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High inspired oxygen concentration (FiO 2  > 0.85) is administered to test pulmonary vascular reactivity in children with pulmonary hypertension (PH). It is difficult to measure oxygen consumption (VO 2 ) if the subject is breathing a hyperoxic gas mixture so the assumption is made that baseline VO 2 does not change. We hypothesized that hyperoxia changes VO 2 . We sought to compare the VO 2 measured by a thermodilution catheter in room air and hyperoxia. A retrospective review of the hemodynamic data obtained in children with PH who underwent cardiac catheterization was conducted between 2009 and 2014. Cardiac index (CI) was measured by a thermodilution catheter in room air and hyperoxia. VO 2 was calculated using the equation CI = VO 2 /arterial-venous oxygen content difference. Data were available in 24 subjects (males = 10), with median age 8.3 years (0.8-17.6 years), weight 23.3 kg (7.5-95 kg), and body surface area 0.9 m 2 (0.4-2.0 m 2 ). In hyperoxia compared with room air, we measured decreased VO 2 (154 ± 38 to 136 ± 34 ml/min/m 2 , p = 0.007), heart rate (91 [Formula: see text] 20 to 83 [Formula: see text] 21 beats/minute, p=0.005), mean pulmonary artery pressure (41 [Formula: see text] 16 to 35 [Formula: see text] 14 mmHg, p=0.024), CI (3.6 [Formula: see text] 0.8 to 3.3 [Formula: see text] 0.9 L/min/m 2 , p = 0.03), pulmonary vascular resistance (9 [Formula: see text] 6 to 7 [Formula: see text] 3 WU m 2 , p = 0.029), increased mean aortic (61 [Formula: see text] 11 to 67 [Formula: see text] 11 mmHg, p = 0.005), pulmonary artery wedge pressures (11 [Formula: see text] 8 to 13 [Formula: see text] 9 mmHg, p = 0.006), and systemic vascular resistance (12 [Formula: see text] 6 to 20 [Formula: see text] 7 WU m 2 , p=0.001). Hyperoxia decreased VO 2 and CI and caused pulmonary vasodilation and systemic vasoconstriction in children with PH. The assumption that VO 2 remains unchanged in hyperoxia may be incorrect and, if the Fick equation is used, may lead to an overestimation of pulmonary blood flow and underestimation of PVRI.

Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardiac catheterization. A single-center, retrospective study of 65 infants and children with PH was performed. Pediatric Functional Class (FC) at diagnosis, at last visit, and change in FC over time were evaluated for their association with mortality and PH-associated morbidity in univariate, time-to-event, and multivariate regression analyses. Median age at PH diagnosis was 5.3 months (0 days-12.7 years). Twenty-five children (38 %) had idiopathic PH or PH secondary to congenital heart disease, one (2 %) had left heart disease, and 39 (60 %) had PH secondary to respiratory disease. Mortality was 25 % (16/63), primarily in the first year of follow-up. FC at diagnosis was not significantly associated with survival (p = 0.22), but higher FC (more impaired) at last visit (p < 0.001) and change in FC over time (HR 2.3, 95 % confidence interval 1.3-4, p = 0.0003) were associated with mortality. Higher FC at last visit was associated with greater days of hospitalization in the intensive care unit per year (p = 0.006) and history of cardiac arrest (p = 0.012) and syncope (p = 0.02). Although pediatric FC at diagnosis was not predictive of mortality, response to therapy (as assessed by change in FC over time and FC at last visit) was associated with morbidity and mortality in this heterogeneous cohort. Multicenter prospective studies are necessary to further validate these findings.

The risks associated with cardiac catheterization in children with pulmonary hypertension (PH) are increased compared with adults. We reviewed retrospectively all clinical data in children with PH [mean pulmonary artery pressure (mean PAp) ≥25 mmHg and pulmonary vascular resistance index (PVRI) ≥3 Wood units m(2)] undergoing cardiac catheterization between 2009 and 2014. Our strategy included a team approach, minimal catheter manipulation and sildenafil administration prior to extubation. Adverse events occurring within 48 h were noted. Seventy-five patients (36 males), median age 4 years (0.3-17) and median weight 14.6 kg (2.6-77 kg), underwent 97 cardiac catheterizations. Diagnoses included idiopathic or heritable pulmonary arterial hypertension (PAH) (29 %), PAH associated with congenital heart disease (52 %), left heart disease (5 %) and lung disease (14 %). Mean PAp was 43 ± 19 mmHg; mean PVRI was 9.7 ± 6 Wood units m(2). There were no deaths or serious arrhythmias. No patient required cardiac massage. Three patients who suffered adverse events had suprasystemic PAp (3/3), heritable PAH (2/3), decreased right ventricular function (3/3), and pulmonary artery capacitance index <1 ml/mmHg/m(2) (3/3) and were treatment naïve (3/3). No patient undergoing follow-up cardiac catheterization suffered a complication. In 45 % of cases, the data acquired from the follow-up cardiac catheterization resulted in an alteration of therapy. Three percent of children with PH undergoing cardiac catheterization suffered adverse events. However, there were no intra or post procedural deaths and no one required cardiac massage or cardioversion. Follow-up cardiac catheterization in patients receiving pulmonary hypertensive targeted therapy is safe and provides useful information.

