Percutaneous Pulmonary Flow Restriction in Infants With Congenital Heart Disease.

Background: Restriction of PBF in infants born with CHD is often required to avoid pulmonary over-circulation prior to definitive intervention. The current standard is to surgically place pulmonary artery bands, but these have limitations and are associated with complications. Objectives: The purpose of this study was to a single-center experience with a relatively novel technique to percutaneously restrict pulmonary blood flow (PBF) in select infants with congenital heart disease (CHD). Methods: Patients were selected to undergo this procedure either due to low birth weight or prematurity. All of them had CHD that would result in over-circulation without control of PBF. By a percutaneous method, modified vascular plug devices were placed in the bilateral branch pulmonary arteries. Results: Seven neonates with CHD resulting in left-sided obstruction underwent this procedure. All patients demonstrated evidence of restricted PBF with a decrease in mean oxygen saturation from 95% to 84%. One patient required pulmonary artery band placement due to over-circulation 5 days after the procedure. All patients proceeded to full surgical intervention without device embolization or need for pulmonary arterioplasty. Hemodynamics demonstrated adequate limitation of PBF in 5 patients who underwent presurgical cardiac catheterization with a mean pulmonary vascular resistance of 1.52 WU × m2 and a mean transpulmonary gradient of 5.9 mm Hg. Conclusions: Percutaneous PBF restriction appears to be safe and a less invasive option to delay surgical intervention in a select population to allow for somatic growth and gestational maturation. It results in a decrease in the total number of sternotomies.

Aortic Arch Reconstruction Using Nonvalved Femoral Vein Homograft in High-Risk Neonates.

Aortic arch obstruction is often present with complex concomitant congenital heart defects (CHDs). The use of nonvalved femoral vein homograft (FVH) to reconstruct the aortic arch has distinct surgical advantages, including simplified reconstruction. We present an intraoperative video of a Yasui procedure utilizing FVH for aortic reconstruction in a 12-day-old (2.2 kg) neonate with right ventricular outflow tract obstruction, malalignment ventricular septal defect, aortic valve atresia, aortic arch hypoplasia, atrial septal defect, and ductal dependent systemic circulation. Further, we report outcomes for a series of three additional neonatal patients with complex CHD and aortic arch obstruction who underwent FVH arch reconstruction.

Report of the 2022 Society of Thoracic Surgeons Congenital Heart Surgery Practice Survey.

BACKGROUND: The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. METHODS: An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. RESULTS: Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being "just right," 74 (42%) reported that their case volume was "too small." Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. CONCLUSIONS: Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

Post-operative troponin levels and left ventricular function in patients with d-transposition of the great arteries following the arterial switch operation.

The aim of this study was to assess the significance of post-operative troponin levels as a surrogate for left ventricular (LV) dysfunction measured by global longitudinal strain (GLS) in patients with dextro-transposition of the great arteries (d-TGA) who undergo an arterial switch operation (ASO), and to explore the LV GLS recovery in the mid-term follow-up period. Seventy-eight neonates were included, of whom 41 had troponin-I measurements and 37 had troponin-T measurements. The primary outcome of LV GLS was assessed and compared with healthy controls at the pre-operative stage and time of discharge, 3 months, 6 months and 12 months of age. Secondary outcomes included deaths or transplantations and other clinical markers such as length of hospital stay. D-TGA patients had worse LV GLS post-operatively compared to age-matched controls (p < 0.01) which improved by 12 months of age (p = 0.53). No association was found between changes in troponin-I or troponin-T levels and LV GLS at the time of discharge (r = 0.4, p = 0.64 and r = -0.5, p = 0.91, respectively). In addition, there were no deaths or transplantations in this cohort over a period of 12 months. LV GLS appears to worsen in the early post-operative period for d-TGA patients who undergo neonatal ASO but this recovers through the first post-operative year. Troponin levels have limited value in predicting early or midterm LV dysfunction and recovery.

Isolated aortic arch anomalies are associated with defect severity and outcome in patients with congenital diaphragmatic hernia.

