Anxiety and Depression in Adults With Congenital Heart Disease.

BACKGROUND: A comprehensive understanding of adult congenital heart disease outcomes must include psychological functioning. Our multisite study offered the opportunity to explore depression and anxiety symptoms within a global sample. OBJECTIVES: In this substudy of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults With Congenital Heart Disease-International Study), the authors we investigated the prevalence of elevated depression and anxiety symptoms, explored associated sociodemographic and medical factors, and examined how quality of life (QOL) and health status (HS) differ according to the degree of psychological symptoms. METHODS: Participants completed the Hospital Anxiety and Depression Scale, which includes subscales for symptoms of anxiety (HADS-A) and depression (HADS-D). Subscale scores of 8 or higher indicate clinically elevated symptoms and can be further categorized as mild, moderate, or severe. Participants also completed analogue scales on a scale of 0 to 100 for QOL and HS. Analysis of variance was performed to investigate whether QOL and HS differed by symptom category. RESULTS: Of 3,815 participants from 15 countries (age 34.8 ± 12.9 years; 52.7% female), 1,148 (30.1%) had elevated symptoms in one or both subscales: elevated HADS-A only (18.3%), elevated HADS-D only (2.9%), or elevations on both subscales (8.9%). Percentages varied among countries. Both QOL and HS decreased in accordance with increasing HADS-A and HADS-D symptom categories (P < 0.001). CONCLUSIONS: In this global sample of adults with congenital heart disease, almost one-third reported elevated symptoms of depression and/or anxiety, which in turn were associated with lower QOL and HS. We strongly advocate for the implementation of strategies to recognize and manage psychological distress in clinical settings. (Patient-Reported Outcomes in Adults With Congenital Heart Disease [APPROACH-IS]; NCT02150603).

Towards improving the lived experience of adult congenital heart patients: suggestions for health care practitioners.

What steps may clinicians looking after adult congenital heart patients take to improve the care they provide? An adult with complex congenital heart disease (CHD) who required a Fontan circulation has developed a discussion paper with the help of his attending clinicians. Drawing on his personal experience and that of others that he has represented, the paper highlights the problems in communication between such patients and their physician, followed by suggestions as to how to improve patient engagement, empowerment, and enablement. Attention to those suggestions may well benefit all in the care provided for this growing population.

Fifteen-minute consultation: Pathophysiology of postural orthostatic tachycardia syndrome in children and adolescents: diagnosis and management strategies.

Postural orthostatic tachycardia syndrome (POTS) affects children and adolescents-normally fit and well and yet troubled by a myriad of everyday symptoms. Understanding the pathophysiology of this not uncommon disorder together with a review of the clinical features may well assist the attending clinicians to arrive at a clear diagnosis without the need for extensive testing and multiple investigations. Simple lifestyle measures such as increasing fluid intake, electrolyte and/or salt supplements, adequate sleep, not missing out on meals and instituting an exercise programme to improve muscle tone and strength may avoid the need of pharmacological management. These simple measures may benefit symptoms which generally improve as adulthood approaches. In addition, it is important to recognise the comorbidities that may be associated with POTS, each of which may require specific management strategies. This paper reviews the pathophysiology, clinical features and recommended treatment in the management of POTS.

The left ventricle in well newborns versus those with perinatal asphyxia, haemodynamically significant ductus arteriosus or fetal growth restriction.

Hemodynamic changes accompanying the initial breaths at the time of birth are especially important for a smooth transition of fetal to neonatal circulation. Understanding the normal transitional physiology and the clinical impact of adverse adaptation is important for delineating pathology so as to guide physiologically relevant therapies. Disorders such as severe perinatal asphyxia, hemodynamically significant patent ductus arteriosus (and its surgical ligation) and utero-placental insufficiency underlying fetal growth restriction, can adversely affect left ventricular (LV) function. The left ventricle is the predominant chamber involved in systemic perfusion during postnatal life. Cardiac output is closely linked to afterload; the latter is determined by arterial properties such as stiffness and compliance. This article outlines normal transition in term and preterm infants. It also highlights the adverse impact of three not uncommon neonatal disorders on LV function. Perinatal asphyxia leads to a reduced LV output, superior vena cava and coronary artery blood flow and an increase in the troponin level. Multiple haemodynamic changes are observed in the premature infant with a large patent ductus arteriosus. They need careful analysis to determine when ligation should proceed. Ligation itself generally results in a dramatic increase in afterload which may lead to a reduction in LV contractility and the need for ionotropic support. Fetal growth restricted infants have a higher systolic pressure, a somewhat hypertrophied heart arising from an increased arterial wall thickness/stiffness and systemic peripheral resistance. Point of care ultrasound (POCUS) helps differentiate normal transition and that resulting from neonatal disorders. It may be increasingly utilized in guiding management.

