Management Options for Congenitally Corrected Transposition: Which, When, and for Whom?

Management strategies for congenitally corrected transposition of the great arteries (ccTGA) historically consisted of a physiologic repair, resulting in the morphologic right ventricle (mRV) supporting systemic circulation. This strategy persisted despite the development of heart failure by middle age because of the reasonable short-term outcomes, and the natural history of some patients with favorable anatomy (felt to demonstrate the mRV's ability to function in the long-term), and due to the less-than-optimal outcomes associated with anatomical repair. As outcomes with anatomical repair improved, and the long-term risk of systemic mRV dysfunction became apparent, more have begun to realize its advantages. In addition to the decision on whether or not to pursue anatomical repair, and the optimal timing, studies demonstrating the nuance to morphologic left ventricle retraining have demonstrated its feasibility. Further considerations in ccTGA have begun to be better understood, including: the management of a poorly functioning mRV, systemic tricuspid valve regurgitation, the utility of morphologic left ventricle outflow tract obstruction (native or surgically created) and pacing strategies. While some considerations are apparent: biventricular pacing is superior to univentricular, tricuspid regurgitation must be managed early with either progression towards anatomical repair (pulmonary artery banding if needed for retraining) or tricuspid replacement (not repair) based on the patient's age; others remain to be completely elucidated. Overall, the heterogeneity of ccTGA, as well as the unique presentation with each patient regarding ventricular and valvular function and center-to-center variability in management strategies has made the interpretation of published data difficult. That said, more recent long-term outcomes favor anatomical repair in most situations.

Paediatrician's guide to post-operative care for functionally univentricular CHD: a review.

IMPORTANCE: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care. OBSERVATIONS: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation. CONCLUSIONS AND RELEVANCE: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Paediatrician's guide to post-operative care for biventricular CHD: a review.

IMPORTANCE: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients. OBSERVATIONS: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation. CONCLUSIONS AND RELEVANCE: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Loss of Consciousness in the Young Child.

In the very young child (less than eight years of age), transient loss of consciousness represents a diagnostic and management dilemma for clinicians. While most commonly benign, syncope may be due to cardiac dysfunction which can be life-threatening. It can be secondary to an underlying ion channelopathy, cardiac inflammation, cardiac ischemia, congenital heart disease, cardiomyopathy, or pulmonary hypertension. Patients with genetic disorders require careful evaluation for a cardiac cause of syncope. Among the noncardiac causes, vasovagal syncope is the most common etiology. Breath-holding spells are commonly seen in this age group. Other causes of transient loss of consciousness include seizures, neurovascular pathology, head trauma, psychogenic pseudosyncope, and factitious disorder imposed on another and other forms of child abuse. A detailed social, present, past medical, and family medical history is important when evaluating loss of consciousness in the very young. Concerning characteristics of syncope include lack of prodromal symptoms, no preceding postural changes or occurring in a supine position, after exertion or a loud noise. A family history of sudden unexplained death, ion channelopathy, cardiomyopathy, or congenital deafness merits further evaluation. Due to inherent challenges in diagnosis at this age, often there is a lower threshold for referral to a specialist.

Cardiac Surgery in Trisomy 13 and 18: A Guide to Clinical Decision-Making.

There has been substantial controversy regarding treatment of congenital heart defects in infants with trisomies 13 and 18. Most reports have focused on surgical outcomes versus expectant treatment, and rarely there has been an effort to consolidate existing evidence into a more coherent way to help clinicians with decision-making and counseling families. An extensive review of the existing literature on cardiac surgery in patients with these trisomies was conducted from 2004 to 2020. The effects of preoperative and perioperative factors on in-hospital and long-term mortality were analyzed, as well as possible predictors for postoperative chronic care needs such as tracheostomy and gastrostomy. Patients with minimal or no preoperative pulmonary hypertension and mechanical ventilation undergoing corrective surgery at a weight greater than 2.5 kg suffer from lower postoperative mortality. Infants with lower-complexity cardiac defects are likely to benefit the most from surgery, although their expected mortality is higher than that of infants without trisomy. Omphalocele confers an increased mortality risk regardless of cardiac surgery. Gastrointestinal comorbidities increased the risk of gastrostomy tube placement, while those with prolonged mechanical ventilation and respiratory comorbidities are more likely to require tracheostomy. Cardiac surgery is feasible in children with trisomies 13 and 18 and can provide improved long-term results. However, this is a clinically complex population, and both physicians and caretakers should be aware of the long-term challenges these patients face following surgery when discussing treatment options.

Outcomes of the Kawashima: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.

