CASE PRESENTATION: We reported a rare case of large papillary fibroelastoma in the right atrium of a young gentleman which was complicated with pulmonary embolism. Transthoracic echocardiography identified a large pedunculated mass measuring 3.4cmX3.4cmX2cm in right atrium with stalk attached to interatrial septum. The intracardiac mass was resected surgically, which revealed papillary fibroelastoma in histology examination.
CONCLUSION: Differential diagnosis of intracardiac masses requires clinical information, laboratory tests and imaging modalities including echocardiography. Incidentally discovered papillary fibroelastomas are treated on the basis of their sizes, site, mobility and potential embolic complications. Due to the embolic risk inherent to intraacardiac masses, surgical resection represents an effective curative protocol in treating both symptomatic and asymptomatic right sided and left sided papillary fibroelastomas, with excellent long term postoperative prognosis.
METHODS: A retrospective analysis was conducted on 24 patients with severe PR post-surgical TOF correction who underwent left anterior mini-thoracotomy PVR in Penang General Hospital from January 2021 to January 2023.
RESULTS: The median age was 23.5 years (I.Q.range 17.6-36.3), with a male:female ratio of 1:4. Majority of patients had mild to moderate symptoms prior to surgery and 19 patients (79.1%) were on regular diuretics medication. All patients had severe free-flow PR with evidence of right ventricular dilatation and dysfunction. Magnetic Resonance Imaging and computed tomography of pulmonary artery were performed prior to surgery. Minimally invasive PVR was performed on all patients via left upper anterior mini-thoracotomy and femoral-femoral bypass without cardioplegic arrest. The operative time and cardiopulmonary bypass time were 208 (I.Q.range 172-324) and 98.6 minutes(I.Q.range 87.4-152.4) respectively. The time to wean off inotropes postoperatively was 6.2 hours (I.Q.range1.4-14.8), and no postoperative arrhythmia and chest re-exploration were reported. Most patients stayed in Intensive Care Unit (ICU) for 10.8 hours (I.Q.range 8.4-36.5), and the total hospital stay was 4.2 days (I.Q.range 3.4-7.6). 2 patients (11.1%) required blood transfusion postoperative. There was no paravalvular leak and no mortality during the follow-up period of up to 28 months.
CONCLUSION: Minimally invasive PVR after surgical correction of TOF is a safe alternative to the conventional redo-sternotomy approach in patients with favorable anatomy. This approach is able to reduce the risks associated with redo-sternotomy, particularly bleeding and injury to mediastinal structures, with the additional benefit of expedited recovery and hospital discharge. Our series has shown a safe and efficient approach in these patients with favorable outcomes.
CASE PRESENTATION: We present a successful aortic valve replacement with cardiopulmonary bypass in a 48 years old lady with antiphospholipid syndrome, who has severe aortic regurgitation as a result of Libman-sacks endocarditis. Antiphospholipid antibodies were positive and the clinical data showed both negative cultures and infective parameters. Surgically resected vegetations revealed sterile fibrinous and verrucous vegetations on aortic valve. Valve replacement and the course of cardiopulmonary bypass was uneventful, and the patient was discharged well.
CONCLUSIONS: Classically Libman-Sacks endocarditis is often and more commonly associated with autoimmune diseases such as systemic lupus erythematosus, although it can occur in both primary and secondary antiphospholipid syndrome. It is not a common entity, and it is a frequent underestimated disease as most clinicians do not routinely screen for valvular lesion in patients with antiphospholipid syndrome unless they are symptomatic. However, due to its high prevalence of cardiac involvement, clinicians should have a high index of suspicion in the attempt to minimize cardiovascular and haemodynamic complications. Valve surgery in patients with antiphospholipid syndrome carries considerable early and late morbidity and mortality, usually caused by thromboembolic and bleeding events. The perioperative anticoagulation management and haemostatic aspect of antiphospholipid syndrome present an exceptional challenges to clinicians, surgeons, anaesthetists and laboratory personnel.