Presurgical pulmonary function tests in the first few days of life in neonates with congenital heart disease, a pilot study.

OBJECTIVE: To compare early pulmonary function tests (PFTs) in neonates with critical congenital heart disease (CHD) compared to a historical reference group. DESIGN: Infants ≥ 37 weeks gestation with critical CHD were studied within the first few days of life, prior to cardiac surgery, and compared to data from a published reference group of healthy term neonates without CHD, studied at the same institution. Passive respiratory resistance (Rrs) and compliance (Crs) were measured with the single breath occlusion technique following specific acceptance criteria. The study was powered for a 30% difference in Rrs. RESULTS: PFTs in 24 infants with CHD were compared to 31 historical reference infants. There was no difference in the Rrs between the groups. The infants with CHD had a significantly decreased Crs (1.02 ± 0.26 mL/cmH2O/kg versus 1.32 ± 0.36; (p < 0.05; mean ± SD)). CONCLUSIONS: Further prospective studies are required to quantify early PFTs in infants with CHD of different phenotypes.

Open repair of a ruptured abdominal aorta with an aortoiliac vein fistula in a 7-month-old infant and review of the literature.

Ruptured abdominal aortic aneurysms are extremely rare in the pediatric population. In this video case report, we describe the successful repair of a ruptured abdominal aortic aneurysm in a 7-month-old female infant.

Presurgical Pulmonary Function Tests in the First Few Days of Life in Neonates with Congenital Heart Disease, A Pilot Study.

Objective: To compare early pulmonary function tests (PFTs) in neonates with critical congenital heart disease (CHD) compared to a historical reference group. Design: Infants > 37 weeks gestation with critical CHD were studied within the first few days of life and prior to cardiac surgery and compared to data from a published reference group. Passive respiratory resistance (Rrs) and compliance (Crs) were measured with the single breath occlusion technique following specific acceptance criteria. The study was powered for a 30% difference in Rrs. Results: PFTs in 24 infants with CHD were compared to 31 historical reference infants. There was no difference in the Rrs between the groups. The infants with CHD had a significantly decreased Crs (1.02 ± 0.26 mL/cmH2O/kg versus 1.32 ± 0.36; (p < 0.05; mean ± SD)). Conclusions: Further prospective studies are required to quantify early PFTs in infants with CHD of different phenotypes.

Nonresuscitation Fluid Accumulation and Outcomes After Pediatric Cardiac Surgery: Single-Center Retrospective Cohort Study.

OBJECTIVES: Postoperative patients after congenital cardiac surgery are at high risk of fluid overload (FO), which is known to be associated with poor outcomes. "Fluid creep," or nonresuscitation IV fluid in excess of maintenance requirement, is recognized as a modifiable factor associated with FO in the general PICU population, but has not been studied in congenital cardiac surgery patients. Our objective was to characterize fluid administration after congenital cardiac surgery, quantify fluid creep, and the association between fluid creep, FO, and outcome. DESIGN: Retrospective, observational cohort study. SETTING: Single-center urban mixed-medical and cardiac PICU. PATIENTS: Patients admitted to the PICU after cardiac surgery between January 2010 and December 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 1,459 postoperative encounters with 1,224 unique patients. Total fluid intake was greater than maintenance requirements on 3,103 of 4,661 patient days (67%), with fluid creep present on 2,624 patient days (56%). Total nonresuscitation intake was higher in patients with FO (defined as cumulative fluid balance 10% above body weight) versus those without. Fluid creep was higher among patients with FO than those without for each of the first 5 days postoperatively. Each 10 mL/kg of fluid creep in the first 24 hours postoperatively was associated with 26% greater odds of developing FO (odds ratio [OR] 1.26; 95% CI, 1.17-1.35) and 17% greater odds of mortality (OR 1.17; 95% CI, 1.05-1.30) after adjusting for risk of mortality based on surgical procedure, age, and day 1 resuscitation volume. Increasing fluid creep in the first 24 hours postoperatively was associated with increased postoperative duration of mechanical ventilation and PICU length of stay. CONCLUSIONS: Fluid creep is present on most postoperative days for pediatric congenital cardiac surgery patients, and fluid creep is associated with higher-risk procedures. Fluid creep early in the postoperative PICU stay is associated with greater odds of FO, mortality, length of mechanical ventilation, and PICU length of stay. Fluid creep may be under-recognized in this population and thus present a modifiable target for intervention.

