P722 Unrepaired complex severe aortic coarctation determining restrictive cardiomyopathy with pulmonary hypertension in adulthood

Abstract

Abstract A 54-year-old female presented to our Emergency Department with acute pulmonary edema. On physical examination she was tachypneic with arterial oxygen saturation of 91% and a grade 3/6 systolic murmur in the apex and left second intercostal space that was irradiated to the intrascapular area. Bilateral femoral and pedal pulses were quite faint on palpation. The blood pressure was 260/120 mmHg. Results of routine blood chemistry were normal with the exception of increased NT-proBNP (2746 pg/mL). Twelve-lead electrocardiogram revealed left ventricular hypertrophy with repolarization abnormalities and left atrial enlargement. The two dimensional (2D) echocardiography showed significant left ventricular hypertrophy (RWT 0.6; LVmass index 150 g/m2), no wall-motion abnormalities and normal left ventricular ejection fraction (EF 60%) but with reduced longitudinal components at TDI evaluation (6 cm/s). Severe ventricular diastolic dysfunction was detected with E/A 2,11 and E/e" 26 with a significant left atrial dilatation (LAVi 73 ml/m2) and severe pulmonary arterial hypertension ( PASP of 85 mmHg) . We were unable to visualize the aortic arch but reduced/absent pulsatile wall motion of abdominal aorta was identified. The patient reported to be affected by unrepaired aortic coarctation diagnosed at the age of 32 during pregnancy. She refused the surgical treatment twice and the she did not undergo any specific follow up but she had several admissions in emergency department for uncontrolled arterial hypertension. Our further evaluation with CT angiography showed a severe narrowing of the post isthmic aortic lumen with significant development collateral vessels (Figure 1). The patient was discharged after resolution of pulmonary edema and titration of hypertension treatment with a planning of hybrid approach to treat the aortic coarctation. The case shows a restrictive cardiomyopathy with post-capillary pulmonary hypertension due to severe unrepaired aortic coarctation. Abstract P722 Figure. CT angiography,echo, right heart cath