How to get cured of peripartum cardiomyopathy?

Question

Hi doctor,

My sister was diagnosed with peripartum cardiomyopathy during her pregnancy. Her age is 31 years, and her weight is 121 pounds. She delivered a baby girl through normal delivery. The 2D echo report says that heart function is 20 % to 25 % (LVF). However, she is not having any external symptoms like swelling, breathing problems, tiredness, or high BP. I have a few questions about her disease and treatment. Is this curable? How much time will it take to get completely out of it?

How frequently should she visit a cardiologist and review 2D echo reports? Will fixing an ICD with surgery help her in handling sudden cardiac arrest? What is the approximate cost? If required, is a heart transplant possible? What precautions should be taken? When can an emergency situation arise? Should we keep some emergency medicines ready at home? Is there a chance for her baby girl to have a similar problem when she grows up? Please help.

Thank you.

Answer 1

Hi,

Welcome to icliniq.com.

Before I answer your questions in detail, I would like to tell you that if she is totally symptom-free and her ejection fraction (EF) is 20 to 25 %, please do get another echo (echocardiogram) done from an independent reliable source to confirm the diagnosis. The condition of peripartum cardiomyopathy, or PPCMP, has no definite cure. A proportion of patients will completely recover, while some may have partial recovery and continue to have low ejection fraction and symptoms.

Some unfortunate patients see no improvement and may not survive for long. There is no definite period of recovery, even for those who recover completely. Continuous improvement of up to five years has been seen. The frequency of visits depends upon her problems and her cardiologist. Whenever she has a problem, she should visit the cardiologist, or whenever the cardiologist calls her, she must visit. The frequency of echo is decided by clinical circumstances and depends upon symptoms.

I do a first follow-up echo after six weeks in my patients if there are no emergent symptoms. ICD, or internal cardiac defibrillator, is indicated in patients with low ejection fraction. It helps prevent death caused by ventricular tachyarrhythmia. However, since low EF may revert to normal in a subset of patients, one should wait for three to six months. In Western countries, a wearable ICD (not surgically implanted) is sometimes used for these three to six months. The cost of ICD implantation is nearly four to five lakhs.

Yes, a heart transplant is possible and is available in major cardiac centers of metros. Do take your medicines on time. Do not over-exert. Stick with the recommended salt and water intake. Avoid alcohol. Avoid steroids and painkillers. Only take medicine under the supervision of your cardiologist. One can tell that when an emergency situation arises. Do take medicine as prescribed by your cardiologist. Visit the health facility in case of an emergency. No emergency medicines can be prescribed as such. Although a number of case reports are available regarding the occurrence of PPCMP in daughters and sisters of patients of PPCMP. However, systematic studies regarding such an association are not available. In fact, routine genetic studies of PPCPM patients are not yet recommended.

I hope this helps.

Please revert so I can assist you further.

Thank you.

Answer 2

Hi,

Welcome back to icliniq.com.

Since she requires standard therapy for heart failure, she should be evaluated accordingly. I normally call my patients every fortnight till optimization of therapy. If the ejection fraction is less than 30 % to 35 % despite optimal medical therapy as per guidelines, even after six to nine months, the patient may be considered for ICD implantation. A heart transplant is indicated if the patient has refractory heart failure (stage D) despite optimal therapy or has circulatory shock requiring mechanical support. However, it does not seem to be a consideration in your case right now.

I hope this helps.

Please revert so I can assist you further.

Thank you.