Measured Oxygen Consumption During Pediatric Cardiac Catheterization is More Accurate than Assumed Oxygen Consumption.

When calculating cardiac index (C.I.) by the Fick method, oxygen consumption (VO2) is often unknown, so assumed values are typically used. This practice introduces a known source of inaccuracy into the calculation. Using a measured VO2 (mVO2) from the CARESCAPE E-sCAiOVX module provides an alternative that may improve accuracy of C.I. calculations. Our aim is to validate this measurement in a general pediatric catheterization population and compare its accuracy with assumed VO2 (aVO2). mVO2 was recorded for all patients undergoing cardiac catheterization with general anesthesia and controlled ventilation during the study period. mVO2 was compared to the reference VO2 (refVO2) determined by the reverse Fick method using cardiac MRI (cMRI) or thermodilution (TD) as a reference standard for measurement of C.I. when available. 193 VO2 measurements were obtained, including 71 with a corresponding cMRI or TD measure of cardiac index for validation. mVO2 demonstrated satisfactory concordance and correlation with the TD- or cMRI-derived refVO2 (ρc = 0.73, r2 = 0.63) with a mean bias of - 3.2% (SD ± 17.3%). Assumed VO2 demonstrated much weaker concordance and correlation with refVO2 (ρc = 0.28, r2 = 0.31) with a mean bias of + 27.5% (SD ± 30.0%). Subgroup analysis of patients < 36 months of age demonstrated that error in mVO2 was not significantly different from that observed in older patients. Many previously reported prediction models for assuming VO2 performed poorly in this younger age range. Measured oxygen consumption using the E-sCAiOVX module is significantly more accurate than assumed VO2 when compared to TD- or cMRI-derived VO2 in a pediatric catheterization lab.

Diagnosis and management of the adult patient with a failing Fontan circulation.

Since the original description, the Fontan operation has been widely used for the palliation of children with single ventricle physiology. Although the Fontan operation revolutionized the survival rates of patients with single ventricle physiology, it carries an inevitable risk for long-term morbidity and mortality that impacts clinical outcomes and quality of life. This review will focus on the evaluation and treatment of the patient with the failing Fontan phenotype, with an emphasis on creating an individualized treatment plan.

Cumulative medical radiation exposure throughout staged palliation of single ventricle congenital heart disease.

Patients with single ventricle heart disease undergoing staged palliation are exposed to ionizing radiation from multiple medical sources. We sought to quantify typical radiation burden in this population and identify risk factors for increased exposure. A retrospective review was performed of single ventricle patients surgically palliated at our institution, in which all studies utilizing ionizing radiation occurring from birth through Fontan completion were compiled. Thirty-eight patients were followed for a median of 33 months. A median of 59 radiation events occurred during follow-up, with a median cumulative effective dose of 25.7 milliSieverts (mSv). On average, cardiac catheterization accounted for 4% of radiation encounters but comprised 78% of total radiation exposure. In a multivariate model, factors associated with increased total radiation exposure included pulmonary artery (PA) stenosis requiring intervention (p = 0.005) and systemic right ventricle (p = 0.02). Risk factors for increased exposure from catheterization included heterotaxy syndrome (p = 0.007), re-coarctation (p = 0.003), and PA stenosis (p = 0.02). At our institution, many single ventricle patients are exposed to substantial radiation throughout staged palliation, most of which derives from cardiac catheterization. PA stenosis was identified as a risk factor for increased total and catheterization-based exposure. As patient survival improves, awareness of this scale of radiation exposure at a vulnerable period is imperative.

Spontaneous rupture of a coronary artery fistula presenting with post-exertional syncope and haemopericardium.

An 8-year-old with a known diagnosis of an asymptomatic coronary artery fistula (CAF) presented with exertional syncope and haemopericardium due to contained rupture of CAF. A transcatheter closure was attempted, but the fistula ruptured again prompting emergency surgical repair. Spontaneous rupture is a catastrophic complication of CAF and warrants a high index of suspicion, timely diagnosis and team management.

Pulmonary histopathology in an experimental model of chronic aspiration is independent of acidity.

