Figure. Chest radiograph showing a 1-cm left apical pneumothorax, left tube thoracostomy with a Foley catheter, and a prominent central right pulmonary artery suggestive of pulmonary hypertension.
Pulmonary hypertension may be either primary (idiopathic) or secondary. The pathogenesis of primary pulmonary hypertension may be (1) neurohormonal vascular hyperreactivity as a central mechanism in which chronic vasoconstriction induces pulmonary hypertension with ensuing intimal and medial vascular hypertrophy, (2) immune collagen vascular diseases, or (3) atherosclerosis.
Secondary pulmonary hypertension may be associated with (1) chronic obstructive or interstitial lung disease, (2) recurrent pulmonary emboli, or (3) antecedent heart disease. As the disease progresses, the right ventricle compensates through hypertrophy. Although the right ventricle can sustain elevated pressures for months to years, decompensation eventually occurs. The diagnostic workup includes electrocardiography, echocardiography, and cardiac catheterization.
Medical management is poorly understood and very complex. Treatment includes a combination of oxygen, diuretics, digoxin (Lanoxin; Glaxo Wellcome, Research Triangle Park, NC), long-term anticoagulation, calcium-channel blockers, and intravenous prostacyclin (Flonan; Glaxo Wellcome).1In addition, vasodilators such as endothelin 1 and the transforming growth factor β superfamily are under investigation.2An additional treatment modality that has been shown to be effective involves the administration of inhaled nitric oxide.3Lung transplantation is the final option, but it is reserved for patients who either have refractory right ventricular heart failure on presentation or develop right ventricular heart failure despite maximal medical therapy.1
Video-assisted thorascopic surgical pleuradesis is recommended for recurrent pneumothorax. Because of the abnormal chest radiograph, diagnostic evaluation showed that this patient had pulmonary hypertension (systolic pressure of 110 mm Hg). Therefore, before video-assisted thorascopic surgical pleuradesis was performed, a trial of prostacyclin therapy was instituted. Unfortunately, the patient did not respond to the prostacyclin therapy and therefore was deemed a candidate for lung transplantation. Pleuradesis in lung transplant candidates is considered a relative contraindication.
This case demonstrates the importance of a plain chest radiograph. Careful evaluation of all diagnostic studies is essential, because additional pathologic conditions may be identified. Moreover, further diagnostic studies may be necessary, thereby altering initial treatment strategies.
Correspondence: Richard J. Hendrickson, MD, Department of Pediatric Surgery, Children's Hospital/University of Colorado Health Sciences Center, 1056 E 19th Ave, B323, Denver, CO 80218 (Hendrickson.Richard@tchden.org).
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Image of the Month—Diagnosis. Arch Surg. 2004;139(9):1017–1018. doi:10.1001/archsurg.139.9.1018