European Journal of Cardiovascular Medicine

Cardiac tumors are rare neoplasms that can be classified as primary or secondary (metastatic), with secondary tumors being more prevalent. Primary cardiac tumors have an incidence of approximately 0.0017% to 0.03% in autopsy series, with benign forms such as myxomas, lipomas, papillary fibroelastomas, and rhabdomyomas constituting the majority (1). Malignant primary tumors, predominantly sarcomas, are less common but associated with a poorer prognosis.

Secondary cardiac tumors are up to 20-40 times more common than primary cardiac tumors and frequently result from metastasis of malignancies such as lung, breast, kidney cancers, melanoma, and lymphomas (2). The clinical presentation of cardiac tumors varies widely depending on size, location, and involvement of cardiac structures, ranging from asymptomatic cases to symptoms of heart failure, arrhythmias, embolic events, or sudden cardiac death (3).

Advancements in imaging techniques, including echocardiography, cardiac MRI, and CT, have significantly improved the detection and characterization of cardiac tumors. Despite these advancements, definitive diagnosis often requires histopathological examination following surgical excision or biopsy.

Understanding the pathophysiology, diagnostic approaches, and management strategies for cardiac tumors is crucial for optimizing patient outcomes, given their potential for significant morbidity and mortality. Complete excision of tumour with a margin of healthy tissue of 3 to 5 mm is must to prevent recurrence and other complications

This retrospective study investigates the course of cardiac tumours operated at tertiary care centre from 2014 to 2024. A total of 25 patients diagnosed with cardiac tumors were included. The study aimed to analyse the patients’ age groups, tumor types, presenting symptoms, diagnostic investigations, surgical management, and outcomes.

Study Design and Patient Selection-

This is a retrospective analysis of all patients diagnosed with cardiac tumors who underwent surgical intervention at a tertiary care centre. Patients were selected based on clinical suspicion and imaging findings that led to the diagnosis of a cardiac tumor. Exclusion criteria included patients with incomplete records, those diagnosed with metastatic cardiac tumors and patients who did not undergo surgery for medical or other reasons.

Preoperative Evaluation

All patients underwent a standardized preoperative workup:

Transthoracic Echocardiography (TTE): This was the first-line imaging modality to evaluate the tumor’s size, location, and hemodynamic impact, such as valvular involvement or embolic risks.

Computed Tomography (CT) Chest Scan: Used to further evaluate the mass, identify extracardiac involvement, and assess the tumor’s relationship with surrounding structures.

Coronary Angiography/CT Coronary Angiography: Performed to assess coronary artery disease, especially in patients aged ≥40 years.

Surgical Technique

The surgical approach was tailored according to the location and characteristics of the tumor. A standard median sternotomy was performed to access the heart. Cardiopulmonary bypass (CPB) was established in all cases to facilitate safe resection by providing cardiac arrest and systemic perfusion. Superior and inferior vena cava cannulation was used for venous return management. Cold blood cardioplegic was applied to arrest the heart and provide a still, bloodless field for tumor resection. Right atrial, left atrial or biatrial approach was used depending on the location of tumor for surgical excision. Right ventricular outflow tract (RVOT) approach was used for a single case. The tumor was carefully excised with margin of 3 to 5 mm (Figure 1), prioritizing the prevention of embolization, especially in cases of myxoma. In cases with concomitant cardiac pathology, such as mitral valve involvement, valve repair or replacement was performed as necessary. Transoesophageal Echocardiography (TEE) was used intraoperatively for real-time guidance, ensuring complete resection and monitoring for any residual tumor or complications.

Figure 1: Excised atrial myxoma with 4mm margin of atrial septum

Postoperative Care

After surgery, patients were closely monitored in the intensive care unit (ICU) for potential complications, such as arrhythmias, bleeding, or thromboembolism. Follow-up care included regular clinical evaluations; echocardiograms done at 6 monthly intervals

Follow-up

Patients were regularly followed up using transthoracic echocardiography (TTE) and, when necessary, CT scans or MRIs to monitor for tumour recurrence.

Case Reports of Interest-

Two cases from this series are particularly noteworthy:

Coexisting Left Atrial and Left Ventricular Myxoma:
A 24-year-old female presented with exertional breathlessness and a prior history of a cerebrovascular accident. Neurological examination and CT of the brain were normal. Echocardiography revealed a left atrial mass arising from the anterior mitral leaflet and a separate left ventricular mass. These findings were confirmed with cardiac MRI. The patient underwent open-heart surgery, which involved the resection of both myxomas, as well as mitral valve replacement (Figure 2 and 3). The patient has been followed for 3 years with no recurrence or complications.

Figure 2: Excised LA and LV mass

Figure 3: Excised LA and LV mass along with excised mitral valve

Cardiac liposarcoma in the right ventricular outflow

A young male presented with a history of recurrent dyspnoea and palpitations on exertion. TEE revealed a large, mobile mass arising from the anterolateral wall of the RVOT. Cardiac MRI confirmed the diagnosis of a neoplastic mass. The patient underwent surgery, and the mass was excised along with part of the pulmonary valve using a minimal-touch technique. Histopathology revealed a diagnosis of liposarcoma. The patient remained asymptomatic on follow-up with no recurrence or metastasis.