Gastroesophageal reflux has become a major health concern in industrialized countries, with drugs aimed at blocking acid production being more frequently prescribed than any other drug. Damage to lung tissue as a result of chronic aspiration of gastric fluid is a primary health risk associated with gastro-esophageal reflux, with such aspiration being suspected in the induction or exacerbation of asthma and other lung diseases. In this study, a rodent model of chronic aspiration was used to characterize the pulmonary histopathology produced by repetitive aspiration events and to investigate the pathologic roles of individual gastric fluid components such as acid and particulate food matter. Rats exposed to chronic aspiration of whole gastric fluid developed a pathology distinct from that of acute lung injury, characterized by granulomatous interstitial pneumonitis with prominent formation of multinucleated giant cells. This pattern of injury could be reproduced with chronic aspiration of particulate food matter and with chronic aspiration of pH-neutralized gastric fluid, but not with chronic aspiration of hydrochloric acid. Thus, since acid-neutralizing therapy is currently the mainstay of treatment for patients with reflux-associated respiratory symptoms, these results strongly suggest that alternative therapeutic approaches aimed at preventing chronic-aspiration induced lung injury may be warranted.

Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit). Median length of follow-up was 5.3 years (interquartile range 1.4 to 11.2), and 30% had follow-up >10 years. Freedom from a composite medical morbidity outcome (protein-losing enteropathy, plastic bronchitis, serious thromboembolic event, or tachyarrhythmia) was 47% at 20 years (95% confidence interval [CI] 38 to 55). Independent risk factors for morbidity included pre-Fontan atrioventricular valve regurgitation (hazard ratio [HR] 1.7, 95% CI 1.2 to 2.4, p = 0.001), pleural drainage >14 days (HR 1.5, 95% CI 1.01 to 2.2, p = 0.04), and longer cross-clamp time (HR 1.2 per 10 minutes, 95% CI 1.06 to 1.3, p = 0.004) at the time of TCPC. Surgical era, Fontan type, and ventricular morphology were not associated with the composite outcome. Presence of Fontan-associated morbidity was associated with a 36-fold increase in the risk of subsequent Fontan takedown, heart transplantation, or death (95% CI 17 to 76, p <0.001). For patients without any component of the composite outcome, freedom from Fontan failure was 98% at 20 years (95% CI 96 to 99). Medical morbidities after TCPC are common and significantly reduce the longevity of the Fontan circulation. However, for those patients who remain free from the composite morbidity outcome, 20-year survival with intact Fontan circulation is encouraging.

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9.8 years. Freedom from reoperation was 69% at 15 years and 63% at 20 years. The most common operations were pacemaker placement and Fontan revision. Risk factors for pacemaker placement included systemic left ventricle (hazard ratio [HR], 2.2; P=0.006) and lateral tunnel Fontan (HR, 4.3; P=0.001). Freedom from interventional catheterization was 53% at 15 years and 50% at 20 years. The most common procedures performed were fenestration closure and pulmonary artery intervention. Catheter intervention for anatomic indications was associated with Fontan after 2002 (HR, 2.1; P=0.007), Norwood operation (HR, 2.3; P=0.001), and longer cardiopulmonary bypass time (HR, 1.1 per 10 minutes; P=0.001). Catheter intervention for physiological indications was associated with prolonged post-Fontan pleural drainage (HR, 4.0; P<0.001) and hypoplastic left heart syndrome (HR, 2.0; P=0.01). CONCLUSIONS: In this study of Fontan survivors, two thirds of patients required surgical or catheter-based reintervention by 20 years. Families should be counseled that the Fontan is typically not the final stage of single-ventricle palliation.

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

OBJECTIVE: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort. METHODS: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013. RESULTS: Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001). CONCLUSIONS: Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.

Tetralogy of Fallot: General Principles of Management.

Repaired tetralogy of Fallot (TOF) is one of the most common diagnoses encountered when caring for adults with congenital heart disease. Although long-term survival after childhood TOF repair is excellent, morbidity is common and most patients require reintervention in adulthood. This review provides an overview of key surveillance and management issues for adults with TOF, including residual right ventricular outflow tract disease and timing of pulmonary valve replacement, arrhythmias and risk stratification, left-sided heart disease and heart failure, and pregnancy management.

