A 29-year-old young female presented in out-patient with complain of breathlessness on exertion, and bilateral lower limb swelling. Transthoracic echocardiography (TTE) showed severely dilated isolated right ventricle with dysfunction without pulmonary hypertension. Endomyocardial biopsy revealed lymphocytic myocarditis with fibrosis.
Right ventricular dysfunction could be acute and/or chronic. Massive pulmonary embolism, severe LV dysfunction and right ventricular infarction commonly with inferior wall myocardial infarction are well known causes of right ventricle dilatation and dysfunction. Atrial septal defect is another common cause of long-standing right ventricle dilatation. Rare causes are arrhythmogenic cardiomyopathy principally with ventricular arrhythmia, giant cell myocarditis, eosinophilic with hypersensitivity reaction and lymphocytic myocarditis. Few cases of isolated right ventricular involvement in sarcoidosis are also reported.
Isolated right ventricle lymphocytic myocarditis is unique diagnosis. Usually the course of disease is acute fulminant and recovers with limited or no damage, as the peak myocardial inflammation subsides; however, some cases show irreversible myocardial damage. Lymphocytic myocarditis is ordinarily caused by a viral infection. Viral infections associated with the condition includes parvovirus B19, adenovirus, coxsackievirus B and enterovirus being the most frequent and many more. Rare to rare, does it progress as chronic inflammation and right ventricular dysfunction in isolation. Endomyocardial biopsy is the gold standard, but not constantly performed in these cases.
A 29 years old young female presented in out-patient with history of breathlessness on exertion (Grade 2) from last 2 years, both lower limb swelling and abdominal distention. No history of chest pain, palpitation, dizziness and syncope. No relevant family or drug history. Physical examination shows regular pulse 80bpm, BP of 124/78mmHg, Spo2 99% on room air, raised jugular venous pressure prominent ‘v’ wave, bilateral grade 3 pitting oedema, and pulsatile liver and systolic murmur in lower left parasternal area. (Figure 1) ECG shows regular rhythm, low-voltage QRS complexes and T wave inversion in anterior precordial leads with few VPCs.
Fig. 1 Showing electrocardiogram
TTE revealed massive right ventricle dilation and dysfunction with severe tricuspid regurgitation. Normal left side systolic, diastolic and valvular function. Mild pulmonary systolic pressure of 36 mmHg noted on echocardiography (Figure 2).
Grossly dilated right ventricular and right attrium |
Severe tricuspid regurgitation |
Dilated right ventricule with volume overload |
Transoesophageal Echocardiography was done revealed normal pulmonary vein drainage and no atrial septal defect. Routine investigations were ordered showed no significant abnormality except borderline positive HsTropT and BNP. Abdominal ultrasound showed mild ascites and mild hepatomegaly.
Computed tomography pulmonary angiography (CTPA) and Bilateral lower limb venous Doppler showed no sign of venous thromboembolism (VTE).
Cardiac Magnetic Resonance imaging (CMR) manifest mild late gadolinium enhancement of right ventricle free wall suggestive of myocyte necrosis on top of right ventricular dysfunction (Figure 3).
Fig.3 Cardiac MR showing LGE of right ventricle free wall.
Genetic testing was done but no pathogenic or likely pathogenic variants causative of the phenotype was detected for genetic cardiomyopathies.
Ultimately, myocardial biopsy was done under fluoroscopic guidance that revealed myocardial tissue with multiple areas of fibrosis with lymphocytic infiltration i.e. chronic myocarditis. No fibrofatty replacement of myocyte. No granulomas, multinucleated giant cells or eosinophilic infiltration. Not at all, evidence of viral cytopathic changes seen (Figure 4).