We use cardiac echocardiography and cardiac magnetic resonance imaging as our primary diagnostic tools with cardiac tumors. Most benign tumors can be recognized from these tests. Malignant tumors tend to be larger, broader based, and more infiltrative. Resection of benign tumors depends on the occurrence or potential occurrence of tumor-related complications. For the most common benign tumor, a left atria myxoma, the most feared risk is systemic embolization and stroke. For myxoma, presence alone is generally accepted as an indication for surgery. Papillary fibroelastomas are generally considered similar to myxoma in indications for surgery. They generally involve cardiac valvular structures but in our experience preservation of the valve has always been possible. Lipomas and hemangiomas when small and not causing symptoms can often be observed. Paragangliomas tend to grow and cause symptoms and should be resected. They are always very vascular with large parasitic vessels from the coronary arteries. Percutaneous biopsy for diagnosis should not be attempted in these tumors. Contraindications for benign tumors are patient physiologic or anatomic conditions that preclude resection at a reasonable risk.

Most malignant tumors are sarcomas. The majority of the rest that we have seen are lymphomas. For suspected lymphomas a biopsy is indicated as these are treated with chemotherapy rather than surgical resection once a diagnosis has been established. We divide sarcomas into their site of origin rather than histology as this dictates their presentation and potential surgical approaches. We categorize them as left heart sarcoma, right heart sarcoma, or pulmonary artery (PA) sarcoma. Many primary cardiac sarcomas present with metastatic disease when initially seen. Left heart sarcoma and PA sarcoma tend to present with obstruction to intracardiac blood flow and heart failure. Surgical resection may need to be considered for palliation even in the face of metastatic disease to allow for subsequent chemotherapy. Survival in these cases without surgery is generally less than a month. For left heart or PA tumors with characteristics of sarcoma but without metastatic disease, a preoperative biopsy to establish a diagnosis is difficult and generally not necessary. Preoperative imaging will usually allow a prediction of the potential for resection with negative margins as these tumors tend to be fairly well defined. For right heart tumors, we have found them to be more exophytic in their growth and causing less heart failure but also more infiltrative at the tumor margins making complete resection more difficult. For right heart sarcoma we would recommend a biopsy and consideration of chemotherapy prior to resection. Contraindications to resection would be metastatic disease that does not respond to chemotherapy, patient physiologic conditions that preclude surgery, or anatomy that would preclude complete resection of the tumor.

Minimally invasive cardiac surgical (MICS) approaches are possible for myxomas and fibroelastomas in appropriate anatomic locations in the right and left atria or atrioventricular (AV) valves. A right chest (nonsternal) approach is used to gain access to the atria or the ventricles through the mitral or tricuspid valves (TVs). Fibroelastomas are also amenable to excision through a MICS approach to the aortic valve and left ventricular outflow tract (LVOT).

MICS approaches are generally performed through a limited right lateral third or fourth intercostal space (ICS) incision (5 to 8 cm) for exposure of the right and left atria. Exposure of the aortic valve and LVOT is achieved via an upper ministernotomy toward the third ICS or anterior thoracotomy with disarticulation of the third rib at the sternum.

In ministernotomy approaches, the patient is positioned supine with arms at the side and full exposure to the femoral and axillary spaces. In right lateral approaches, the patient is positioned supine with a bump placed vertically to elevate the right hemithorax; exposing the lateral ribs as well as the axillary and femoral spaces.

Safe and complete cardiopulmonary bypass (CPB) is essential to achieve optimal clinical outcomes. Complete tumor resection, adequate venous drainage, aortic clamping, and complete de-airing are essential for a successful result. In left-sided tumors, CPB can be safely performed via femoral vessel Selinger access (percutaneously or cut down) and multistage venous drainage. In patients where right-sided cardiac access is necessary, a separate superior vena cava (SVC) cannula can be inserted and snared separately via the right minithoracotomy. Optimal venous drainage is essential for adequate visualization and right ventricular (RV) myocardial protection. Arterial cannulation can be performed femorally, directly into the ascending aorta or via the axillary artery.

Myocardial protection in MICS procedures, similar to MICS valve surgery, is dependent on delivery of cardioplegia. Antegrade cardioplegia is usually administered directly into the ascending aorta. Retrograde cardioplegia is performed via direct catheterization of the coronary sinus (CS) through the right atrium. Alternatively, a percutaneous CS catheter can be inserted via a neck approach prior to surgery. Aortic clamping is optimally performed via standard Chitwood clamp inserted through an axillary stab. Endovascular balloon aortic occlusion may also be performed if the aortotomy is not required. Venting of the left ventricle (LV) may be performed through direct cannulation of the LA/LV via right superior pulmonary vein or percutaneously through PA drainage.

Excellent preoperative imaging (optimally through CMR) is necessary to delineate tumor anatomy and involvement prior to MICS procedures and surgical planning. If performed adequately, MICS tumor resection is a viable and safe option for complete removal of tumors. With these approaches, patients may benefit from decreased transfusions, length of stay, and pain medication requirement.

Many benign and all malignant tumors are best resected using a mediastinotomy approach. Patient positioning is supine with the arms at the side.