Third-Grade Academic Performance and Episodes of Cardiac Care Among Children with Congenital Heart Defects.

Children with congenital heart defects (CHDs) are at risk for poor academic performance. The degree to which receipt of health care services is associated with adverse academic outcomes is not known. We examined the association between episodes of cardiac care and third-grade performance in children with CHD. We identified subjects between 1/1/2008 and 4/30/2012 among 5 centers in North Carolina. We classified children by CHD type and linked subjects to the state educational records. Any inpatient or outpatient cardiac encounter on a date of service was considered an encounter. We calculated the number of encounters by adding the number of inpatient or outpatient cardiac visits prior to the date of the end-of-grade (EOG) tests. We estimated the odds of failing third-grade reading or math EOG tests by episodes of care stratified at the 50th percentile, controlling for CHD type, maternal education, sex, race/ethnicity, birth weight, and gestational age. A total of 184 children had third-grade EOG scores linked to health care records. The median number of episodes of care was 4 (range: 1-60). Those with visits ˃ 50th percentile (> 4 encounters/year over the 4.3 year observation period) had 2.09 (95% CI 1.04, 4.21) greater odds of failing the math EOG compared to those ≤ 50th percentile (1-4 encounters). The third-grade math score declined by 1.5 points (P < 0.008) for every 10 episodes of care. There was no association of episodes of care on third-grade reading performance. Children with CHD with > 4 episodes of cardiac care/year may be at risk for delays in third-grade academic performance. Strategies to minimize school absenteeism may improve academic success in this population.

Impact of Prenatal Diagnosis of Critical Congenital Heart Disease on Preoperative and Postoperative Outcomes.

The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008 and 2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. There were 715 patients with STS records; 558 linked to the NC-CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.

Body mass index and age are associated with ventricular end-diastolic pressure in adults with a Fontan circulation.

INTRODUCTION: Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population. METHODS: All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analysed to identify variables associated with end-diastolic pressure. RESULTS: Forty-two patients were included. Median age was 24.0 years (20.9-29.0) with a body mass index of 23.7 kg/m2 (21.5-29.7). 10 (23.8%) patients had a systemic right ventricle. The median (Interquartile range) and mean pulmonary artery pressure were 11.0 mmHg (9.0-12.0) and 16.0 mmHg (13.0-18.0), respectively. On univariate analysis, end-diastolic pressure was positively associated with body mass index (p < 0.01), age > 25 years (p = 0.04), symptoms of heart failure (p < 0.01), systemic ventricular systolic pressure (p = 0.03), pulmonary artery mean pressure (p < 0.01), and taking diuretics (p < 0.01) or sildenafil (p < 0.01). End-diastolic pressure was negatively associated with aortic saturation (p < 0.01). On multivariate analysis, end-diastolic pressure was positively associated with age ≥ 25 years (p < 0.01), and body mass index (p = 0.04). CONCLUSIONS: In a cohort of adult patients with Fontan circulation undergoing catheterisation, end-diastolic pressure was positively associated with age ≥ 25 years and body mass index on multivariate analysis. Maintaining a healthy body mass index may offer haemodynamic benefit in adults with Fontan physiology.

Ductal stent endocarditis resulting in a large aortic pseudoaneurysm.

An infant with ductal dependent pulmonary blood flow who underwent neonatal ductal stenting and, 4 months later, developed ductal stent endocarditis due to Streptococcus gallolyticus subsp. pasteurianus was described. The infection was associated with a moderate aortic pseudoaneurysm and the patient was treated with antibiotics as well as surgical aortic pseudoaneurysm repair. This novel and unusual complication of ductal stent placement warrants reporting.

