There Is Still Hope for a Failing Heart

Colleen Steele avatar

by Colleen Steele |

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Up until age 6, my son Cullen was a healthy little boy. Earaches, wellness checks, and immunizations were the only reasons I took him to the pediatrician. So I was shocked when he began complaining that his heart felt funny and he was short of breath while exercising.

Asthma was the first thing my husband and I thought of, and given the relatively minor symptoms Cullen was experiencing, his pediatrician agreed. We allowed Cullen to continue participating in T-ball, soccer, and karate, but always with an inhaler on hand. Unfortunately, his symptoms intensified and began occurring while he was at rest, too.

I have long QT syndrome, a heart rhythm disorder, and mitral valve prolapse, a type of heart disease. I saw similarities in some of my symptoms and Cullen’s episodes. Testing by Cullen’s pediatric cardiologist confirmed the conditions and indicated a high risk of sudden death. Cullen was devastated to learn it meant an end to all of his athletic activities.

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Despite being placed on inhalers and beta blockers to control his symptoms, Cullen began to present with other symptoms as well, such as blue lips, chronic fatigue, cough, and lightheadedness. Additional testing eventually ruled out asthma, recurring croup, and panic attacks.

Finally, when Cullen was 8, an echocardiogram and right-heart catheterization unveiled the worst of the horrible truths: Cullen had pulmonary arterial hypertension (PAH).

Cullen’s lungs were already in critical condition at the time of diagnosis, and the right side of his heart was severely enlarged. This was devastating to hear, but it was almost a relief to have finally found the cause of Cullen’s symptoms.

As with many patients newly diagnosed with PAH, the next step for us was coming to grips with the knowledge that Cullen was fighting a disease that ravages both the lungs and the heart. It is common for newly diagnosed PAH patients to lack a clear understanding of the correlation between the two organs and the disease.

Although the condition is characterized by high blood pressure in the lungs, it is essential to understand how it affects the pulmonary arteries, which are the vessels responsible for carrying blood from the heart to the lungs. Without this understanding, it can feel like having a post-diagnosis aftershock when a patient is told they have an enlarged heart and are in right-heart failure.

My son’s PAH is idiopathic, meaning there is no known cause, but for others, it can be attributed to an underlying condition, with the most common being left-heart failure.

As a Pulmonary Hypertension News resource page explains: “Left heart disease and failure affect the left ventricle and consequently the overall capacity of the heart to properly pump blood.”

Here’s how the website explains how and when right-heart failure becomes part of the picture:

Patients develop inflammation and mutations in the cells that line the pulmonary arteries. Due to these alterations, the vessels become narrowed and blood flow becomes obstructed. As a result, the heart cannot properly pump blood through the pulmonary arteries into the lungs, which increases the pressure in the arteries. Since the heart needs to work harder, the right ventricle becomes enlarged and weakened. As the heart’s function is progressively affected, it may result in right heart failure.”

Now that the frightening details are out of the way, I want to offer some consolation, perhaps even hope, regarding what I am most familiar with: right-heart failure.

I know many patients who are long-term PAH and heart failure survivors. It can be managed with a healthy diet, combined PAH and heart failure medications, procedures, and medical devices.

Cullen’s PAH was diagnosed as severe, and statistically, he was unlikely to survive to celebrate his next birthday. His condition went from critical to managed in a few months thanks to continuous intravenous Flolan (epoprostenol GM), triple PAH oral therapy, diuretics, beta blockers, and magnesium.

He did well for five years before he stopped responding to his PAH treatments. Cullen’s doctors placed him on the IV vasodilator milrinone to treat his worsening heart failure. It kept him stable enough to receive a heart and double-lung transplant a year later, and he also climbed metaphorical mountains while on it by attending and graduating from eighth grade.

A pacemaker also was an option to improve his quality of life, but the day he was supposed to have it placed, he received a transplant instead.

We don’t think of Cullen’s former heart and lungs as failures. To us, they were wounded warriors who battled on longer than expected with the help of medical science. This has been the case for many other PAH patients as well. If you are in a similar situation, I hope it will be the case for you, too.

Did you find this column helpful? Please share in the comments below. 

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.


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