Hedinger Syndrome with Intraoperative Carcinoid Crisis — Understanding the Pathophysiology for a Successful Management

Abstract Carcinoid tumors can be a cause for right heart valve disease, also known as Hedinger syndrome or carcinoid heart disease. Proper understanding of the pathophysiology is of the uttermost importance for adequate treatment of these patients, especially during heart surgery.

Hedinger Syndrome with Intraoperative Carcinoid Crisis - Understanding the Pathophysiology for a Successful Management.

Carcinoid tumors can be a cause for right heart valve disease, also known as Hedinger syndrome or carcinoid heart disease. Proper understanding of the pathophysiology is of the uttermost importance for adequate treatment of these patients, especially during heart surgery.

Surgical management of a giant right coronary artery aneurysm with coronary arteriovenous fistula: case report.

BACKGROUND: Coronary artery aneurysms (CAAs) are uncommon, and giant aneurysms (>2 cm) are even more unusual. Coronary atherosclerosis and Kawasaki disease are the leading causes for this pathology. The treatment for this condition is controversial because the evidence is based on case report series. CASE SUMMARY: We describe the case of a 77-year-old female patient who presented with heart failure symptoms. She was diagnosed with a giant saccular aneurysm arising from the right coronary artery (RCA) ostium and a fistula between the RC and the left anterior descending artery (LAD) to the coronary sinus. And an atrial septal defect (ASD) and severe tricuspid regurgitation were also found. The patient underwent surgery through a medium sternotomy, the aneurysm was opened and resected under cardiopulmonary bypass. The RCA was ligated at the distal end of the aneurysm, and a saphenous vein graft bypass was performed. A coronary arteriovenous fistula from the distal portion of RC and LAD artery to a severely enlarged coronary sinus was found and corrected with an autologous pericardial patch. Closure of the ASD was performed with a pericardial patch and a tricuspid ring annuloplasty was done. Post-operative course was uneventful. DISCUSSION: There are few cases of giant coronary aneurysms associated with fistulas reported in the literature. Despite the endovascular percutaneous techniques available to treat these patients, we believe that surgical treatment was the best option for this particular case. We consider that surgical treatment is a very good option for giant CAAs associated with AV fistulas that are not susceptible for current endovascular available devices. The literature lacks evidence regarding the best approach for these cases, and we think that invasive treatment should be tailored according to the heart's anatomy and patient risk.