BACKGROUND: We aimed to evaluate the effect of age at operation on postoperative outcomes in children undergoing a Kawashima operation. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for Kawashima procedures from January 1, 2014, to June 30, 2020. Patients were stratified by age at operation in months: 0 to <4, 4 to <8, 8 to <12, and >12. Subsequently, outcomes for those in whom the Kawashima was not the index operation and for those undergoing hepatic vein incorporation (Fontan completion or hepatic vein-to-azygos vein connection) were evaluated. RESULTS: We identified 253 patients who underwent a Kawashima operation (median age, 8.6 months; median weight, 7.4 kg): 12 (4.7%), 0 to <4 months; 96 (37.9%), 4 to <8 months; 81 (32.0%), 8 to <12 months; and 64 (25.3%), >12 months. Operative mortality was 0.8% (n = 2), with major morbidity or mortality in 17.4% (n = 44), neither different across age groups. Patients <4 months had a longer postoperative length of stay (12.5 vs 9.3 days; P = .03). The Kawashima was not the index operation of the hospital admission in 15 (5.9%); these patients were younger (6.0 vs 8.4 months; P = .05) and had more preoperative risk factors (13/15 [92.9%] vs 126/238 [52.9%]; P < .01). We identified 173 patients undergoing subsequent hepatic vein incorporation (median age, 3.9 years; median weight, 15.0 kg) with operative mortality in 6 (3.5%) and major morbidity or mortality in 30 (17.3%). CONCLUSIONS: The Kawashima is typically performed between 4 and 12 months with low mortality. Morbidity and mortality were not affected by age. Hepatic vein incorporations may be higher risk than in traditional Fontan procedures, and ways to mitigate this should be sought.

Creation of a Neo-Mitral Valve With Right Atrial Appendage Tissue in an Infant.

Mitral valve replacement in neonates and infants is a challenging operation with few good options. Neo-mitral valve reconstruction with right atrial appendage (RAA) may overcome some of the limitations of existing options.

Evolution of Pulmonary Valve Management During Repair of Tetralogy of Fallot: A 14-year Experience.

BACKGROUND: The optimal repair strategy for tetralogy of Fallot remains controversial. This report presents a 14-year evolution of management of the pulmonary valve (PV) from transannular patch to valve-sparing repair to neovalve creation using living right atrial appendage tissue. METHODS: A retrospective review of 172 consecutive patients undergoing complete repair for TOF between January 2007 and June 2021 was performed. Clinical and follow-up data were analyzed by repair group. Neopulmonary valve (NPV) creation using right atrial appendage tissue was introduced in 2019. Failure of valve-sparing repair was defined as needing reintervention for recurrent right ventricular outflow tract obstruction (RVOTO). RESULTS: Median age and weight at repair were 4.9 months and 6 kg, respectively. Median preoperative PV size and z-score were 6.4 mm (5.2-8.3 mm) and -3.2 (-4.1 to -2.1), respectively. Patients who underwent valve-sparing repair had larger PV size and z-score compared with patients who underwent transannular patch procedures (8 mm vs 5.6 mm; -2.1 vs -3.2; both P < .001). There were no hospital mortalities. Overall follow-up was 44 months. At last follow-up, 10% of patients who underwent valve-sparing repair had repeat intervention for recurrent RVOTO. Patients who had failed valve-sparing repair had significantly lower PV z-scores (-2.6 vs -1.9; P = .01). An NPV was used in 8 patients with a median PV z-score of -4 (-4.7 to -3.9). At 6 months, 6 patients (75%) had mild or trivial pulmonary insufficiency after NPV placement. CONCLUSIONS: Repair of tetralogy of Fallot is a safe operation with excellent outcomes. Valve-sparing repair avoids right ventricular dilation but may fail for RVOTO at a PV z-score <-2. NPV creation offers an alternative option in patients with a small PV.

The American Association for Thoracic Surgery (AATS) 2022 Expert Consensus Document: Management of infants and neonates with tetralogy of Fallot.

OBJECTIVE: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. METHODS: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. RESULTS: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. CONCLUSIONS: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.

Technique for Neo-Pulmonary Valve Creation With Living Tissue for Repair of Atrioventricular Septal Defect and Tetralogy of Fallot.

Long-standing effects of pulmonary regurgitation after transannular patch repair in Tetralogy of Fallot (ToF) can be especially deleterious in the setting of combined ToF and complete atrioventricular septal defect (CAVSD). We present a technique for a complete repair of combined ToF/CAVSD using right atrial appendage tissue to create a competent neo-pulmonary valve. This technique provides advantages of right heart protection via pulmonary valve competence and the use of living tissue capable of growth with the patient, potentially obviating the need for repeat interventions.

Pediatric heart-lung transplantation: Technique and special considerations.

