Cardiac papillary fibroelastomas (PFEs), the second most common intracardiac tumor, are frequently encountered in valvular structures (aortic valve [AV] > mitral valve > tricuspid valve) along with a pedunculated, mobile, and avascular tumor characteristics.1 Less than 10% of these primary benign tumors can be found elsewhere in the myocardium. These locations include the right and left atrial and ventricular endocardium, Chiari network, the proximal left ventricular outflow tract/ interventricular septum, and coronary ostia.2 Most patients with PFEs are asymptomatic, with a mean age of 60 years, and are diagnosed incidentally; however, symptomatic patients commonly have evidence of embolic events such as transient ischemic attacks (TIAs), myocardial infarction, and sudden death or valvular dysfunction.1 The standard procedure for removal of PFEs involving the left ventricle (LV) cavity entails performing a ventriculotomy for exposure and complete resection of the mass; however, although one group has performed an endoscopically assisted resection of two PFEs using a flexible scope and snaring of the tumor stalk, one through the AV and the other through the mitral valve, a novel and direct resection through the AV has not been described.3,4 Performing a ventriculotomy can potentially lead to several complications intraoperatively and postoperatively, including bleeding, conduction dysfunction, and impaired blood flow to the myocardium, whereas endoscopic techniques with the assistance of sorbitol solution in the LV could lead to less resected margins that are free of neoplasm. This case and its procedural approach to the resection of LV cavity tumors represent a novel minimally invasive technique, using a mediastinoscope placed through the AV to directly visualize and resect the tumor.
This 68-year-old gentleman experienced an episode of TIA whereby right-sided eye blindness was appreciated for 20 seconds. Echocardiogram demonstrated a pedunculated mass in the LV apex and was referred to cardiology. While waiting to be seen by cardiology, the patient experienced another TIA with right lower facial numbness. Computed tomography scan of the brain showed no acute intracranial abnormalities. Angiogram and cardiac catheterization showed no evidence of coronary artery disease along with normal pulmonary arterial pressures. Transthoracic echocardiography (TTE) revealed a 16 × 11–mm mobile pedunculated mass in the LV apex. Cardiac magnetic resonance imaging demonstrated a 10 × 5–mm mass located in the anterolateral apex of the LV (Fig. 1). The patient was referred to cardiac surgery and consent was obtained for resection of the pedunculated LV mass.
A median sternotomy was performed and the pericardial sac was excised to expose the beating heart. An epicardial ultrasound study was carried out and revealed normal LV wall thickness and systolic and diastolic function, along with the 16 × 11–mm pedunculated mobile mass with irregular borders arising from the anterolateral wall of the LV apex. Arterial and venous lines were placed as per usual fashion. Cardiopulmonary bypass (CPB) was initiated along with antegrade cardioplegia. The aorta was cross-clamped and a horizontal aortotomy was performed (slightly more distal compared with aortotomy for normal AV replacement). A mediastinoscope was used to look through the AV and the lesion was easily seen arising from the lateral wall of the LV apex (Figs. 2A, B). The stalk of the lesion appeared to be a cord with a thickness slightly greater than a nondiseased mitral cord. This lesion was not attached to any valvular structures. A large biopsy forcep was placed through the AV and into the LV through the mediastinoscope (Fig. 2). The lesion, along with nascent myocardium, was completely and easily excised through the scope. There was no bleeding internally (LV chamber) and externally (LV epicardial apex). The aortotomy was then closed and a warm shot of cardioplegia was given and the aortic cross-clamp was removed. The total CPB and cross-clamp times were 37 and 22 minutes, respectively. Two chest tubes were placed in the mediastinum along with atrial and ventricular pacing wires. The patient initially came off CPB with atrioventricular pacing and quickly entered in normal sinus rhythm. The pericardial sac was easily reapproximated and the sternum was closed with sternal wires. Pathology report concluded that the lesion was a PFE with a pedicle arising from an unusual site, the LV apex.
The postoperative course included a short stay in the cardiovascular intensive care unit. On postoperative day 4, the patient had a TTE, which showed again normal LV function. A chord was seen in the LV cavity measuring 2 to 5 mm in thickness along with small echodensities in the pericardial space (likely fat or small clots); these were thought to be benign, and repeat TTE was arranged at a later date. The patient was then discharged home (postoperative day 4) on diuretics, pain control medication, warfarin (international normalized ratio target at 2–3 for prevention of clots on the raw LV endocardial surface for 3 months), crestor, zantac, ativan as needed, and slow K. At the 3-month follow-up, the patient had not experienced any more TIAs and physical examination was within normal limits. The TTE demonstrated normal LV function, no pericardial effusions, and resolution of postoperative findings. At 1-year follow-up, the patient had no limitation with respect to exercise but had some chest and left arm discomfort with variable frequency and left-handed pruritus. Work-up for this chest discomfort was negative for cardiac disease, and at 1-year TTE follow-up, no abnormalities were observed. The patient continued on his preoperative medications.
This case demonstrated a novel minimally invasive technique to remove intracardiac tumors within the LV cavity under direct visualization. Instead of accessing the LV cavity through a ventriculotomy, thereby causing disruption of normal LV physiology (conduction, muscle, and blood flow), a mediastinoscope can easily be placed through the AV into the LV, allowing sufficient LV cavitary exposure for complete resection.3 Previous groups have demonstrated resection of tumor and stalk, along with minimal complications, using a flexible endoscope to visualize and snare off the tumor. However, resection is indirectly performed, likely limited to a snare, and may jeopardize clear tumor margins.4 In summary, this particular case and procedure demonstrated to be an effective and efficient novel approach to the direct resection of intracardiac tumors in the LV cavity with a short procedural time, minimal complications, and a speedy discharge home with quick recovery. This technique could easily be applied to patients with intracardiac tumors in the LV cavity and to achieve acceptable resection of the tumor margins.
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