Aortic valve atresia and type C interrupted aortic arch with circle of Willis dependent circulation: a case of successful biventricular repair in the neonatal period.

We report a rare case of aortic valve atresia and type C interrupted aortic arch with retrograde filling of the ascending aorta via the right common carotid artery through intracranial collateralisation and a presumed intact circle of Willis, who successfully underwent a complete biventricular repair in the neonatal period.

Hospital Care for Adult Patients with Congenital Heart Diseases.

OBJECTIVE: The ideal type of hospital to care for adult congenital heart disease (ACHD) patients is not well known. Hospital competitiveness, clinical volume and market structure can influence clinical outcomes. We sought to understand how hospital competitiveness affects clinical outcomes in ACHD patients in the era prior to the Adult Congenital Heart Association accreditation program. METHODS: Patient discharges with ACHD diagnosis codes were filtered between 2006-2011 from an all-payer inpatient healthcare database. Hospital-level data was linked to market structure patient flow. A common measure of market concentration used to determine market competitiveness-the Herfindahl-Hirschman Index (HHI)-was stratified into: more competitive (HHI ≤25th percentile), moderately competitive (HHI 25th to <75th percentile), and less competitive (HHI ≥75th percentile) hospital. Any complication, home discharge and mortality were analyzed with clustered mixed effects logistic regression. The combined impact of HHI and any complication on mortality by interaction was assessed. RESULTS: A total of 67,434 patient discharges were isolated. More competitive hospitals discharged the least number of patients (N = 15,270, 22.6%) versus moderately competitive (N = 36,244, 53.7%) and less competitive (N = 15,920, 23.6%) hospitals. The adjusted odds of any complication or home discharge were not associated with hospital competitiveness strata. Compared to more competitive hospitals, mortality at moderately competitive hospitals (Adjusted Odds Ratio (AOR) 0.79, 95% CI: 0.66-0.94) and less competitive hospitals (AOR 0.79, 95% CI: 0.63-0.98) were lower (p = 0.025). Age, race, elective admission, transfer status, and payer mix were all significantly associated with adjusted odds of any complication, home discharge and mortality (p ≤ 0.05). Having any complication independently increased the adjusted odds of mortality more than 6-fold (p < 0.001), and this trend was independent of HHI strata. Failure to rescue an ACHD patient from mortality after having any complication is highest at less competitive hospitals. Sensitivity analysis which excluded the transfer status variable, showed that any complication (p = 0.047) and mortality (p = 0.01) were independently associated with HHI strata. CONCLUSIONS: Whether lower competition allow hospitals to focus more on quality of care is unknown. Hospital competitiveness and outcome seem to have an inverse trend relationship among ACHD patients. Since medical care is frequently provided away from the home area, hospital selection is an important issue for ACHD patients. Further research is needed to determine why competitiveness is linked to surgical outcomes in this population.

Racial Disparity: The Adult Congenital Heart Disease Surgery Perspective.

