Obstructive Hypertrophic Cardiomyopathy: The Challenges and Hope of Modern Treatments

Obstructive hypertrophic cardiomyopathy (oHCM) is a condition in which the heart muscle thickens excessively, becoming stiff and obstructing blood flow. In an interview with The Educated Patient, Srihari S. Naidu, M.D., director of the Hypertrophic Cardiomyopathy Program at Westchester Medical Center, with nearly two decades of experience in treating oHCM, emphasizes its diagnostic challenges. Historically, oHCM was considered rare, leading to frequent misdiagnoses. Symptoms like shortness of breath, dizziness, fatigue and chest pain mimic those of other heart conditions, and traditional imaging often misses the thickened heart muscle. Additionally, the misconception that oHCM predominantly affects young people has delayed diagnosis in middle-aged and older adults, who form the majority of cases.

Treatment for oHCM has evolved significantly over the years. Previously, beta-blockers and surgical options like muscle shaving or ablation were standard but carried risks and limited efficacy. Recently, targeted medications like mavacamten (Camzyos), which address the underlying cellular dysfunction, have shown promise in reducing obstruction and improving symptoms. Treatment goals focus on alleviating specific symptoms, such as fatigue or shortness of breath, to restore patients’ quality of life.

Naidu highlights the importance of patient advocacy and collaboration with specialists to ensure accurate diagnosis and effective treatment. Success is measured by a patient’s ability to resume everyday activities and enjoy an improved quality of life. While newer treatments are transformative, ongoing monitoring is essential to manage potential risks. Naidu advises patients to seek second opinions if necessary and find a trusted specialist to partner with in managing their condition.

A Patient Perspective

As a former respiratory therapist and paramedic, Barb was no stranger to medical challenges, but nothing could have prepared her for the escalating symptoms that left her breathless, unable to perform daily tasks and facing an overwhelming sense of despair. For months, she endured inconclusive tests and countless hospital visits until a cardiologist diagnosed her with symptomatic oHCM, a condition often mistaken for other ailments.

Despite the initial fear, the diagnosis brought hope—hope that a treatment plan could be found. With the help of her “superhero,” Naidu, Barb began treatment with mavacamten, and her life transformed. Now, two years later, Barb celebrates the return of small joys — walking her dog, climbing stairs and enjoying the beauty of the world around her.

Through her experience, she champions the importance of education and persistence in medical care, urging others to trust themselves, fight for answers and never lose hope.