Causes of Death in Infants and Children with Congenital Heart Disease.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients < 20 years old were evaluated. There were 15,277 infants with an annual mortality rate of 3.5 deaths per 100 live births. The most frequent COD in infants (age < 1 year) were CHD (31.7%), lung disease (16.1%), and infection (11.4%). In 20,265 children (age 1 to < 20 years), there was annual mortality rate of 9.7 deaths per 1000 at risk. The most frequent COD in children were CHD (34.2%), neurologic disease (10.2%), and infection (9.5%). In the severe subtype, CHD was the most common COD. In infants with shunt-type CHD disease, lung disease (19.5%) was the most common COD. The mortality rate in infants was three times higher when compared to children. CHD is the most common underlying COD, but in those with shunt-type lesions, extra-cardiac COD is more common. A multidisciplinary approach in CHD patients, where development of best practice models regarding comorbid conditions such as lung disease and neurologic disease could improve outcomes in this patient population.

Identification of patient variables that are associated with ventricular end-diastolic pressure before the bidirectional Glenn operation.

INTRODUCTION: Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely identified. METHODS: All patients who underwent bidirectional Glenn operation operation at our centre between January 2007 and March 2017 were retrospectively identified and patient variables were extracted. For patients who had undergone Fontan operation at the time of this study, post-Fontan patient variables were also extracted. RESULTS: One-hundred patients were included with a median age at pre-bidirectional Glenn operation catheterisation of 4.5 months. In total, 71 (71%) patients had a systemic right ventricle. At the pre-bidirectional Glenn operation catheterisation, the mean systemic ventricular end-diastolic pressure was higher amongst those with systemic right ventricle compared to left ventricle (9.1 mmHg ± 2.1 versus 7.7 ± 2.7 mmHg, p < 0.01). On univariate analysis, pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01), history of recoarctation (p = 0.03), history of Norwood operation (p = 0.04), and ventricular systolic pressure (p < 0.01). On multivariate analysis, systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01) and ventricular systolic pressure (p < 0.01). Amongst those who had undergone Fontan operation at the time of study (n = 49), those with a higher pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure were more likely to have experienced death, transplantation, or listed for transplantation (p = 0.02) and more likely to have had heart failure symptoms (p = 0.04) at a mean time from Fontan of 5.2 years ± 1.3. CONCLUSIONS: In patients undergoing bidirectional Glenn operation operation, the volume-loaded, pre-bidirectional Glenn operation state may expose diastolic dysfunction that has prognostic value.

Here today, gone tomorrow: Outcomes of residual leak following secundum atrial septal defect closure with the GORE CARDIOFORM Septal Occluder.

OBJECTIVE: To assess the outcomes and potential predictors of residual leak after implantation of the GORE CARDIOFORM Septal Occluder (GSO) in secundum atrial septal defects (ASDs). BACKGROUND: The non-self-centering design of the GSO could lead to residual leak at the edge of the device. Outcomes of residual leak are poorly understood. METHODS: Retrospective, multicenter review of patients treated with the GSO for the pivotal and continued access study. Procedural, echocardiographic, and follow up data were reviewed. Multivariate analysis was performed to determine predictors of leak at implant and outcomes of these leaks. RESULTS: There were 370 total ASD device closures with the GSO, 65 (17.56%) of which were found to have residual leak at implantation. Patients with residual leak had larger defects (10.33 ± 3.05 mm vs. 9.13 ± 2.89 mm, p = .006) and smaller aortic rims (4.87 ± 3.33 mm vs. 6.17 ± 3.78 mm, p = .019), as compared to those without leak. Larger devices were implanted (p = .008) and more devices per case (p = .007) were utilized in the residual leak cohort as compared to those without leak. Overall, among patients with residual leak, there was a significant decrease in leak size over 1 year, from 1.55 ± 0.75 mm to 0.25 ± 0.74 mm (p < .001), with the majority (87.6%) disappearing by 12-month follow-up. CONCLUSIONS: Residual leak at implantation can be seen after ASD device closure with the GSO, more commonly in larger defects with smaller retroaortic rims. The vast majority of these leaks completely resolve by 1 year follow up.

Use of the GORE® CARDIOFORM Septal Occluder for percutaneous closure of secundum atrial septal defects: Results of the multicenter U.S. IDE trial.