PURPOSE: Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients. METHODS: Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality. RESULTS: Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χ2 p < 0.001). CDH + IAAA were less likely to undergo diaphragm repair (72 vs. 87%, p < 0.001), and overall mortality was higher for CDH + IAAA infants (58 vs. 24%, p < 0.001). When controlling for defect size, birth weight, and Apgar, IAAA were significantly associated with mortality (OR 3.3, 95% CI 2.2-5.0; p < 0.01) but not associated with ECLS (OR 0.98, 95% CI 0.65-1.50; p = 0.90). Only 17% (n = 28) of CDH + IAAA patients underwent aortic intervention. CONCLUSIONS: IAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

Impact on clinical outcomes from transcatheter closure of the Fontan fenestration: A systematic review and meta-analysis.

Background: Meta-analysis of the impact on clinical outcome from transcatheter closure of Fontan fenestration. Methods: Cochrane, Embase, MEDLINE, and Open-Gray were searched. Parameters such as changes in oxygen saturation, cavo-pulmonary pressure, maximum heart rate during exercise, exercise duration, and oxygen saturation after fenestration closure were pooled and statistical analysis performed. Results: Among 922 publications, 12 retrospective observational studies were included. The included studies involved 610 patients, of which 552 patients (90.5%) had a fenestration. Of those patients, 505 patients (91.5%) underwent attempt at trans-catheter closure. When it could be estimated, the pooled overall mean age at trans-catheter fenestration closure was 6.6 ± 7.4 years, and the mean follow-up time was 34.4 ± 10.7 months. There were 32 minor (6.3%) and 20 major (4.0%) complications during or after trans-catheter Fontan fenestration closure. The forest plots demonstrate that following fenestration closure, there was a significant increase in the mean arterial oxygen saturation of 7.9% (95% CI 6.4-9.4%, p < 0.01). There was also a significant increase in the mean cavo-pulmonary pressure of 1.4 mmHg (95% CI 1.0-1.8 mmHg, p < 0.01) following fenestration closure. The exercise parameters reported in 3 studies also favored closing the fenestration as well, yet the exercise duration increase of 1.7 min (95% CI 0.7-2.8 min, p < 0.01) after fenestration closure is probably clinically insignificant. Conclusion: Late closure of a Fontan fenestration has the impact of improving resting oxygen saturation, exercise oxygen saturation, and a modest improvement of exercise duration. These clinical benefits, however, may be at the expense of tolerating slightly higher cavo-pulmonary mean pressures.

Virtual Simulated Implantation of an Adult-Sized Left Ventricular Assist Device in a Pediatric Patient.

There is very limited experience with simulated virtual implantation of left ventricular assist devices (LVADs) to assess device fitness in pediatric patients. In this clinical vignette, we report the case of a 9-year-old male patient with dilated cardiomyopathy who underwent successful placement of an LVAD after virtual simulated implantation was performed. (Level of Difficulty: Advanced.).

Children who stroke on VAD support: When is it safe to transplant and what are their outcomes?

OBJECTIVE: Ventricular assist devices (VADs) increase waitlist survival, yet the risk of stroke remains notable. The purpose of this study was to analyze how strokes on VAD support impact post-transplant (post-Tx) outcomes in children. METHODS: About 520 pediatric (<18 years) heart transplant candidates listed from January 2011 to April 2018 with a VAD implant date were matched between the United Network of Organ Sharing and Pediatric Health Information System databases. Patients were divided into pre-Tx Stroke and No Stroke cohorts. RESULTS: About 81% of the 520 patients were transplanted; 28% (n = 146) had a pre-Tx Stroke; and 59% (n = 89) of the Stroke patients were transplanted at a median of 57 (IQR 17-102) days from stroke. Significantly more No Stroke cohort (90%) were transplanted (p < 0.001). There was no difference in post-Tx survival between the Stroke and No Stroke cohorts (p = 0.440). Time between stroke and transplant for patients who died within 1 year of transplant was 32.0 days (median) compared to 60.5 days for those alive >1 year (p = 0.18). Regarding patients in whom time from stroke to transplant was more than 60 days, one-year survival of Stroke vs. No Stroke patients was 96% vs. 95% (p = 0.811), respectively. CONCLUSION: Patients with stroke during VAD support, once transplanted, enjoy similar survival compared to No Stroke patients. We hypothesize that allowing Stroke patients more time to recover could improve post-Tx outcomes. Unfortunately, the ideal duration of time between stroke and safe transplantation could not be determined and will require more detailed and larger studies in the future.

Peri-operative changes in diastolic function and outcomes in congenital aortic valve surgery.

