Clinico-radiological profile of arrhythmogenic right ventricular dysplasia at a tertiary care center: two year experience

Arrythmogenic right ventricular dysplasia [ARVD/C] refers to fibro fatty infiltration replacement of ventricular myocardium especially that of right ventricle. The clinical presentation varies from asymptomatic state to ventricular tachycardia, heart failure and even sudden death. Diagnosis is established using modified ARVD/C taskforce criteria. Among all the various modalities of diagnosis, magnetic resonance imaging [MRI] gives most comprehensive evaluation of both morphological and functional abnormalities in this disease. MRI may not only obviate need for myocardial biopsy but also give insights into the nature of disease like presence of left ventricular myocardial involvement. We present our 2 years experience of ARVD/C patents who were admitted in our center and in whom diagnosis of ARVD/C was supported by excellent MR imaging. This study was conducted by Department of Radiology and Cardiology SKIMS, a tertiary care center for a period of 2 years. Patients with suspected ARVD/C based on clinical, electrophysiological and echocardiographic findings were subjected to MR imaging. Patients were excluded if they had history metallic implants, claustrophobia or were uncooperative. In this study stress was laid on diagnostic role of MRI in ARVD/C. The median age at presentation was 31 years [range 21-43 years]. 80% of patients were males. Most common clinical presentation was palpatations [40%]. Syncope was present in 27% and heart failure in 13%. EKG suggestive of ARVD was seen in 87%. Echocardiographic features suggestive of ARVD/C was seen in all 15 patients. Family history of premature sudden death less than 35 years old was present in one patient only. MRI evidence classical for ARVD/C was seen in 80%. Demographic features and mode of presentation of our patients is consistent with what has been rest of the world. We performed MRI in all patients to increase the specificity of our diagnosis. MR imaging allows a three-dimensional evaluation of the right ventricle and provides the most important anatomic, functional, and morphologic criteria for diagnosis of ARVD/C within one single study. MR imaging appears to be the optimal imaging technique for detection and follow-up of clinically suspected ARVD/C

Involvement of the cervical cord and medulla in posterior reversible encephalopathy syndrome

The posterior reversible encephalopathy syndrome [PRES] is characterized by patchy cortical and subcortical lesions in the distribution of the posterior circulation. The lesions are classically reversible. This syndrome has multiple etiologies, most of which cause acute hypertension. We present a case of PRES with involvement of the medulla and cervical cord [apart from the typical parieto-occipital lesions]-an extremely rare imaging manifestation of PRES. It is important to recognize the imaging findings of PRES in spinal cord, and avoid misdiagnosis as myelitis by proper clinical correlation. Typically patients with myelitis have a profound neurodeficit, while patients with spinal manifestations of PRES are asymptomatic. Involvement of the cord in PRES has probably been an underrecognized entity as spinal imaging is not routinely performed in posterior reversible encephalopathy syndrome

Congenital lobar emphysema associated with polysplenia syndrome

Polysplenia, or left isomerism, is a rare heterotaxy syndrome characterized by bilateral bi-lobed lungs, bilateral pulmonary atria, a symmetrical midline liver, and multiple aberrant splenic nodules. We report a case of polysplenia associated with congenital lobar emphysema apart from other typical anomalies. Such an association has not been previously reported. The patient was a young male with progressive exertional breathlessness referred for high resolution CT of the lungs. CT, MRI and echocardiography revealed [in addition to congenital lobar emphysema of right lung] a hemiazygos continuation of the inferior vena cava, a persistent left superior vena cava, multiple splenunculi in the right hypochondrium, midline liver, bilateral bilobed lungs, a large pulmonary artery [suggestive of severe pulmonary artery hypertension] and a large VSD- a typical constellation of findings described in polysplenia syndrome