The influence of race and ethnicity on clinical outcomes in medicine are widely acknowledged. However, the effect of race on adult congenital heart disease (ACHD) surgery is not known. We sought to evaluate the possible association between race and outcomes following ACHD operations. Discharge records for patients who underwent ACHD surgery between 2005 and 2014, were isolated from an all-payer voluntary database in the United States. Hierarchical case-mix regression models and sensitivity analyses examined any complication, in-hospital mortality, and discharge disposition (home/non-home) by race (white-WP, black-BP, non-white non-black-NWNB). Of the 174,370 patients (WP: 80.8%, BP: 5.8%, NWNB: 13.4%), black patients were youngest to undergo surgery (WP: 57.9 ± 15.8 years, BP: 50.2 ± 16.1 years, NWNB: 51.6 ± 16.9 years, P < 0.0001), the most likely to have a comorbidity (WP: 70.3%, BP: 74.3%, NWNB: 68.6%, P < 0.0001), and most likely to have had a post-operative cardiac complication (WP: 9.4%, BP: 15.3%, NWNB: 10.9%, P < 0.0001). BP had similar odds of having any complication (AOR = 0.99, 95%CI = 0.94-1.04), while NWNB had significantly decreased odds of a major complication (AOR = 0.90, 95%CI = 0.87-0.93). BP had equivalent in-hospital mortality compared to WP (AOR = 1.03, 95%CI = 0.91-1.18), while NWNB had significantly increased odds of in-hospital mortality (AOR = 1.29, 95%CI = 1.18-1.41). Among survivors, BP were less likely to discharge home (AOR = 0.88, 95%CI = 0.82-0.94), and NWNB were more likely to discharge home than WP (AOR = 1.26, 95%CI = 1.19-1.33). Race and clinical outcomes are associated among patients undergoing surgery for ACHD. Understanding why and how these factors are impactful will help improve care for this complex population.

Patent foramen ovale in children: Unique pediatric challenges and lessons learned from adult literature.

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea-orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

Heart Rate Induced Dynamic Coronary Ischemia Secondary to Aortic Valve Insufficiency from a Diminutive Aortic Valve Leaflet: Diagnosis and Repair.

Hemodynamically significant congenital obstruction of the coronary arteries is a rare phenomenon. The potential etiology of these obstructions is variable including coronary os stenosis, occlusive fibrous webs, or a dysplastic aortic valve leaflet resulting in obstruction. In infants, diagnosis of these coronary lesions is often difficult due to the small anatomic size and limitations of imaging modalities in the setting of the underlying rapid heart rates. We present a case of an infant presenting in heart failure secondary to dynamic coronary artery insufficiency from a diminutive aortic valve leaflet and aortic sinus and variable levels of aortic valve insufficiency based on heart rate.

Short- and Mid-Term Autograft Outcomes Following the Reinforced Ross Operation.

BACKGROUND: The Ross operation for aortic valve replacement continues to be a controversial option because of concerns related to late autograft dilation and progressive neo-aortic insufficiency. In 2005, the reinforced Ross procedure was described at our institution to address this problem. We aim to analyze the short and mid-term outcomes following this procedure. METHODS: This is a retrospective study of patients who underwent the reinforced Ross operation between 2004 and 2019. A comprehensive chart review was performed. Echocardiograms were independently reviewed by an adult congenital cardiologist. The time to reintervention was evaluated with a Kaplan-Meier curve. Analysis was conducted in JMP 15.1 (SAS Inc., Cary, NC). RESULTS: Twenty-five patients underwent the reinforced Ross operation. Twenty-three patients (92%) had bicuspid aortic valve and the most common indication for surgery was a combination of aortic insufficiency and stenosis (n = 18, 72%). The mean follow-up was 6.1 ± 5.0 years. All patients were alive at the time of follow-up. Six patients (24%), from early in our experience, required subsequent aortic reintervention. Median time to reintervention was 41.8 months (0-81.5 months). Sixteen (64%) patients had less than moderate aortic insufficiency at last follow-up. Additionally, average aortic root measurements remained unchanged. CONCLUSIONS: The reinforced Ross technique was initially proposed as a way to mitigate aortic root dilation seen in the traditional Ross procedure. Our experience suggests an associated learning curve with the majority of aortic reinterventions occurring within the first few years following surgery. Continued follow-up is warranted to assess its long-term durability and functionality.

Incidence and Recovery of Vocal Fold Immobility Following Pediatric Cardiac Operations.