A total of 25 patients with cardiac tumors underwent surgery at our institution between 2014 and 2024. Of these, 23 patients had atrial myxomas, 1 had LA with LV myxoma, and 1 had liposarcoma. The most common age group for presentation was between 20 and 40 years, with dyspnea and palpitations being the most frequent presenting symptoms.

Table No. 1- Different type of tumour

Table No. 2- Age Distribution

Table No. 3- Gender Distribution

Table No. 4- Site Distribution

The diagnosis of cardiac tumors was confirmed using Transthoracic Echocardiography (TTE) and Transoesophageal Echocardiography (TEE). These imaging modalities were pivotal in determining the size, location, and nature of the tumors, guiding surgical planning.

The standard surgical approach for all patients involved complete excision of the tumor with a 3-5 mm margin of surrounding normal tissue to ensure clear margins and minimize the risk of recurrence. The surgical approaches varied based on tumor location, with right and left atriotomy, biatrial and right ventricular outflow tract approach, being utilized as needed for optimal access to the tumors.

Postoperative management included routine echocardiograms before discharge to assess cardiac function and ensure there were no complications such as residual tumor or valve dysfunction. The maximal follow-up duration was five years, with the minimum follow-up being 6 months after surgery.

The management of cardiac tumors presents significant clinical challenges due to their rarity, variable presentation, and potential for severe complications. Benign tumors like myxomas, though non-malignant, can cause obstructive symptoms and systemic embolization, necessitating prompt surgical resection. The surgical approach often results in excellent outcomes with low recurrence rates, particularly when complete excision is achieved (1).

In contrast, malignant primary cardiac tumors, predominantly sarcomas, have a poor prognosis due to their aggressive nature and high potential for local invasion and metastasis. Treatment typically involves a multimodal approach, including surgical resection when feasible, chemotherapy, and radiotherapy. However, the overall survival remains limited, often less than one-year post-diagnosis (4).

Secondary cardiac tumors are managed based on the primary malignancy and the extent of cardiac involvement. Systemic therapy targeting the primary cancer is often the mainstay, with palliative measures to address cardiac symptoms. The role of surgical intervention is generally limited to cases with life-threatening complications (5).

Emerging therapies, such as targeted molecular treatments and immunotherapy, offer potential benefits, particularly for specific sarcoma subtypes and metastatic cancers involving the heart. Advances in imaging modalities continue to enhance early detection, guiding timely intervention and improving prognostic outcomes (6).

The role of genetic and molecular profiling in cardiac tumors is an evolving area of interest. Understanding the genetic mutations and pathways involved in tumor pathogenesis may help identify novel therapeutic targets. For instance, mutations in the PRKAR1A gene are associated with Carney complex, a condition predisposing to cardiac myxomas (7). Additionally, research into the tumor microenvironment and immune landscape of cardiac tumors may provide insights into the efficacy of immunotherapeutic strategies (8).

Prognostic factors for cardiac tumors include tumor size, histological subtype, degree of local invasion, and presence of metastases. Timely diagnosis and multidisciplinary management involving cardiologists, oncologists, and cardiothoracic surgeons are crucial in improving patient outcomes (9).

The diagnostic approach to cardiac tumors often begins with non-invasive imaging techniques. Transthoracic echocardiography (TTE) is typically the first-line modality due to its accessibility and effectiveness in detecting intracardiac masses (10). Transoesophageal echocardiography (TEE) provides superior resolution for posterior cardiac structures and is particularly useful in assessing tumor attachment and mobility (11).

Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are critical for further characterizing cardiac tumors, offering detailed information on tissue composition, vascularity, and the extent of local invasion (12). Positron emission tomography (PET) can be valuable in differentiating benign from malignant lesions and assessing metastatic spread (13).

Treatment strategies for cardiac tumors are tailored based on tumor type, location, and patient condition. Surgical resection remains the cornerstone of treatment for resectable primary tumors, particularly benign ones. Complete excision with clear margins is associated with favourable outcomes.

For malignant tumors, especially unresectable sarcomas, a combination of chemotherapy and radiotherapy is often employed. Agents such as doxorubicin and ifosfamide are commonly used in sarcoma protocols (14). Heart transplantation has been considered in select cases with isolated cardiac malignancies, although recurrence rates are high (15).

Palliative care, including pericardiocentesis for effusions and arrhythmia management, is crucial for symptom relief in advanced disease stages. Ongoing clinical trials are exploring the role of novel therapeutics, including tyrosine kinase inhibitors and immune checkpoint inhibitors, in improving outcomes for patients with cardiac tumors.

Cardiac tumors, though rare, represent a fascinating and important aspect of cardiology. The clinical presentation can range from asymptomatic to life-threatening, making early diagnosis critical. Imaging techniques, including echocardiography, MRI, and PET, have enhanced our ability to diagnose these tumors earlier, but histopathological examination remains essential for definitive diagnosis. While benign tumors such as myxomas can be successfully managed with surgery, malignant tumors pose significant challenges, often requiring multimodal treatment approaches. Ongoing research into the molecular mechanisms underlying cardiac tumors may provide new insights into diagnosis, treatment, and prognosis in the future.

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