Abstract
The article describes a surgical treatment of a rare congenital heart disease – isolated pulmonary artery stenosis. Clinical progression
requires observation due to the progressive development of right ventricular dysfunction. This patient has critical pulmonary stenosis
(gradient 92 mmHg, which is absolutely indication for correction), with funnel-shaped valve, supravalvular stenosis and orifice
stenosis of left pulmonary artery with hypoplasia determined our tactic choice. Long-term results indicate the effectiveness of our
methodology. Early diagnostic and surgical correction of defect allows to achieve encouraging results.
References
1. Gay B.B., French R.H., Shuford W.H. et al. The roentgenologic
features of simple and multiple coarctations of the pulmonary
artery and branches. Am. J. Roentgenol. Radium.
Ther. Nucl. Med. 1963; 90: 599–613.
2. Maugars A. Description d'une artere pulmonaire considerable
naissant de l'aorte abdominale. Rec. Period. Soc. Med. Paris.
1802; 13: 74.
3. Schwalbe E. Die Morphologie der Missbidugen des Menschen
und der Tiere. Vol. 1. Jena: Fischer; 1909: 426.
4. Nickerson E., Greenberg F., Keating M.T. et al. Deletions of the
elastin gene at 7q11.23 occur in 90% of patients with Williams
syndrome. Am. J. Hum. Genet. 1995; 56: 1156–61.
5. Matsumoto A.H., Delany D.J., Parker L.A. et al. Massive
hemoptysis associated with isolated peripheral pulmonary
artery stenosis. Cathet. Cardiovasc. Diagn. 1987; 13: 313–6.
6. Allen H.D., Driscoll D.J., Shaddy R.E., Feltes T.F. Moss and
Adams' heart disease in infants, children, and adolescents:
including the fetus and young adults. 7th ed. Lippincott
Williams & Wilkins; 2008: 836–57.
7. Trivedi K.R., Benson L.N. Interventional strategies in the management
of peripheral pulmonary artery stenosis. J. Interven.
Cardiol. 2003; 16: 171–88.
8. Fraser C.D., Latson L.A., Mee R.B.B. Surgical repair of severe
bilateral branch pulmonary artery stenosis. Ann. Thorac.
Surg. 1995; 59: 738–40.
About the authors
- Didyk Vadim Petrovich, Researcher, Cardiovascular Surgeon, orcid.org/0000-0002-8438-6339;
- Ediev Murat Osmanovich, Junior Researcher, orcid.org/0000-0002-1902-7524;
- Kim Aleksey Ivanovich, Dr Med. Sc., Professor, Head of Department, orcid.org/0000-0002-1759-9895;
- Biryukova Maria Viktorovna, Junior Researcher, orcid.org/0000-0002-6750-1286
