Introduction

Did you know that approximately 7 out of 10 primary heart tumours are cardiac myxomas? In people with carney complex, a rare inherited heart condition, these tumours are more likely to occur at a younger age, in multiple heart chambers and can return after treatment. Blood flow is blocked, and stroke can occur as a result of cardiac myxomas and carney complex, which is why surgery is always the recommended treatment after diagnosis. 

What are cardiac myxomas in the carney complex?

Carney complex (CNC) is a rare genetic syndrome that causes tumours in the heart, skin, endocrine glands, and other organs. Cardiac myxomas are the most common heart tumours seen in the carney complex, with roughly 7% of all cardiac myxomas occurring in people with CNC.¹ Cardiac myxomas seen in the CNC appear earlier in life, grow in more than one chamber of the heart and have a high risk of returning after surgery. Common symptoms of cardiac myxomas include:

• Breathlessness
• Fainting 
• Chest discomfort
• Stroke-like episodes: including blood clots breaking off the tumour

Diagnosis is usually made using an echocardiography (ultrasound of the heart), as well as magnetic resonance imaging (MRI), and computed tomography (CT) scan for second confirmation. 

Why early surgery matters

Cardiac myxomas pose serious risks because they can block blood flow to the heart, break off to cause strokes, and produce general symptoms like fever or weight loss. Surgery is therefore the first-choice treatment and often urgent, even when symptoms are not apparent.^2,3 Surgery is usually urgent if the tumour is:

• Causing obstruction to the blood flow in the heart
• Linked to an embolic event (stroke)
• Large, mobile, or located in a position likely to block a valve

Preparing for surgery

Before the operation, patients should have:

• A full cardiovascular assessment, including echocardiogram, MRI, or CT scan 
• Imaging to check for tumours in all heart chambers
• Consultations with the genetic and endocrine specialists to address other carney complex-related health issues, particularly for the PRKAR1A mutations linked to CNC
• A review of any medications, particularly blood thinners

Surgical approaches

The main surgical approaches taken for cardiac myxomas are:

Standard open-heart surgery

It is the most common procedure where a chest incision (median sternotomy) grants access to the heart.⁴ Patients are placed on a heart-lung bypass machine and the tumour is removed with a margin of healthy tissue, to reduce the chance of recurrence. Any affected heart structures, like the wall separating chambers or heart valves, are also repaired.

Minimally invasive techniques 

Some patients benefit from less invasive approaches like the mini-thoracotomy or robot-assisted surgery. These offer faster recovery, less pain, and smaller scars but they are not suitable for all cases, especially if the tumours are large or located in multiple chambers.²

Handling multiple or recurrent tumours

Because CNC patients frequently develop multiple myxomas or see tumours recur, surgeons may:^2,3

• Remove multiple tumours in one operation when feasible 
• Use real-time ultrasound during surgery to guide complete removal
• Plan additional procedures if the tumours reappear in different areas

Considering future recurrences

In CNC patients, recurrence rates are significantly higher (12-22%) compared to sporadic cases (1-4%).² Future surgeries may be required, so surgery planning carefully considers heart health and scar tissue.

Heart transplantation in refractory cases

Persistent and recurrent cardiac myxomas that is unresponsive to surgery requires an orthotopic heart transplantation (OHT). A 2025 case highlighted that even post-transplant, residual native tissue could lead to tumour recurrence, which underscores the importance of meticulous surgical technique and regular lifelong monitoring.⁷

Risks and recovery

All of the surgical approaches involve certain risks and require a period of recovery. The risks of surgery include:

• Stroke or tumour fragment embolism during the surgical procedure
• Irregular heartbeat (arrhythmias)
• Infection or bleeding
• Damage to heart valves, requiring repair or replacement⁵

Recovery typically includes:

• Hospital stays, around one week
• Several more weeks of rest at home 
• Encouragement to return to normal activities once cleared, unless there is heart failure or other issues occur⁶

Long-term follow-up

Because of the high recurrence rate and other CNC-related tumours developing, lifelong monitoring is essential. Typical follow-ups include:

• Echocardiography: annually starting in childhood years and every 6 months, if a cardiac myxoma has occurred
• Regular clinical exams to check for skin, endocrine, and other tumours^6,7
• Collaboration with specialists for comprehensive care: endocrinologists, geneticists, and oncologists^6,7 

With prompt surgery, most people make a full recovery and can return to normal activities. However, day-to-day monitoring is key. Detecting and treating new tumours early greatly improves long-term outcomes and quality of life.⁸

What to watch for after surgery

Seek medical help right away if you notice:

• Sudden shortness of breath or chest pain
• Stroke-like symptoms: such as weakness, slurred speech, or facial drooping
• Dizziness, fainting, or abnormal heart rhythm⁹

Summary

Surgical removal of cardiac myxomas is lifesaving for people with carney complex (CNC). Open-heart surgery remains the gold standard, whilst minimally invasive options are emerging for select patients. Myxomas tend to come back in CNC therefore, lifelong monitoring and regular check-ups with specialist collaboration are key to maintaining heart health and overall well-being.