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Pulmonary Vascular Disease |

A Clinicopathological Study of Cardiopulmonary Complications of Sickle Cell Disease

Archana Sinha, MD; Marie Fidelia-Lambert, MD; Oswaldo Castro, MD; Rajan Kumar, MBBS; Nandita Shetty, MBBS; Christelle Tchiendji, MD; Alem Mehari, MD
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Department of Medicine, Washington, DC


Chest. 2013;144(4_MeetingAbstracts):861A. doi:10.1378/chest.1704919
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Abstract

SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 30, 2013 at 01:30 PM - 02:30 PM

PURPOSE: Pulmonary hypertensions (PH), systolic and diastolic dysfunction are markers of early mortality in adults with sickle cell disease (SCD). SCD associated PH is characterized by modest elevation of mean pulmonary artery pressure and is pre-capillary or post-capillary in nature. Despite seemingly favorable disease, subjects with SCD and PH often have histopathological changes more severe than might be expected from relatively modest hemodynamic abnormalities. Although, the cardiovascular and pulmonary complications are clinically recognized, very few clinicopathological studies have been done to date.The aim of the study is to characterize the cardiovascular and pulmonary findings at autopsy in adults with SCD

METHODS: A retrospective review of 17 patients with SCD deceased between 2004- 2012 at Howard University Hospital. Their clinical, laboratory, echocardiogram and autopsy findings were reviewed

RESULTS: Ten patients (59%) were males and the median age was 41 years (range 25- 69yrs). Most of the patients (94%) were African Americans and SS was the most common sickle cell genotype (88%). Thirteen of the patients (76%) were admitted with vaso-occlusive pain crisis. Echocardiogram showed EF of 51.2±12.5 %( mean±SD), the TRJV 3.3± 0.5m/s and RVSP of 55.5±18.6mmHg. The most frequent histopathological findings in the lung included vascular changes of PH grades I-VI in 11 (65%), pulmonary thromboembolism in 5 (35 %) and pulmonary edema in 16(94%).The gross examination of heart showed cardiomegaly, mean weight 536±123.7gms (normal 350gm), right ventricular thickness 0.4±0.5 cm (normal 0.3cms), left ventricular thickness 1.62±0.5 cm (normal 1.2cms). Thirteen (76%) had RV dilation and 10(59%) had LV dilatation. Mild coronary atherosclerosis was present in eleven (65%) and four (24%) had evidence of old myocardial infarction

CONCLUSIONS: Our findings indicate that cardiopulmonary abnormalities are commonly seen at autopsy in adults with SCD. Histopathological features at autopsy are typical of pulmonary arterial hypertension with intimal and medial hyperplasia and plexogenic changes. Cardiomegaly with chamber dilation and hypertrophy were frequently seen.

CLINICAL IMPLICATIONS: Further clinical research with intervention targeted at the heart and lung is warranted.

DISCLOSURE: The following authors have nothing to disclose: Archana Sinha, Marie Fidelia-Lambert, Oswaldo Castro, Rajan Kumar, Nandita Shetty, Christelle Tchiendji, Alem Mehari

No Product/Research Disclosure Information


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