<p>Multimodality treatment with catheter-based intervention is still commonly used for pulmonary atresia with ventricular septal defect (PA/VSD) or hemi-truncus with major aortopulmonary artery collateral arteries (MAPCAs) requiring unifocalization. A total of 12 patients with PA/VSD with MAPCAs (<i>n</i> = 10) or hemi-truncus (<i>n</i> = 2) who had undergone unifocalization since 1994 were enrolled. The development of the central pulmonary artery (cPA) was absent in 2 patients, diminutive (&lt; 2&#xa0;mm) in 3, sizable (&gt; 2&#xa0;mm) in 5, and unilateral (hemi-truncus) in 2. Treatment strategy was determined according to the morphology of the central PA. VSD closure was not performed at the same time of unifocalization. The source of pulmonary blood flow from completion of unifocalization to VSD closure was either systemic to pulmonary artery shunt (SPS) or right ventricle-to-pulmonary artery (RV-PA) conduit. A total of 39 catheterizations were performed. Median follow-up was 13.2 years [interquartile range: 2.3–16.5 years]. All patients achieved VSD closure. Cumulative survival rate was 83.3% at 10 years. Median RV to aortic pressure (AoP) ratio during VSD closure was 0.57 [0.51–0.75]. After a patient with partial anomalous pulmonary venous connection and pulmonary hypertension was eliminated RV/AoP was lower in RV-PA conduit (<i>n</i> = 5) than in SPS cases (<i>n</i> = 6) (0.49 vs. 0.76, <i>p</i> = 0.014). RV-PA conduit as a source of pulmonary blood flow facilitates blood flow control and effective catheter-based intervention to reconstructed PA after unifocalization then contributed maintaining low RV pressure at VSD closure.</p>

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Efficacy of Right Ventricle-to-Pulmonary Artery Conduit as a Pulmonary Blood Source at Unifocalization of Major Aortopulmonary Collateral Arteries

  • Erika Yuasa,
  • Takaya Hoashi,
  • Takuro Kojima,
  • Akinori Hirano,
  • Ryusuke Hosoda,
  • Yuji Fuchigami,
  • Yukino Iijima,
  • Takaaki Suzuki

摘要

Multimodality treatment with catheter-based intervention is still commonly used for pulmonary atresia with ventricular septal defect (PA/VSD) or hemi-truncus with major aortopulmonary artery collateral arteries (MAPCAs) requiring unifocalization. A total of 12 patients with PA/VSD with MAPCAs (n = 10) or hemi-truncus (n = 2) who had undergone unifocalization since 1994 were enrolled. The development of the central pulmonary artery (cPA) was absent in 2 patients, diminutive (< 2 mm) in 3, sizable (> 2 mm) in 5, and unilateral (hemi-truncus) in 2. Treatment strategy was determined according to the morphology of the central PA. VSD closure was not performed at the same time of unifocalization. The source of pulmonary blood flow from completion of unifocalization to VSD closure was either systemic to pulmonary artery shunt (SPS) or right ventricle-to-pulmonary artery (RV-PA) conduit. A total of 39 catheterizations were performed. Median follow-up was 13.2 years [interquartile range: 2.3–16.5 years]. All patients achieved VSD closure. Cumulative survival rate was 83.3% at 10 years. Median RV to aortic pressure (AoP) ratio during VSD closure was 0.57 [0.51–0.75]. After a patient with partial anomalous pulmonary venous connection and pulmonary hypertension was eliminated RV/AoP was lower in RV-PA conduit (n = 5) than in SPS cases (n = 6) (0.49 vs. 0.76, p = 0.014). RV-PA conduit as a source of pulmonary blood flow facilitates blood flow control and effective catheter-based intervention to reconstructed PA after unifocalization then contributed maintaining low RV pressure at VSD closure.