A rare heart with 5 chambers detected in a 51-year-old man in Dubai

Aster Hospital, Mankhool recently made a rare detection when a 51-year-old man presented himself with complaints of chest pain. Upon thorough medical investigation the man was diagnosed with an unusual heart condition known as cor triatriatum sinister, a rare anomaly resulting in a heart with three atria (upper chambers of the heart, most people have two atria). This rare finding was managed successfully without the need for surgery, highlighting the hospital’s expertise in comprehensive cardiac care.

Cor triatriatum sinister is an extremely rare congenital anomaly where the left atrium is divided into two chambers by a membrane, resulting in a total of three atria within the heart. In this particular patient, alongside the two ventricles (right ventricle and left ventricle), an additional chamber has formed within the left atrium due to the presence of the membrane characteristic of cor triatriatum sinister. Therefore, while there were technically four chambers within the heart, the abnormality pertained to the left atrium, resulting in an overall count of five chambers in the heart of this patient.

It’s important to note that patients with Cor triatriatum may have other associated congenital heart defects, requiring careful evaluation and imaging. In fact, in 70% of cases, an associated Atrial Septal Defect (ASD) is present.[3] The Male to Female ratio of Cor triatriatum is 1.5:1, indicating a slightly higher prevalence in males. The size of the communication between the chambers of the left atrium determines the type of Cor triatriatum. If the blood flow is restricted due to a narrow opening, the patient may experience symptoms early in life. Conversely, if the opening is non-restrictive, patients may remain asymptomatic until later in life. However, the communicating orifice may undergo changes over time due to fibrosis and calcification, potentially leading to symptoms. Therefore, continuous observation and regular follow-up are necessary for these patients.

Despite its rarity, the medical team at Aster Hospital, Mankhool, led by Dr. Sachin Upadhyaya, Cardiology Specialist promptly identified and treated the condition in the patient, enabling him to resume his normal life in the shortest period of time.

Dr. Upadhyaya emphasized the importance of thorough investigation when dealing with atypical cardiac symptoms. He stated, “This case underscores the significance of comprehensive heart checks in adults after the age of 35 years, especially when symptoms appear unusual. It’s crucial for both medical professionals and the public to recognize that uncommon symptoms may signify underlying conditions that require careful examination and timely intervention to avoid possible complications.”

The patient presented with chest pain lasting two weeks, accompanied by occasional chest heaviness exacerbated by activity. Despite the atypical nature of the symptoms, a thorough evaluation including ECG and echocardiography revealed the presence of cor triatriatum sinister. Further confirmation was obtained through cardiac magnetic resonance imaging (CMR), which showed a non-restrictive type of cor triatriatum with no associated anomalies.

“The successful management of this case without surgery highlights the effectiveness of comprehensive evaluation and non-surgical approaches in treating rare cardiac conditions,” Dr. Upadhyaya added. “With detailed evaluation and medical management, the patient became symptom-free within a few days and continues to do well.”

Cor triatriatum sinister is an exceedingly rare condition, occurring in only 0.004% of the general population and accounting for 0.1 to 0.4% of all congenital heart anomalies. Its detection, especially in adults, can be incidental and requires careful evaluation to determine its subtype and associated symptoms, as well as to plan further treatment.

Aster Hospital Mankhool reaffirms its commitment to providing exceptional cardiac care, leveraging its expertise and advanced diagnostic capabilities to address rare and complex heart conditions effectively.

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