Abstract
Introduction. Idiopathic pulmonary arterial hypertension (IPAH) in children is one of the rare and understudied diseases. Literature as a rule contains only presentations of individual cases. Our study is aimed at research of morphological and morphometrical peculiarities of pulmonary intraacinar arteries of children suffering from IPAH.
Material and methods. Between 1980 and 2015 year we studied lung speciments of 12 patients (9 autopsies and 3 biopsies), 6 male and 6 female aged between 5 month and 14 years 6 months old. 1x1x1.5 sm of pulmonary tissue was embedded in paraffin and the serial sections (10-15) were colored using routine methods. In addition intraacinar arteries were analised using quantitative morphometric techniques.
Results. A wide spectrum of morphological changes of pulmonary arteries was found, from II to V-VI grades of Heath-Edwards classification. We discovered, that only 4 раtients had the slightest changes: infant aged 5 months had changes II grade of Heath-Edwards classification, two children aged 11 months and one patient aged 1 year had changes of the III grade of Heath-Edwards classification. 7 children, among them those aged 1-2 years, had irreversible morphological changes of IV-VI grades of Heath-Edwards classification, which are common for pulmonary-vascular disease (plexyform artheriopathy) developing very fats and resulting into death in 2 or 3 years if not treated. Thickness index of intraacinar arteries (d 100-300 mm) measures up to 37.6 and 50.1% in some cases, which overruns the media hypertrophy greater level of those having congenital heart disease.
Conclusion. Pulmonary-vascular disease in children having IPAH can develop faster than among those having congenital heart disease, pulmonary-vascular disease appear at the age of 11-12 months. Mean arterial medies thickness in patients with IPAH greater in patients with congenital heart disease. Two types of congenital pathology connected with geneous abnormalities were discovered under IPAH from children. The first type is characterized by appearance of plexiform structures following the development of obstructive lesions. Under the second more rare type the plexiform structures are initially rooted in, but the disease does not develop immediately after birth, but later, when obstruction of plexiform structures appears without development of obstructive lesions in intraacinar arteries.
References
1. Есипова И.К. (ред.). Некоторые вопросы патологии
легких в свете новейших данных об их нормальном
строении, развитии, регенерации. Новосибирск:
Изд-во Сиб. отд-ния АН СССР; 1962.
2. Крючкова Г.С., Еремеева А.С. Первичная гипертония
малого круга кровообращения. Архив
патологии. 1976; 5: 84—9.
3. Мухарлямов Н.М., Рыфф И.М. Некоторые вопросы
клиники и патологии первичной легочной
гипертонии. Терапевтический архив. 1974;
2: 27-35.
4. Wagenvoort C.A., Wagenvoort N. Primary pulmonary
hypertension a pathologic study of the lung
vessels in 156 clinically diagnosed cases. Circulation.
1970, 42: 1163-84.
5. Wagenvoort C.A. UnexpMned pulmonary hypertension.
Pathol. Microbiol. 1975; 13 (23): 239-41.
6. Yamaki S., Wagenvoort C.A. Cоmparison of primary
plexogenic arteriopathy in adults and children.
Brit. Heart J.1985; 54: 428-34.
7. Yamaki S. Pulmonary vascular disease associated
with pulmonary hypertension in 445 patients: diagnosis
from lung biopsy and autopsy. Gеn. Thorac.
Cardiovasc. Surg. 2013; 61: 24-31.
8. Shephard J.T., Edwards J.E., Burchell H.B.,
Swan H.J.C., Wood E.H. Clinical, physiological
and pathological considerations in patients with
idiopathic pulmonary hypertension. Br. Heart J.
1957; 19 (1): 70-82.
9. Tompson J.R., Machado R.D., Pauciulo M.W,
Morgan N.V., Humbert M., Elliott G.C. et al.
Spоradic primary pulmоnary hypertension is associated
with gernline mutations оf the gene encording
BMPR-2 f receptor member of the TGF- beta
family. J. Med. Gеnet. 2000; 37: 741-5.
10. Neuman J.H., Wheelier L., Lane K.B., Loyd E.,
Gaddipat R., Phillips J. Mutation of the gene for
bone morfogenetic protein receptor-2 as a cause of
primary hypertension in a large kindred. N. Engl. J.
Med. 2001; 345: 319 S.
11. Steel P.M., Fuster V., Cohen M., Ritter D.G., Mac-
Goon D.C. Isolated atrial septal defect with pulmonary
vascular obstructive disease-long term follow
up prediction of outcome after surgical correction.
Circulation. 1987; 76: 1037-42.
12. Therrien J., Rambihar S., Newman B., Siminovitc
K., Langleben D., Webb C., Crarton J. Eisenmenger
syndrome and atrial septal defect: nature or
nurture? Can. J. Cardiol. 2006; 22 (13): 1133-6.
13. Горчакова А.И., Серов Р.А., Горбачевский С.В.
Морфологический анализ изменений легочных
сосудов в биопсиях и аутопсиях у больных с дефектом
межжелудочковой перегородки и легочной
гипертензией. Бюллетень НЦССХ
им. А.Н. Бакулева РАМН. 2014; 15 (2): 17-26.
14. Горчакова А.И., Серов Р.А., Горбачевский С.В.,
Хугаев ГА. Сочетание идиопатической легочной
гипертензии с дефектом межпредсердной
перегородки у ребенка одного года одного месяца:
редкий случай. Детские болезни сердца и
сосудов. 2015; 2: 38-42.
15. Wagenvoort C.A., Keutel J., Mool WT., Wagenvoort
N. Longitudinal smooth muscle in pulmonary
arteries occurence in congenital heart disease.
Virchows Arch. (Pathol. Anat.). 1984; 404: 265-74.
About the authors
- Gorchakova Alla Ivanovna, Cand. Med. Sc., Senior Researcher;
- Serov Roman Andreevich, Dr. Med. Sc., Professor, Head of Department; orcid.org/0000-0002-7962-7273;
- Gorbachevskiy Sergey Valer'evich, Dr. Med. Sc., Professor, Head of Department, orcid.org/0000-0002-4193-3320;
- Khugaev Georgiy Аleksandrovich, Junior Researcher, orcid.org/0000-0002-7384-8040
