Giant Left and Right Atrium in Rheumatic Mitral Stenosis and Tricuspid Regurgitation

Abstract

Dilation of atria occurs in patients with valvular heart disease, especially in rheumatic mitral regurgitation, mitral stenosis, or tricuspid valve abnormalities. We report a case of giant left and right atrium in the context of rheumatic mitral stenosis and severe tricuspid regurgitation in a 68-year-old woman.

INTRODUCTION

Rheumatic valvular heart disease still continues to be a burden in developing nations. Giant enlargement of atria in adults is rare and most commonly occurs in rheumatic valvular heart disease. Despite massive enlargement many patients may remain asymptomatic.. We report a case of giant atria in the context of rheumatic mitral stenosis and severe tricuspid regurgitation in an elderly lady

CASE REPORT

A 68-year-old woman with a history of rheumatic mitral stenosis and atrial fibrillation presented with dyspnea NYHA class III, palpitations, and ankle edema for 3 months. She had worsened during the past 6 days. She had been diagnosed with rheumatic mitral stenosis 30 years back, on irregular follow-up and not compliant with injectable penicillin. Physical examination revealed a blood pressure of 100/80 mm Hg and an irregular heart rate of 136 beats/min. Auscultation revealed a loud S1 at the apex, a diastolic rumble at the apex, and a holosystolic murmur at the lower left sternal border. Chest radiography revealed a marked cardiomegaly with a cardiothoracic ratio of 95% suggesting massively dilated right atrium (RA) and left atrium (LA).

Two-dimensional echocardiogram [Figures 1 and 2] revealed a giant LA measuring 15.2 cm × 10.1 cm, an area of 170 cm², and a volume of 1326 mL. RA was 13.1 cm × 7.05 cm, with a volume of 760 ml. The mitral valve was thickened with a planimetered mitral valve area of 1.2 cm². The mean LA-left ventricular diastolic gradient was 4.5 mm Hg. The left ventricular size was small with an ejection fraction of 60%. The right ventricle was small with right ventricular dysfunction which was evident from a reduced tricuspid annular plane systolic excursion of 10 mm and a right ventricular ejection fraction of 35%. Tricuspid valve was thickened with noncoaptating leaflets and severe tricuspid regurgitation as evident from vena contracta width of 0.8 cm and hepatic vein systolic flow reversal [Video 1–2].

Figure 1.

Echocardiogram apical four-chamber view with giant left atrium 15.2 cm × 10.1 cm

Figure 2.

Echocardiogram apical four-chamber view with giant right atrium 13.1 cm × 7.05 cm

The patient was stabilized with intravenous furosemide, amiodarone, oral digoxin, warfarin, and spironolactone. The patient did not consent to surgery and hence was discharged home after medical stabilization.

DISCUSSION

Massive enlargement of atria in adults is rare and most commonly occurs in rheumatic valvular heart disease.[1] Giant RA in adults[2,3,4,5] must be differentiated from idiopathic right atrial aneurysm.[6] The most common causes of enlarged RA in adults are chronic pulmonary disease, severe mitral valvular abnormalities with pulmonary hypertension, pulmonary emboli, and tricuspid valvular abnormalities.[3] Mitral valve disease (mitral regurgitation more than mitral stenosis) often leads to giant LA.[5,6]

Hurst[5] defined a giant LA as “one that touches the right lateral side of the chest wall” on chest X-ray and that “the condition is almost always caused by rheumatic mitral valve disease.” The definition proposed by Kawazoe et al.[7] is widely accepted which defines giant LA by the following two echocardiography criteria:

1. Large LA depicted by M-mode echocardiography with diameter >65 mm,

2. Left ventricular posterobasal wall bent inward and lying between the dilated left atrial cavity and left ventricular cavity.

In our case, the right atrial enlargement was due to the severe pulmonary hypertension as a consequence of mitral stenosis and severe tricuspid regurgitation.

Kelesidis et al.[8] reported a giant RA (volume of 760 mL) in an 84-year-old woman with severe tricuspid regurgitation and severe pulmonary hypertension. Patra et al. also reported a similar case of giant RA in a patient with rheumatic heart disease.[9] According to Bando et al.,[10] about 19% of patients requiring surgery for mitral valve disease had giant atria. Many of the surgeons believe that the effect of rheumatic process on left atrial elastic fibers is irreversible, and hence they tend to go only for mitral valve surgery without LA reduction. Complications of giant atria include thromboembolism, atrial fibrillation, and Ortner's syndrome. Postoperative morbidity and mortality are also influenced by the presence of giant atria.

CONCLUSION

Giant atria due to rheumatic etiology are still prevalent in developing nations. Despite massive enlargement many patients may remain asymptomatic. Pressure symptoms, atrial fibrillation, and thromboembolism risk are very high among symptomatic patients. Early recognition and appropriate primary and secondary prophylaxis of rheumatic fever could mitigate such complications of rheumatic heart disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.