The relationship between left ventricular dilation and right ventricular diastolic function in children with a patent ductus arteriosus.

The impact of a dilated left ventricular (LV) on right ventricular (RV) diastolic function has not been investigated. We hypothesized that in patients with a patent ductus arteriosus (PDA), LV dilation causes elevation of the RV end-diastolic pressure (RVEDP) through ventricular-ventricular interaction. We identified patients' ages 6 months to 18 years who underwent transcatheter PDA closure at our center from 2010 to 2019. One hundred and thirteen patients were included with a median age of 3 years (0.5-18). The median LV end-diastolic dimension (LVEDD) Z-score was 1.6 (-1.4-6.3). RVEDP was positively associated with RV systolic pressure (0.38, P < 0.01), ratio of pulmonary artery/aortic systolic pressure (0.4, P < 0.01), and pulmonary capillary wedge pressure (0.71, P < 0.01). RVEDP was not associated with LVEDD Z-score (0.03, P = 0.74). In children with a PDA, RVEDP was not associated with LV dilation, but was positively associated with RV systolic pressure.

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.

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.

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.

How to perform combined cutting balloon and high pressure balloon valvuloplasty for dogs with subaortic stenosis.

Subvalvular aortic stenosis (SAS) is one of the most common congenital cardiac malformations in dogs. Unfortunately, the long term success rate and survival data following either open heart surgery or catheter based intervention has been disappointing in dogs with severe subaortic stenosis. Medical therapy is currently the only standard recommended treatment option. A cutting balloon dilation catheter has been used successfully for resistant coronary artery and peripheral pulmonary arterial stenoses in humans. This catheter is unique in that it has the ability to cut, or score, the stenotic region prior to balloon dilatation of the stenosis. The use of cutting balloon valvuloplasty combined with high pressure valvuloplasty for dogs with severe subaortic stenosis has recently been reported to be a safe and feasible alternative therapeutic option. The following report describes this technique, outlines the materials required, and provides some 'tips' for successful percutaneous subaortic balloon valvuloplasty.

Utility of store-and-forward pediatric telecardiology evaluation in distinguishing normal from pathologic pediatric heart sounds.

Because pediatric cardiologists can accurately diagnose innocent murmurs by physical exam alone, the authors developed a system for remote cardiac auscultation. They hypothesized that their system could accurately classify auscultatory findings as normal/innocent or pathologic. Patients undergoing evaluation underwent examination, echocardiography, and heart sound recording. Pediatric cardiologists evaluated the heart sounds and classified the case as either normal/innocent or pathologic. They reviewed103 heart sound data sets; 85% of the cases were accurately classified as either normal/innocent or pathologic, with a sensitivity of 82% and specificity of 86%. However, when accounting for clinical diagnosis, reviewer uncertainty, and ECG abnormalities, the sensitivity and specificity improved to 91% and 88% (accuracy 89%), respectively. Degree of certainty with the telecardiology diagnosis correlated with correct interpretation (P < .005). Digital heart sound recordings evaluated via telemedicine can distinguish normal/innocent murmurs from pathologic ones. Such a system could improve the use of pediatric cardiology services.