BACKGROUND: Loss of laryngeal function after congenital cardiac surgery causes morbidity and prolongs hospitalization. Early diagnosis of vocal fold immobility (VFI) and referral to pediatric otolaryngology (pOTO) aids in laryngeal rehabilitation. Understanding the incidence and recovery rates of VFI enables counseling for families of infants undergoing high-risk surgery. METHODS: A retrospective chart review from November 2014 to July 2019 of infants postcardiac surgery where the aortic arch or surrounding structures were manipulated and were screened via flexible fiberoptic laryngoscopy (FFL) at a single institution was performed. Patients were divided into five surgical categories: Norwood procedure, aortic arch augmentation via median sternotomy, arterial switch operation, coarctation repair via lateral thoracotomy, and cardiac surgeries including ligation of a patent ductus arteriosus (PDA). Patients undergoing isolated PDA ligation were excluded. RESULTS: One hundred ninety-nine qualifying operations occurred during this period; 28 patients did not undergo FFL before discharge and were excluded from the analysis. Immediately following cardiac surgery, 34% (58 of 171 patients) had VFI. Follow-up was completed by 38 of 58 patients with VFI. Complete recovery was demonstrated in 63% (24 of 38) of patients by 6 months and in 86% (33 of 38) within 18 months. The highest risk occurred with the Norwood procedure and arch augmentation via median sternotomy. CONCLUSIONS: Infants undergoing surgery involving the aortic arch and surrounding structures have high rates of VFI. Follow-up by pOTO is recommended to optimize laryngeal rehabilitation. Most patients have spontaneous recovery within 18 months of cardiac surgery.

Mediastinal Mass: An Unusual Location for a Paraganglioma.

A 50-year-old man presented to the hospital for workup of a symptomatic inguinal hernia. At presurgical workup, findings from a contrast material-enhanced CT of the chest, abdomen, and pelvis revealed a large, well-defined and enhancing middle mediastinal mass arising from the right ventricular outflow tract. The mass was ultimately deemed resectable due to preserved fat planes between the mass and other mediastinal structures and the preservation of the right and left coronary arteries. The tumor was diagnosed as a mediastinal paraganglioma at histologic assessment. Keywords: Adults, Angiography, CT-Angiography, MR-Angiography, Cardiac, Neoplasms-Primary ©RSNA, 2021.

Randomized Pilot Trial of Acute Normovolemic Hemodilution in Pediatric Cardiac Surgery Patients.

BACKGROUND: Due to the substantial improvement in survival among pediatric patients undergoing congenital heart surgery, reducing early and long-term morbidity is becoming the major focus of care. Blood transfusion is associated with worse postoperative outcomes after cardiac surgery. Acute normovolemic hemodilution (ANH) is a blood conservation strategy that aims to reduce allogenic blood transfusion during cardiac surgery. However, there are scant data regarding its efficacy for pediatric cardiac surgery patients. METHODS: We designed a single-center, controlled, randomized, pilot trial in patients between 6 and 36 months old undergoing pediatric heart surgery. Patients were equally assigned to undergo ANH prior to initiation of cardiopulmonary bypass or to be managed per usual care. The primary end point was the amount of blood product transfused perioperatively. Secondary end points were markers of morbidity: postoperative bleeding, hematocrit, inotropic agents use, intensive care unit, and hospital stay. The analysis was by intention-to-treat. Estimates of differences between groups are presented with 95% CIs. RESULTS: Twelve pediatric heart surgery patients were randomized to each group, ANH and usual care. Baseline characteristics were similar between groups. Acute normovolemic hemodilution implementation did not result in a reduction in the administration of blood product transfused (difference between ANH and usual care among patients transfused = -1.4 mL [-29.4 to 26.6], P = .92). Secondary end points were not different between groups. CONCLUSIONS: In this small trial of pediatric cardiac surgery patients, ANH as a strategy to reduce blood component therapy was safe; however, the study failed to show a reduction in perioperative transfusion or other postoperative outcomes.

Reinforced Ross operation and intermediate to long term follow up.

