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To compare early pulmonary function tests (PFTs) in neonates with critical congenital heart disease (CHD) compared to a historical reference group.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 RESULTS: PFTs in 24 infants with CHD were compared to 31 historical reference infants.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.

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To compare early pulmonary function tests (PFTs) in neonates with critical congenital heart disease (CHD) compared to a historical reference group.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 Results: PFTs in 24 infants with CHD were compared to 31 historical reference infants.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.

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.

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.

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.

Current Epidemiology of Vocal Cord Dysfunction After Congenital Heart Surgery in Young Infants.

OBJECTIVES: Surgery of the aortic arch poses risk of recurrent laryngeal nerve injury due to the anatomic proximity and can manifest as vocal cord dysfunction after surgery. We assessed risk factors for vocal cord dysfunction and calculated surgical procedure associated rates in young infants after congenital heart surgery. DESIGN: Cross section analysis. SETTING: Forty-four children's hospitals reporting administrative data to Pediatric Health Information System. PARTICIPANTS: Cardiac surgical patients less than or equal to 90 days old and discharged between January 2004 and June 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Overall, 2,319 of 46,567 subjects (5%) had vocal cord dysfunction, increasing from 4% to 7% over the study period. Of those with vocal cord dysfunction, 75% had unilateral partial paralysis. Vocal cord dysfunction was significantly more common in newborn infants (74%), those with aortic arch procedures (77%) and with greater surgical complexity. Rates of vocal cord dysfunction ranged from 0.7% to 22.4% across surgical procedure groups. Vocal cord dysfunction was significantly associated with greater use of: prolonged mechanical ventilation (53% vs 40%), diaphragmatic plication (3% vs 1%), feeding tube use (32% vs 8%), surgical airways (4% vs 2%), and prolonged length of stay (44 vs 21 d). Vocal cord dysfunction testing increased significantly over the study (6-14 %), and vocal cord dysfunction diagnosis increased almost two-fold (odds ratio, 1.9; 95% CI, 1.7-2.1) comparing the last to first study quarters with the increase in vocal cord dysfunction diagnosis occurring predominately in surgeries to the aortic arch supported by cardiopulmonary bypass. However, aortic procedures without cardiopulmonary bypass and nonaortic arch procedures were common surgeries accounting for 27% and 23% of vocal cord dysfunction cases despite low overall vocal cord dysfunction rates (3.7% and 2.6%). CONCLUSIONS: Vocal cord dysfunction complicated all cardiac surgical procedures among infants including those without aortic arch involvement. Increased efforts to determine appropriate indications for prevention, screening and treatment of vocal cord dysfunction among young infants after congenital heart surgery are needed.

Right ventricular remodeling in response to volume overload in fetal sheep.

The fetal myocardium is known to be sensitive to hemodynamic load, responding to systolic overload with cellular hypertrophy, proliferation, and accelerated maturation. However, the fetal cardiac growth response to primary volume overload is unknown. We hypothesized that increased venous return would stimulate fetal cardiomyocyte proliferation and terminal differentiation, particularly in the right ventricle (RV). Vascular catheters and pulmonary artery flow probes were implanted in 16 late-gestation fetal sheep: a right carotid artery-jugular vein (AV) fistula was surgically created in nine fetuses, and sham operations were performed on seven fetuses. Instrumented fetuses were studied for 1 wk before hearts were dissected for component analysis or cardiomyocyte dispersion for cellular measurements. Within 1 day of AV fistula creation, RV output was 20% higher in experimental than sham fetuses ( P < 0.0001). Circulating atrial natriuretic peptide levels were elevated fivefold in fetuses with an AV fistula ( P < 0.002). On the terminal day, RV-to-body weight ratios were 35% higher in the AV fistula group ( P < 0.05). Both left ventricular and RV cardiomyocytes grew longer in fetuses with an AV fistula ( P < 0.02). Cell cycle activity was depressed by >50% [significant in left ventricle ( P < 0.02), but not RV ( P < 0.054)]. Rates of terminal differentiation were unchanged. Based on these studies, we speculate that atrial natriuretic peptide suppressed fetal cardiomyocyte cell cycle activity. Unlike systolic overload, fetal diastolic load appears to drive myocyte enlargement, but not cardiomyocyte proliferation or maturation. These changes could predispose to RV dysfunction later in life. NEW & NOTEWORTHY Adaptation of the fetal heart to changes in cardiac load allows the fetus to maintain adequate blood flow to its systemic and placental circulations, which is necessary for the well-being of the fetus. Addition of arterial-venous fistula flow to existing venous return increased right ventricular stroke volume and output. The fetal heart compensated by cardiomyocyte elongation without accelerated cellular maturation, while cardiomyocyte proliferation decreased. Even transient volume overload in utero alters myocardial structure and cardiomyocyte endowment.

Ross procedure for patient with Marfan syndrome.

The most prominent long-term complication after the Ross procedure is the risk of autograft dilatation, and therefore its application in patients at increased perceived risk of autograft dilatation (those with bicuspid aortic valve disease, aortic insufficiency [AI] with dilated aorta, collagen vascular diseases such as Marfan syndrome) has been discouraged. We reported a modified Ross procedure in 2005 in which the autograft was completely encased in a polyester graft before implantation to prevent further dilatation of the autograft. This case report describes follow-up of a patient with Marfan syndrome who underwent this modified Ross procedure in July 2005.

Minimizing reperfusion injuries: successful resuscitation using eCPR after cardiac arrest on a post-operative Norwood patient.

