Social determinants of health and outcomes for children and adults with congenital heart disease: a systematic review.

BACKGROUND: Social determinants of health (SDH) can substantially impact health outcomes. A systematic review, however, has never been conducted on associations of SDH with congenital heart disease (CHD) outcomes. The aim, therefore, was to conduct such a systematic review. METHODS: Seven databases were searched through May 2020 to identify articles on SDH associations with CHD. SDH examined included poverty, uninsurance, housing instability, parental educational attainment, immigration status, food insecurity, and transportation barriers. Studies were independently selected and coded by two researchers based on the PICO statement. RESULTS: The search generated 3992 citations; 88 were included in the final database. SDH were significantly associated with a lower likelihood of fetal CHD diagnosis, higher CHD incidence and prevalence, increased infant mortality, adverse post-surgical outcomes (including hospital readmission and death), decreased healthcare access (including missed appointments, no shows, and loss to follow-up), impaired neurodevelopmental outcomes (including IQ and school performance) and quality of life, and adverse outcomes for adults with CHD (including endocarditis, hospitalization, and death). CONCLUSIONS: SDH are associated with a wide range of adverse outcomes for fetuses, children, and adults with CHD. SDH screening and referral to appropriate services has the potential to improve outcomes for CHD patients across the lifespan. IMPACT: Social determinants of health (SDH) are associated with a wide range of adverse outcomes for fetuses, children, and adults with congenital heart disease (CHD). This is the first systematic review (to our knowledge) on associations of SDH with congenital heart disease CHD outcomes. SDH screening and referral to appropriate services has the potential to improve outcomes for CHD patients across the lifespan.

Development of a biventricular conversion program: A new paradigm.

BACKGROUND: Borderline small ventricular size or technical issues precluding the use of both ventricles may lead to single ventricle palliation. Fontan complications have led some centers to look for alternatives to the traditional pathway. The objective of this study is to evaluate the essential philosophy and outcomes of a new biventricular (BiV) conversion program. METHODS: The prospectively collected Children's Memorial Hermann Heart Institute Society of Thoracic Surgeon's Database was retrospectively reviewed between July 2017 and July 2020. RESULTS: Thirteen patients met inclusion criteria and underwent BiV conversion during that time. The most frequent diagnosis was malposed great arteries and a ventricular septal defect (VSD) in 4 (31%) patients. Seven (54%) patients were in the first interstage, and 1 (8%) patient had already undergone a Fontan operation before their BiV conversion operation. One or more risk factors for single ventricle palliation (genetic syndrome ≥ moderate atrioventricular valve regurgitation ≥ moderate ventricular dysfunction, presence of signs of Fontan failure) were present in 3 (23%) patients. The median left ventricular end diastolic pressure increased from 5.5 mmHg (4-10 mmHg) to 10 mmHg (6-20 mmHg) postoperatively (p < .05). The right ventricular pressure (RVP) was estimated as less than half systemic in all six patients who were able to be estimated. At a median follow-up of 22.6 months (0.3-36.4 months), 12 (92%) patients are alive. CONCLUSIONS: BiV conversion is feasible with reasonable short-term clinical outcomes. Mortality risk is low, but as seen in other studies, the risk of reintervention is high.

Impact of gender in congenital heart surgery: Results from a national survey.

OBJECTIVE: There are limited data regarding the impact of gender within congenital heart surgery. Our aim was to assess gender-related experiences by surgeons in this field. METHODS: A cross-sectional survey was emailed to practicing congenital heart surgeons to ascertain the perception of gender in 5 domains: training, professional career, clinical practice, personal life, and career outlook. RESULTS: The survey response rate was 94% (17/18) for women and 44% (112/257) for men. More than half of women (53%) were discouraged from pursuing congenital heart surgery (P < .001) and reported a negative impact of gender in attaining their first congenital heart surgery job (P < .001) compared with men. Despite similar demographics, women reported lower starting annual salaries ($150K-$250K vs $250K-$400K), lower current annual salaries ($500K-$750K vs $750K-$1M), lower academic ranks (clinical instructor 6% vs 4% [P = .045], assistant professor 35% vs 19% [P = .19], associate professor 41% vs 25% [P = .24], and professor 6% vs 41% [P = .005]) along with lower annual salaries at the associate professor ($500K-$750K vs $1M-$1.25M) and professor levels ($1M-$1.25M vs >$1.5M) compared with men. Sexual harassment was experienced more frequently by women both in training (65% vs 6%, P < .001) and in practice (65% and 4%, P < .001). CONCLUSIONS: This survey highlights many areas of gender-related differences: discouragement due to gender to pursue congenital heart surgery, sexual harassment in training and practice, salary and academic rank differentials, negative gender perception at work, and lower career satisfaction for women. Despite various differences between both genders, the majority in each group would choose to enter this profession again as well as encourage others to do so.

