Abstract 18305: Right Ventricular Stemi as a Cause of Death in Idiopathic Pulmonary Arterial Hypertension

• Published in: Circulation (New York, N.Y.) Vol. 134; no. Suppl_1 Suppl 1; p. A18305
• Main Authors: Zhan, Yang, Burstein, Barry, Abualsaud, Ali, Nosair, Mohamed, Langleben, David
• Format: Journal Article
• Language: English
• Published: by the American College of Cardiology Foundation and the American Heart Association, Inc 11.11.2016
• ISSN: 0009-7322

IntroductionThe cause of death in advanced idiopathic pulmonary arterial hypertension (IPAH) is commonly cardiogenic shock or arrhythmia. We describe a 33 year old female with advanced IPAH on IV epoprostenol and oxygen, who had a normal coronary angiogram 12 months prior to admission. She presented with two days of stuttering angina that became constant. Initial ECG showed new ST Elevation in leads 1, aVL, V2-V6. (Fig 1-A). Rapid CT pulmonary angiogram excluded pulmonary embolism. Urgent cardiac catheterization showed discrete obstruction of the first and second acute marginal branches supplying most of her right ventricle (RV) (Fig 1-C, D). Angioplasty and stenting was performed, with partial reperfusion. Echocardiography showed a severely dilated and hypokinetic right ventricle with no evidence of right to left shunt. She subsequently died of refractory cardiogenic shock before heart-lung transplant could be performed. Right ventricular ischemia is a major cause of chest pain in IPAH, and may cause myocardial necrosis with high troponins. However, a witnessed right ventricular STEMI, particularly in a young female, has not been described. She developed coronary lesions within a 12 month period, and one explanation might be that her massive right ventricular dilatation resulted in compression of her acute marginals between the RV myocardium and chest wall, with endothelial trauma and thrombus formation. In addition, the dilated right ventricle overlaid the whole anterior chest wall explaining the ECG findings on presentation (Figure 1-B).ConclusionAcute myocardial infarction should be suspected in patients with severe IPAH and ongoing chest discomfort, even without common cardiovascular risk factors. The dilated right ventricle occupies much of the precordium, making ECG localization unreliable. Severe right ventricle dilation might cause coronary compression leading to vascular injury and thrombus formation.