Abstract
Experience of palliative surgeries for cyanotic congenital heart defects with unilateral pulmonary artery lack is presented.
Palliative surgeries were performed in 29 patients with unilateral pulmonary artery lack between 1983 and 2011 at
the A.N. Bakulev SCCVS RAMS. All the patients lacked left pulmonary artery and had congenital heart defects with
inadequate blood flow: Fallot's tetrad (n=26), right ventricle origin of aorta and pulmonary artery with pulmonary
artery stenosis (n=3). Mean age of patients was 2.6 years (between 40 days and 37 years). Nine (31%) patients
needed palliative reoperations.
Spectrum of 42 palliative surgeries performed in 29 patients included: systemic pulmonary anastomoses (n=13),
right ventricle outflow tracts reconstructions without ventricular septal defect closure (n=19) and roentgen surgical
procedures (n=10).
Hospital mortality following palliative surgeries was 6/9% (2/29). There was no hospital mortality following systemic
pulmonary anastomoses.
Hospital mortality following 19 palliative reconstructive surgeries of the right ventricle outflow tract in 17 patients
was 11.8% (2/17). Complications following palliative reconstructive surgeries of the right ventricle outflow tract
were in 6 patients out of 15 survivals. Right ventricle outflow tract diameter gauged on the operating table upon
completion of the palliative reconstruction was 10.7+2.3 mm, pulmonary artery systolic pressure was 32.6+8.8
mm. Hg, normal median Z-score of the pulmonary valve diameter was -1.5 (-2.9;-1.1).
Angiometric values of the pulmonary artery central portions following systemic pulmonary anastomoses and right
ventricle outflow tracts reconstructions indicated more significant and steady increase of all the segments of the
pulmonary artery following reconstructions (pulmonary artery segments increase was 57.2+5.1%) then after systemic
pulmonary anastomoses (pulmonary artery segments increase was 32.4+9.5%).
Radical correction of concomitant congenital heart defects following palliative surgeries was performed in 17
(62.9%) out of 27 survivals including 55.5% (10/18) cases after one palliative procedure and 77.8% (7/9) after multistage
palliative treatment.
Palliative surgeries for unilateral pulmonary artery lack were accompanied with hospital mortality rate 6.9% and
in 69% cases they were limited to only one surgical intervention. Creation of anastomosis between systemic artery
and single main pulmonary artery was associated with minimal risk.
Right ventricle outflow tract palliative reconstruction is preferable for patients with marked «hypoplasia» of the single
pulmonary artery.
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