Status of women in congenital heart surgery: Results from a national survey.

OBJECTIVE: Gender disparity in cardiothoracic surgery, but not specifically in congenital heart surgery, has been a growing focus. The aim of this study was to describe the experiences related to gender for women in this field. METHODS: This cross-sectional survey was designed to assess gender perception in 5 domains: training, professional career, clinical practice, personal life, and career outlook. Inclusion criteria were women who trained and are practicing congenital heart surgery in the United States. RESULTS: Eighteen women were identified with a survey response rate of 94% (17/18). The majority of respondents were aged more than 40 years (94%) and White (69%), chose their career in congenital heart surgery during residency (77%), and completed more than 10 years of postgraduate education (56%). More than half were discouraged from congenital heart surgery because of their gender and reported its negative impact on their ability to obtain their first job. The need to "outperform" their male colleagues was nearly universal (94%), and gender pay disparity was reported by 81%. The majority (65%) reported sexual harassment both during surgical training and as staff surgeons. CONCLUSIONS: This survey highlights many areas (discouragement due to gender, sexual harassment, and gender disparities in pay and leadership opportunities) that women may perceive as barriers to a successful career in congenital heart surgery. There is an increasing call to action to mitigate these hurdles for women, both to enter and succeed. It is encouraging that the women surveyed would repeat their career choice and are actively mentoring other women to join this field.

Primary Sternal Cleft Repair in First Week of Life.

We present a full-term neonate with a postnatal diagnosis of a sternal cleft. Cardiac evaluation was otherwise remarkable for a patent ductus arteriosus and patent foramen ovale. Computed tomography scan confirmed the absence of a sternum except for a small inferior portion, including the xyphoid process. The patient underwent primary repair at five days of age through a midline incision where an absence of pericardium anteriorly was noted. She was extubated successfully the next day and discharged home on postoperative day 3. On follow-up examination, her sternum has been healing well without any clinical concerns. We advocate early repair of such defects in order to allow primary closure in a tension-free manner.

Results of Less Than 5-mm Right Ventricle-Pulmonary Artery Conduits in Single Ventricle Palliation.

BACKGROUND: Our programmatic approach to single ventricle (SV) neonatal palliation has evolved to using smaller-size right ventricle to pulmonary artery (RV-PA) conduits for pulmonary blood flow. Therefore, we sought to compare the early outcomes of less than 5-mm versus 5-mm RV-PA conduits in SV patients undergoing neonatal palliation. METHODS: We performed a retrospective review of SV patients who had neonatal palliation with either a less than 5-mm or 5-mm RV-PA connection, constructed using a waterproof, breathable fabric membrane graft (Gore-Tex, Flagstaff, AZ). Data before, during, and after surgery were analyzed using SPSS software. RESULTS: A total of 26 patients in group 1 (n = 11; <5 mm) and group 2 (n = 15; 5 mm) were operated on, with a mean follow-up of 13 months. Statistical differences (P ≤ .05) were noted with renal near-infrared spectroscopy (mean, 56 versus 44), and oxygen saturation at discharge (mean, 80% versus 85%) in groups 1 and 2, respectively. We found no significant difference in patient weight, peak lactate, cerebral near-infrared spectroscopy, length of intubation, length of hospitalization, or need for RV-PA conduit intervention after stage 1 along with age, branch PA sizes, or need for PA plasty at bidirectional Glenn between groups. CONCLUSIONS: Our early results of less than 5-mm conduits support further exploration into the tailoring of the RV-PA conduit size for each patient. A smaller-diameter conduit may improve systemic cardiac output and net oxygen delivery. Larger patient cohorts and longer follow-up with assessment of pulmonary artery growth are warranted to guide clinical practice.