BACKGROUND: The ratio of early diastolic mitral inflow velocity (E) to early diastolic mitral annular tissue velocity (e'), or E/e', is an echocardiographic measure of left ventricular filling pressure. Peri-operative changes in E/e' and association with outcomes have been demonstrated in adults undergoing surgery for aortic stenosis (AS). We sought to explore changes in E/e' and other diastolic indices in the setting of congenital AS surgery and to assess for association with post-operative outcomes among children and young adults. METHODS: A retrospective, single-center study was performed among patients 6 months to 30 years of age who underwent congenital AS surgery from 2006 to 2018. Tissue Doppler indices were collected from pre- and post-operative echocardiograms. Post-operative outcomes were reviewed. RESULTS: Sixty-six subjects with subvalvar (45%), valvar (47%), and supravalvar (8%) AS underwent surgery at a median age of 9.5 years (IQR: 4.0-14.8). Pre-operatively, the lateral E/e' ratio was 8.6 (6.7-11.0); 33% had E/e'≥10. Post-operatively, the lateral e' decreased to 9.9 cm/s (8.0-11.4), the E/e' ratio increased to 10.4 (8.3-13.1); and 53% had E/e'≥10 (p-values < 0.0001, 0.0072, and < 0.001, respectively). Pre-operative lateral e' correlated modestly with duration of intubation (ρ = -0.24, p-value 0.048) and post-operative lateral e' correlated modestly with duration of intubation and length of hospital stay (ρ = -0.28 and -0.26, p-values = 0.02 and 0.04, respectively). CONCLUSIONS: Children and young adults who underwent congenital AS surgery had echocardiographic evidence of diastolic dysfunction pre-operatively that worsened post-operatively. Lateral e' may be a sensitive indicator of impaired ventricular relaxation in these patients and may impact duration of intubation and hospital stay.

Aortic Valve Neocuspidization in a Pulmonary Autograft.

Management of aortic valve (AV) disease in the pediatric population is challenged by a lack of attractive valve replacement options. The Ross procedure has been prominent for these patients; however, long-term reports have revealed concerns, namely neoaortic root dilation and regurgitation. More recently, AV neocuspidization has emerged as an alternative to the Ross operation or other valve replacement procedures in pediatric patients with congenital AV disease. We report a successful case of AV neocuspidization of a previously implanted pulmonary autograft.

Isolated aortic valve replacement with bio-prostheses in patients age 50 to 65 years: a decade of statewide data on cost and patient outcomes.

BACKGROUND: Guidelines for choice of replacement valve-mechanical versus bio-prosthetic, are well established for patients aged <50 and >65 years. We studied the trends and implications of aortic valve replacement (AVR) with mechanical versus bioprosthetic valve in patients aged 50 to 65 years. METHODS: STS and cost database of 17 centers for isolated AVR surgery were analyzed by dividing them into bioprosthetic valve (BV) or mechanical valve (MV) groups. RESULTS: From 2002 to 2011, 3,690 patients had AVR, 18.6% with MV and 81.4% with BV. Use of BV for all ages increased from 71.5% in 2002 to 87% in 2011. There were 1127 (30.5%) patients in the age group 50-65 years. Use of BV in this group almost doubled, 39.6% in 2002 to 76.8% in 2011. Mean age of patients in BV group was higher (59.2±4.2 years vs. 56.7±4.3 years, P≤0.0001). Preoperative renal failure, heart failure and chronic obstructive pulmonary disease favored use of BV, whereas preoperative atrial fibrillation favored AVR with MV. Mortality (MV 2.2% vs. BV 2.36%) and other postoperative outcomes between the groups were similar. Cost of valve replacement increased for both groups (MV $26,191 in 2002 to $42,592 in 2011; BV $27,404 in 2002 to $44,257 in 2011). CONCLUSIONS: Use of bioprostheses for AVR has increased; this change is more pronounced in patients aged 50-65 years. Specific preoperative risk factors influence the choice of valve for AVR. Postoperative outcomes between the two groups were similar. Long-term implications of this changing practice, in particular, reoperation for bioprosthetic valve degeneration should be examined.

Modern advances regarding interatrial communication in congenital heart defects.

