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.

Outcomes of surgical treatment for carcinoid heart disease: A systematic review and meta-analysis.

BACKGROUND: Carcinoid heart disease (CaHD) develops from vasoactive substances released by neuroendocrine tumors, which can cause significant patient morbidity and mortality without surgical intervention. We performed a systematic review and meta-analysis to elucidate granular perioperative details and long-term outcomes in these patients. METHODS: Electronic search of Ovid, Scopus, Cumulative Index of Nursing and Allied Health Literature, and Cochrane Controlled Trials Register was performed to examine surgical treatment of carcinoid disease. Nine articles comprising 416 patients were selected. Study-level data were extracted and pooled for meta-analysis. RESULTS: Mean patient age was 63 years (95% confidence interval, 57-70) with 53% (95% confidence interval, 46-61) of patients being male. In addition, 75% (95% confidence interval, 54-96) of neuroendocrine tumors originated from the small bowel or colon and 98% (95% confidence interval, 93-100) had liver metastases. Right heart failure was present in 48% (95% confidence interval, 14-81). Moderate or severe regurgitation was present in 97% (95% confidence interval, 95-99) of tricuspid and 72% (95% confidence interval, 58-83) of pulmonary valves. In addition, 99% (95% confidence interval, 98-100) of tricuspid and 59% (95% confidence interval, 38-79) of pulmonary valves were replaced. Bioprosthetic valves were used in 80% (95% confidence interval, 68-93) of tricuspid positions. Mean hospital duration of stay was 16 days (95% confidence interval, 7-25). Thirty-day mortality was 9% (95% confidence interval, 6-12). Mean follow-up was 25 months (95% confidence interval, 11-39). Median survival was 3 years (95% confidence interval, 2.5-3.5). CONCLUSION: For patients >18 years of age, surgical treatment of carcinoid heart disease can be performed with a reasonable safety profile. However, overall survival appears to have ongoing effects of the primary disease.

Quadruple Valve Replacement for Carcinoid Heart Disease.

INTRODUCTION: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. There are only four previous isolated case reports in the literature. METHODS: All three patients underwent quadruple valve replacement during a single operation. Right ventricular outflow tract reconstruction with a pericardial patch was performed in all patients. For 24 hours prior to surgery, all patients received intravenous octreotide, which continued in intensive care for at least 24 hours. RESULTS: Mean cross-clamp and bypass times were 175 (range 164-197 minutes) and 210 (range 195-229 minutes) minutes, respectively. Mean intensive treatment unit (ITU) and inpatient stays were 2.3 (range 2-3 days) and 12 (range 9-16 days) days, respectively. One patient was reopened for bleeding 4 hours postoperatively from a ventricular pacing wire site. None required a permanent pacemaker postoperatively. There were no other complications in any patient. The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1. Postoperative echocardiography showed no paravalvular leaks and well-functioning prostheses in all cases. CONCLUSION: Surgery to replace all four valves is feasible with excellent medium-term survival and a very low rate of complications. Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement.

Quadruple valve replacement for carcinoid heart disease

Abstract Introduction: Carcinoid heart disease most frequently involves the tricuspid or, more rarely, the pulmonary valve and presents with right heart failure as 5-HT is metabolized by the lung. Left-sided valve involvement is quite rare. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. There are only four previous isolated case reports in the literature. Methods: All three patients underwent quadruple valve replacement during a single operation. Right ventricular outflow tract reconstruction with a pericardial patch was performed in all patients. For 24 hours prior to surgery, all patients received intravenous octreotide, which continued in intensive care for at least 24 hours. Results: Mean cross-clamp and bypass times were 175 (range 164-197 minutes) and 210 (range 195-229 minutes) minutes, respectively. Mean intensive treatment unit (ITU) and inpatient stays were 2.3 (range 2-3 days) and 12 (range 9-16 days) days, respectively. One patient was reopened for bleeding 4 hours postoperatively from a ventricular pacing wire site. None required a permanent pacemaker postoperatively. There were no other complications in any patient. The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1. Postoperative echocardiography showed no paravalvular leaks and well-functioning prostheses in all cases. Conclusion: Surgery to replace all four valves is feasible with excellent medium-term survival and a very low rate of complications. Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement.

[Tricuspid and pulmonary valve involvement in carcinoid syndrome. Report of two cases]. / Síndrome carcinoide y compromiso valvular cardiaco, a propósito de 2 casos.

Carcinoid is a rare neuroendocrine tumor typically arising in the gastrointestinal tract that can cause heart valve involvement. We report two patients with carcinoid syndrome and tricuspid/pulmonary valve lesions. A 33-year-old male presenting with fatigue and weight loss: A tumor in the tail of the pancreas was found on an abdominal CAT scan. The percutaneous biopsy was informed as a carcinoid tumor. A trans-esophageal echocardiogram showed a tricuspid and pulmonary valve involvement, which was replaced surgically. The biopsy informed an extensive fibrous and myxoid degeneration of the valves. A 35-year-old male with a carcinoid syndrome and cardiac failure: An echocardiogram showed a severe tricuspid stenosis and severe pulmonary regurgitation. The patient was subjected to a double surgical valve replacement. The pathology report of the excised valve showed a deforming fibrous and myxoid valvulopathy.

Síndrome carcinoide y compromiso valvular cardiaco, a propósito de 2 casos: Report of two cases / Tricuspid and pulmonary valve involvement in carcinoid syndrome

Carcinoid is a rare neuroendocrine tumor typically arising in the gastrointestinal tract that can cause heart valve involvement. We report two patients with carcinoid syndrome and tricuspid/pulmonary valve lesions. A 33-year-old male presenting with fatigue and weight loss: A tumor in the tail of the pancreas was found on an abdominal CAT scan. The percutaneous biopsy was informed as a carcinoid tumor. A trans-esophageal echocardiogram showed a tricuspid and pulmonary valve involvement, which was replaced surgically. The biopsy informed an extensive fibrous and myxoid degeneration of the valves. A 35-year-old male with a carcinoid syndrome and cardiac failure: An echocardiogram showed a severe tricuspid stenosis and severe pulmonary regurgitation. The patient was subjected to a double surgical valve replacement. The pathology report of the excised valve showed a deforming fibrous and myxoid valvulopathy.

Sindrome carcinoide: achados ecocardiográficos na evolução clínica de um caso de síndrome carcinoide / Carcinoid syndrome: echocardiography findings in a clinical evolution of a carcinoid syndrome case

O comprometimento cardíaco na síndrome carcinoide ocorre em 50% dos pacientes, sendo o ecocardiograma um importante meio diagnóstico da doença. Os achados de exame físico podem passar despercebidos, como o sopro tricúspide e pulmonar, em virtude dabaixa pressão no território pulmonar. Apesar de alguns casos responderem bem aos análogos da somastotina, como o octeotride, a doença cardíaca evolui, inexoravelmente, para insuficiência cardíaca, sendo a indicação cirúrgica uma opção para determinados casos.