Aims Valvular surgery has improved long-term prognosis in severe carcinoid heart disease (CaHD). Experience is limited and uncertainty remains about predictors for survival and strategy regarding single vs. double-valve surgery. The aim was to review survival and echocardiographic findings after valvular surgery for CaHD at our institution. Methods and results Between 1986 and 2019, 60 consecutive patients, median age 64 years, underwent valve surgery for severe CaHD. Operations involved combined tricuspid valve replacement (TVR) and pulmonary valve replacement (PVR) in 42 cases, and TVR-only or TVR with pulmonary valvotomy (no PVR) in 18 patients. All implanted valves were bioprosthetic. Preoperative echocardiography, creatinine, NT-pro-brain natriuretic peptide (NT-pro-BNP), and 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) were obtained. 30-Day mortality was 12% (n=7), and 8% for the most recent decade 2010–2019. Median survival was 2.2 years and maximum survival 21 years. Patients undergoing combined TVR and PVR had significantly higher survival compared with operations without PVR (median 3.0 vs. 0.9 years, P = 0.02). Preoperative levels of NT-pro-BNP and 5-HIAA in the top quartile predicted poor survival. On preoperative echocardiograms, pulmonary regurgitation was severe in 51% and indeterminate in 17%. Postoperative echocardiography confirmed relatively good durability of bioprostheses, relative to the patients’ limited oncological life expectancy. Conclusion Valvular surgery in CaHD has an acceptable perioperative risk. Survival for combined TVR and PVR was significantly higher compared with operations without PVR. Bioprosthetic valve replacement appears to have adequate durability. Preoperative echocardiography may underestimate pulmonary pathology. Combined TVR and PVR should be considered in most patients.

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Background Valvular surgery for carcinoid heart disease (CaHD) has been found to improve long-term prognosis. Experience is limited and questions about single versus double-valve surgery as well as selection of prosthetic valves remain under debate. Purpose We reviewed the surgical experience after valvular surgery for CaHD in our institution, which is a national referral center. Methods We reviewed clinical records and echocardiograms. Perioperative death was defined as mortality within 30 days of operation. Results Sixty consecutive patients (32 men, 62±9 years) underwent valve surgery between 1986 and 2019, of whom 59/60 had an intestinal carcinoid disease with hepatic metastases. Mean time from diagnosis to heart surgery was 3.8±3.7 years. Preoperatively, 6 patients were in NYHA class I/II, the others were in class III or IV. All 60 surgical procedures involved tricuspid valve replacement (TVR). In 47 cases, the pulmonary valve was treated surgically: Early in the series, five patients underwent pulmonary valvotomy or commissurotomy, and more recently, 42 patients received pulmonary valve replacement (PVR). All valves were replaced with bioprostheses, except for one pulmonary homograft. Concomitant significant aortic and mitral regurgitation occurred in 2 patients who received quadruple valve replacement. Two re-operations were performed due to degeneration of bioprostheses. Mean hospital stay was 13±9 days. Overall 30-day mortality was 11.6% (n=7) but was reduced to 8.3% in the last decade. The overall median survival was 2.2 years. Maximal survival free of reoperation was 18 years, and maximal survival for a single patient was 21 years. Median survival for combined TVR and PVR was significantly better than for single TVR or TVR combined with pulmonary valvotomy (3.0 years vs. 0.94 years, respectively, p=0.02; see Figure). Preoperatively, left ventricular ejection fraction (EF) was normal in 83%. Severe tricuspid regurgitation was always present. The right ventricle (RV) was dilated (RVD1 49 mm ± 5 mm) as well as the right atrium (60±16 ml/m2). The TAPSE was in normal range (17 to 29 mm). Pulmonary regurgitation was mild, moderate, severe, or indeterminate in 6%, 26%, 51%, and 17% respectively. In 17% the transpulmonary velocity was >2.5 m/s, indicating stenosis. On last available postoperative echo (mean time 2.1 years) EF was found unchanged. RV diameter was nearly normalized (RVD1 41 mm ± 5 mm). TAPSE was reduced (12 mm ± 4 mm). No significant regurgitation was detected in any TVR. There was significant stenosis (mean gradient >5 mmHg) in 23% of the TVR. No regurgitation of significance was detected in any PVR. A mean gradient >20 mmHg was found in 11% of the PVR. Conclusions While perioperative risk of valve replacement in CaHD remains substantial, this study shows relatively favourable outcomes of surgical valve replacement with bioprostheses. PVR together with TVR had better outcomes than those undergoing TVR only. Figure. Survival by Surgical Treatment. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Department of Medical Sciences, Uppsala University. Department of Surgical Sciences, Uppsala University

Subjects with asymptomatic moderate-to-severe or severe primary mitral regurgitation are closely observed for signs of progression or symptoms requiring surgical intervention. The role of myocardial metabolic function in progression of mitral regurgitation is poorly understood. We used 11 C-acetate PET to noninvasively measure myocardial mechanical external efficiency (MEE), which is the energetic ratio of external cardiac work and left ventricular (LV) oxygen consumption. Methods: Forty-seven patients in surveillance with mitral regurgitation and no or minimal symptoms prospectively underwent PET, echocardiography, and cardiac MRI on the same day. PET was used to simultaneously measure cardiac output, LV mass, and oxygen consumption to establish MEE. PET findings were compared between patients and healthy volunteers (n 5 9). MEE and standard imaging indicators of regurgitation severity, LV volumes, and function were studied as predictors of time to surgical intervention. Patients were followed a median of 3.0 y (interquartile range, 2.0-3.8 y), and the endpoint was reached in 22 subjects (47%). Results: MEE in patients reaching the endpoint (23.8% 6 5.0%) was lower than in censored patients (28.5% 6 4.5%, P 5 0.002) or healthy volunteers (30.1% 6 4.9%, P 5 0.001). MEE with a cutoff lower than 25.7% was significantly associated with the outcome (hazard ratio, 7.5; 95% CI, 2.7-20.6; P , 0.0001) and retained independent significance when compared with standard imaging parameters. Conclusion: MEE independently predicted time to progression requiring valve surgery in patients with asymptomatic moderate-to-severe or severe primary mitral regurgitation. The study suggests that inefficient myocardial oxidative metabolism precedes clinically observed progression in mitral regurgitation.