Constrictive Pericarditis, A Hardening Of The Heart Lining

“Only those who will risk going too far can possibly find out how far one can go.” 

T.S.Eliot

Constrictive Pericarditis Following Heart Transplant

It appears that Wednesday, my brave new heart, is not happy. In fact she or should I say we are in heart failure. It appears we have developed either Constrictive Pericarditis or Cardiomyopathy. Either way, both conditions cause heart failure.

This post provides a deep dive into constrictive pericarditis, both in general and following heart transplantation. It highlights the importance of early recognition and intervention. These actions ensure optimal patient outcomes. A recent post unpacks Cardiomyopathy.  

Complications Following Heart Transplant

If you regularly read my blog, you might have noticed my absence. I have been MIA for the last 4 weeks through August 2024. I would love to say this is because I won Lotto and have been travelling the world. Sadly, I have had a significant setback. 

Some hospital admissions later and diagnostic testing reveals the pressures are off in my heart. Results suggest that the heart is functioning abnormally. A.K.A. Heart Failure. However, the cause is yet to be concluded. Thorough testing which is discussed below means the diagnosis options have been narrowed down to two. These include: Constrictive Pericarditis or Cardiomyopathy. 

What Does Pericarditis Mean For Wednesday?

Essentially the heart is not expanding and contracting as it should. This in effect is heart failure. Heart failure means blood is not circulating around the body as designed. This leads to organ and vascular damage, extreme fluid retention and ultimately death. 

Heart failure can be partially managed via diet, fluid restriction, exercise and medications. Sounds simple and breezy but it is not. The symptoms and treatments of heart failure are distressing and traumatic. Life becomes pretty miserable. Ultimately, there is no cure other than heart transplant or surgical intervention. 

Heart transplantation is a life-saving procedure

Heart transplantation is a last resort and life saving treatment for patients with end-stage heart failure. While this surgery has significantly improved survival rates, it is not without complications. One such rare but serious complication is constrictive pericarditis, which can occur following a heart transplant. This condition, characterised by the stiffening of the pericardium, can significantly impact the transplant’s success. It can also affect the patient’s quality of life. Read on to delve into the causes, symptoms, diagnosis, and management of constrictive pericarditis following heart transplantation.

To read about other complications and transplant related issues, follow these links. Learn about hair loss after heart transplant. Discover what you need to know about a heart transplant. Understand the infection risk from donated organs. Explore Heart Transplant Rejection for more information.

What is Constrictive Pericarditis?

Constrictive pericarditis is a condition where the pericardium, the double-layered sac surrounding the heart, becomes thickened and scarred. This thickening restricts the normal movement of the heart, leading to reduced cardiac output and heart failure symptoms. You can develop constrictive pericarditis without a transplant.  In the context of heart transplantation, this condition is concerning. It can compromise the function of the transplanted heart.

Causes of Constrictive Pericarditis After Heart Transplant

The exact cause of constrictive pericarditis following heart transplantation is not always clear, but several factors have been identified:

Surgical Trauma: 

The pericardium may become inflamed and scarred due to surgical manipulation during the transplant procedure.

Infection: 

Postoperative infections can lead to inflammation and subsequent scarring of the pericardium.

Rejection Episodes: 

Acute or chronic rejection of the transplanted heart can result in pericardial inflammation and fibrosis.

Radiation Therapy: 

Patients who have undergone radiation therapy for conditions like lymphoma before transplantation may experience constrictive pericarditis. This can occur as a late complication.

During the work up for heart transplant, I was required to have a mammogram for breast cancer screening. I failed the mammogram and was diagnosed with breast cancer. Ultimately I had aggressive and preventative breast surgery. Radiation treatment followed with 6 weeks of radiation therapy.

As the radiation therapy occurred before the heart transplant, my new heart, Wednesday has not been impacted by radiation therapy. However, radiation therapy did cause scaring to my left lung. This has improved and eventually healed over time.

