Abstract
Objective. To present outcomes of diagnosis and experience of surgical treatment of patients with malposition of pulmonary arteries in the presence of congenital heart diseases (CHD) with pulmonary artery stenosis.
Material and methods. From 1992 to 2013 in A.N. Bakoulev Scientific Center for Cardiovascular Surgery of Russian Academy of Medical Sciences 10 cases were diagnosed as having malposition of pulmonary arteries in the presence of CHD with reduced pulmonary blood flow. All 10 patients have had concomitant ventricular septal defect (VSD) and stenosis of PA.
The total of 6 patients underwent palliative operations, while three of them underwent 2 or 3 palliative interventions. Median age of
patients in this group made up 2.4 years. Three patients underwent radical repair of concomitant CHD after palliative interventions.
Mean interval between palliative and radical operations made up 1.7 years. Seven patients underwent radical repair of concomitant CHD. Median age of 7 patients at the time of performance of radical repair made up 3.7 years.
Results. Malposition of pulmonary arteries in the total of 10 patients was diagnosed by Doppler echocardiographic and angiocardio graphic examination. Malposition with crisscross PA was diagnosed in 7 patients, malposition without crisscross PA was diagnosedin 3 patients. There was no hospital mortality after performance of palliative interventions and radical repair. Cardiopulmonary insufficiency having developed in postoperative period requested the administration of large doses of inotropic agents and prolongedmechanical ventilation of lungs, i.e. in 2 patients after palliative operations and in 5 patients after radical repair.
Right ventricular outflow tract diameter measured on the operation table after finishing of repair of right ventricular outflow tract without closure of VSD in 3 patients made up 9.0 ± 1.0 mm (from 8 to 10 mm), Z-score from diameter of PA valve in the norm made up-2.7; -1.5 and -1.9 respectively, systolic pressure in PA after operation made up 29.0 ± 1.7 mm Hg with the systolic pressure in theaorta 96.7 ± 20.2 mm Hg.
Before radical repair the Nakata index made up 280 ± 37 mm2/ m2, median Z-score of normal value of the Nakata index – –1.4. The
systolic pressure in right ventricle made up 45.0 ± 14.6 mm Hg and the ratio of systolic pressure in ventricles after radical repair made up 0.50 ± 0.18.
Conclusion. Recent methods of diagnosis allows in patient’s lifetime to diagnose the malposition of pulmonary arteries. The presence of pathology should not change the treatment strategy and repair of intracardiac defects has involved minimal risk.
References
1. Подзолков В.П., Иваницкий А.В., Махачев О.А., Алекян Б.Г., Митина И.Н., Сафонова Н. И. Врожденный порок сердца, сочетающий-
ся с criss-cross легочными артериями. Грудная и сердечно-сосудистая хирургия. 1995; 5: 74–7.
2. Хириев Т.Х. Перекрещивающиеся (сriss-сross) легочные артерии. Детские болезни сердца и сосудов. 2012; 3: 15–23.
3. Jue K.L., Lockman L.A., Edwards J.E. Anomalous origins of pulmonary arteries from pulmonary trunk («crossed pulmonary arteries»):
observation in a case with 18 trisomy syndrome. Am. Heart J. 1966; 71 (6): 807–12.
4. Xiong Y., Gan H.J., Liu T. et al. Prenatal diagnosis of crossed pulmonary arteries. Ultrasound Obstet. Gynecol. 2010; 36 (6): 776–7.
5. Bockeria L.A., Makhachev O.A., Khiriev T.Kh. Crossed pulmonary arteries with malposition of the great arteries: an accidental anatomic
situation or a new syndrome? 62st IC European Society of Cardiovascular and Endovascular Surgery. 2013, April 11–13; Regensburg,
Germany. 54 (2, suppl. 1): P. 87.
6. Bockeria L.A., Makhachev O.A., Khiriev T.Kh., Podzolkov V.P. Malposition of the pulmonary arteries in congenital heart defects with pulmonary
stenosis: diagnosis and surgical treatment results: 62st IC European Society of Cardiovascular and Endovascular Surgery. 2013,
April 11–13; Regensburg, Germany. 54 (2, suppl. 1): 89.