The five "S's" and the "SNOO" Smart Sleeper-non-pharmacological interventions (NPI) to promote sleep and reduce crying of infants: a scoping review.

Background: Sleeping and crying are normal activities of infants. Infant crying and night wakings can be both distressing and exhausting for parents. At its worse it may be associated with an increased risk of maternal depression and psychosocial stress for both parents. Strategies for reducing crying and improving sleep include the five "S's"-swaddling, side/stomach position, sucking, swinging and shushing sounds simulating "womb-like" sensations. The "SNOO" Smart Sleeper (SNOO), a "smart" bassinet, incorporates 3 of the five "S's", swaddling, swinging (rocking) and emits soothing sounds while demonstrating safe infant sleep practices. This paper explores the effectiveness of the five "S's" and the SNOO. Methods: References for the five "S's" were obtained from various sources while a scoping review of publications from PubMed, Embase and Web of Science was undertaken to seek out relevant studies to document the efficacy of the SNOO. Results: The five "S's" appear to help soothe infants, reduce their crying and improve their sleep. In addition, infant obesity rates fell. Infants also experienced less pain following immunisations. Of the 66 papers gleaned from the database in mid-2021 for the scoping review, only those which provided clear outcomes and conclusions, were complete and related to infants were included. That resulted in only 2 studies that fitted the criteria imposed. They suggested that the SNOO incorporating 3 of the five "S's" had similar beneficial effects. Conclusions: The five "S's" were effective non-pharmacological strategies to help reduce crying and improve sleep in infants. Confounding factors included normal crying of infants, triggers of hunger or tiredness, or recognised causes of crying. The 2 studies reviewed suggested that the SNOO was helpful in reducing crying and improving the sleep duration of normal infants. Further studies have suggested it may be used therapeutically for distressed or ill infants.

Persistent Tachypnoea in Early Infancy: A Clinical Perspective.

Tachypnoea in the newborn is common. It may arise from the many causes of the respiratory distress syndrome such as hyaline membrane disease, transient tachypnoea of the newborn, meconium aspiration etc. Congenital heart disease rarely presents with early tachypnoea on day one or two, in contrast to the early presentation of cyanosis, unless there is "pump" (ventricular) failure such as may occur in a cardiomyopathy/myocarditis, or as a result of severe obstruction to either ventricle. Space-occupying lesions within the chest, for example from a diaphragmatic hernia or a congenital cystic adenomatoid malformation, may present with early tachypnoea, as can a metabolic cause resulting in acidosis. The aim of this paper, however, is to focus on infants where the tachypnoea persists or develops beyond the newborn period, at times with minimal signs but occasionally with serious underlying pathology. They include causes that may have originated in the newborn but then persist; for example, arising from pulmonary hypoplasia or polycythemia. Many congenital cardiac abnormalities, particularly those causing left sided obstructive lesions, or those due to an increasing left to right shunt from large communications between the systemic and pulmonary circulations, need be considered. Respiratory causes, for example arising from aspiration, primary ciliary dyskinesia, cystic fibrosis, or interstitial lung disease, may lead to ongoing tachypnoea. Infective causes such as bronchiolitis or infantile wheeze generally are readily recognisable. Finally, there are a few infants who present with persistent tachypnoea over the first few weeks/months of their life who remain well and have normal investigations with the tachypnoea gradually resolving. How should one approach infants with persistent tachypnoea?

Influence of accelerated arterial aging in growth-restricted cohorts on adult-onset cardiovascular diseases.

Epidemiologists have long documented a higher risk of adult-onset cardiovascular diseases (CVDs) such as stroke, hypertension, and coronary artery disease, as well as mortality from circulatory causes in low birth-weight cohorts (poor in utero substrate supply). Utero-placental insufficiency and in utero hypoxemic state-induced alterations in arterial structure and compliance are important initiating factors for adult-onset hypertension. The mechanistic links between fetal growth restriction and CVD include decreased arterial wall elastin-to-collagen ratio, endothelial dysfunction, and heightened renin-angiotensin-aldosterone system (RAAS). Systemic arterial thickness on fetal ultrasound and vascular changes in placental histopathology in growth restricted cohorts indicate fetal/developmental origins of adult-onset circulatory diseases. Similar findings of impaired arterial compliance have been noticed across age groups (neonates through to adults). Such changes augment what occurs as "normal arterial aging," resulting in accelerated arterial aging. Data from animal models suggest that hypoxemia-associated vascular adaptations enacted in utero are region specific, reflecting long-term vascular pathology. In this review, we explore the influence of birthweight and prematurity on blood pressure and arterial stiffness, demonstrating impaired arterial dynamics in growth-restricted cohorts across age groups, explain how early arterial aging influences adult-onset CVDs, describe pathophysiology data from experimental models and finally, discuss interventions which may influence aging by way of altering various cellular and molecular mechanisms of arterial aging. Age-appropriate interventions which have noted efficacy include prolonged breastfeeding and high polyunsaturated fatty acids dietary intake. Targeting the RAAS seems a promising approach. New data indicate activation of sirtuin 1 and maternal resveratrol may have beneficial effects.

