Summary
TWO patients with the CRST syndrome and pulmonary hypertension are described. They were treated with hydralazine given orally in a dosage of 200 mgs daily. One patient improved subjectively and had a better exercise capacity but the signs of pulmonary hypertension and tricuspid regurgitation persisted. The other patient has shown a marked clinical improvement and a significant reduction of the pulmonary hypertension has been demonstrated on repeat cardiac catheterisation.
Similar content being viewed by others
References
Camerini, F., Alberti, E., Klugmann, S. and Salvi, A. 1980. Primary pulmonary hypertension: Effects of nifedipine. Br. Heart J. 44, 352.
Daoud, F. S., Reeves, J. T. and Kelly, D. 1978. Isoproterenol as a potential pulmonary vasodilator in primary pulmonary hypertension. Am. J. Cardiol. 42, 817.
Fahey, P. J., Utell, M. J., Condemi, J. J., Green, R. M. and Hyde, R. W. 1980. Raynaud’s phenomenon of the pulmonary vasculature. Chest 78, 515.
Hall, D. R. and Petch, M. C. 1981. Remission of primary pulmonary hypertension during treatment with diazoxide. Br. Med. J. 282, 1118.
Honey, M., Cotter, L., Davies, N., Denison, D. 1980. Clinical and haemodynamic effects of diazoxide in primary pulmonary hypertension. Thorax 35, 269.
Howard, T. P., Solomon, D. A., Germain, B. and Goldman, A. L 1980. Clinically silent pulmonary hypertension in the CRST syndrome. Chest 78, 522.
Rubin, L. J., Peter, R. H. 1980. Oral hydralazine therapy for primary pulmonary hypertension. N. Eng. J. Med. 302, 69.
Sackner, M. A., Akgun, N., Kimbel, P. and Lewis, D. H. 1964. The pathophysiology of scleroderma involving the heart and respiratory system. Ann. Int. Med. 60, 611.
Salerni, R., Rodnan, G. P., Leon, D. F. and Shaver, J. A. 1977. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). Ann. Int. Med. 86, 394.
Shettigar, U. R., Hultgren, H. N., Specter, M., Martin, R. and Davies, D. H. 1976. Primary pulmonary hypertension favourable effect of isoproterenol. N. Eng. J. Med. 295, 1414.
Velayos, E. E., Masi, A. T., Stevens, M. B. and Shulman, L. E. 1979. The ’CREST syndrome. Comparison with systemic sclerosis (scleroderma). Arch. Intern. Med. 139, 1240.
Wang, S. W. S., Pohl, J. E. F., Rowlands, D. J. and Wade, E. G. 1978. Diazoxide in treatment of primary pulmonary hypertension. Br. Heart J. 40, 572.
Winterbauer, R. H. 1964. Multiple telangiectasia, Raynaud’s phenomenon, sclerodactyly and subcutaneous calcinosis: A syndrome mimicking hereditary hemorrhagic telangiectasia. Bull. Johns Hopkins Hosp. 114, 361.
Young, R. H. and Mark, G. J. 1978. Pulmonary vascular changes in scleroderma. Am. Med. J. 64, 998.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
McWeeney, J., Finnegan, P. Vasodilator therapy of pulmonary hypertension in the crst syndrome. Ir J Med Sci 151, 151–154 (1982). https://doi.org/10.1007/BF02940166
Issue Date:
DOI: https://doi.org/10.1007/BF02940166