Type IV- Reno-Cardiac syndrome in a female child: Heart and kidney dysfunction, intertwined.

Abstract

Renal artery stenosis is the major cause of renovascular hypertension, can be found isolated or have association with syndromes or autoimmune conditions. It can be due to atherosclerotic deposition in vessels, autoimmune etiology or fibromuscular dysplasia, the latter is the commonest cause, however, needs biopsy for diagnosis and exclusion of other possible causes. Patients can have diverse presentation, disease may be discovered as incidental finding, with hypertensive crises, Nephropathy or cardiac manifestations. We report a case of 7 years female patient presented with severe respiratory distress, hypertension, Grade 1 BL pitting edema, hepatomegaly, basal crackles in chest and gallop. He had no history of any diagnosed medical condition prior. However, symptoms were evident for last 2 months with Headache, fatigue, cough, exertional dyspnea, edema and decreased urine output. She had no history of arthralgia, frothy urine, hematuria or any skin rashes. Evaluation for Hypertension revealed small sized right kidney with decreased perfusion in segmental arteries. Echocardiography showed Biventricular dilation, dysfunction with Ejection fraction of 40%. She initially managed with iv labetalol infusion later started enalapril and amlodipine then referred to specialty where percutaneous transangioplasty planned abut deferred by pediatrics nephrology as patient responded with medical management and it is considered for resistant hypertension or persistent high renin and aldosterone. The case concluded with diagnosis of Reno cardiac type-iv syndrome, as manifestations are concordant with chronic kidney disease – right SSK due to hypoperfusion that resulted in renovascular hypertension and Cardiac failure.