (ilio-caval fistulas) are extremely rare. Apart from aortic aneurysms, etiologies are predominantly traumatic and iatrogenic, although other causes have been reported. Clinical presentation is variable and may result in delayed diagnosis. However, specific clinical and imaging findings can establish the diagnosis. Doppler ultrasound, owing to its immediate availability in the emergency department, and contrast-enhanced computed tomography angiography (CTA) of the abdomen are essential diagnostic imaging modalities. Doppler ultrasound often allows visualization of the site of communication between the artery and the vein, while CTA confirms the diagnosis. Treatment is surgical, either via open or endovascular approaches. We report the case of a 69-year-old female patient, followed in cardiology for multivalvular heart disease for six years, with a history of uterine fibroids with recurrence, and treated for a leg ulcer, who presented to the emergency department with worsening dyspnea associated with orthopnea and abdominal pain. On examination, she exhibited signs of global heart failure. Given the abdominal pain, an urgent ultrasound examination was performed, demonstrating a dilated IVC with a “yin-yang” sign on color Doppler, associated with dilatation of the iliac veins, more pronounced on the right side. A rapid Doppler survey of the veins of both lower limbs revealed arterialization of flow within the deep and superficial venous systems, with multiple bilateral pulsatile varicose clusters exhibiting arterialized flow. Careful assessment of the walls of the major abdominal vessels identified a breach in continuity establishing communication between the right common iliac artery and the IVC, located immediately above the confluence of the two common iliac veins and a few millimeters distal to the aortic bifurcation. In the context of pulsatile varicosities, hypotension, development of distal cyanosis of the lower limbs, and worsening peripheral venous insufficiency, together with Doppler ultrasound findings, the diagnosis was retained as a contributing cause of cardiac decompensation in this patient. Emergency abdominal CTA was indicated; however, hemodynamic deterioration with associated renal failure necessitated immediate admission to the intensive care unit, preceding the patient’s death due to cardiogenic shock. Early diagnosis and surgical management of such fistulas prior to the onset of shock may double survival rates, underscoring the importance of prompt recognition [1-5]. Subsequently, a brief review of the literature is presented.