OBJECTIVE: To evaluate safety and efficacy of the GORE® CARDIOFORM Septal Occluder for percutaneous transcatheter closure of ostium secundum atrial septal defects. BACKGROUND: The GORE® CARDIOFORM septal occluder is a double-disc, low profile, soft, conformable device, with distinct advantages over the GORE® HELEX® Septal Occluder. METHODS: Subjects were enrolled in this single arm prospective study from 21 U.S. sites, and followed for 3 years. Primary endpoint was 6 month composite clinical success, comprised of technical success (implantation and retention of device), closure success (normalization of right heart size), no 30 day serious adverse events, and no device embolization or reintervention. Secondary endpoints included technical success, procedure success (technical success and ≤ 2 mm residual shunt at procedure conclusion), closure success (clinically insignificant or no residual shunt), and safety (freedom from 30 day serious adverse events and 6-month device events). RESULTS: Between October 2012 and May 2015, 50 pivotal and 350 continued access subjects underwent attempted transcatheter GORE® CARDIOFORM Septal Occluder implantation. Median age was 6.9 years, and mean static defect diameter 9.7 ± 3.1 mm. Device placement was achieved in 93.5% (374/400). Composite clinical success was 90.2% and clinical closure success was 98.8% at 6 months. Freedom from serious adverse events was 98.3% at 30 days, with no device embolizations or reinterventions through 6 months. CONCLUSIONS: The GORE® CARDIOFORM Septal Occluder has high composite clinical success and safety, performing well in defects ≤17 mm by stop flow stretched diameter. Single, multifenestrated, and deficient retroaortic tissue defects were well represented and successfully treated.

ASSURED clinical study: New GORE® CARDIOFORM ASD occluder for transcatheter closure of atrial septal defect.

OBJECTIVE: Assess clinical performance of a new device for transcatheter closure of atrial septal defect (ASD). BACKGROUND: Previously-approved ASD Closure devices have known limitations. Device erosion has been associated with the AMPLATZER® septal occluder in patients with retro-aortic rim deficiency (<5 mm), while defects ≥18 mm are too large for the GORE® CARDIOFORM septal occluder. The GORE® CARDIOFORM ASD occluder (GCA), a hybrid of the approved devices, was designed to expand the eligible ASD population. METHODS: One-hundred and twenty-five ASD patients were enrolled in a prospective, multicenter registry. Descriptive clinical endpoints included: technical implant success, closure success, serious adverse events (SAE), clinically significant new arrhythmia, and wire frame fracture. Procedural outcomes and adverse events were adjudicated by an Echocardiography Core Lab and Independent Data Review Board, respectively. RESULTS: Median subject age was 12.3 years (range 2.9-84.7), with 72% of patients ≤18 years old. Median ASD stop-flow diameter was 17.0 mm (8.0-30.0), with 43% ≥18 mm. Deficient retro-aortic rim occurred in 57% of subjects, and 30% had both diameter ≥ 18 mm and deficient rim. Technical Implant Success was achieved in 120/125 (96%), though three devices were removed within 24 hr. At the scheduled 6-month evaluation, 112/117 returned for evaluation. All 112 had closure success. SAE occurred in 6/125 (4.8%) and 6/125 (4.8%) had clinically significant new arrhythmia. Wire frame fractures occurred in 37/104 (36%), without any associated clinical sequelae, residual shunt, or device instability. CONCLUSION: The GCA performed effectively and safely in this initial cohort, and led to FDA approval based on this data.

Renal thromboembolism from a large pulmonary artery to a pulmonary vein fistula in an asymptomatic adolescent.

Pulmonary arteriovenous fistula is a rare vascular anomaly that can cause significant morbidity and mortality. The presence and significance of symptoms are dependent on the size of the right-to-left shunt. Thromboembolic events may result in cerebrovascular accidents or systemic vascular occlusions. We present a case of an adolescent without cardiorespiratory symptoms, who developed flank pain due to renal infarction, followed by a brief literature review.