Pulmonary artery aneurysm: Harbinger of an ominous disease.

An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51. A diagnosis of Hughes-Stovin syndrome (vascular variant of Behcet's syndrome) was confirmed, and she was started on immunosuppression, on which there was improvement.

Cardiac magnetic resonance imaging in right ventricular outflow tract arrhythmia: a retrospective analysis from a tertiary care centre in South India.

INTRODUCTION: Arrhythmia arising from right ventricular outflow tract (RVOT) is the most common cause of idiopathic ventricular arrhythmia. Previous studies involving cardiac magnetic resonance imaging (MRI) in these patients are known to show abnormalities in up to 60% of patients. METHODOLOGY: This was a retrospective descriptive analysis of patients having idiopathic RVOT arrhythmia who underwent cardiac MRI between January 2010 and December 2020. Clinical and demographic details were recorded from the electronic medical records and cardiac MRI of all patients were reviewed. RESULTS: Among 214 patients with RVOT arrhythmia, 64 underwent cardiac MRI. A total of 41 patients, who did not have any abnormality on baseline echocardiogram were included in the study. There was male preponderance (56.1%), with median age of 43 years. About 43.9% had syncope. Twenty-four-hour Holter study revealed a premature ventricular complex (PVC) burden of 26.3 ± 11.7%. MRI showed structural abnormalities in 51.2% of patients, which commonly included RV sacculations and aneurysms. Late gadolinium enhancement (LGE) was seen in six patients, which was mostly seen in RV free wall. Right ventricular (RV) systolic dysfunction was evident in 29.3%. About 9.8% fulfilled the criteria for arrhythmogenic RV cardiomyopathy (ARVC). Out of 23 patients with baseline abnormalities on ECHO, 87% had structural abnormalities on MRI, and 43.5% fulfilled the criteria for ARVC. CONCLUSIONS: Additional imaging by cardiac MRI helps to identify structural abnormalities in 51.2% of patients with RVOT arrhythmias, even with normal baseline echocardiogram and electrocardiogram (ECG). It is useful tool to rule out ARVC in this subset of patients, and can help in increasing the diagnostic yield in the early stages.

Septo-optic dysplasia.

A seven-month-old child with congenital poor vision was referred for evaluation. Fundus examination revealed bilateral optic nerve hypoplasia with disc macula distance of approximately ten disc diameters. Neuroimaging revealed finding consistent with septo-optic dysplasia.