In patients with hypoplastic left heart syndrome (HLHS), the left ventricle is too small to circulate adequate oxygenated blood. If left untreated, HLHS is fatal.A 3-staged palliative procedure ultimately leading to a single ventricle physiology is the preferred management strategy for HLHS in most pediatric cardiac centers in the United States. In this report, a 1-month-old infant developed cardiac arrest 3 weeks after undergoing a Norwood procedure as an initial palliation for HLHS. After 151 minutes of cardio-pulmonary resuscitation (CPR) with intermittent, but non-sustainable return of spontaneous circulation, extracorporeal cardio-pulmonary resuscitation (eCPR) was used. Utilizing the carotid artery and internal jugular vein for cannulation, we connected our extracorporeal membrane oxygenation (ECMO) circuit to the patient. To minimize reperfusion injury, immediate cooling, arterial/venous shunting, minimal calcium, and hemodilution strategies were used. Once paCO2/pvCO2 gradients were minimized, we instituted sweep gas and gradually increased fiO2 as pH normalized. The patient was successfully weaned from ECMO and discharged, eCPR was used successfully in the resuscitation of this patient and reperfusion injuries were minimized despite prolonged CPR.

Modified Ross procedure to prevent autograft dilatation.

The most prominent, long-term complication after the Ross procedure is autograft dilatation that can present within 1 to 2 years after the Ross operation. We describe a modified Ross procedure in which the autograft is completely encased in a Dacron graft (Hemashield; Maquet Cardiovascular, Wayne, NJ) prior to implantation. We have performed 30 modified Ross procedures since October 2004. There has been no mortality, and at follow-up none of the patients showed autograft dilatation. This article describes our current technique, which we believe is consistently reproducible and may be especially applicable to adults who are at risk for autograft dilatation after the Ross procedure.

Renal complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the renal system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. Although renal dysfunction and renal failure are known risks of congenital heart surgery, accurate estimates of the incidences of these complications are limited. This lack of knowledge is in part due to the lack of uniform definitions of these postoperative complications. The purpose of this effort is to propose consensus definitions for renal complications following congenital cardiac surgery so that collection of such data can be standardized. Clinicians caring for patients with congenital heart disease will be able to use this list for databases, quality improvement initiatives, reporting of complications, and comparing strategies for treatment.

Gastrointestinal complications associated with the treatment of patients with congenital cardiac disease: consensus definitions from the Multi-Societal Database Committee for Pediatric and Congenital Heart Disease.

A complication is an event or occurrence that is associated with a disease or a healthcare intervention, is a departure from the desired course of events, and may cause, or be associated with, suboptimal outcome. A complication does not necessarily represent a breech in the standard of care that constitutes medical negligence or medical malpractice. An operative or procedural complication is any complication, regardless of cause, occurring (1) within 30 days after surgery or intervention in or out of the hospital, or (2) after 30 days during the same hospitalization subsequent to the operation or intervention. Operative and procedural complications include both intraoperative/intraprocedural complications and postoperative/postprocedural complications in this time interval. The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease has set forth a comprehensive list of complications associated with the treatment of patients with congenital cardiac disease, related to cardiac, pulmonary, renal, haematological, infectious, neurological, gastrointestinal, and endocrinal systems, as well as those related to the management of anaesthesia and perfusion, and the transplantation of thoracic organs. The objective of this manuscript is to examine the definitions of operative morbidity as they relate specifically to the gastrointestinal system. These specific definitions and terms will be used to track morbidity associated with surgical and transcatheter interventions and other forms of therapy in a common language across many separate databases. Although serious gastrointestinal complications are relatively uncommon after congenital cardiac surgery, accurate estimates of the incidences of these complications are limited, in part due to lack of standardized reporting and the absence of universal nomenclature that defines organ-specific complications. The Multi-Societal Database Committee for Pediatric and Congenital Heart Disease has prepared and defined a list of gastrointestinal complications that may be temporally associated with congenital cardiac surgery. Clinicians caring for patients with congenital cardiac disease will be able to use this list for databases, initiatives to improve quality, reporting of complications, and comparing strategies of treatment.

Retrospective analysis comparing hollow fiber and silicone membrane oxygenators for neonates on ECMO.

There is little information showing the use of microporous polypropylene hollow fiber oxygenators during extra-corporeal life support (ECLS). Recent surveys have shown increasing use of these hollow fibers amongst ECLS centers in the United States. We performed a retrospective analysis comparing the Terumo BabyRx hollow fiber oxygenator to the Medtronic 800 silicone membrane oxygenator on 14 neonatal patients on extracorporeal membrane oxygenation (ECMO). The aim of this study was to investigate the similarities and differences when comparing pressure drops, prime volumes, oxygenator endurance, and gas transfer capabilities between the two groups.

Recurrent pulmonary artery sarcoma.

Pulmonary artery sarcomas are a diagnostic and therapeutic challenge. Most patients are initially thought to have pulmonary emboli, and during embolectomy, a sarcoma is found. Given the significant morbidity and mortality of cardiac sarcomas, an aggressive strategy for resection is indicated, as this leads to benefits in disease-free and overall survival. Imaging tests and clinical signs and symptoms may assist in accurate preoperative determination of pulmonary artery sarcoma. We present an interesting case of a patient with pulmonary artery sarcoma who underwent successful re-resection, along with a brief discussion regarding preoperative imaging and the surgical resection of these tumors.