Adolescents and Adults With Congenital Heart Disease: Why Are They Lost to Follow-Up?

Almost 90% of infants with congenital heart disease (CHD) now reach adulthood but require long-term surveillance to recognize and manage residual and/or evolving lesions. Yet many are lost to follow-up. A scoping review identified four specific domains that pose barriers to consistent follow-up. There are multiple issues associated with transition from pediatric to adult care which included-the lack of a seamless transfer, the establishment of a new trusting relationship, promoting the right balance of patient autonomy and addressing knowledge gaps. Additional issues related to logistic problems of time, distance, cost, and the availability of specialized care, are further compounded by the psychosocial factors and the heterogeneity of the cardiac abnormality affecting our patients. Further study of all these issues is warranted to improve ongoing engagement.

How do Adults With Congenital Heart Disease Evaluate Their Clinical Care?

BACKGROUND: Almost 90% of patients with congenital heart disease (CHD) now reach adulthood. How do they evaluate the care they received? METHODS: Adults with CHD (ACHD) recruited for an international multi-center study (APPROACH-IS II) were posed 3 additional "questions" to determine their perceptions of the positive, negative, and areas for improvement of their clinical care. The findings underwent a thematic analysis. RESULTS: Of the 210 recruited, 183 completed the questionnaire, 147 answered the 3 "questions." Most appreciated open communication and support, a holistic approach, continuity of and readily accessible care conducted by experts, and with good outcomes. Less than half reported negative concerns which included loss of autonomy, distress from multiple and/or painful investigations, restricted lifestyles, medication side-effects, and anxiety about their CHD. Others found their reviews time-consuming with long travel times. Some complained of limited support, poor accessibility to services in rural areas, shortage of ACHD specialists, absence of tailored rehabilitation programs, and at times their own as well as their clinicians' limited understanding of their CHD. Suggestions for improvement included better communication, further education about their CHD, availability of simplified written information, mental health and support services, support groups, seamless transition to adult care and providing better prognostications, financial assistance, flexible appointments, telehealth reviews, and greater access to rural specialist care. CONCLUSIONS: In addition to providing optimal medical and surgical care for ACHD, clinicians need to be cognizant of their patients' concerns and proactive in addressing them.

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603.

Cardiorespiratory adaptation to low-dose dexamethasone for lung disease in extremely preterm infants: A prospective echocardiographic study.

The cardiovascular impact of dexamethasone (Dex) is not well understood. Most data are obtained from a 6 week, high-dose regimen, and are limited to findings of hypertension and cardiac hypertrophy. The present study ascertained the impact of low-dose Dex on cardiac indices when administered to extremely preterm infants for lung disease. A pre-post intervention prospective echocardiographic (Echo) study was undertaken, with cardiac assessments performed before and within 24 h after completion of first course of therapy (10 day regimen, cumulative 0.89 mg kg-1 ). Thirty infants with a gestational age of 24.6 ± 1.1 weeks and birthweight of 612 ± 125 g, respectively, were studied. The age at Dex administration was 20 ± 9 days. Fractional inspired oxygen decreased from 0.7 ± 0.23 to 0.35 ± 0.14 (P < 0.001). Patent ductus arteriosus was noted in 20 infants at Echo1. At Echo2, the ductal diameter decreased from 2.16 ± 0.8 to 1.1 ± 0.8 mm (P = 0.0003), with complete closure in 7/20 (35%). A reduction in left pulmonary artery end-diastolic velocity was noted (17 ± 12 to 9 ± 10 cm s-1 , P < 0.001). Pulmonary vascular resistance decreased (increased time to peak velocity/right ventricular ejection time, 0.2 ± 0.03 to 0.23  ± 0.03, P = 0.0001) and right ventricular systolic performance improved (tricuspid annular plane systolic excursion, 4.9 ± 0.8 to 5.5 ± 0.9 mm, P = 0.02). No significant changes in fractional shortening and left ventricular mass were noted. A significant increase in blood pressure was noted. As a percentage of pre-treatment baseline, the mean increase for systolic blood pressure was 20.3% (95% confidence interval = 14-26) on day 2 (P = 0.008). Low-dose Dex influenced cardiovascular parameters related to pulmonary circulation. KEY POINTS: Corticosteroid therapy is frequently used in preterm infants who are dependent on ventilator support. Echocardiographic studies in infants administered a 6 week course of steroids have noted left ventricular hypertrophy, outlet obstruction and hypertension, but no information is available on right heart indices. The cardiopulmonary effects of the current, significantly lesser cumulative dose (10 day regimen, commonly described as 'DART') have not been evaluated. The present study noted a significant influence on ductal and pulmonary circulation indices. Left heart architecture and function was maintained, whereas a significant but transient increase in blood pressure was noted.