Effect of Atrioventricular Valve Repair on Multistage Palliation Results of Single-Ventricle Defects.

BACKGROUND: The presence of significant atrioventricular valve (AVV) regurgitation results in unfavorable conditions that affect the success of single-ventricle (SV) multistage palliation. We report our institution's AVV repair experience. METHODS: We examined incidence of AVV repair in 603 infants who underwent initial SV palliative surgery from 2002 to 2012. We explored patients' characteristics and anatomic and operative details associated with death, transplantation, and AVV reoperation. RESULTS: AVV repair was performed in 60 patients during first-stage (n = 10), Glenn (n = 27), and Fontan (n = 23). Median age at AVV repair was 6.9 months (interquartile range, 4.2-24.1 months). Underlying SV anomaly was hypoplastic left heart syndrome (n = 30), heterotaxy (n = 15), and other (n = 15). The AVV was tricuspid (n = 34), mitral (n = 6), or common (n = 20). Preoperatively, all patients had AVV regurgitation moderate or higher, and 7 (12%) had ventricular dysfunction moderate or higher. After the repair, AVV regurgitation was none/trivial in 21 (35%), mild in 21 (35%), and moderate or higher in 17 (30%). Competing risks analysis showed that 10 years after AVV repair, 18% of patients had undergone AVV reoperation, 26% had died or undergone transplantation, and 56% were alive without subsequent reoperation. Transplant-free survival was 38%, 65%, and 100% for AVV repair at first-stage, Glenn, or Fontan (P = .0011), respectively, and was 74%, 83%, and 56% for tricuspid, mitral, and common AVV repair (P = .344), respectively. Factors associated with transplant-free survival were timing of AVV repair, underlying SV anomaly, and systemic ventricle function. CONCLUSIONS: AVV repair at the first-stage operation and reduced systemic ventricle function are associated with poor outcomes. In those high-risk patients, different approaches that involve initial palliation mode, timing of AVV repair, or listing for transplantation might be warranted.

Management outcomes of primary pulmonary vein stenosis.

OBJECTIVES: Primary pulmonary vein stenosis (PPVS) is increasingly diagnosed in children with no prior pulmonary vein intervention history, and management is challenging. We describe characteristics of patients who underwent surgical repair of PPVS at our center, and examine factors associated with treatment failures. METHODS: A retrospective review of all patients who underwent surgical intervention for PPVS (2002-2016) was completed. Patients who had undergone prior cardiac surgery involving the pulmonary veins or atrial switch were excluded. Regression analyses were performed to examine characteristics, PPVS features, including severity score, and surgical details associated with treatment failures. RESULTS: Thirty-four children underwent initial surgical intervention for PPVS. Median age was 8.9 months (interquartile range, 5.9-18.4 months). Most patients (n = 31; 91%) had unilateral pulmonary vein involvement and the median PPVS severity score was 3.5 (interquartile range, 3-5). On competing risk analysis, 1 year following surgical repair, 9% of patients had died, 14% had undergone reintervention, and 77% were alive without reintervention; at 5 years the numbers were 9%, 30%, and 61%, respectively. Factors associated with mortality included bilateral disease and PPVS severity score >6. Bilateral disease and PPVS severity score >5 were associated with reintervention risk. CONCLUSIONS: Multidisciplinary management strategy is required for PPVS. Despite satisfactory early repair, patients continue to be at risk for recurrence and subsequent mortality, especially those with extensive primary involvement. The disappointing results underscore the need for multi-institutional collaborations to better understand this complex disease, establish management and follow-up protocols, and explore investigational treatment modalities that could modify the unfavorable outcome of this uncommon and challenging disease.

Resuscitating the Chimney Graft to Innominate Artery for Straightforward Cannulation During Infancy.

Arterial cannulation with a chimney polytetrafluoroethylene graft to the innominate artery is commonly used for antegrade cerebral perfusion during neonatal aortic arch surgery. When properly retained and prepared before sternal closure, resuscitation of the polytetrafluoroethylene graft to innominate artery can be performed months later during sternal reentry. It is a safe and reproducible technique for expeditious arterial cannulation at stage II palliation in single-ventricle patients or complete intracardiac repair of biventricular lesions. We report our experience utilizing this technique successfully during reoperation in 90 of 92 patients, with no adverse thromboembolic events identified.

Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score "cut-off" in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was -1.7 (0 to -4.9) with a median re-intervention rate of 4.7% (0-36.8%) during a median follow-up of 2.83 years (1.4-15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of -0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

Outcomes and risk factors associated with tricuspid valve repair in children with hypoplastic left heart syndrome.

OBJECTIVES: Tricuspid valve (TV) competence is important for successful palliation of hypoplastic left heart syndrome (HLHS). We report our experience with TV repair in HLHS patients with a focus on TV and right ventricular (RV) function and associated clinical outcomes. METHODS: From 2002 to 2012, 219 neonates with HLHS underwent the Norwood operation. Thirty patients who underwent TV repair at various stages comprised our current series cohort. Echocardiographic and clinical data were reviewed to determine the effectiveness of TV repair and outcomes of the patients. RESULTS: Thirty patients received TV repair during Norwood (n = 4), Glenn (n = 17) and Fontan (n = 9) operations. Median age at TV repair was 188 days (range 3-1498). Preoperatively, all patients had ≥moderate TV regurgitation and 4 (13%) patients had ≥moderate RV dysfunction. After repair, TV regurgitation was none or trivial (n = 12, 40%), mild (n = 8, 27%), ≥moderate (n = 10, 33%), whereas 10 (33%) had ≥moderate regurgitation at last follow-up; ≥moderate RV dysfunction was present in 5 (17%) patients following TV repair and 10 (33%) patients at last follow-up. Competing risk analysis showed that 10 years following TV repair, 21% of patients had TV reoperation, 18% died or underwent transplantation and 61% were alive without subsequent reoperation. Overall, 10-year survival, transplant-free survival and freedom from second TV reoperation were 89%, 71% and 78%, respectively; ≥moderate RV dysfunction following TV repair was associated with diminished transplant-free survival (P = 0.0277). CONCLUSIONS: Although TV repair is successful in reducing regurgitation in the majority of HLHS patients, outcomes are restricted by limited repair durability with recurrent significant regurgitation in one-third of the patients. RV dysfunction in these patients is progressive and a major determinant of transplant-free survival.

Mechanical Circulatory Support for Single Ventricle Failure.

Mechanical circulatory support (MCS) for failing single ventricle (SV) physiology is a complex and challenging problem, which has not yet been satisfactorily addressed. Advancements in surgical strategies and techniques along with intensive care management have substantially improved the outcomes of neonatal palliation for SV physiology, particularly for hypoplastic left heart syndrome (HLHS). This is associated with a steady increase in the number of SV patients who are susceptible to develop heart failure (HF) and would potentially require MCS at a certain stage in their palliation. We have reviewed the literature regarding the reported modalities of MCS use in the management of SV patients. This includes analysis of various devices and strategies used for failing circulation at distinct stages of the SV pathway: after neonatal palliation, after the superior cavo-pulmonary connection (SCPC), and after total cavo-pulmonary connection (TCPC).

A comparison of aortic valve replacement via an anterior right minithoracotomy with standard sternotomy: a propensity score analysis of 492 patients.

OBJECTIVES: Right anterior minithoracotomy with central arterial cannulation is our preferred technique of minimally invasive aortic valve replacement (AVR). We compared perioperative outcomes with this technique to those via sternotomy. METHODS: Between March 1999 and December 2013, 492 patients underwent isolated AVR via either sternotomy (SAVR, n = 198) or minimally invasive right anterior thoracotomy (MIAVR, n = 294) in our institution. Univariate comparisons between groups were made to evaluate overall outcomes and adverse events. To control treatment selection bias, propensity scores were constructed from core patient characteristics. A propensity score-stratified analysis of outcome and adverse events was then performed. RESULTS: Overall mortality was 2.5 and 1.0% in the SAVR and MIAVR groups, respectively. Hospital and ICU stays were shorter, there was less intraoperative blood product usage, and fewer wound infections in the MIAVR group. There were no differences in other adverse events, including strokes. The composite end-point of alive and adverse event-free was significantly more common in the MIAVR group (83 vs 74%, P = 0.002). After adjusting for the propensity score, hospital and ICU stays remained shorter and intraoperative blood product usage remained less in the MIAVR group. There was no difference in mortality, stroke or other adverse events between groups. CONCLUSION: Minimally invasive AVR via an anterior right thoracotomy with predominately central cannulation can be performed with morbidity and mortality similar to that of a sternotomy approach. There appear to be advantages to this minimally invasive approach when compared with sternotomy in terms of less intraoperative blood product usage, lower wound infection rates and decreased hospital stays. If mortality and the occurrence of adverse events are taken together, MIAVR may be associated with better outcomes. As minimally invasive AVR becomes more common, further long-term follow-up is needed and a prospective multicentre randomized trial would be warranted.