Causes of Constrictive Pericarditis In Non Transplant Patients

Causes of Constrictive Pericarditis In Non Transplant Patients can be diverse and include:

Infections:

• Tuberculosis: Historically one of the most common causes, especially in developing countries.
• Viral infections: Such as those caused by Coxsackievirus or other viral agents.
• Bacterial infections: Such as staphylococcal or streptococcal infections.

Post-Cardiac Surgery:

• Constrictive pericarditis can develop after heart surgery. This includes procedures like heart valve repair or replacement. It also includes coronary artery bypass grafting (CABG).

Radiation Therapy:

• Radiation therapy to the chest area is often used in the treatment of cancers like lymphoma or breast cancer. It can lead to pericardial fibrosis and subsequent constriction.

Autoimmune and Inflammatory Diseases:

• Conditions such as rheumatoid arthritis, lupus, or scleroderma can cause inflammation of the pericardium, leading to constriction over time.

Trauma:

• Chest trauma, whether from an accident or a surgical procedure, can lead to scarring and thickening of the pericardium.

Idiopathic:

• In many cases, no specific cause can be identified, and the condition is termed idiopathic.

Neoplasms:

• Metastatic cancer or primary tumours of the pericardium can lead to constrictive pericarditis.

Uremic Pericarditis:

• Seen in patients with chronic kidney disease, especially those on dialysis.

Each of these causes leads to fibrosis and scarring of the pericardium. This process eventually results in the restrictive physiology characteristic of constrictive pericarditis.

Constrictive Pericarditis, Signs And Symptoms

The general symptoms of constrictive pericarditis can mimic those of heart failure and include:

Dyspnea (Shortness of Breath): Due to reduced cardiac output.

Fatigue: Caused by poor blood circulation.

Oedema (Swelling): Especially in the legs and abdomen. Read more about oedema in my the post on Fluid Retention.

Ascites: Accumulation of fluid in the abdomen.

Jugular Venous Distention: Due to increased pressure in the veins.

Presentation Of Heart Failure Symptoms For Wednesday and Me

Change in blood flow and pressure

At the start of February this year I noticed a change in the blood vessels in my legs. This started prominently in the right leg but quickly impacted the left leg. I suddenly developed painful and unsightly varicose veins, extensive broken capillaries and small blood vessels. This became worse around my lower calves and ankles. 

During monthly visits this year to the transplant clinic I would raise my concerns with consultants. One consultant actually thought I had been kicked in the shins. They also considered whether I had twisted my ankles or experienced some other trauma to my legs. 

Varicose Veins

The transplant team referred me to a vascular surgeon. An ultrasound of the veins around the iliac/pelvis, abdomen, and legs was performed. It discovered that my inferior vena cava was fully blown or dilated while laying down. Usually, the *inferior vena cava (IVC) is partially collapsed. This apparently means that the heart pressures are forcing too much blood into my legs. The blood is then trapped and pooling in the lower legs causing blood vessels and capillaries to swell and distend.

Inter-cranial Pressure

As it turns out my heart is forcing too much blood up into my brain via the **superior vena cava (SVC). This causes increased pressure in my head resulting in a pressure like headache. This gets worse while laying or bending down. I had ophthalmology checks, a Brain MRI and Contrast CT Scan to check for intracranial pressures. Results show increased blood pressure and flow to the optic veins.

This increased brain pressure can lead to partial or complete blindness. If you have read my post on infections after an organ transplant/, you will know that I have monocular vision. This condition is due to a glioma (brain tumour). This resulted in the removal of my left eye. Therefore, I cannot risk losing the vision in my right eye.

* The Inferior Vena Cava (IVC) a large blood vessel responsible for transporting deoxygenated blood from the lower limbs and extremities and abdomen back to the right atrium of the heart.

**The superior vena cava(SVC) is a large, significant vein responsible for returning deoxygenated blood collected from the upper body to the heart’s right atrium.

Swelling (especially in the abdomen and lower legs) from fluid buildup.

Weight gain in the form of fluid retention is a symptom of heart failure. This is not like a few extra grams but kilograms of water stored within the cells body wide. The result of too much fluid means water around the organs. Particularly the lungs and heart. The extra fluid around my heart is causing the difficulty in breathing, sleeping and eating.