Accurate measurement of oxygen consumption (VO2) is important to precise calculation of blood flow using the Fick equation. This study aimed to validate the breath-by-breath method (BBBM) of measuring oxygen consumption VO2 compared with respiratory mass spectroscopy (MS) for intubated children during cardiac catheterization. The study used MS and BBBM to measure VO2 continuously and simultaneously for 10 min in consecutive anesthetized children undergoing cardiac catheterization who were intubated with a cuffed endotracheal tube, ventilated mechanically, and hemodynamically stable, with normal body temperature. From 26 patients, 520 data points were obtained. The mean VO2 was 94.5 ml/min (95 % confidence interval [CI] 65.7-123.3 ml/min) as measured by MS and 91.4 ml/min (95 % CI 64.9-117.9 ml/min) as measured by BBBM. The mean difference in VO2 measurements between MS and BBBM (3.1 ml/min; 95 % CI -1.7 to +7.9 ml/min) was not significant (p = 0.19). The MS and BBBM VO2 measurements were highly correlated (R (2) = 0.98; P < 0.0001). Bland-Altman analysis showed good correspondence between MS and BBBM, with a mean difference of -3.01 and 95 % limits of agreement ranging from -26.2 to +20.0. The mean VO2 indexed to body surface area did not differ significantly between MS and BBBM (3.4 ml/min m(2); 95 % CI -1.4 to 8.2; p = 0.162). The mean difference and limits of agreement were -3.8 ml/min m(2) (range, -19.9 to 26.7). Both MS and BBBM may be used to measure VO2 in anesthetized intubated children undergoing cardiac catheterization. The two methods demonstrated excellent agreement. However, BBBM may be more suited to clinical use with children.

Background: Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN)., Objective: We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN., Results: Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen., Conclusions: We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.

Pulmonary arteriovenous malformations (PAVMs) are a common source of morbidity after bidirectional superior cavopulmonary anastomosis (Glenn). The diversion of hepatic venous effluent away from the pulmonary circulation after Glenn appears to play a significant role in the pathogenesis of PAVMs. Although the liver is known to produce factors that regulate vascular development, specific hepatic inhibitors of angiogenesis have not been described in the post-Glenn population. Endostatin, produced from its precursor collagen XVIII, is a potent inhibitor of angiogenesis produced by the liver. This study aimed to investigate the hypothesis that endostatin levels decrease in patients after Glenn. Levels of endostatin and its precursor, long-type collagen XVIII, were determined by enzyme-linked immunoassay and immunoprecipitation, respectively, for serum samples from 38 patients undergoing Glenn, total cavopulmonary anastomosis (Fontan), or biventricular repair of cardiac defects. Samples were obtained before surgery and 24 h afterward. In patients undergoing a bidirectional Glenn procedure, endostatin levels decreased after surgery (n = 17; 4.42 vs 3.34 ng/ml; p < 0.001), and long type-collagen XVIII levels increased by 200 % (n = 10; p = 0.0001). However, endostatin levels did not change after surgery in patients undergoing Fontan (n = 13) or biventricular repair (n = 8). In patients undergoing Fontan, long-type collagen XVIII increased by 18 % (p < 0.01), whereas in control subjects, the levels were unchanged. These data suggest that the diversion of hepatic blood flow away from the pulmonary circulation in patients after the Glenn procedure inhibits endostatin production from collagen XVIII, resulting in decreased circulating serum endostatin levels. A decrease in endostatin may promote angiogenesis. The mechanism whereby the pulmonary circulation processes endostatin and its potential role in the pathogenesis of PAVMs warrant further study.

A neonate with pulmonary interstitial glycogenosis, pulmonary hypertension, and hypertrophic cardiomyopathy is described. The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis. To the authors' knowledge, the association of pulmonary interstitial glycogenosis and hypertrophic cardiomyopathy has not been reported previously. The authors have broadened the phenotype of pulmonary interstitial glycogenosis and demonstrate the diagnostic value of lung biopsy in cases of unexplained neonatal pulmonary hypertension.

Objective: To evaluate whether a trial of inhaled nitric oxide (NO) differentiates reversible pulmonary vasoconstriction from fixed anatomic obstruction to pulmonary blood flow after surgery for congenital heart disease in patients at risk for pulmonary hypertension., Design: Prospective cohort study., Setting: Tertiary care children's hospital., Patients: 15 neonate and infants with elevated pulmonary artery or right ventricular pressure or with clinical signs suggestive of high pulmonary vascular resistance in the early postoperative period following repair of congenital heart disease., Intervention: 30-min trial of 40 ppm inhaled NO., Results: 5 patients responded to inhaled NO, 2 patients were weaned from extracorporeal support with NO. Four were maintained on continuous inhaled NO for 3 to 5 days. All the responders survived. Ten patients did not respond to NO. An important anatomic obstruction was found with echocardiography or angiography in all 10 patients. Reintervention was performed in 6/10 (4 stent placement, 1 balloon angioplasty of pulmonary arteries and 1 revision of systemic to pulmonary shunt). Six of the nonresponders died., Conclusion: A trial of inhaled NO after cardiac surgery in neonates and infants may be useful to differentiate reversible pulmonary vasoconstriction from fixed anatomic obstruction and may provide useful information if temporary support with extracorporeal membrane oxygenation is considered. Failure to respond to inhaled NO should prompt further investigations to rule out a residual obstruction.

This report illustrates the rare occurrence of a pulmonary embolus in a neonate during cardiac catheterization. The patient was a term newborn who underwent repair of obstructed infradiaphragmatic total anomalous pulmonary venous connection. Postoperative risk factors for pulmonary embolism included severe pulmonary hypertension unresponsive to nitric oxide therapy, an indwelling venous catheter, and young age. Successful management was achieved by initial mechanical fragmentation with streptokinase infusion and monitoring by serial lung perfusion scans.