Down Syndrome as a Factor Influencing Hemodynamic Response to Pulmonary Artery Banding

Abstract

The hemodynamic response to pulmonary artery banding (PAB) in relation to the preoperative pulmonary/systemic vascular resistance (Rp/Rs) ratio and to the timing of surgery, with special regard to Down syndrome, was investigated in 56 nonconsecutive pediatric patients aged 3 days to 6 months (mean 2.5 months) with simple and complex congenital shunt-related cardiac malformations. Among the non-Down patient group (39 patients; mean age 6.9 weeks) there was a good hemodynamic response in all but three cases, irrespective of the preoperative Rp/Rs ratio; these three poor responders had preoperatively normal or nearly normal Rp/Rs ratios (Rp/Rs < 0.3) and were affected postoperatively by lung complications. In the Down patient group (17 patients; mean age 8.2 weeks) the mean preoperative as well as the mean postoperative Rp/Rs ratio was higher than in the non-Down patient group (preoperative Rp/Rs 0.49 versus 0.32; postoperative Rp/Rs 0.31 versus 0.18). There was a good hemodynamic response in all five patients with Down syndrome who had preoperative normal or nearly normal pulmonary vascular resistance ratios (Rp/Rs < 0.3). Among 12 patients with Down syndrome and preoperative increased resistance ratios (Rp/Rs > 0.3) PAB did not cause a reduction in pulmonary vascular resistance (PVR) in five patients (postoperative Rp/Rs 0.49-1.00), all operated on at more than 6 weeks of age. PAB resulted in effective reduction of postoperative Rp/Rs ratios (range 0.10-0.27) in seven patients, six of them younger and one older than 6 weeks at the time of the banding procedure. In conclusion, patients with Down syndrome and shunt-related cardiac malformations (predominantly total atrioventricular canal cases) in general have higher pre- and postoperative Rp/Rs ratios than non-Down children and also have a higher potential for developing pulmonary vascular obstructive disease despite hemodynamically effective PAB. Especially in children with Down syndrome and pathologically high resistance ratios, PAB, if indicated, should be performed as early as possible.

Individuals with down syndrome exhibit reduced skin thermo sensitivity response during intermittent physical exercise

“…Indeed, previous studies have shown that DS individuals may have elevated peripheral vascular resistance 7 and reduced peripheral vasodilator response. 37…”

Adult Presentation of Complete Atrioventricular Septal Defect

“…4 Late presentation in patients with trisomy 21 is also unusual as the development of elevated pulmonary vascular resistance is more severe and rapid in these patients. 5,6 One of the factors that allowed this patient to progress relatively symptom-free into adulthood may have been the protective vasoconstrictive effect of hypoxia secondary to sleep apnea. Hypoxic vasoconstriction is a defining characteristic of the pulmonary circulation, and a study has indeed found vasoconstriction in the pulmonary vasculature during obstructive sleep apnea.…”

Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

“…In the lung biopsies at PAB, the index of pulmonary vascular disease was less than 1.4 in all patients, which classified them as operable on the basis of previous studies. 17,18 Five patients (3 AVSD cases (1, 4, 6), 2 VSD cases (13,14)) had PVOD. All AVSD patients with PVOD underwent PAB when they were older than 1.5 months, whereas all VSD patients with PVOD underwent PAB when they were older than 2 months.…”

“…PAB was planned to be performed in early infancy (within 2 months after birth 13 in principle), and ICR approximately 1 year later. Most of the patients underwent lung biopsy during both PAB and ICR, but some who underwent biopsy only during PAB were also included in the study.…”