The Clinical Impact of Patent Foramen Ovale in Patients With Carcinoid Heart Disease

Background: Carcinoid heart disease (CaHD) typically affects the right-sided valves. Rarely, CaHD affecting the left-sided valves has been observed in the setting of patent foramen ovale (PFO). However, the impact of PFO on valve disease progression and prognosis in CaHD is not well understood. Objectives: The purpose of this study was to evaluate the impact of PFO on CaHD progression and clinical outcomes. Methods: Patients diagnosed with neuroendocrine tumor and carcinoid syndrome (CS), and with serial transthoracic echocardiograms, were included. The primary endpoint was progression of left-sided valve disease. Secondary endpoints included the requirement for left-sided cardiac valve surgery, progression of right-sided valve disease, mortality, and stroke. Results: Of 413 patients with neuroendocrine tumor and CS, 175 met the inclusion criteria; mean age 61.3 ± 11.3 years, 54.9% male, and 67 patients had a PFO. Through 3.6 years (1.1-5.9) between serial transthoracic echocardiograms, patients with PFO were more likely to have progression of left-sided valve disease (adjusted HR [aHR]: 1.93; 95% CI: 1.09-3.42; P = 0.025) and require left-sided valve replacement (13.6% vs 2.1%, log-rank; P = 0.012). Patients with PFO did not have a significant increase in right-sided progression (aHR: 1.51; 95% CI: 0.91-2.51; P = 0.111). Patients with a PFO also had an increased risk of stroke (aHR: 5.42; 95% CI: 1.57-18.70; P = 0.007). Conclusions: Patients with CS and PFO were at increased risk of progression of left-sided valve disease and valve surgery. PFO presence was also associated with an increased risk of stroke, highlighting the importance of surveillance and consideration of potential early intervention.