BACKGROUND: The interatrial communication, one of the most frequent congenital heart defects, represents an important intracardiac shunt between systemic and pulmonary circulations. Direction and magnitude of the interatrial shunting depends upon several features, including defect size, shape and location, pressure difference between right and left atrium, and difference in right and left ventricular compliance. METHODS: In this review article, the presence or absence of interatrial communication, and its role, have been analyzed, as they can have a critical impact on the cardiovascular physiopathology, and the interatrial communication can prove to be either clinically harmful, useful or indispensable. Accordingly, the utility and role of the interatrial communication in modern congenital, pediatric and adult, disease has evolved, with modification of the indications to close, maintain patency, or create an interatrial communication. RESULTS: The interatrial communication and shunting can be manipulated to maximize the oxygen delivery to the tissues, accordingly with the underlying congenital heart defect. While not always relevant to patients with bi-ventricular circulations, this becomes extremely important in children and adults with complex congenital heart defects. CONCLUSIONS: With improving long-term survival for the vast majority of congenital heart patients, an advanced understanding of the role and utility of the interatrial communication, and of all the possibilities of its manipulation, is essential to improve the patient outcomes.

A narrative review of modern approach and outcomes evaluation in congenital heart defects.

OBJECTIVE: The purpose of this review is to highlight critical advances and innovative approaches to the most challenging clinical situations in congenital heart surgery, to establish a new perspective from which to evaluate current clinical practice patterns and contemporary United States program ranking systems. BACKGROUND: The past decades have witnessed substantial advances in the treatment of congenital heart defects. New strategies are deeply rooted in calculated risk-taking innovations. Pioneer surgeons developed, improved and refined critical operative skills and techniques to optimize cardiovascular physiology, decrease operative mortality and improve clinical outcomes. METHODS: Unfortunately, in the modern surgical era, supportive environments to allow surgeons to make similar gains and innovative contributions remain scarce. In the current practice, overall procedure volume is prioritized to safeguard quality metrics, including hospital survival and length of stay, surgical complications, and neurocognitive outcomes. As a result, exceptional surgical results have become translated and defined by public ranking systems such as the US News and World Report Best Children's Hospital National Ranking (USNWR) and the Congenital Heart Surgery Database of the Society for Thoracic Surgeons (CHSD-STS), primary based upon early post-operative mortality. This reality places surgeons in a vulnerable position where pressure to achieve a high clinical ranking contrasts with a surgeon definition of "acceptable" surgical risk. Currently, the most frequently used risk stratification tools do not factor in important differences in strategies, such as staged palliation versus complete repair, or bi-ventricular versus uni-ventricular physiology. This favors hospitals pursuing multistage surgical approaches, even if the result is worse long-term morbidity, mortality and increased resource utilization. This economy of ranking-based decision-making causes surgeons either avoid operating altogether or accept less advantageous multi-staged treatment strategies for patients with elevated expected mortality. Such an environment also might present much farther-reaching negative impacts on the growth and development of junior surgeons and trainees, as well as on the pursuit of new surgical innovations to aid future generations of patients. CONCLUSIONS: Risk aversive surgical behavior is creating an environment not favorable for the children born with truly complex congenital heart defects. KEYWORDS: Biventricular conversion; congenital heart surgery; multidisciplinary approach; risk-stratification; surgical outcomes.

Cardiac transplantation in adult congenital heart disease with prior sternotomy.

BACKGROUND: Adult congenital heart disease (ACHD) patients who require orthotopic heart transplantation are surgically complex due to anatomical abnormalities and multiple prior surgeries. In this study, we investigated these patients' outcomes using our institutional database. METHODS: ACHD patients who had prior intracardiac repair and subsequent heart transplant were included (2008-2018). Adult patients without ACHD were extracted as a control. A comparison of patients with functional single ventricular (SV) and biventricular (BV) hearts was performed. RESULTS: There were 9 SV and 24 BV patients. The SV group had higher central venous pressure/pulmonary capillary wedge pressure (P = .028), hemoglobin concentration (P = .010), alkaline phosphatase (P = .022), and were more likely to have liver congestion (P = .006). Major complications included infection in 16 (48.5%), temporary dialysis in 12 (36.4%), and graft dysfunction requiring perioperative mechanical support in 7 (21.2%). Overall in-hospital mortality was 15.2%. Kaplan-Meier analysis showed a higher, but not statistically significant, survival after 10 years between the ACHD and control groups (ACHD 84.9% vs. control 67.5%, P = .429). There was no significant difference in 10-year survival between SV and BV groups (78% vs. 88%, P = .467). CONCLUSIONS: Complex ACHD cardiac transplant recipients have a high incidence of early morbidities after transplantation. However, long-term outcomes were acceptable.