Heart-lung transplantation has historically been used as a definitive treatment for children with end-stage cardiopulmonary failure, although the number performed has steadily decreased over time. In this review, we discuss current indications, preoperative risk factors, outcomes, and heart-lung transplantation in unique patient subsets, including infants, children with single-ventricle physiology, tetralogy of Fallot/major aortopulmonary collateral arteries, and prior Potts shunt palliation. We also describe the different surgical techniques utilized in pediatric heart-lung transplantation.

Outcome of the pancreatic remnant following segmental pancreatectomy for non-invasive intraductal papillary mucinous neoplasm.

OBJECTIVES: Intraductual papillary mucinous neoplasms (IPMNs) are often multifocal and involve the entire pancreas. Because of the morbidity associated with total pancreatectomy, surgeons will perform segmental pancreatectomy, resecting only the most 'threatening' IPMN lesion(s). We sought to determine whether the presence of residual IPMN following segmental pancreatectomy for non-invasive IPMN increases the risk for subsequent development of invasive pancreatic cancer and decreases survival. METHODS: Data on patients undergoing segmental resection of non-invasive IPMN during the period 1991-2010 at a high-volume academic institution were prospectively accrued. RESULTS: Of 243 patients who underwent segmental resection for IPMN, 191 (79%) demonstrated non-invasive pathology. Of these, 153 (80%) showed the absence and 38 (20%) the presence of residual IPMN at the initial operation. Of the 38 patients with residual IPMN, eight had positive IPMN margins, 23 had radiographic evidence of IPMN, and seven had both. During a mean follow-up of 73 months, 31 (20%) of 153 patients without residual IPMN developed a new radiographic lesion consistent with IPMN and, of these, three (10%) were found to represent invasive cancer. One (3%) of 38 patients with residual IPMN developed invasive cancer. In summary, in 191 initially non-invasive cases of IPMN, four invasive cancers (2%) developed during follow-up. The mean progression-free interval in these four patients was 54 months (range: 20-99 months). CONCLUSIONS: Compared with patients undergoing complete operative IPMN clearance, patients with residual IPMN after segmental pancreatectomy do not demonstrate increased risk for the development of invasive disease or reduced survival. In patients without residual IPMN who later develop new IPMN, the risk for invasive IPMN is increased.

Behavioral and Attitudinal Correlates of Trusted Sources of COVID-19 Vaccine Information in the US.

There is a critical need for the public to have trusted sources of vaccine information. A longitudinal online study assessed trust in COVID-19 vaccine information from 10 sources. A factor analysis for data reduction revealed two factors. The first factor contained politically conservative sources (PCS) of information. The second factor included eight news sources representing mainstream sources (MS). Multivariable logistic regression models were used. Trust in Dr. Fauci was also examined. High trust in MS was associated with intention to encourage family members to get COVID-19 vaccines, altruistic beliefs that more vulnerable people should have vaccine priority, and belief that racial minorities with higher rates of COVID-19 deaths should have priority. High trust in PCS was associated with intention to discourage friends from getting vaccinated. Higher trust in PCS was also associated with participants more likely to disagree that minorities with higher rates of COVID-19 deaths should have priority for a vaccine. High trust in Dr. Fauci as a source of COVID-19 vaccine information was associated with factors similar to high trust in MS. Fair, equitable, and transparent access and distribution are essential to ensure trust in public health systems' abilities to serve the population.

Childhood experiences and adult health: the moderating effects of temperament.

Existing literature demonstrates a strong relationship between childhood experiences and adult health outcomes. The Differential Susceptibility to Environment Theory suggests that there are several factors, including personality, that affect a child's sensitivity to adverse and advantageous experiences. A sample of 246 adults (ages 19-57) were asked questions about extroverted personality characteristics, adverse and advantageous childhood experiences (ACEs and counter-ACEs), and several indicators of adult health, including executive functioning, perceived stress levels, depression, and past smoking habits. The sample was then stratified based on level of extroversion scores with the top quartile being labeled as "extroverts", the bottom quartile as "introverts", and those in between as "ambiverts". Regression analyses were then used to assess the relationship between childhood experiences and each adult health outcome. The results of the study showed that the relationship between childhood experiences and adult health was generally stronger among extroverted individuals. These results suggest that extroverts may be more sensitive to environmental influences in childhood as compared to introverts and ambiverts. More research is needed to understand the neurobiological mechanisms that increase environmental sensitivity among extroverts.

Executive functions and health behaviors associated with the leading causes of death in the United States: A systematic review.

Research indicates that executive functioning may predict health behavior. This systematic review provides an overview of the relationship between domains of executive functioning and health behaviors associated with the leading causes of death in the United States. A total of 114 articles met the inclusion criteria (adult sample, published in English between 1990 and November 2016) and were reviewed and synthesized. Results indicated that although many studies had mixed findings, at least one executive function component was associated with every health behavior. Based on these results, health professionals should consider the role of executive functions in behavior change interventions.

