key: cord-0024391-prr23iki authors: Karuru, Uma Devi; Relan, Jay; Verma, Mansi; Kumar, Sanjeev; Tripathi, Madhavi; Gupta, Saurabh Kumar; Ramakrishnan, Sivasubramanian; Saxena, Anita; Kothari, Shyam S. title: Heart Failure in a Child: Multimodality Approach Leading to an Unusual Cause date: 2021-12-01 journal: JACC Case Rep DOI: 10.1016/j.jaccas.2021.10.007 sha: b1399cb15cbf0d242e3ac7ddd77e17e0cfa57c48 doc_id: 24391 cord_uid: prr23iki Heart failure secondary to isolated pulmonary artery vasculitis is rarely described in children. We describe a 10-year-old child who presented with right heart failure symptoms, severe pulmonary hypertension, and bilateral branch pulmonary artery stenosis secondary to isolated pulmonary artery vasculitis. (Level of Difficulty: Advanced.) hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension secondary to peripheral pulmonary artery stenosis (PPS). To formulate a differential diagnosis of right heart failure with pulmonary hypertension in a child. To recognize the importance of a multimodality approach to the treatment of patients with right heart failure and pulmonary hypertension. To discuss the management options for isolated pulmonary artery vasculitis. We made a presumptive diagnosis of isolated PA vasculitis secondary to TA based on the age, gender, ethnicity, duration of illness, raised inflammatory biomarkers, and radiologic evidence of active vessel wall inflammation. The child was initially given decongestive measures and oral prednisolone 1mg/kg per day. After 3 months of steroids, the inflammatory biomarkers normalized, and the symptoms of right heart failure reduced, but exertional dyspnea persisted. Therefore Unusual Case of Pediatric Heart Failure The patient was discharged to take oral prednisolone with a plan to serially monitor RV pressure and function and consider repeat balloon dilatation or stenting in the future, in case of worsening or no further improvement. The maintenance dose of steroid was continued. In children, heart failure with severe pulmonary hypertension is secondary to left-to-right shunt lesions. PPS is an uncommon cause of heart failure in children and is usually congenital. PA vasculitis, an acquired cause of PPS, is extremely rare and is most commonly associated with TA. Unusual Case of Pediatric Heart Failure Unusual Case of Pediatric Heart Failure immunosuppressant therapy and repeated balloon dilatation/stenting in case of inadequate response. We describe a rare case of a child who presented with heart failure secondary to isolated PA vasculitis. The combination of immunosuppression and endovascular intervention can help in treating this rare and difficult entity. The authors have reported that they have no relationships relevant to the contents of this paper to disclose. The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis Massive hemoptysis in an 11-year-old girl with isolated pulmonary arteritis Clinical and radiographic features mimicking pulmonary embolism as the first manifestation of Takayasu's arteritis Initial isolated Takayasu's arteritis of the right pulmonary artery: MR appearance Isolated pulmonary arterial stenosis caused by Takayasu's arteritis in an elderly male Percutaneous transluminal angioplasty and stenting for pulmonary stenosis due to Takayasu's arteritis: clinical outcome and four-year follow-up Isolated large vessel pulmonary vasculitis as a cause of chronic obstruction of the pulmonary arteries Takayasu's arteritis with isolated pulmonary artery involvement in a middle-aged Asian woman with hepatitis B and latent tuberculosis infection Isolated peripheral pulmonary stenosis in Takayasu arteritis Takayasu arteritis-associated pulmonary hypertension Percutaneous transluminal angioplasty for symptomatic pulmonary stenosis in Takayasu arteritis