Rationale, design and methodology of APPROACH-IS II: International study of patient-reported outcomes and frailty phenotyping in adults with congenital heart disease.

BACKGROUND: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardiovascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II. METHODS/DESIGN: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments. DISCUSSION: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD.

Mitral valve Doppler for cardiac output assessment in preterm neonates.

INTRODUCTION: Cardiac output (CO) assessment in neonates is commonly done by echocardiography. It is unclear which is the best site to measure the left ventricular (LV) outflow tract for CO assessment (the aortic valve [AV] aortic sinus [AS] or the sinotubular junction [STJ]). In the normal heart, the blood flow entering the LV equals the blood ejected from it. Therefore, measuring the blood flow into the LV through the mitral valve (MV) is an alternative way to measure CO. METHODS: In stable preterm infants the MV CO was compared with the right ventricular (RV) CO and the three ways to measure LV CO, in 30 stable preterm neonates. Interobserver variability for MV CO was established. RESULTS: In the 30 neonates studied, MV CO was best correlated and had a minimal bias to the RV CO and LV CO measured at the STJ. Left ventricular CO measured at the AV and AS had significant bias relative to RV CO and MV CO. MV CO inter-observer variability was similar to other echocardiographic CO assessment methods. CONCLUSION: MV CO may be used as an alternative way to assess CO. The STJ may be the optimal site to measure LV outflow tract.

Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries.

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents. Patient-report outcomes were: perceived health status (12-item Short Form Health Survey), quality of life (Linear Analogue Scale and Satisfaction with Life Scale), sense of coherence-13, psychological distress (Hospital Anxiety and Depression Scale), and illness perception (Brief Illness Perception Questionnaire). In this sample, 137 (3.5%) had HF at the time of investigation, 298 (7.5%) had a history of HF, and 3524 (89.0%) had no current or past episode of HF. Patients with current or past HF were older and had a higher prevalence of complex congenital heart disease, arrhythmias, implantable cardioverter-defibrillators, other clinical comorbidities, and mood disorders than those who never had HF. Patients with HF had worse physical functioning, mental functioning, quality of life, satisfaction with life, sense of coherence, depressive symptoms, and illness perception scores. Magnitudes of differences were large for physical functioning and illness perception and moderate for mental functioning, quality of life, and depressive symptoms. Conclusions HF in adults with congenital heart disease is associated with poorer patient-reported outcomes, with large effect sizes for physical functioning and illness perception. Registration URL: https://clinicaltrials.gov; Unique identifier: NCT02150603.

Melody valve insertion for relief of "Tricuspid" stenosis in an unbalanced atrioventricular septal defect.

A Melody valve was successfully placed across a very stenotic right-sided component of a common atrioventricular valve because of ongoing troublesome arrhythmias in a young woman with an unbalanced atrioventricular septal defect, a very dilated right atrium and a hypoplastic right ventricle. Four years later, she remains well.

Fifteen-minute consultation: How to spot serious heart disease in the newborn.

Congenital heart disease (CHD) is common and important as it remains a leading cause of neonatal morbidity and appreciable mortality. Prenatal diagnosis, the presence of a murmur, cyanosis, tachypnoea and/or poor or differential peripheral pulses raise the suspicion of CHD aided by differential pre/postductal saturations. Yet even serious CHD may not be considered when such clues are absent. Nevertheless, there are clinical cues which may alert the clinician to the possibility of a significant CHD which may lead to an early and accurate diagnosis and appropriate intervention to achieve best results. This paper addresses these issues which become especially difficult if caring for infants away from facilities provided by tertiary centres. Tachypnoea on the first postnatal day is generally non-cardiac in origin. Exceptions include large arteriovenous fistulae and/or 'pump' (ventricular) failure. In addition, attention is drawn to two important confounding factors in the newborn, namely the patency of the ductus arteriosus and the initially high pulmonary vascular resistance, both of which alter and may mask the clinical findings of a serious cardiac abnormality. An appreciation of the physiological changes that occur in early infancy will aid the clinician's understanding of CHD as it affects the newborn.

