Hedinger Syndrome Secondary to Carcinoid Tumor of the Ovary: Anesthetic Challenge and Echocardiographic Illustration

摘要

Carcinoid syndrome is the conglomeration of symptoms mediated by humoral factors produced by a neuroendocrine tumor. It is characterized by gastrointestinal hypermotility, bronchospasm, hypotension, and vasomotor symptoms. It is associated with carcinoid heart disease (CHD) in >50% of patients. We are reporting a case of a 69-year-old female who presented with congestive heart failure, platypnea-orthodeoxia syndrome with refractory diarrhea. After medical stabilization and diagnosing the female as a case of CHD with a primary tumor located at the ovary; she was planned for exploratory laparotomy, bilateral salpingo-oophorectomy, and retroperitoneal pelvic lymph node removal, followed by tricuspid valve (TV) intervention. Carcinoid tumors are neuroendocrine tumors arising primarily from the small bowel. The incidence is 1-2 per lakh people. Around 20-30% of individuals develop carcinoid syndrome due to the release of vasoactive substances like histamine, serotonin, bradykinin, and prostaglandin. It is manifested in the form of episodic vasomotor symptom, hypotension, diarrhea, bronchospasm, and right heart failure due to CHD. Carcinoid heart disease (CHD) develops in 20-30% of patients with a carcinoid tumor, which has a very poor outcome. The median life expectancy is approximately 5.9 years without CHD, whereas reduced to 2.6 years with CHD. Therefore, screening for cardiac involvement is vital in patients with novel carcinoid syndrome. Carcinoid tumors of the small bowel, followed by the lung, large bowel, pancreas, appendix,and ovaries, have the highest incidence of HD. It is characterized by the deposition of carcinoid plaque (myofibroblast and collagen in the myxoid matrix), mainly around the right heart structure. Tricuspid regurgitation and mixed pulmonary stenosis and regurgitation are the major manifestations that may result in right heart failure.