Dispositional forgiveness and stress as primary correlates of executive functioning in adults.

The purpose of this study was to explore the physical, social, and mental health correlates of executive functioning in adults. Our sample consisted of 250 adults aged 18-55 years who participated in a survey. Participants reported on their physical health behaviors, family closeness, and mental health. Using hierarchical linear regression, the final model explained 41 percent of executive functioning in adults. Dispositional forgiveness of situations, stress, and living in a single-family home were the only significant correlates of executive functioning. These results are useful for better understanding possible mechanisms through which to improve executive functioning in adults.

ACEs and counter-ACEs: How positive and negative childhood experiences influence adult health.

BACKGROUND: Numerous studies over the past two decades have found a link between adverse childhood experiences (ACEs) and worse adult health outcomes. Less well understood is how advantageous childhood experiences (counter-ACEs) may lead to better adult health, especially in the presence of adversity. OBJECTIVE: To examine how counter-ACEs and ACEs affect adult physical and mental health using Resiliency Theory as the theoretical framework. PARTICIPANTS AND SETTING: Participants were Amazon mTurk users ages 19-57 years (N = 246; 42% female) who completed an online survey. METHODS: We conducted a series of regression analyses to examine how counter-ACEs and ACEs predicted adult health. RESULTS: Corresponding to the Compensatory Model of Resiliency Theory, higher counter-ACEs scores were associated with improved adult health and that counter-ACEs neutralized the negative impact of ACEs on adult health. Contrary to the Protective Factors Model, there was a stronger relationship between ACEs and worse adult health among those with above average counter-ACEs scores compared to those with below average counter-ACEs scores. Consistent with the Challenge Model, counter-ACEs had a reduced positive effect on adult health among those with four or more ACEs compared to those with fewer than four ACEs. CONCLUSIONS: Overall, the findings suggest that counter-ACEs protect against poor adult health and lead to better adult wellness. When ACEs scores are moderate, counter-ACEs largely neutralize the negative effects of ACEs on adult health. Ultimately, the results demonstrate that a public health approach to promoting positive childhood experiences may promote better lifelong health.

An overview of mechanical circulatory support in single-ventricle patients.

The population of people with a single-ventricle is continually increasing due to improvements across the spectrum of medical care. Unfortunately, a proportion of these patients will develop heart failure. Often, for these patients, mechanical circulatory support (MCS) represents the only available treatment option. While single-ventricle patients currently represent a small proportion of the total number of patients who receive MCS, as the single-ventricle patient population increases, this number will increase as well. Outcomes for these complex single-ventricle patients who require MCS has begun to be evaluated. When considering the entire population, survival to hospital discharge is 30-50%, though this must be considered with the significant heterogeneity of the single-ventricle patient population. Patients with a single-ventricle have unique anatomy, mechanisms of failure, indications for MCS and the type of support utilized. This has made the interpretation and the generalizability of the limited available data difficult. It is likely that some subsets will have a significantly worse prognosis and others a better one. Unfortunately, with these limited data, indications of a favorable or poor outcome have not yet been elucidated. Though currently, a database has been constructed to address this issue. While the outcomes for these complex patients is unclear, at least in some situations, they are poor. However, significant advances may provide improvements going forward, including new devices, computer simulations and 3D printed models. The most important factor, however, will be the increased experience gained by the heart failure team to improve patient selection, timing, device and configuration selection and operative approach.

Rituximab Induced Pulmonary Edema Managed with Extracorporeal Life Support.

Though rare, rituximab has been reported to induce severe pulmonary edema. We describe the first report of ECLS utilization for this indication. A 31-year-old female with severe thrombotic thrombocytopenic purpura developed florid pulmonary edema after rituximab infusion. Despite advanced ventilatory settings, she developed severe respiratory acidosis and remained hypoxemic with a significant vasopressor requirement. Since her pulmonary insult was likely transient, ECLS was considered. Due to combined cardiorespiratory failure, she received support with peripheral venoarterial ECLS. During her ECLS course, she received daily plasmapheresis and high dose steroids. Her pulmonary function recovered and she was decannulated after 8 days. She was discharged after 23 days without residual sequelae.

Anomalous Systemic Artery to the Left Lower Lobe: Literature Review and a New Surgical Technique.

Anomalous systemic arterial supply to the basal segments of the left lower lobe without coexisting pulmonary artery connection is a rare anomaly. Most feel treatment is necessary; however, the ideal strategy is unclear. Treatments described include embolization, pulmonary resection, or anastomosis to the native pulmonary artery. We recently encountered an infant with this anomaly and present a literature review summarizing all recent reports. Additionally, we describe a novel surgical technique to create a tension-free anastomosis utilizing segmental aortic translocation that we employed in our patient due to a large distance between the anomalous vessel and native left pulmonary artery.