Major Complication Following Kawasaki Disease in an Infant-The Development of Apical Infarction and Aneurysm Formation.

Considerable advances have occurred in the understanding of Kawasaki disease, with a substantial drop in morbidity and mortality following the infusion of gamma globulin during the acute phase. Nevertheless, major complications may still occur. A 27-year-old male presented as an infant of 11 weeks when he was diagnosed as having Kawasaki disease. He was appropriately treated with aspirin and a gamma globulin infusion following his diagnosis 5 days after the onset of his illness. Despite that, he went on to develop coronary aneurysms. He represented a few weeks later with a history of inconsolable crying associated with pallor, suggestive of ischaemic chest pain. A repeat echocardiogram revealed infarction of the apex of the left ventricle with localised thrombus formation. There were persistent aneurysms within both coronary artery systems. A further infusion of gamma globulin was given. In view of the thrombus formation, he was started on warfarin. The thrombus gradually resolved with the development of a clearly defined left ventricular apical aneurysm. He has remained on warfarin, aiming for an international normalised ratio (INR) level of 2 to 2.5. He developed mild left ventricular dysfunction during late childhood, which improved following the commencement of an angiotensin-converting enzyme (ACE) inhibitor. Despite his ventricular aneurysm, there has been no documented evidence of ventricular tachycardia over the years. Repeated testing initially by nuclear perfusion scans and then by stress echocardiograms failed to show any inducible ischaemia apart from the apical ventricular aneurysm. A recent computed tomography (CT) coronary angiogram revealed an ectatic origin of the left main and the right coronary arteries with mild calcification involving the mid-portion of the latter and slight calcification of the former. His raised cholesterol level has responded well to a statin. Despite the persistence of the ventricular aneurysm, he continues to be managed conservatively, as he has remained well. The question arises as to what the long-term implications are of his left ventricle apical aneurysm. Should it be excised? Is he at risk for ventricular tachycardia and sudden death? In addition, although the coronary aneurysms have resolved, the CT coronary angiogram shows calcium plaques in both coronary arteries at the site of the earlier aneurysms. This finding raises the question as to whether all children who develop coronary artery aneurysms following Kawasaki disease should have a CT coronary angiogram performed in adulthood.

Long-term outcomes of right ventricle-to-pulmonary artery conduit insertion in adults with congenital heart disease: survival analysis by National Death Index.

OBJECTIVES: The aim of this study was to investigate the long-term outcomes following right ventricle-to-pulmonary artery (RV-to-PA) conduit insertion of Medtronic Freestyle® porcine valve (MFV) or pulmonary allograft valve (PAV) in adult patients with congenital heart disease. METHODS: Retrospective medical record review of consecutive RV-to-PA conduit insertion, using either PAV or MFV from 1991 to 2017. Perioperative data and clinic reports were collected. Cause and date of death were obtained from the Australian National Death Index to obtain survival function. RESULTS: In total, 232 patients (median age 31.5 years, interquartile range 25-41 years) underwent RV-to-PA conduit insertion (PAV = 84 and MFV = 148) and were eligible for inclusion [63.8% tetralogy of Fallot (TOF); 11.6% congenital pulmonary stenosis (PS); 24.6% other diagnoses]. The overall median follow-up time was 9.1 years (interquartile range 5.3-12.6 years). The mean gradient was 11.8 ± 7.1 mmHg in PAV and 16.6 ± 9.6 mmHg in MFV patients. Congenital PS patients had 100% survival at 20 years, TOF patients at 5, 10, 15 and 20 years had 99%, 97%, 96% and 96% survival, respectively. Patients with other primary diagnoses at 5, 10, 15 and 20 years had 93%, 91%, 87% and 87% respectively. Freedom from reintervention did not differ significantly at 5 and 10 years between pulmonary allograft (98.6%, 98.6%) and Freestyle® porcine bioprosthesis (97.5%, 93%). CONCLUSIONS: Both valves perform equally well with regard to patients' freedom from reoperation, although transvalvular gradient was higher for Freestyle® patients. Congenital PS and TOF patients had better survival than patients with other primary diagnoses.