Home›Cardiology› Double-chambered left ventricle in adulthood with severe mitral regurgitation and refractory heart failure
Double-chambered left ventricle in adulthood with severe mitral regurgitation and refractory heart failureComplex heart structure can cause severe heart failure in adults
Frontiers in MedicinePublished August 27, 2026DOI ↗Editorial oversight: Dr. Amelia Tan, PhD · Internal Medicine & Chronic Disease
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Key Takeaway
Consider DCLV in adults with refractory heart failure and severe mitral regurgitation; transplant evaluation may be warranted.
This case report describes a 50-year-old man with longstanding atrial fibrillation and heart failure with reduced ejection fraction, who was found to have a double-chambered left ventricle (DCLV), a rare congenital anomaly. The patient also had severe mitral regurgitation, left ventricular non-compaction, and biventricular systolic dysfunction. The report illustrates that DCLV can present in adulthood with refractory heart failure, rather than only in childhood.
At baseline, the patient's ejection fraction was 29.4%, with an intraventricular gradient of 7 mmHg and a premature ventricular complex burden of 52.9%. After one month of guideline-directed medical therapy for heart failure with reduced ejection fraction, the report notes these parameters, though the direction of change is not specified. The long-term response to medical therapy remains to be determined.
The authors highlight that DCLV can be associated with severe mitral regurgitation and refractory heart failure in adults. They suggest that for cases where structural repair is not feasible due to complex anatomy, evaluation for orthotopic heart transplantation should be considered. This recommendation is based on the single case and should be interpreted cautiously.
Limitations include the small sample size inherent to a case report. No adverse events, funding sources, or conflicts of interest were reported. The findings are hypothesis-generating and do not establish a standard of care.
How this fits prior evidence
This case report extends prior coverage by illustrating a rare structural cause of heart failure in an adult with atrial fibrillation, complementing earlier findings that postoperative atrial fibrillation increases thromboembolic risk. It also underscores the diagnostic complexity in heart failure, aligning with prior evidence that speckle-tracking echocardiography may better characterize ventricular mechanics than LVEF alone. The report does not address anticoagulation strategies, but the patient's atrial fibrillation and heart failure context is relevant to prior guidance favoring anticoagulation monotherapy over adding antiplatelet therapy.
Imagine your heart having an extra chamber where it should not be. For one 50-year-old man, this structural issue, called a double-chambered left ventricle, made managing his heart health incredibly difficult. He also lived with atrial fibrillation, which is an irregular and often rapid heart rate.
Doctors found that his condition led to severe heart failure and significant issues with his heart valves. His heart's pumping ability was measured at 29.4 percent. Because of the complex way his heart was built, standard repairs were not possible. This case highlights how rare structural problems can appear in adulthood and cause serious complications.
While this report only looks at one patient, it is important for doctors to recognize these complex cases early. For patients with such complicated anatomy, experts suggest looking into options like a heart transplant when standard surgeries cannot fix the damage.
What this means for you:
A rare double-chambered heart can cause severe heart failure and may require advanced transplant options.
Common questions
What is a double-chambered left ventricle?
It is a rare condition where the main pumping chamber of the heart, the left ventricle, has an extra wall or chamber. In this case, it caused severe heart failure and problems with the heart's valves in a 50-year-old man.
How does this affect heart function?
The patient had an ejection fraction of 29.4 percent, which means his heart was not pumping blood effectively. He also experienced mitral regurgitation and other issues caused by the complex structure of his heart.
What are the treatment options for this condition?
Because the anatomy was so complex, standard repairs were not possible. In cases like this where surgery cannot fix the structure, doctors may evaluate the patient for a heart transplant.
BackgroundDouble-chambered left ventricle (DCLV) is an extremely rare congenital anomaly characterized by an aberrant muscular or fibromuscular septum dividing the left ventricular cavity. Most cases present in childhood or adolescence. Adult presentations are exceptional, particularly when complicated by severe valvular dysfunction and advanced heart failure.Case summaryA 50-year-old male with longstanding atrial fibrillation presented with one month of exertional chest tightness and progressive dyspnea, including paroxysmal nocturnal dyspnea. Physical examination revealed a displaced apex and a grade 5/6 holosystolic murmur consistent with severe mitral regurgitation. Electrocardiography showed permanent atrial fibrillation with an extremely high premature ventricular complex burden (52.9%). Transthoracic echocardiography was diagnostic, demonstrating a hypertrophied muscular septum dividing the mid-to-distal left ventricle into a basal and an apical chamber (peak gradient 7 mmHg), severe mitral regurgitation due to restrictive leaflet closure, left ventricular non-compaction, and severe biventricular systolic dysfunction (left ventricular ejection fraction 29.4%). Despite optimization of guideline-directed medical therapy for heart failure with reduced ejection fraction, the patient's symptoms and functional capacity showed no improvement over one month of follow-up. Given the surgically complex anatomy, diffuse myocardial disease, and refractory biventricular failure, the multidisciplinary heart team recommended referral for orthotopic heart transplantation evaluation.ConclusionThis case highlights that double-chambered left ventricle (DCLV), though typically a pediatric diagnosis, can rarely present in adulthood with severe mitral regurgitation and refractory heart failure, adding to the limited reports of this presentation. In such cases where structural repair is not feasible due to complex anatomy and low intraventricular gradient, heart transplantation evaluation may represent the most appropriate therapeutic strategy, although the long-term response to medical therapy remains to be determined.
