Key Takeaways
A rare case of cor triatriatum was diagnosed in a 34-year-old woman using transoesophageal echocardiography after initial transthoracic echocardiography findings suggested right heart abnormalities.
The case report by cardiologist Professor Ruchika Meel and colleagues from the University of Witwatersrand, Johannesburg, Johannesburg, South Africa, highlighted the importance of considering congenital heart disease as a cause of heart failure in adults and demonstrated the diagnostic value of echocardiography.
The Patient and Her History
The patient presented with progressively worsening dyspnoea, bipedal swelling, and chronic productive cough at a hospital in Johannesburg, South Africa. Her pre-existing condition was HIV infection, which was being treated with antiretroviral therapy.
Additionally, she had heart failure classified as New York Heart Association functional class IV, which was treated with medication. About a year prior, the patient had developed post-partum cardiomyopathy. Since then, she had experienced the aforementioned symptoms. A detailed investigation revealed that community-acquired pneumonia had further worsened the symptoms.
The other medical, medication, family, social, drug, and travel history was unremarkable.
Findings and Diagnosis
The patient’s vital parameters were measured upon admission. Blood pressure was normal at 118/80 mm Hg. A heart rate of 122 beats/min indicated sinus tachycardia. Her body temperature and respiratory rate were within normal ranges. Inspection, auscultation, palpation, and percussion confirmed the medical history.
Laboratory tests showed normocytic, normochromic anaemia with a haemoglobin level of 11.8 g/dL and a CD4+ count of 945 cells/µL. An elevated C-reactive protein level of 108 mg/L and abnormal liver function tests attributed to cardiac congestion were also observed.
The initial chest x-ray showed pulmonary congestion, characterised by an enlarged cardiothoracic ratio, prominent pulmonary arteries, right pleural effusion, and consolidation of the right lower lobe.
A 12-lead ECG indicated left atrial enlargement, right ventricular hypertrophy, and tricuspid regurgitation.
Transthoracic echocardiography revealed right atrial enlargement and right ventricular hypertrophy (right ventricular free wall thickness was 7 mm). Moderate to severe tricuspid regurgitation was also noted. The pulmonary arterial systolic pressure was elevated to 100 mm Hg. The ventricular ejection fraction was significantly reduced to 20%.
For a more accurate diagnosis, a detailed transoesophageal echocardiogram of the heart revealed a small atrial septal defect (three-dimensional [3D] area of 0.42 cm²) with left-to-right shunting of blood contaminating the tricuspid regurgitation jet. Additionally, a membrane with a defect measuring 1.3 cm² on multiplanar 3D reconstruction was confirmed in the left atrium above the mitral valve. The mean gradient across the defect was 3 mm Hg and was underestimated due to the low-flow, low-gradient state associated with biventricular failure. Moderate to severe eccentric functional mitral regurgitation (vena contracta width of 0.6 cm) jet impinging on the membrane as a consequence of underlying dilated cardiomyopathy was also noted. A large thrombus was also observed in the right atrial appendage.
On the basis of overall findings, cor triatriatum was diagnosed.
Given her late presentation, poor cardiac function, and complex congenital heart defects, which could significantly complicate interventional procedures, the patient was considered to be at high surgical risk with limited benefits. The final diagnosis was made at a facility that lacked surgical expertise in complex adult congenital heart disease. Consequently, she was managed medically with diuretics, particularly furosemide, and anti-remodelling therapy consisting of enalapril, spironolactone, and carvedilol. The patient was also administered rivaroxaban as an anticoagulant. At the 6-month follow-up, her ejection fraction increased to 34%, and her symptoms improved significantly.
Discussion
Globally, the incidence of cor triatriatum is only 0.1%-0.4%. Cor triatriatum is a rare congenital cardiac anomaly characterised by a fibromuscular membrane that partitions the atrium into two distinct chambers. The presence of a septum in the left atrium results in cor triatriatum sinister, whereas a septum in the right atrium results in cor triatriatum dexter.
This is the “first documented case from Southern Africa of an adult female presenting with heart failure with an incidental finding of cor triatriatum sinister discovered through advanced echocardiographic imaging,” the authors wrote. Its “purpose” is to “raise awareness about the presence of congenital heart disease in adulthood as a potential cause of heart failure.” It also demonstrates the critical role of echocardiography in identifying congenital defects, particularly in developing nations.
This article was translated from Univadis Germany using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.