The Ross operation for aortic valve replacement continues to be a controversial option because of concerns related to late autograft dilation and progressive neo-aortic regurgitation. We described a technique in 2005 to address this problem, in which we place the entire autograft in a Dacron tube which makes it theoretically unlikely, if not impossible, for it to dilate-the reinforced Ross procedure. Since 2004, we have operated on 25 patients using this technique. Median length of follow-up in our cohort was 6 years, with 14 patients having 5 years or more of follow-up. Our data demonstrate the externally supported, or reinforced Ross technique using a straight graft is a safe and effective technique in older children, adolescents, and young adult patients. At intermediate follow-up, patients who underwent a reinforced Ross technique were less likely to have neoaortic root dilatation.

Prevalence and Anatomy of Retroesophageal Major Aortopulmonary Collateral Arteries.

BACKGROUND: Major aortopulmonary collateral arteries (MAPCAs) are the sole source of pulmonary blood flow in patients with pulmonary atresia and absent ductus arteriosus. The anatomy of MAPCAs can be highly variable, both in the number of MAPCAs supplying each lung and the anatomic origin and course of the MAPCAs. This study evaluated the prevalence and anatomy of retroesophageal MAPCAs in patients undergoing repair of pulmonary atresia/ventricular septal defect/MAPCAs. METHODS: This was a concurrent analysis of 68 consecutive patients (March 2013 through October 2015) undergoing a primary surgical procedure for pulmonary atresia/ventricular septal defect/MAPCAs. A detailed analysis of the MAPCA anatomy was made intraoperatively for each patient, including the total number of MAPCAs to each lung and the presence or absence of a retroesophageal course. These data were correlated with the preoperative cardiac catheterization images. RESULTS: A retroesophageal MAPCA was identified during the operation in 45 of the 68 patients (67%), all of which were located on the side opposite the arch. For the 36 patients with a left aortic arch, 77% had a retroesophageal MAPCA compared with 53% of patients with a right arch. Forty-six percent of retroesophageal MAPCAs coursed within the muscular fibers of the esophagus (intraesophageal) and were more common to the left lung than the right (72% vs 32%). A midsegment stenosis was present in 84% of the retroesophageal MAPCAs, and this was more severe when the MAPCAs were intraesophageal than when they were not (80% vs 42%). CONCLUSIONS: These data demonstrate that two-thirds of patients had a retroesophageal MAPCA and that there were significant differences in prevalence and anatomy depending on the side of the aortic arch. These data provide important insights into the origin and course of retroesophageal MAPCAs.

Surgical Quality Predicts Length of Stay in Patients with Congenital Heart Disease.

Historically, the primary marker of quality for congenital cardiac surgery has been postoperative mortality. The purpose of this study was to determine whether additional markers (10 surgical metrics) independently predict length of stay (LOS), thereby providing specific targets for quality improvement. Ten metrics (unplanned ECMO, unplanned cardiac catheterization, revision of primary repair, delayed closure, mediastinitis, reexploration for bleeding, complete heart block, vocal cord paralysis, diaphragm paralysis, and change in preoperative diagnosis) were defined in 2008 and subsequently collected from 1024 consecutive index congenital cardiac cases, yielding 990 cases. Four patient characteristics and 22 case characteristics were used for risk adjustment. Univariate and multivariable analyses were used to determine independent associations between each metric and postoperative LOS. Increased LOS was independently associated with revision of the primary repair (p = 0.014), postoperative complete heart block requiring a permanent pacemaker (p = 0.001), diaphragm paralysis requiring plication (p < 0.001), and unplanned postoperative cardiac catheterization (p < 0.001). Compared with patients without each metric, LOS was 1.6 (95 % CI 1.1-2.2, p = 0.014), 1.7 (95 % CI 1.2-2.3, p = 0.001), 1.8 (95 % CI 1.4-2.3, p < 0.001), and 2.0 (95 % CI 1.7-2.4, p < 0.001) times as long, respectively. These effects equated to an additional 4.5-7.8 days in hospital, depending on the metric. The other 6 metrics were not independently associated with increased LOS. The quality of surgery during repair of congenital heart disease affects outcomes. Reducing the incidence of these 4 specific surgical metrics may significantly decrease LOS in this population.

Heart Failure in Adult Congenital Heart Disease: Nonpharmacologic Treatment Strategies.

