LVNC: Addressing a serious condition that is estimated to be 99.8% less prevalent than coronary artery disease.

Left Ventricular Non-compaction Cardiomyopathy (LVNC) is still a widely under-recognized heart condition, with a much lower prevalence compared to more common heart diseases. For example, coronary artery disease affects about 7% of adults, making it nearly 500 times more common than LVNC. Hypertrophic cardiomyopathy, which affects roughly 0.2% of people, is about 14 times more frequent. These numbers highlight just how rare LVNC is and why more attention and research are needed.

The left ventricle, the heart’s main pumping chamber, normally develops into a strong, solid muscle. In LVNC, this process doesn’t fully finish, leaving the heart muscle with a spongy texture and deep grooves. During fetal development, the heart starts off spongy and then compacts into muscle before birth. When that compaction is incomplete, it results in the unusual heart appearance seen on scans (Towbin). However, not every heart that looks spongy is sick. Studies using advanced cardiac magnetic resonance imaging (MRI) found that up to 15% of people may have some non-compaction, especially athletes or pregnant women, where the heart changes naturally due to stress (Petersen et al.). For many, this is harmless. But for others, LVNC can cause serious problems, like weak pumping, irregular heartbeats, or blood clots that can cause strokes (Jenni et al.). Around 12% of affected adults may die or need a heart transplant, while others require ongoing care (Oechslin et al.). LVNC can also run in families due to genetic mutations in certain heart muscle genes such as MYH7, MYBPC3, and TTN (Klaassen et al.). We noticed that even people with the same mutation can have very different outcomes, some live without symptoms, while others develop severe complications. This unpredictability makes LVNC difficult to fully understand.

LVNC exists on a spectrum, sometimes it’s a disease, and other times it’s just a harmless heart variation. That’s why doctors don’t rely on imaging alone. They look at symptoms, family history, and genetic tests to understand what the diagnosis means for each person (Aras et al.). The condition isn’t straightforward. While it may not be a breakthrough technology like AI, learning about LVNC could be life-changing for patients and families.

When we started researching LVNC, we thought it would be mostly technical terms and statistics. Instead, we found many stories filled with emotion. People diagnosed range from marathon runners who fainted once, to newborns who suffer heart failure soon after birth, to those who never had symptoms until they collapsed unexpectedly. Some live 80 years without problems, while others end up in the emergency room as teenagers. There’s no typical case. Almost half of patients show no symptoms at diagnosis but later develop serious issues like arrhythmias, heart failure, or strokes (Towbin). Some need devices like defibrillators in their 20s. Diagnosis can feel like detective work—one doctor may see LVNC on an MRI, another may not (Petersen et al.). Some say it’s overdiagnosed, others say it’s missed. Genetic mutations linked to LVNC can also be found in people with other heart conditions or none at all (Klaassen et al.). It’s a confusing, debated, and still not fully understood condition. For patients, this means a difficult journey full of questions about their future and safety. Our goal isn’t to have all the answers but to provide clarity, share real patient stories, and build a community where people can learn and support each other.

Studies estimate that about 1 in 700 people have LVNC, though using broader criteria suggests it may be more common (Petersen et al.). One major problem is that many doctors and cardiologists aren’t familiar with LVNC or other rare heart diseases. This leads to patients being misdiagnosed, told their symptoms aren’t serious, and sent to many appointments without answers. This is dangerous. For children, delayed diagnosis means the disease can progress unchecked, harming their fragile bodies. Adults without diagnosis may suffer years of unexplained fatigue, shortness of breath, or palpitations without knowing why. One study found that nearly 20% of children with LVNC died or needed a heart transplant within five years of diagnosis (Jenni et al.). For those who survive, not knowing what’s wrong with their heart can be deeply stressful. There is no single standard test—some doctors diagnose LVNC on MRI, others miss it with ultrasound (Petersen et al.). In places without access to advanced imaging or genetic testing, people may never understand why their heart beats irregularly. This means they’re fighting an invisible battle. That’s why awareness is so important. Better awareness leads to better research, better diagnoses, and better care. Our hope is that by raising awareness, patients and families can recognize signs earlier, feel empowered to advocate for themselves, and connect with doctors who understand this complex condition.

A heart attack can be a week, a day, even minutes away when you’re not pumping the blood you need and that’s why this cause is so important. Learning new things is always fun. But learning is just the beginning.

Start by following our work on Instagram @nexusheartfoundation. On our page we analyze the latest breakthroughs for biotech with LVNC and heart disease, uncover patient stories, and will interview top cardiologists to make space for real conversations about what it’s like to live with or care for someone with LVNC.

We’re also building something bigger. A full website where you’ll be able to access medically reviewed resources, hear directly from those with LVNC, and find information that doesn’t require a PhD or MD to read. Through interviews, real-life experiences, and expert insight, we’re making sure LVNC gets the awareness and support it has long been underserving of. All of the donations we raise will be given directly to Cardio-Myopathy funds such as the Lori Fund and the Heart Failure Society of America so we can support families and fuel research into better treatment and earlier diagnoses. Though we are in our emerging stage of development, we maintain committed in aiding and supporting patients with LVNC. The more people who know, the more lives we can change.

Awareness leads to action. Action leads to care. And real, timely, human care can be the difference between fear and hope. Join us in bringing LVNC out of the shadows.

This starts with you.

Citations:

Aras, D., et al. “Clinical Features of Isolated Ventricular Noncompaction in Adults: Long-Term Clinical Course, Echocardiographic Properties, and Predictors of Mortality.” Journal of Cardiac Failure, vol. 12, no. 9, 2006, pp. 726–733.

Jenni, R., E. Oechslin, J. Schneider, et al. “Echocardiographic and Pathoanatomical Characteristics of Isolated Left Ventricular Non-Compaction: A Step Towards Classification as a Distinct Cardiomyopathy.” Heart, vol. 86, 2001, pp. 666–671.

Klaassen, Stephan, et al. “Mutations in Sarcomere Protein Genes in Left Ventricular Noncompaction.” Circulation, vol. 117, no. 22, 3 June 2008, pp. 2893–2901. doi:10.1161/CIRCULATIONAHA.107.746164. https://pubmed.ncbi.nlm.nih.gov/18506004/.

Oechslin, E., et al. “Long-Term Follow-Up of 34 Adults with Isolated Left Ventricular Noncompaction: A Distinct Cardiomyopathy with Poor Prognosis.” Journal of the American College of Cardiology, vol. 36, no. 2, 2000, pp. 493–500.

Petersen, Stephan, et al. “Left Ventricular Non-Compaction: Insights from Cardiovascular Magnetic Resonance Imaging.” Journal of the American College of Cardiology, vol. 46, no. 1, July 2005, pp. 101–105. https://doi.org/10.1016/j.jacc.2005.03.045.

Towbin, Jeffrey A. “Left Ventricular Noncompaction: A New Form of Heart Failure.” Heart Failure Clinics, vol. 6, no. 4, 2010, pp. 453–469. https://www.sciencedirect.com/science/article/pii/S1551713610000802.

Article Written By - Peter Smalley, Dillon Colaso, Akshaj Katragunta