Development of a cardiac surgery simulation curriculum: from needs assessment results to practical implementation.

OBJECTIVE: A paradigm shift in surgical training has led to national efforts to incorporate simulation-based learning into cardiothoracic residency programs. Our goal was to determine the feasibility of developing a cardiac surgery simulation curriculum using the formal steps of curriculum development. METHODS: Cardiothoracic surgery residents (n = 6) and faculty (n = 9) evaluated 54 common cardiac surgical procedures to determine their need for simulation. The highest scoring procedures were grouped into similarly themed monthly modules, each with specific learning objectives. Educational tools consisting of inanimate, animate, and cadaveric facilities and a newly created virtual operating room were used for curriculum implementation. Resident satisfaction was evaluated by way of a 5-point Likert scale. Perceived competency (scale of 1-10) and pre-/post-self-confidence (scale of 1-5) scores were collected and analyzed using cumulative mean values and a paired t-test. RESULTS: Of the 23 highest scoring procedures (mean score, ≥ 4.0) on the needs assessment, 21 were used for curriculum development. These procedures were categorized into 12 monthly modules. The simulation curriculum was implemented using the optimal simulation tool available. Resident satisfaction (n = 57) showed an overwhelmingly positive response (mean score, ≥ 4.7). The perceived competency scores highlighted the procedures residents were uncomfortable performing independently. The pre-/post-self-confidence scores increased throughout the modules, and the differences were statistically significant (P < .001). CONCLUSIONS: It is feasible to develop and implement a cardiac surgery simulation curriculum using a structured approach. High-fidelity, low-technology tools such as a fresh tissue cadaver laboratory and a virtual operating room could be important adjuncts.

Progressive mucosal injury in patients with gastroesophageal reflux disease and increasing peripheral blood eosinophil counts.

HYPOTHESIS: Peripheral blood eosinophil count increases with the degree of mucosal injury associated with gastroesophageal reflux disease (GERD). DESIGN: Retrospective review. SETTING: Single-institution tertiary hospital. PATIENTS: Two hundred ninety-five patients (215 men and 80 women; median age, 57 years [interquartile range (IQR), 46-66 years]). One hundred had GERD without intestinal metaplasia, 100 had GERD with intestinal metaplasia, 40 had GERD with dysplasia, and 55 had GERD with intramucosal carcinoma. Results of complete blood count with differential and serum chemistry studies were compared among the groups using a nonparametric test for trend. RESULTS: Patients with a higher degree of mucosal injury were older (P < .001). There were no differences between white blood count, percent neutrophil count, absolute neutrophil count, and hematocrit levels among the groups. Serum albumin level decreased as the degree of mucosal injury increased (P = .04) but lost significance when controlled for age (P = .53). Percent eosinophil counts were 2.0 (IQR, 1.3-2.8) in patients with GERD without intestinal metaplasia, 2.5 (IQR, 1.6-3.7) in GERD with intestinal metaplasia, 2.6 (IQR, 1.7-4.4) in GERD with dysplasia, and 2.7 (IQR, 1.5-4.3) in GERD with intramucosal carcinoma. This progressive increase in the percent eosinophil count was statistically significant (P = .006), remained significant after controlling for age (P = .04), and was also significant when measuring the absolute eosinophil count. CONCLUSION: There is a progressive increase in the percent and absolute peripheral blood eosinophil count associated with progressive mucosal injury in patients with GERD.