In early stages, heart failure (HF) in adult congenital heart disease (ACHD) remains an elusive diagnosis. Many ACHD patients seem well-compensated owing to chronic physical and psychological adaptations. HF biomarkers and cardiopulmonary exercise tests are often markedly abnormal, although patients report stable health and good quality of life. Treatment differs from acquired HF. Evidence for effective drug therapy in ACHD-related HF is lacking. Residual ventricular, valvular, and vascular abnormalities contribute to HF pathophysiology, leading to an emphasis on nonpharmacologic treatment strategies. This article reviews emerging perspectives on nonpharmacologic treatment strategies, including catheter-based interventions, surgical correction, and palliative care.

High flow velocity through congenital cardiac lesions predicts preoperative platelet dysfunction.

BACKGROUND: Platelet dysfunction resulting from abnormal fluid shear stress has been reported in adults with aortic stenosis. Blood flowing through a congenital heart defect at greater than normal velocity is subjected to increased shear stress. The primary aim was to determine whether peak flow velocity through congenital cardiac lesions predicts preoperative platelet dysfunction. METHODS: The charts of 402 patients who underwent cardiopulmonary bypass and had preoperative platelet function analysis were evaluated. Platelet dysfunction was measured as a prolonged closure time (CT) in seconds with a platelet function analyzer. Echocardiography was used to determine peak velocity. The relationship between peak velocity and CT was analyzed using linear regression and Kaplan-Meier estimation. RESULTS: The distribution of peak velocity was bimodal. The mean velocity of the lower group was 1.9 m/second and the higher group was 4.2 m/second. Univariate analysis showed age, weight, peak velocity, hematocrit, and Risk Adjustment for Congenital Heart Surgery score to be associated with prolonged CT. Using multivariable analysis, prolonged CT was significantly associated with peak velocity (p < 0.001). For each 1m/second increase in peak velocity the CT increased by over 9 seconds (p < 0.001). In addition, a median CT increase of more than 6 seconds was also associated with a 5 percentage point drop in hematocrit (p = 0.04). CONCLUSIONS: Platelet dysfunction is associated with high blood flow velocity through congenital cardiac lesions. Lower preoperative hematocrit was associated with prolonged CT, which may suggest subclinical bleeding secondary to platelet dysfunction.

A defined management strategy improves early outcomes after the Fontan procedure: the PORTLAND protocol.

BACKGROUND: Patients undergoing the Fontan procedure may have extended hospital stay due to various postoperative factors including prolonged chest tube drainage. Our aim was to determine the efficacy of our Fontan management protocol in reducing chest tube drainage and length of stay. METHODS: Patients who underwent a Fontan procedure at our institution from June 2008 to September 2013 were analyzed (n = 42). We currently manage our patients according to the PORTLAND protocol: Peripheral vasodilation, Oxygen, Restriction of fluids, Technique of surgery, Low-fat diet, Anticoagulation (including antithrombin III management), No ventilator, and Diuretics. Group A (n = 28) had surgery prior to initiation of this protocol; group B (n = 14) had surgery during the current protocol era. RESULTS: The median number of chest tube days was lower in group B (6 vs 11 days, p < 0.001) as was the total indexed drainage (126 vs 259 mL/kg, p < 0.001). Patients in group B had shorter intensive care unit length of stay (4 vs 7 days, p = 0.004) and hospital length of stay (8 vs 13 days, p = 0.001). Group B had higher preoperative common atrial pressures (7.0 vs 5.8 mm Hg, p = 0.017), end-diastolic pressures (9 vs 7 mm Hg, p = 0.026), and trended toward higher pulmonary artery pressures (11.5 vs 9.5 mm Hg, p = 0.077). There was no statistically significant difference in age, weight, transpulmonary gradient, or pulmonary vascular resistance between groups. CONCLUSIONS: The PORTLAND protocol has improved early outcomes after the Fontan procedure. Chest tube drainage and duration, and both intensive care unit and hospital length of stay have been reduced since initiation of this protocol.