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Large and Medium-Sized Pulmonary Artery Obstruction Does Not Play a Role of Primary Importance in the Etiology of Sickle-Cell Disease-Associated Pulmonary Hypertension^*

Eduard J. van Beers, MD; Berthe L. F. van Eck-Smit, MD, PhD; Melvin R. Mac Gillavry, MD, PhD; Charlotte F. J. van Tuijn, RN; Joost W. J. van Esser, MD, PhD; Dees P. M. Brandjes, MD, PhD; Mies C. Kappers-Klunne, MD, PhD; Ashley J. Duits, MS, PhD; Bart J. Biemond, MD, PhD; John-John B. Schnog, MD, PhD; on Behalf of the CURAMA Study Group

^* From the Department of Hematology (Drs. van Beers, van Tuijn, and Biemond), Academic Medical Center, Amsterdam, the Netherlands; Department of Nuclear Medicine (Dr. van Eck-Smit), Academic Medical Center, Amsterdam, the Netherlands; Department of Cardiology (Dr. Mac Gillavry), Slotervaart Hospital, Amsterdam, the Netherlands; Department of Hematology (Drs. van Esser and Kappers-Klunne), Erasmus Medical Center, Rotterdam, the Netherlands; Department of Internal Medicine (Dr. Brandjes and Schnog), Slotervaart Hospital, Amsterdam, the Netherlands; and The Red Cross Blood Bank Foundation (Dr. Duits), Curaçao, the Netherlands Antilles. The CURAMA study group is a collaborative effort studying SCD in the Netherlands Antilles and the Netherlands. Participating centers include The Red Cross Blood Bank Foundation, Curaçao, the Netherlands Antilles; The Antillean Institute for Health Research, Curaçao, the Netherlands Antilles; The Department of Internal Medicine, Slotervaart Hospital, Amsterdam, the Netherlands; the Department of Vascular Medicine and the Department of Hematology, Academic Medical Center, Amsterdam, the Netherlands; the Department of Hematology, Erasmus Medical Center, Rotterdam, the Netherlands; the Department of Pathology, Groningen University Hospital, Groningen, the Netherlands; the Department of Internal Medicine, the Laboratory of Clinical Thrombosis and Hemostasis, and the Cardiovascular Research Institute, Academic Hospital Maastricht, Maastricht, the Netherlands.

Correspondence to: John-John B. Schnog, MD, PhD, Department of Hematology, Erasmus Medical Center, s-Gravendijkwal 230, 3015 CE, Rotterdam, the Netherlands; e-mail: jbschnog{at}mac com

Abstract

Background: Pulmonary hypertension (PHT) occurs in approximately 30% of adult patients with sickle-cell disease (SCD) and is a risk factor for early death. The potential role of pulmonary artery obstruction, whether due to emboli or in situ thrombosis, in the etiology of SCD-related PHT is unknown.

Methods: Consecutive SCD patients were screened for PHT (defined as a tricuspid regurgitant jet flow velocity 2.5 m/s) employing echocardiography and were evaluated for pulmonary artery obstruction with ventilation-perfusion (VQ) scintigraphy.

Results: Fifty-three HbSS, 6 HbSβ⁰-thalassemia, 20 HbSC, and 6 HbSβ⁺-thalassemia patients were included. The overall prevalence of PHT was 41% in HbSS/HbSβ⁰-thalassemia patients and 13% in HbSC/HbSβ⁺-thalassemia patients. High-probability VQ defects (Prospective Investigation of Pulmonary Embolism Diagnosis criteria) were detected in two patients, one of whom had PHT. In HbSS/HbSβ⁰-thalassemia patients with PHT, 19 patients (86%), 2 patients (9%), and 1 patient (5%) had low-, intermediate-, or high-probability scan results as compared to 30 patients (97%), 1 patient (3%), and 0 patients (0%) in HbSS/HbSβ⁰-thalassemia patients without PHT (p = 0.31). In HbSC/HbSβ⁺-thalassemia patients with PHT, 3 patients (100%), 0 patients (0%), and 0 patients (0%) had low-, intermediate-, and a high-probability scan as compared to 19 patients (90%), 1 patient (5%), and 1 patient (5%) in HbSC/HbSβ⁺-thalassemia patients without PHT (p = 0.86). There were no statistical differences in irregular distribution of the radiopharmaceutical or nonspecific signs associated with PHT between patients with and without PHT.

Conclusions: Although small pulmonary artery obstruction cannot be excluded, large to medium-sized pulmonary artery obstruction is an unlikely primary causative factor in SCD-related PHT.

Key Words: echocardiography • pulmonary artery obstruction • pulmonary artery thrombosis • pulmonary hypertension • sickle-cell disease • ventilation-perfusion scintigraphy