The burden of psychological trauma and post-traumatic stress disorder among adults with congenital heart disease: PTSD in ACHD.

Psychological trauma, symptoms of post-traumatic stress disorder (PTSD), and mental health conditions are common in adult congenital heart disease (ACHD). There is a gap in research examining PTSD in ACHD using the current Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) criteria in assessing patient characteristics and experiences with trauma-focused treatment. Surveys were offered to outpatients over a 6-month enrollment period to be completed by way of a QR code on their personal smart phone. Patient-reported items include a detailed medical and psychosocial history, the Oslo social support scale, adverse childhood experiences survey, and the PTSD checklist for DSM-5. Of 158 patients (77% moderate or complex heart disease) who provided complete data, a provisional diagnosis of PTSD was found in 48 patients (30%) using a PTSD checklist for DSM-5 cut-off score of ≥31. A positive PTSD screen was associated with younger age, nonwhite race, presence of heart failure, lower New York Heart Association functional class, lower linear quality of life score, lower Oslo social support scale score, an insecure caregiver relation, period of unemployment, emergency department visits, medication nonadherence, and coexisting mental health disorders. Complexity of heart disease and number of surgical and/or catheter-based interventions were not associated with PTSD, although having undergone no cardiac surgeries until adulthood (aged ≥18 years) was associated with a lower prevalence of PTSD. Those who screened positive for PTSD were more likely to report multiple traumatic events, including noncardiac traumatic events. Only 14 of 48 patients (29%) reported a known diagnosis of PTSD, although 44 patients (92%) reported having ever seen a mental health provider. A total of 18 patients (38%) reported currently having a mental health provider. A total of 30 patients (62%) had heard of at least 1 evidence-based trauma-informed therapy, and 14 (29%) had tried at least 1. In conclusion, using the DSM-5 criteria, we observed a high prevalence of potential PTSD in ACHD associated with several novel cardiac and psychosocial patient factors. Future longitudinal studies will be necessary to establish causality. Few patients with ACHD have been formally diagnosed with PTSD or have experience with evidence-based trauma-informed therapies.

Considerations for Gender-Affirming Hormonal and Surgical Care Among Transgender and Gender Diverse Adolescents and Adults With Congenital Heart Disease.

BACKGROUND: Transgender and gender diverse (TGD) individuals and long-term survivors with adult congenital heart disease (ACHD) are both growing populations with specialized needs. No studies assess temporal trends or evaluate the care of TGD individuals with ACHD. METHODS AND RESULTS: Meetings between congenital cardiology and gender-affirming care specialists identified unique considerations in TGD individuals with ACHD. A retrospective chart review was then performed to describe patient factors and outpatient trends in those with an ACHD diagnosis undergoing gender-affirming hormonal or surgical care (GAHT/S) at 1 adult and 1 pediatric tertiary care center. Thirty-three TGD individuals with ACHD were identified, 21 with a history of GAHT/S. Fourteen (66%) had moderate or complex ACHD, 8 (38%) identified as transgender male, 9 (43%) transgender female, and 4 (19%) other gender identities. Three had undergone gender-affirming surgery. There were zero occurrences of the composite end point of unplanned hospitalization or thrombotic event over 71.1 person-years of gender-affirming care. Median age at first gender-affirming appointment was 16.8 years [interquartile range 14.8-21.5]. The most common treatment modification was changing estradiol administration from oral to transdermal to reduce thrombotic risk (n=3). An increasing trend was observed from zero TGD patients with ACHD attending a gender diversity appointment in 2012 to 14 patients in 2022. CONCLUSIONS: There is a growing population of TGD patients with ACHD and unique medical and psychosocial needs. Future studies must fully evaluate the reassuring safety profile observed in this small cohort. We share 10 actionable care considerations for providers with a goal of overseeing a safe and fulfilling gender transition across all TGD patients with ACHD.

End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

BACKGROUND: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. OBJECTIVES: The authors aimed to determine factors associated with survival in a large cohort of such individuals. METHODS: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). RESULTS: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. CONCLUSIONS: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

A life-threatening dental implant: A case report.

BACKGROUND: Intraoral bleeding complications in implant surgery are infrequent; nevertheless, serious and life-threatening complications have been described, generally related to mandibular implants. PURPOSE: This article presents the exceptional case of a 24-year-old woman who underwent repeated delayed life-threatening episodes of intraoral bleeding following uncovering of a maxillary dental implant. Local measures afforded only temporary control. RESULTS: The episodes were successfully managed by embolising the greater descending palatine artery, and the patient remains now under close follow-up. CONCLUSIONS: Early recognition of shock secondary to hemorrhage following intraoral surgery is of paramount importance.

COVID-19 in Adults With Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. OBJECTIVES: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. METHODS: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. RESULTS: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. CONCLUSIONS: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity.

Dyslipidemia in childhood and adolescence: from screening to management.

PURPOSE OF REVIEW: To summarize types of dyslipidemia frequently encountered during childhood and adolescence, with a focus on screening, diagnosis, and management. RECENT FINDINGS: It is important that screening for atherosclerotic cardiovascular disease (ASCVD) begin in childhood. Genetic testing allows for increased awareness of dyslipidemia and more targeted intervention. Pharmacologic treatment of pediatric dyslipidemias has a good safety profile and can reduce adult ASCVD risk. SUMMARY: Much of what is known about pediatric dyslipidemia has been extrapolated from adult data, but recently, there have been increasing investigations within the pediatric population to better guide diagnosis and management of these disorders.

Open versus percutaneous tracheostomy in COVID-19: a multicentre comparison and recommendation for future resource utilisation.

