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Objective: Stenting of ostial pulmonary artery stenosis presents several unique challenges. These include difficulty in defining anatomy and need for precise stent placement in order to avoid missing the ostial stenosis or jailing either the contralateral branch pulmonary artery or the ipsilateral upper lobe branch., Design: A retrospective review of outcomes was conducted in 1.5 or 2-ventricle patients who underwent stent placement for ostial branch pulmonary artery stenosis. Specific catheterisation lab techniques were reviewed., Results: Forty-seven branch pulmonary arteries underwent stent placement for ostial stenosis in 43 patients. The median age and weight were 3.7 (0.3-18.1) years and 14.2 (5.6-70.0) kg, respectively. Three (2-8) angiographic projections were needed to profile the ostial stenosis. Open-cell stents were used in 23 and stents were modified in 5 cases. Following stent implantation, the minimum diameter improved from 3.6 (0.8-10.5) to 8.1 (4.2-16.5) mm (p < 0.001). The gradient improved from 21 (0-66) to 4 (0-27) mmHg (p < 0.001). Stent malposition occurred in eight (17%) of the stents placed. Five migrated distally causing suboptimal ostial coverage necessitating placement of a second stent in four. Three migrated proximally and partially jailed the contralateral pulmonary artery. Intentional jailing of the upper lobe branch occurred in four additional cases. At a follow-up of 2.4 (0.3-4.9) years, 15 stents underwent further dilation and 1 had a second stent placed within the exiting stent., Conclusion: Ostial branch pulmonary artery stenosis may require additional angiography to accurately define the ostial stenosis. Treatment with stents is effective but carries high rates of stent malposition. more...

We describe a case of anomalous left coronary artery from the pulmonary artery in association with total anomalous pulmonary venous return. The infant was diagnosed with total anomalous pulmonary venous return at 6 weeks of age and underwent successful surgical repair. On routine follow-up, he was found to have an anomalous left coronary artery from the pulmonary artery without evidence of mitral regurgitation or left ventricular dysfunction. The presence of the left-to-right shunt and secondary elevation in pulmonary artery pressures likely masked the usual findings associated with this coronary anomaly. more...

Pulmonary artery wedge pressure (PAWp) as a surrogate for left atrial pressure (LAp) has been validated in patients with fully pulsatile two-ventricle circulations. This study was to validate PAWp as an accurate estimate of LAp for calculating pulmonary vascular resistance in single-ventricle patients after the bidirectional Glenn operation. A prospective study measuring direct branch pulmonary artery pressures, ipsilateral PAWp, and direct LAp to calculate pulmonary and systemic blood flows was conducted. Pulmonary vascular resistance (PVR) was calculated for each patient using the PAWp and compared to the PVR calculated by direct LAp measurements. Twenty-eight patients were enrolled; 27 were eligible for study inclusion. Ages ranged from 21 to 60 months and weights ranged from 9.5 to 20.8 kg. Underlying congenital heart defect diagnoses varied, with all patients either functionally or anatomically univentricular. Left mPAWp measured 4.4 ± 2.6, statistically different from simultaneous direct mLAp of 2.5 ± 2.4 mmHg (P < 0.001. Right mPAWp measured 4.3 ± 3.2 mmHg, statistically different from simultaneous direct mLAp of 2.8 ± 2.6 mmHg (P < 0.001. The PVR calculated using mPAWp was 1.2 ± 0.7 W.U., statistically different to 1.8 ± 0.95 W.U. using mLAp (P < 0.001). The average PVR measurement calculated using mPAWp underestimated the PVR calculated using mLAp by 0.6 W.U. It can be extrapolated that a PVR of 2.3 W.U. calculated using wedge measurements would estimate a PVR of 3 W.U. calculated using direct LA measurements. In this study, the PVR calculated using PAWp as a surrogate for LAp either equaled or underestimated the true PVR, up to 33% different. This difference is important in patients with univentricular physiology who rely on low PVR for cardiac output. Thus, based on this study, a PVR measurement of greater than 2.3 W.U. by the mPAWp method should prompt a direct LA pressure measurement, in order to more appropriately determine true surgical risk. more...

Objectives: The Norwood procedure with right ventricle-pulmonary artery conduit is thought to improve postoperative hemodynamics in hypoplastic left heart syndrome, but its effects on pulmonary artery growth are unknown. This study evaluated pulmonary artery growth after the Norwood procedure in patients with a right ventricle-pulmonary artery conduit as compared with patients with a modified Blalock-Taussig shunt., Methods: A total of 159 patients at our institution underwent the Norwood procedure between January 2000 and September 2005. Patients were divided into group A or B if they had a modified Blalock-Taussig shunt (n = 103) or a right ventricle-pulmonary artery conduit (n = 56). Angiograms from the pre-Glenn catheterizations were used to measure pulmonary artery size and assess shunt stenosis (n = 64)., Results: Fifty-five (53.4%) patients in group A versus 40 (71.4%) in group B underwent Glenn surgery. Group B patients often required an additional shunt (modified Blalock-Taussig) before the Glenn procedure because of hypoxemia (8/40 vs 1/55; P = .004). Branch pulmonary artery growth was better in group B patients who did not require an additional shunt (Nakata index 212 vs 169 mm(2)/m(2); P = .004) and more balanced than in group A (right pulmonary artery/left pulmonary artery ratio = 1.02 vs 1.39; P = .001) as a result of greater left pulmonary artery size (29 vs 19 mm(2); P = .001). However, group B experienced more shunt stenosis (8/32 vs 2/32; P = .001), underwent the Glenn operation earlier (192 vs 246 days; P = .03), and had central pulmonary artery hypoplasia develop more often than group A patients (25/32 vs 14/32; P = .01)., Conclusion: The Norwood procedure with a right ventricle-pulmonary artery conduit promotes better distal left pulmonary artery growth resulting in more balanced branch pulmonary artery size, but central pulmonary artery hypoplasia occurs more often. Early right ventricle-pulmonary artery conduit stenosis also increases the need for additional shunting or early Glenn surgery. more...