7. Chaturvedi R., Mikailian H., Freedom R.M. Crossed pulmonary arteries in tetralogy of Fallot. Cardiol. Young. 2005; 15: 537.
8. Ho S.Y., Wilcox B.R., Anderson R.H., Lincoln J.C.R. Interrupted aortic arch – anatomical features of surgical significance. Thorac.
Cardiovasc. Surg. 1983; 31: 199–205.
9. Siwik E.S., Everman D., Morrison S. Crossed pulmonary arteries, ventricular septal defect, and chromosome 22q11 deletion. Heart. 2002;
88: 88.
10. Butto F., Lucas R.V., Edwards J.E. Persistent truncus arteriosus: pathologic anatomy in 54 cases. Pediatr. Cardiol. 1986; 7 (2): 95–101.
11. Collett R.W., Edwards Y.E. Persistent truncus arteriosus. A classification according to anatomic subtypes. Surg. Chin. North Am. 1949; 29:
1245.
12. Wells T.R., Takahashi M., Landing B.H. et al. Branching patterns of right pulmonary artery in cardiovascular anomalies. Pediatr. Pathol.
1993; 13 (2): 213–23.
13. Бокерия Л.А., Махачев О.М., Панова М.С., Филиппкина Т.Ю. Нормативные параметры сердца и его структур. М.: НЦССХ
им. А.Н. Бакулева РАМН. 2010.
14. Van Meurs-Van Woezik H., Debets T., Klein H.W. Growth of the internal diameters in the pulmonary arterial tree in infants and children.
J. Anat. 1987; 151: 107–15.
15. Becker A.E., Becker M.J., Edwards J.E. Malposition of pulmonary arteries (crossed pulmonary arteries) in persistent truncus arteriosus.
Am. J. Roentgenol. Radium. Ther. Nucl. Med. 1970; 110 (3): 509–14.
16. Van Praagh R., Van Praagh S. The anatomy of common aortic-pulmonary trunk (Truncus Arteriosus communis) and its embryonic implications.
A study of 57 necropsy cases. Am. J. Cardiol. 1965; 16: 406–25.
17. Recto M.R., Parness I.A., Gelb B.D. et al. Clinical implications and possible association of malposition of the branch pulmonary arteries with
DiGeorge syndrome and microdeletion of chromosomal region 22q11. Am. J. Cardiology. 1997; 80: 1624–7.
18. Wolf W.J., Casta A., Nichols M. Anomalous origin and malposition of the pulmonary arteries (crisscross pulmonary arteries) associated
with complex congenital heart disease. Pediatr. Cardiol. 1986; 6: 287–91.
19. Koca B., OztunC F., YalCKn Y. Crossed pulmonary arteries in conjunction with tetralogy of Fallot. TЯrk. Kardiyol. Derneg¤i Ars, ivi. 2011; 39
(6). 499–500.
20. Shunmugasundaram P., Bijual S., Titus T., Tharakan J.A. Crisscross pulmonary artery with double aortic arch: an unusual association.
Pediatr. Cardiol. 2012; 33 (5): 857–8.
21. Zimmerman F.J., Berdusis K., Wright K.L., Alboliras E.T. Echocardiographic diagnosis of anomalous origins of the pulmonary arteries from
the pulmonary trunk (crossed pulmonary arteries). Am. Heart J. 1997; 133: 257–62.
22. Babaoglu K., Binnetoglu F.K., Altun G. et al. Echocardiographic and three-dimensional computed tomographic diagnosis of crossed pulmonary
arteries: report of three cases. Pediatr. Cardiol. 2010; 31 (5): 720–2.
23. Chen B.B., Hsieh H.J., Chiu I.S. et al. Crossed pulmonary arteries: report of two cases with emphasis on tree-dimensional helical computed
tomographic imaging. J. Formos. Med. Assoc. 2008; 107 (3): 265–9.
24. Miyahara Y., Kataoka K., Kawada M. Crossed pulmonary arteries associated with interruption of aortic arch on three-dimensional computed
tomographic imaging. Ann. Thorac. Surg. 2011; 91: 929.
25. Sivakumar K., Prasad R., Francis E. Crossed pulmonary arteries. Cardiol. Young. 2007; 17: 572–3.
