Abstract
IntroductionCarcinoid 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.MethodsAll 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.ResultsMean 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.ConclusionSurgery 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.
Highlights
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
The quality of life was excellent at 6-16 months clinic follow-up as they were in NYHA 1
Patients with carcinoid heart disease should always be considered for surgery irrespective of the extent of valvular involvement
Summary
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. We describe our experience of 3 patients presenting with heart failure secondary to carcinoid heart disease affecting all four cardiac valves. Cardiac involvement in carcinoid disease generally results in tricuspid insufficiency and pulmonary stenosis. Carcinoid tumours must be invasive or metastasise to produce carcinoid syndrome, which is characterized by facial flushing, diarrhea, and bronchospasm. The incidence of carcinoid tumours is approximately 1 in 75,000 of the population[2], of whom about 50% develop carcinoid syndrome. Once carcinoid syndrome has developed, approximately 50% of these patients will go on to develop carcinoid heart disease or Hedinger syndrome
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