Dear Doctors, Today I would like to share an interesting and uncommon case of right sided heart failure due to severe pulmonary hypertension secondary to a connective tissue disorder. A 29 year old married female hailing from Thane presented to us with complaints of breathlessness on exertion, orthopnea, PND and cough for 15 days. She had a saturarion of 81% on room air in my OPD and was admitted to ICU urgently under my care. She had tachycardia with pulse of 110/ min and BP of 130/80 mm hg. She had a pansystolic murmur in tricuspid area, crepts bilaterally. There was no pedal edema. We put her on nasal prongs with oxygen @ 2litres/ min. Central line inserted. CVP was 30 cm h2o. Serum PRO NT BNP levels were raised 2084. 2d echo showed severe pulmonary hypertension with pasp/ tr jet of 100 mmhg. ( normal is less than 25). Severe triscuspid regurgitation was also present with severe RA and RV dilatation. CT chest showed signs of severe PAH. D dimer was elevated to 1.41 We started her on ambrisentan+ sildenafil citrate combination titrating with systolic BP. Lasix infusion was given for 24 hours. Clexane was given for raised D dimer . Work up for causes of severe PAH was sent. ANA and dsDNA antibodies were positive suggestice of SLE. We started her on low dose steroid and hydroxychloroquine for the same. She had a history of joint pains and facial erythema on sun exposure in the past which corelates with SLE. Minimal proteinuria was present in urine. However creatinine levels were normal. Direct Coombs test reports are awaited. Day 3 of ICU the condition improved. Symptoms improved. CVP was 8 to 10 cm h2o. Serum ProNT BNP levels dropped to 700. D dimer levels dropped. She was still dependent on oxygen and was transferred out of the icu on 3rd day. After another 2 days in genersl wards she was discharged to home on oxygen at 1 litre/ min. She has been advised a heart lung transplant in view of her pathology. Dr Hardik Thakker, MD Med, ECFMG (USA).