Development of severe intrapulmonary shunting in a patient with carcinoid heart disease after closure of a persistent foramen ovale: a case report

Background Neuroendocrine tumors (NETs) can affect the cardiopulmonary system causing carcinoid heart disease and valve destruction. Persistent foramen ovale (PFO) occlusion is indicated in patients with carcinoid heart disease and shunt-related left-heart valve involvement. Case Summary We report the case of a 54-year-old female patient with metastatic NET originating from the small bowel. The patient was on medication with octreotide and telotristat. One year after diagnosis, cardiac involvement of carcinoid developed with regurgitation of right-sided and, due to PFO, left-sided heart valves. Closure of PFO was performed (Occlutech 16/18 mm). One year later she presented with recurrent severe dyspnoea. The PFO-occluder was in situ without residual shunt. Valvular heart disease, including left-sided disease, and metastatic spread of NET were stable. Blood gas analysis revealed arterial hypoxemia (pO2 = 44 mmHg/5.87 kPa), which was related to extensive intrapulmonary shunting (31% shunt fraction) confirmed using contrast-enhanced echocardiography. The patient was prescribed long-term oxygen supplementation as symptomatic therapy and anti-tumoral therapy was intensified with selective internal radiotherapy of the liver metastases in order to improve biochemical control of the carcinoid syndrome. Discussion An echocardiographic assessment of the presence of a PFO is recommended in patients with NET as PFO closure minimizes the risk of left-sided carcinoid valve disease. Deterioration of symptomatic status in metastasized NET might also be due to a hepatopulmonary-like physiology with intrapulmonary shunting and arterial desaturation thought to be caused by vasoactive substances secreted by the tumor. This is a rare case describing the development of this syndrome after PFO closure.

Atrial fibrillation screening on holter monitoring after percutaneous patent foramen ovale closure: a prospective study

Background An increased risk of new-onset atrial fibrillation (AF) after patent foramen ovale (PFO) closure was observed in randomized trials. However, systematic screening of AF was not performed in these studies. Purpose We aimed to evaluate incidence of AF in patients undergoing percutaneous PFO closure with serial 24-hours ambulatory electrocardiogram (AECG) monitoring during a 6-months follow-up. Methods All consecutive patients undergoing PFO closure for cryptogenic stroke or decompression sickness (DSC) were prospectively included in 2 centers. AF was defined as an irregular rhythm without discernible P waves lasting at least 30 seconds on 24-hours AECG performed at day 0, 1 month and 6-months follow-up. Primary endpoint was incidence of AF within the study period. Secondary endpoints evaluated clinical outcomes (hospitalization for cardiovascular causes, new stroke or bleeding) within 6-months follow-up. Results Between February 2018 and March 2019, a total of 62 patients underwent PFO closure: 59 (95.2%) for cryptogenic stroke and 3 (4.8%) for DSC. In the overall population, 40 were male (64.5%) and the mean age was 48±9.5 years. An atrial septal aneurysm was present in 37 patients (64.9%). The PFO device mean size was 26±5.0 mm. After a mean follow-up of 33.3±12.2 weeks, new-onset AF occurred in 3 patients (4.8%), all within 1-month after closure and paroxysmal. While symptomatic ectopic atrial heart beats occurred in one fourth of patients, all AF episodes asymptomatic. Two patients (3.2%) required oral anticoagulant therapy according to CHA2DS2-VASc score. No adverse outcomes occurred. Age (RR 1.26 [0.98; 1.61] p=0.3), male sex (p=0.6) and device (p=1.0) were not associated with occurrence of AF but all patients with AF were men and received an Amplatzer device. Conclusion Incidence of AF, evaluated with serial systematic 24-hours AECG during a 6-months follow-up after PFO closure, was relatively low (<5%). Always paroxysmal, AF always occurred within one month after the procedure and was not associated with adverse outcomes. FUNDunding Acknowledgement Type of funding sources: None.

Patent Foramen Ovale and Cryptogenic Stroke: Challenges in Diagnosis and Management

A patent foramen ovale (PFO) is a common disorder that affects between 20-34% of the adult population. This condition is a benign finding for most people. However, In some the PFO can open widely and enabling paradoxical embolism to transit from venous to arterial circulation, which is associated with stroke and systemic embolization. There are still unclear to date regarding the effectiveness of pharmacological anticoagulant therapy, defined as antithrombin or antiplatelet therapy, which has proven to be more beneficial for patients with PFO and cryptogenic stroke. In addition, surgical and transcutaneous PFO closure has been proposed for secondary prevention of stroke in patients with cryptogenic stroke with PFO. Both catheter-based and surgical modes of closure have been shown to reduce the incidence of subsequent embolism substantially. This review will discuss the evidence regarding the relationship between PFO and cryptogenic stroke and decision making for management strategies.

Cryptogenic Stroke: A Challenge in Diagnosis and Management

Ischemic stroke is responsible for 85% of all stroke globally. However, the etiology of around a quarter of ischemic stroke are undetermined, this is called cryptogenic stroke. This kind of stroke affects younger population. Several mechanism are associated with the incidence of cryptogenic stroke such as paroxysmal atrial fibrillation, patent foramen ovale, atherosclerosis, and atrial cardiopathy. Despite many advanced knowledge on stroke generally, cryptogenic stroke is still a challenge in clinical settings. To understand more about cryptogenic stroke, a new term of embolic strokes of undetermined source (ESUS) is proposed and may need a specific workup. Specific workup aims to detect any silent risk factors and also to evaluate the cardiac structure. The term of ESUS also leads to the understanding that cryptogenic stroke is highly related to embolic mechanism and anticoagulation administration might benefit the patients. However, the result of several recent studies showed that anticoagulant was not superior to antiplatelet, and antiplatelet is still the preferred treatment. Studies on PFO closure also shows different result, but the majority of the trials showed benefit of PFO closure in reducing the risk of stroke recurrence.

Contemporary Management of Patent Foramen Ovale: A Multinational Survey on Cardiologists’ Perspective

Objectives. The purpose of our survey is to analyze the clinical approach used by interventional and imaging cardiologists to diagnose, treat, and follow-up patients with PFO-related left circulation thromboembolism in different parts of the world with particular emphasis on adherence to current guidelines. Background. Firm guidelines do not cover many aspects of PFO-related patient care. Consequently, very disparate approaches exist among clinicians in the real-world. Methods. A 24-item electronic questionnaire was sent directly to experienced cardiology specialists practicing at consultant/attending positions directly involved in PFO closure management in the United States, United Kingdom, Gulf countries, and other countries. There were no unanswered questions. Responses were recorded between October 2019 and July 2020. Results. Seventy-one responses were obtained: 31 from the UK, 19 from the US, 16 from Gulf countries, 2 from Poland, and 1 response from Australia, Italy, and Switzerland. The overall response rate was 76%. Significant differences between regions were noted in the duration of ECG monitoring during the diagnostic process, PFO closure for left circulation thromboembolism other than stroke/transient ischemic attack, and intraoperative use of intracardiac echocardiography. A similar pattern was noted in the lack of routine screening for thrombophilia and the use of the long-term single antiplatelet therapy. Conclusions. The study shows a vast spectrum of opinions on the optimal approach to PFO closure with significant differences between the US, UK, and Gulf countries. The results stress the need for systematic, high-quality data on the diagnostic work-up and follow-up strategies to inform the standardized approach.