P1719 A rare complication of homozygous sickle cell disease and high output cardiac failure

Abstract

Abstract Introduction/Background Sickle cell disease (SCD) is an autosomal recessive blood disorder characterised by chronic anaemia and abnormally shaped red blood cells. SCD impacts multiple organ systems causing effects on morbidity and mortality, including cardiovascular (CV). Long term SCD can have a significant impact on the heart through multiple pathways, including chronic anaemia, microvascular dysfunction and the development of hypertension and chronic kidney disease, leading to the development of a high-output state and adverse cardiac remodelling. We report a rare case of homozygous SCD with the development of high output cardiac failure complicated by the development of associated pulmonary hypertension. Case summary A 58 yr old female patient (Height 158cm, weight 51.25kg, cardiac index 4.01 L/min/m²) with known homozygous SCD (HbSS) undergoing regular transfusions for chronic anaemia was admitted following a routine transthoracic echocardiogram (Figure 1a & 1b) due to the presence of a moderate sized global pericardial effusion, severe tricuspid regurgitation, dilated right heart with impaired longitudinal function, and good left ventricular (LV) systolic function (LVEF Simpson’s biplane = 62 +/- 5%). In the months preceding this, the patient described progressive dyspnoea associated with decreasing workload and orthopnoea. On clinical examination there was evidence of right sided heart failure. Blood results demonstrated a longstanding anaemia (Hb 57 g/L) with MCV 109.6. NT-proBNP was 10233 ng/L. Cardiac magnetic resonance imaging demonstrated LV dilatation with hyperdynamic function, severe LV hypertrophy, and impaired longitudinal function (global longitudinal strain -9.7%). Cardiac output was elevated at 6.5 L/min. There was no late gadolinium enhancement and T2* mapping did not demonstrate cardiac iron loading (29.6ms). Right heart catheterisation confirmed pulmonary hypertension (pulmonary artery pressure 55/33, mean 46mmHg) due to left heart disease (PC wedge pressure (24/30, mean 24mmHg). The pericardial effusion was drained and the aspirate demonstrated a cellular infiltrate reflecting pericardial inflammation (moderate pus cells, no growth after 48hrs, total protein = 56 g/L, no histological evidence of malignancy). Additionally, the patient underwent intravenous diuresis and blood transfusion with improvement in clinical status. Discussion We report a rare cause of pulmonary hypertension due to elevated left ventricular end-diastolic pressure as a result of long-term high cardiac output in a patient who has established homozygous SCD. Long-term adverse cardiac remodelling in SCD is well described. Pulmonary hypertension is a rare multifactorial complication of this process and the precise mechanisms remain unclear. It confers a poor prognosis and the only current treatment is the management of the underlying condition. Pulmonary hypertension should always be considered in SCD patients with clinical and cardiac imaging evidence. Abstract P1719 Figure 1