PURPOSE: The COVID-19 pandemic placed an unprecedented demand on critical care services for the provision of mechanical ventilation. Tracheostomy formation facilitates liberation from mechanical ventilation with advantages for both the patient and wider critical care resource, and can be performed using both percutaneous dilatational and surgical techniques. We compared outcomes in those patients undergoing percutaneous dilatational tracheostomy to those undergoing surgical tracheostomy and make recommendations for provision of tracheostomy services in any future surge. METHODS: Multicentre multidisciplinary retrospective observational cohort study including 201 patients with COVID-19 pneumonitis admitted to an ICU in one of five NHS Trusts within the South London Adult Critical Care Network who required mechanical ventilation and subsequent tracheostomy. RESULTS: Percutaneous dilatational tracheostomy was performed in 124 (62%) of patients, and surgical tracheostomy in 77 (38%) of patients. There was no difference between percutaneous dilatational tracheostomy and surgical tracheostomy in either the rate of peri-operative complications (16.9 vs. 22.1%, p = 0.46), median [IQR(range)] time to decannulation [19.0 (15.0-30.2 (5.0-65.0)] vs. 21.0 [15.5-36.0 (5.0-70.0) days] or mortality (13.7% vs. 15.6%, p = 0.84). Of the 172 patients that were alive at follow-up, two remained ventilated and 163 were decannulated. CONCLUSION: In patients with COVID-19 pneumonitis that require tracheostomy to facilitate weaning from mechanical ventilation, there was no difference in outcomes between those patients that had percutaneous dilatational tracheostomy compared with those that had surgical tracheostomy. Planning for future surges in COVID-19-related critical care demands should utilise all available resource and expertise.

Robot-Assisted Reconstruction in Head and Neck Surgical Oncology: The Evolving Role of the Reconstructive Microsurgeon.

Transoral robotic surgery (TORS) is gaining more widespread use among head and neck surgical procedures. As experience grows with this technique, so do the indications of when and in which patients it can be used. Already established in the treatment of small oral cavity tumours, it is expanding into larger multi-site resections and resections, such as through-and-through-into-the-neck defects, that will require reconstruction. With robot-assisted surgery advancing, so robot-assisted reconstruction (RAR) is evolving. In this paper, we discuss the evolving role of reconstruction in post-TORS defects as well as the role of RAR in today's practice.

Novel Treatment Planning of Hemimandibular Hyperplasia by the Use of Three-Dimensional Computer-Aided-Design and Computer-Aided-Manufacturing Technologies.

RATIONALE AND AIM: Hemimandibular hyperplasia is characterized by an obvious overgrowth in the size of the mandible on one side, which can extend up to the midline causing facial asymmetry. Surgical resection of the overgrowth depends heavily on the skill and experience of the surgeon. This report describes a novel methodology of applying three-dimensional computer-aided-design and computer-aided-manufacturing principles in improving the outcome of surgery in 2 mandibular hyperplasia patients. METHODOLOGY: Both patients had their cone beam computer tomography (CBCT) scan performed. CMF Pro Plan software (v. 2.1) was used to process the scan data into virtual 3-dimensional models of the maxilla and mandible. Head tilt was adjusted manually by following horizontal reference. Facial asymmetry secondary to mandibular hypertrophy was obvious on frontal and lateral views. Simulation functions were followed including mirror imaging of the unaffected mandibular side into the hyperplastic side and position was optimized by translation and orientation functions. Reconstruction of virtual symmetry was assessed and checked by running 3-dimensional measurements. Then, subtraction functions were used to create a 3-dimensional template defining the outline of the lower mandibular osteotomy needed. Precision of mandibular teeth was enhanced by amalgamating the CBCT scan with e-cast scan of the patient lower teeth. 3-Matic software (v. 10.0) was used in designing cutting guide(s) that define the amount of overgrowth to be resected. The top section of the guide was resting on the teeth hence ensuring stability and accuracy while positioning it. The guide design was exported as an .stl file and printed using in-house 3-dimensional printer in biocompatible resin. CONCLUSION: Three-dimensional technologies of both softwares (CMF Pro Plan and 3-Matic) are accurate and reliable methods in the diagnosis, treatment planning, and designing of cutting guides that optimize surgical correction of hemimandibular hyperplasia at timely and cost-effect manner.

Lapse of care as a predictor for morbidity in adults with congenital heart disease.

BACKGROUND: There is a need for timely transition of patients with congenital heart disease who have reached adulthood. While the American Heart Association guidelines dictate that patients with moderate or great complexity lesions be seen at least every 2 years, lapse in care is common. We sought to assess the frequency and clinical impact of lapse of medical care in adults with moderate or great complexity cardiac lesions diagnosed in childhood. METHODS: All patients, fulfilling the above criteria, who were seen in an Adult Congenital Heart Disease regional clinic from 2002 to 2005 were questioned as to the length of time from leaving care at a pediatric institution to establishment of subsequent cardiac care. Lapse of medical care, defined as > or = 2 year interval from leaving a pediatric cardiac care facility, was determined. Variables associated with lapse of medical care were sought. Variables associated with adverse outcome were identified. RESULTS: Of 400 patients seen, 158 met inclusion criteria. Of these, lapse of medical care was present in 99 (63%) with a median duration of lapse of medical care of 10 (2-50 years). The most common reason cited for lapse of medical care was that the patient was told there was no need for follow-up (32%). Patients with lapse of medical care were 3.1x(p=0.003) more likely to require urgent cardiac intervention (p=0.003). CONCLUSIONS: Lapse of medical care is common in adults with congenital heart disease and is associated with adverse outcome.