Upon onset of New York Heart Association (NYHA) heart failure class III or IV symptoms in patients with CaHD, 2‑year survival has been recorded as low as 10% [
39]. Valve replacement is the only effective treatment option for symptomatic CaHD patients and is associated with symptomatic improvement [
20,
40] and increased survival [
20,
39‐
44]. Recently, 200 CaHD patients (of which 87 underwent cardiac surgery) were analysed and all-cause mortality was assessed [
41]. The average age was 63 years and the majority of patients were in NYHA class II or III. Predictors of 10-year all-cause mortality by multivariate Cox proportional hazard analysis were age, urinary 5‑HIAA excretion, moderate or severe RV dilation, and cardiac surgery. Cardiac surgery was associated with a risk reduction of 0.48 (95% CI 0.31 to 0.73,
p < 0.001). However, these data should be interpreted in light of the non-randomised study design with patients diagnosed in a large time frame from 1981–2000. Importantly, the percentage of patients who underwent cardiac surgery has increased over the years. It is therefore conceivable that the beneficial effect of surgery is influenced by other factors, such as improved medication, experience, and patient selection. A more recent study analysed outcomes after surgical valve replacement in 19 patients [
42]. The mean age was 56 years and the average NYHA class was III. A 5-year survival rate of 43% was found. No predictors for mortality were identified although preoperative 5‑HIAA levels were lower in patients who were still alive during data analysis than in those who died (not significant). In a similar study short- and long-term outcomes of CaHD following valve replacement were retrospectively assessed [
39]. In total 195 patients were analysed. The mean age was 61 years and 70% of the patients were classified in NYHA class III and IV. All patients underwent tricuspid valve replacement, and 81% pulmonary valve replacement. Survival rates at 1, 5, and 10 years were 69%, 35%, and 24%, respectively. Univariate predictors of overall mortality included age, preoperative creatinine, NYHA class, use of loop diuretics, preoperative chemotherapy, ascites, diabetes mellitus, tobacco use, left-sided valve disease, and right-sided heart size and function.
Patients who are ineligible for cardiac surgery may benefit from pulmonary balloon valvuloplasty. Case studies have been reported where balloon valvuloplasty was performed with major clinical improvements afterwards [
45,
46], although relapsing stenosis poses a significant threat [
47]. Therefore, surgery should be preferred.