Neonatal Cone Valvuloplasty for Pulmonary Atresia/Intact Ventricular Septum and Ebsteinoid Valve.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve, and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the right ventricle-to-pulmonary artery conduit as the patient grows. We present a successful complete repair cone tricuspid valvuloplasty in a 9-day-old (2.8 kg) neonate with trisomy 21, pulmonary atresia with intact ventricular septum, and an Ebsteinoid tricuspid valve.

Center Reputation and Experience Relate to Industry Payments to Congenital Heart Disease Providers.

BACKGROUND: The Physician Payments Sunshine Act was enacted to understand financial relationships with industry that might influence provider decisions. We investigated how industry payments within the congenital heart community relate to experience and reputation. METHODS: Congenital cardiothoracic surgeons and pediatric cardiologists were identified from the Open Payments Database. All payments from 2013 through 2017 were matched to affiliated hospitals' U.S. News & World Report (USNWR) rankings, The Society of Thoracic Surgeons-Congenital Heart Surgery Public Reporting Star Ratings, and Optum Center of Excellence (COE) designation. Surgeon payments were linked to years since terminal training. Univariable analyses were conducted. RESULTS: The median payment amount per surgeon ($71; interquartile range [IQR], $41-$99) was nearly double the median payment amount per cardiologist ($41; IQR, $18-$84; P < .05). For surgeons, median individual payment was 56% higher to payees at USNWR top 10 children's hospitals ($100; IQR, $28-$203) vs all others ($64; IQR, $23-$140; P < .001). For cardiologists, median individual payment was 26% higher to payees at USNWR top 10 children's hospitals ($73; IQR, $28-$197) vs all others ($58; IQR, $19-$140; P < .001). Findings were similar across The Society of Thoracic Surgeons-Congenital Heart Surgery star rankings and Optum Center of Excellence groups. By surgeon experience, surgeons 0 to 6 years posttraining (first quartile) received the highest number of median payments per surgeon (17 payments; IQR, 6.5-28 payments; P < .001). Surgeons 21 to 44 years posttraining (fourth quartile) received the lowest median individual payment ($51; IQR, $20-132; P < .001). CONCLUSIONS: Industry payments vary by hospital reputation and provider experience. Such biases must be understood for self-governance and the delineation of conflict of interest policies that balance industry relationships with clinical innovation.

Trisomy 21 Patients Undergoing Cavopulmonary Connections Need Improved Preoperative and Postoperative Care.

BACKGROUND: Patients with trisomy 21 (T21) often have elevated pulmonary vascular resistance, which may result in a suboptimal cavopulmonary connection (CPC) after a Glenn or Fontan operation. The purpose of this study was to assess, in a nationwide, multiinstitution cohort of patients with CPC, the impact of T21 on patient morbidity, mortality, and resource use. METHODS: A total of 23,271 pediatric patients with CPC (2004 to 2019) at 50 US hospitals were evaluated using the Pediatric Health Information System database. Univariable and multivariable regression analyses were used to assess risk-adjusted associations between Down syndrome and other risk factors and postoperative measures of morbidity, mortality, lengths of stay, and cost of hospitalization. RESULTS: The overall prevalence of T21 among patients who had undergone Glenn and Fontan procedures was 1.5% (199 of 13,268) and 0.8% (78 of 1003), respectively. Among both CPC cohorts, T21 status significantly increased unadjusted mortality, hospital lengths of stay, and total costs of hospitalization compared with the non-T21 CPC cohort (all P < .001). Patients with T21 also had a higher incidence of prolonged mechanical ventilation compared with patients without T21 in both Glenn and Fontan groups (P < .001). Multivariable regression analysis further estimated that patients with T21 are associated with a 5.5-fold increase in mortality (P < .001) compared with patients without T21. Finally, patients with T21 had increased long-term mortality compared with their peers. CONCLUSIONS: T21 significantly increases risk-adjusted morbidity, inpatient mortality, long-term mortality, and resource use after cavopulmonary connections. Further investigation is needed to clarify modifiable patient-level and center-specific risk factors to improve outcomes for patients with T21.