Updated: May 3
Case 2: "I would like to buy a valve, please."
A thirtysomething year old female presented to our center with complaints of shortness of breath, fatigue, and palpitations.
On physical exam, her pulse was irregular. Her abdomen was notably distended. EKG revealed atrial fibrillation.
Echocardiogram provided the patient's diagnosis. Her inferior vena cava (black tubular structure in image below) was plethoric with minimal respiratory variation. Her abdomen was distended due to ascites.
Her left atrium was ginormous. And, perhaps most notably, her mitral valve was abnormal- with failure of coaptation and a characteristic "hockey-stick" deformity.
All of the above findings are consistent with chronic RHEUMATIC HEART VALVE DISEASE (RHVD).
A brief primer on RHD:
Rheumatic heart disease (RHD) is a chronic heart valve condition affecting up to 40 million people worldwide, predominantly poverty-stricken children and young adults. RHD is caused by a preventable infection of the bacterium Group A Streptococcus (Strep A) which may lead to an autoimmune response in the body, namely acute rheumatic fever (ARF). If Strep A and ARF go untreated, repeated infections are more likely to occur. While fever and other rheumatic symptoms often resolve, ARF-associated carditis can result in permanent damage to at least one of the four heart valves. Long-term consequences of RHD may include stroke, heart failure, and premature mortality
While the global incidence of ARF and RHD has declined, it remains endemic in regions which experience barriers to healthcare as well as crowded living environments. These factors enable the spread of Strep A and development of RHD, causing half a million deaths annually.
Peters F, Karthikeyan G, Abrams J, Muhwava L, Zühlke L. Rheumatic heart disease: current status of diagnosis and therapy. Cardiovasc Diagn Ther. 2020 Apr;10(2):305-315. doi: 10.21037/cdt.2019.10.07. PMID: 32420113; PMCID: PMC7225445.
Upon further questioning, our patient reported onset of symptoms approximately twenty years ago. She had been evaluated in the capital city at some point and offered valve replacement surgery. However, per patient report, the financial cost associated with the surgery was insurmountable and she ultimately never had operative intervention. Her valvular heart disease had been managed medically, but she had been without medications "for a while."
Placing the patient back on medications to avoid fluid overload, control her heart rate, and anti-coagulate her blood produced a marked improvement in her symptoms. We continue to follow her fairly closely in clinic. Unfortunately, after years of valvulopathy, valve replacement would likely be of limited value as her "broken heart" has changed structurally in such a dramatic and largely irreversible fashion. Had she been able to "buy a valve" fifteen or twenty years ago (or better still, avoided RHD altogether), her health and subsequently her life may have been significantly different.
The above case, while interesting, is also obviously sad. It highlights the importance of prevention as well as early detection of Rheumatic Heart Disease (RHD) in endemic areas.
One of Wachalal's board members- friend, colleague, and hero- Dr. Michelle Yates, is a physician serving in Ethiopia. She and her squad are doing amazing and inspiring work on that front- screening children and adolescents for asymptomatic/early RHD. Here's a link to one her blog posts describing their program.
May we all do our part to help heal the broken hearts we encounter.
After initially posting the above case, Dr. Yates put us in contact with a Cardiologist who has extensive experience with mitral balloon valvuloplasty. He believes our patient may benefit from such a procedure. Her appropriateness as a candidate for this procedure largely hinges on the severity of her mitral regurgitation, which I hope to further quantify at a future visit. Thus, there is a glimmer of hope for our patient and her broken heart. To be continued...