SESSION TITLE: Cardiovascular Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary hypertension(PHTN) is one of the markers of early mortality in sickle cell disease. Multiple co-morbidities pre-existent in the patient can worsen PHTN resulting in chronic hypoxia which itself contributes to PHTN 1, resulting in a vicious cycle terminating in early death. Early detection and treatment of such conditions can reduce the frequency of sickle cell crises(SCC) and slow the progression of PHTN, as we learned in our case.
CASE PRESENTATION: 35 year old African American female with a past medical history of sickle cell disease(SCD) and protein C deficiency(PCD) on chronic warfarin, presented with worsening shortness of breath on exertion for 2 months. She had also noticed swelling in her legs and an inability to lie down flat. She had a history of multiple admissions in the past 2 years for sickle cell crises. Heart auscultation revealed a pan-systolic murmur best heard in the left sternal border. Chest Xray showed clear lungs and CTA thorax ruled out pulmonary embolism. Echo showed severe dilatation of right atrium, severe tricuspid regurgitation(TR), moderate pulmonary hypertension and bubble study revealed an patent foramen ovale(PFO). Mitral valve was normal. Because of her worsening right heart failure and severe TR, she underwent a successful TR replacement surgery with porcine valve, despite her multiple co-morbidities. During the surgery, the tricuspid valve showed extremely thickened septal leaflet and calcified subvalvular apparatus raising a possibility of rheumatic heart disease(RHD). Two years after surgery patient has had only 1 admission for SCC.
DISCUSSION: The reason for organic TR in our patient could be multiple including isolated rheumatic TR 2, previous non-infective endocarditis from protein C deficiency 3 or infective endocarditis from recurrent transfusions that may have scarred the tricuspid valve irreparably. Presence of significant PHTN and severe TR can cause transient right to left shunting across PFO resulting in hypoxia and precipitating SCC. Correction of these co-morbidities reduced the frequency of SCC and symptoms of right heart failure drastically.
CONCLUSIONS: 1.PHTN is a marker of early mortality in SCD. 2.Early detection and treatment of co-morbidities predisposing to PHTN can improve survival and quality of life
Reference #1: Kapitan KS, Buchbinder M, Wagner PD, Moser KM. Mechanisms of hypoxemia in chronic thromboembolic pulmonary hypertension. Am Rev Respir Dis. 1989;139(5):1149-1154.
Reference #2: Fujii S, Funaki K, Denzumi N. Isolated rheumatic tricuspid regurgitation and stenosis. Clin Cardiol. 1986;9(7):353-355.
Reference #3: Ozkutlu S, Saraclar M, Atalay S, Demircin M, Ruacan S. Two-dimensional echocardiographic diagnosis of tricuspid valve noninfective endocarditis due to protein C deficiency (lesion mimicking tricuspid valve myxoma). Jpn Heart J. 1991;32(1):139-145.
DISCLOSURE: The following authors have nothing to disclose: Gaurang Vaidya, Kan Liu
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