Left-sided scimitar vein causing cyanosis after Fontan operation: successful transcatheter device occlusion using magnetic resonance imaging X-ray fusion.

We present the case of a 3-year-old boy with asplenia-type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left-sided scimitar vein. This pattern of pulmonary venous return has not been previously reported in a patient with asplenia. MRI x-ray fusion was used to guide transcatheter device occlusion of the scimitar vein, resulting in marked clinical improvement.

Cumulative exposure to medical sources of ionizing radiation in the first year after pediatric heart transplantation.

BACKGROUND: Pediatric heart transplant recipients undergo a variety of radiologic tests with the attendant risk of exposure to ionizing radiation. We sought to quantify and describe the cumulative exposure to all forms of medical radiation during the first year after pediatric heart transplantation and identify factors associated with higher exposure. METHODS: Pediatric patients who received a heart transplant between January 2009 and May 2012 with follow-up at our institution were retrospectively reviewed. Patients were included if they survived through 1 year and the first coronary angiography. All medical testing using ionizing radiation performed during follow-up was compiled, and exposures were converted to effective dose (mSv). RESULTS: Included were 31 patients who underwent heart transplantation at a median age of 13.6 years (range, 0.3-18.3 years). The median number of radiologic tests performed was 38 (range, 18-154), including 8 catheterizations (range, 2-12), and 28 X-ray images (range, 11-135). Median cumulative effective dose was 53.5 mSv (range, 10.6-153.5 mSv), of which 91% (range, 34%-98%) derived from catheterizations, 31% (range, 8%-89%) of the exposure occurred during the transplant admission, 59% (range, 11%-88%) during planned follow-up, and 3% (0%-56%) during unplanned follow-up. Older age at transplant was a risk factor for increased exposure (p = 0.006). When adjusted for age, a trend toward increased exposure was shown for congenital heart disease as the indication for transplant (p = 0.08), pre-sensitization (p = 0.12), and positive crossmatch (p = 0.09). CONCLUSIONS: Pediatric heart transplant patients are exposed to significant amounts of ionizing radiation during the first post-transplant year, most during scheduled catheterization. As survival improves, considering the long-term risks associated with these levels of exposure is important.

Accuracy of conventional oximetry for flow estimation in patients with superior cavopulmonary connection: a comparison with phase-contrast cardiac MRI.

BACKGROUND: Cardiac catheterization is routinely used as a diagnostic tool in single ventricle patients with superior cavopulmonary connection. This physiology presents inherent challenges in applying the Fick principle to estimate flow. We sought to quantitatively define the error in oximetry-derived flow parameters using phase-contrast cardiac MRI (CMR) as a reference. METHODS AND RESULTS: Thirty patients with superior cavopulmonary connection who underwent combined CMR and catheterization between July 2008 and June 2012 were retrospectively analyzed. Estimates of flow and resistance calculated using the Fick equation were compared with CMR measurements. Oximetry underestimated CMR-measured pulmonary blood flow (Qp) by an average of 1.1 L/min per m(2) or 32% of the CMR value (P<0.0001). Oximetry overestimated systemic blood flow (Qs) by an average of 0.5 L/min per m(2) or 15% of the CMR value (P=0.009). There was no correlation between the Qp:Qs ratio derived by Fick and that measured by CMR (ρ(c)=0.01). The error in the Fick Qp correlated moderately with the measured systemic-to-pulmonary arterial collateral flow (r=0.39). The median total oxygen consumption calculated using combined CMR and oximetry data was 173 mL/min per m(2), higher than the assumed values used to calculate flows by the Fick equation. The upper body circulation received on average 51% of systemic blood flow while conducting only 39% of total body metabolism. CONCLUSIONS: Fick-derived estimates of flow are inherently unreliable in patients with superior cavopulmonary connections. Integrating flows measured by CMR and pressures measured by catheter will provide the best characterization of superior cavopulmonary connection physiology.