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Medline ® Abstract for Reference 67

of 'Pulmonary atresia with intact ventricular septum (PA/IVS)'

67
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Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty.
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Schwartz MC, Glatz AC, Dori Y, Rome JJ, Gillespie MJ
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Pediatr Cardiol. 2014 Jan;35(1):22-9. Epub 2013 Jun 19.
 
Radiofrequency perforation and valvuloplasty (RFV) is an effective initial treatment in patients with pulmonary atresia and intact ventricular septum (PA-IVS) and mild to moderate right ventricle and tricuspid valve hypoplasia. Outcomes and risk factors for the need for additional interventions in these patients are poorly defined. All patients with PA-IVS who underwent RFV at our center between January 2000 and July 2011 were reviewed. Twenty-three patients met the inclusion criteria. All patients underwent successful valvuloplasty with no procedural deaths and one major complication. Excluding two patients with limited follow-up, 6 (29 %) patients underwent no subsequent interventions, whereas 9 (42 %) patients underwent surgical right-ventricular outflow tract augmentation. All except one patient with adequate follow-up have a biventricular circulation with saturation>92 %. Patients who did not undergo any right-ventricular outflow tract intervention after valvuloplasty had a significantly lower gradient across the pulmonary valve after valvuloplasty (9.9 mmHg ± 8.4 vs. 19.1 mmHg ± 10.4, p = 0.05). Significantly more patients who received a supplemental source of pulmonary blood flow had a tricuspid valve z-score<-0.7 compared with patients who did not receive supplemental blood flow [2 (15 %) vs. 7 (70 %), p = 0.008]. In our cohort of patients with PA-IVS, radiofrequency perforation with valvuloplasty was an effective and safe first step in establishing a biventricular circulation. Postvalvuloplasty pulmonary valve gradient may be predictive of subsequent outflow tract intervention, and tricuspid hypoplasia may be predictive of the need for a supplemental source of pulmonary blood flow.
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Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA, matthew.schwartz@orlandohealth.com.
PMID