A recent post on Fluid Retention unpacks the causes, symptoms and treatments for water retention. Look out for my next post. It will explain how to cope with Fluid Restrictions. This is a common pain point for transplant recipients.

Chest pain.

Since February, I noticed that I could not find a comfortable position to sleep or even lie down to rest. I could feel my heart thumping in my head, chest, throat and stomach. This pounding would keep me awake and restless or wake me up during sleep. I find it distressing to sleep or lie down.

I began to develop sharp clenching heart or chest pain. This started mainly when I was moving around. It has now escalated to being present even at rest.

Shortness of breath (Dyspnea)

It has become harder for me to do simple tasks like showering and dressing without exhaustion and shortness of breath. I am not able to do my usual weight training required in the cardiac rehabilitation program. The length of my walks have become shorter, slower even on flatter ground. Sharp chest pain is common now. It feels like I am receiving a defibrillator shock. This occurs despite its removal during the heart transplant in May 2023. 

During transplant clinic visits this year, the consultant would monitor the Jugular Venous Distention of my left internal jugular (LIJ). My Right Internal Jugular (RIJ) is blocked from the transplant surgery. Therefore, blood is only draining from my brain via the LIJ. The increased pressure in the veins is due to abnormal heart function. This makes my LIJ far more prominent than it should be. As listed above, Jugular Venous Distention is another sign of constrictive pericarditis. 

Loss of muscle mass

Given my shortness of breath and quickness to feel exhaustion, it is hard to maintain my cardiac rehab program. This has quickly resulted in a loss of muscle mass, strength and overall fitness.

Loss of appetite and feeling full even when eating very little.

It is increasingly difficult to eat meals. I tend to skip breakfast as I am still feeling full after dinner the night before. I find that I only eat a child’s serving size. If I am eating out, I share a meal with my husband. Alternatively, I order a small entree as a main meal. I try to prioritise protein in each meal and opt for nutrient dense whole foods to maximise nutrition.

Diagnosing constrictive pericarditis

Diagnosing constrictive pericarditis is a challenge as the condition can mirror rejection or cardiomyopathy.  Diagnosis requires a thorough evaluation. The diagnostic testing process may include:

1. Echocardiography: An ultrasound to assess pericardial thickening and impaired heart function.
2. Right Heart Catheterisation: Measures pressures within the heart to confirm the diagnosis.
3. CT Scan: Useful in assessing the extent of pericardial calcification.
4. Lumbar Puncture: Helps diagnose neurological, infectious or autoimmune conditions.
5. PET Scans: Is an imaging test that uses radioactive material. It is used to diagnose, monitor and treat a variety of conditions and diseases such as cancer and sarcoidosis.
6. MiBi Scans: Evaluates blood flow to the heart at rest and under stress.

Diagnosing Wednesday’s Heart Failure Symptoms

Throughout July 2024, I had all of these tests as an outpatient. The results were suggestive of constrictive pericarditis or cardiomyopathy. The transplant team discussed the results at the multidisciplinary meeting (each Friday morning at 8 am). It was decided that the tests should be repeated in the hospital. I was admitted to St Vincent’s Cardiac Care Unit for the week, last week.

Initially, I was treated with IV Furosemide (a diuretic). This treatment aimed to remove excess fluid from around the organs, especially the heart and lungs. After I had been “dried out” I had lost 4 kilos in 3 days. The testing was repeated again. This included an echo, MRI, Left and Right Heart Catheters. Again the pressures were markedly out of normal range, though improved with the fluid loss.

Decisions Or Indecision?

The transplant multidisciplinary team was to meet last Friday to determine a diagnosis. The surgical doctor in the hospital told me that the results indicated abnormal heart function. However, there was no clear diagnosis. Hence the challenge of diagnosing constrictive pericarditis or cardiomyopathy. The surgeon stated that the only way to really know for sure was to look and feel the heart. Obviously the team do not perform open heart surgery just to look at the heart. The team needs to make an educated guess based on the diagnostic testing results.

The next step involved a third hospital admission. All the tests were repeated for a third time. This also involved several new procedures. I needed a Lumbar Puncture, along with MiBi and Pet Scan. Additionally, I required an advanced cardiac MRI scan.

If it is pericarditis then the lining of the heart can be removed. If it is cardiomyopathy then another heart transplant will be my best option. As you can see these are difficult calls or decisions to make. My greatest fear and threat is indecision.

Management and Treatment Of Pericarditis

The management of constrictive pericarditis can be challenging and often requires a multidisciplinary approach:

Medical Management: 

Initially, diuretics may be used to manage symptoms of fluid overload. However, this is often only a temporary measure. It is to manage symptoms but not a cure.

Diuretics can be IV or oral. Usually patients will also endure fluid restrictions of 1.2 Litres or as low as 800 ml per day. This is torturous and ineffective as a long term solution.

Surgical Intervention: 

The definitive treatment for constrictive pericarditis is pericardiectomy, a surgical procedure to remove the thickened pericardium. This procedure can significantly improve symptoms and cardiac function in healthy patients.

Current research about pericardiectomy seems positive. However, I was told by the consultant that, in her experience, no heart transplant recipient had a successful outcome. I was told that it did not improve quality of life nor extend life. The consultant felt that another heart transplant had better potential for long term success.  

Monitoring and Follow-up: 

Regular follow-up with echocardiography is crucial. Clinical assessments are essential to monitor the progression of the disease. They also evaluate the success of the treatment.

After being discharged from hospital I have fortnightly appointments with the transplant team. I am yet to hear if a decision has been made about future surgery. I will keep you updated. 

Please connect with our community

Constrictive pericarditis is a serious complication that can arise after heart transplantation, though it remains relatively rare: 0.04% of all heart transplants. Early diagnosis and appropriate management are essential to improve patient outcomes. By understanding the potential risks and recognising the symptoms early, healthcare providers can intervene promptly to prevent long-term complications.

I wish you all the luck if you are impacted by pericarditis or cardiomyopathy.

If you have a story or advice to share please connect with our community via the form below.

If you found value in this post please let me know. Other posts include: How To Claim TPD Insurance and Infections After An Organ Transplant. All transplant recipients need to be informed about Preventing Infections

Love and Light

X

Wednesday

References

Andreis, A., Imazio, M., Casula, M., Avondo, S., & Brucato, A. (2021). Recurrent pericarditis: An update on diagnosis and management. Internal and Emergency Medicine, 16(3), 551–558. https://doi.org/10.1007/s11739-021-02639-6

Blanding, W. M., & Balsam, L. B. (2019). Is the ratio of pulmonary artery to aortic diameter akin to heart failure’s hemoglobin A1c? The Journal of Heart and Lung Transplantation, 38(1), 104–106. https://doi.org/10.1016/j.healun.2018.09.005

Gillombardo, C. B., & Hoit, B. D. (2024). Constrictive pericarditis in the new millennium. Journal of Cardiology, 83(4), 219–227. https://doi.org/10.1016/j.jjcc.2023.09.003

Imazio, M., & Gaita, F. (2015). Diagnosis and treatment of pericarditis. Heart, 101(14), 1159–1168. https://doi.org/10.1136/heartjnl-2014-306362

Renner, J., Lorenzen, U., Borzikowsky, C., Schoeneich, F., Cremer, J., Haneya, A., Hensler, J., Panholzer, B., Huenges, K., & Broch, O. (2017a). Unilateral pulmonary oedema after minimally invasive mitral valve surgery: A single-centre experience. European Journal of Cardio-Thoracic Surgery, 53(4), 764–770. https://doi.org/10.1093/ejcts/ezx399

Renner, J., Lorenzen, U., Borzikowsky, C., Schoeneich, F., Cremer, J., Haneya, A., Hensler, J., Panholzer, B., Huenges, K., & Broch, O. (2017b). Unilateral pulmonary oedema after minimally invasive mitral valve surgery: A single-centre experience. European Journal of Cardio-Thoracic Surgery, 53(4), 764–770. https://doi.org/10.1093/ejcts/ezx399