Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective.

BACKGROUND: Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). METHODS: From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings. RESULTS: Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD. CONCLUSIONS: Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

Pediatric cardiology in India - In search of a holistic solution.

In response to the survey among early career pediatric cardiologists from India and the accompanying editorial, we invited comments and suggestions from thought leaders and senior functionaries in the field. We have summarized the thoughts and suggestions as a mini-symposium.

Hybrid stage I palliation for hypo-plastic left heart condition without a hybrid suite: suggestions for developing nations.

Cardiac hybrid procedures are performed in modern, spacious, and highly equipped hybrid suites in developed countries. Organizing such expensive suites in countries with an emerging economy is difficult from both a financial and logistics point of view. We share our experience of safely performing a Hybrid stage I palliation procedure for Aortic atresia with ventricular septal defect on a 2-month-old infant weighing 3.35 kg using minimal resources in a conventional catheterization laboratory.

Avalanches in cardiology.

Sudden cardiac death (SCD) accounts for 15%-60% of mortality in patients with heart disease. Generally, this has been attributed to ventricular tachyarrhythmia. However, ventricular tachyarrhythmia has been documented or strongly suspected on clinical grounds in a relatively small proportion of SCD patients (8%-50%). Attempted prophylaxis of SCD by implantation of cardioverter-defibrillator is associated with variable success in different subsets of high-risk cardiac patients (30%-70%). A significant number of SCD, therefore, appear to be due to catastrophic circulatory failure. Multiple interdependent compensatory mechanisms help to maintain circulation in advanced cardiac disease. Rapid, unexpected, and massive breakdown of the compensated state can be precipitated by small and often imperceptible triggers. The initial critical but stable state followed by rapid circulatory failure and death has been considered to be analogous to snow avalanches. It is typically described in patients with left ventricular (LV) dysfunction (ischemic or nonischemic). It is now recognized that SCD can also happen in conditions where the right ventricle (RV) takes the brunt of the hemodynamic load. Advanced pulmonary arterial hypertension and operated patients of tetralogy of Fallot with pulmonary regurgitation are of particular interest to pediatric cardiologists. A large amount of data is available on LV changes and mechanics, while relatively little information is available on the mechanisms of RV adaptation to increased load and RV failure. Whether the triggers and the decompensatory processes are similar for the two ventricles is a moot point. This article highlights the currently available knowledge on the pathophysiology of SCD in RV overload states, with special reference to RV adaptive and decompensatory mechanisms, and therapeutic measures that can potentially interrupt the vicious downward course (cardiac avalanches).

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

3D Printing to Model Surgical Repair of Complex Congenitally Corrected Transposition of the Great Arteries.

We report the use of three-dimensional (3D) modeling to plan surgery for physiologic repair of congenitally corrected transposition of the great arteries with pulmonary atresia, dextrocardia, and complex intra cardiac anatomy. Based on measurements made from the 3D printed model of the actual patient's anatomy, we anticipated using a composite valved conduit (Dacron tube graft, decellularized bovine jugular vein, and aortic homograft) to establish left ventricle-to-pulmonary artery continuity with relief of stenosis involving the pulmonary artery confluence and bilateral branch pulmonary arteries.

Religion and spirituality as predictors of patient-reported outcomes in adults with congenital heart disease around the globe.

AIMS: Religion and spirituality can be resources for internal strength and resilience, and may assist with managing life's challenges. Prior studies have been undertaken primarily in countries with high proportions of religious/spiritual people. We investigated (i) whether being religious/spiritual is an independent predictor of patient-reported outcomes (PROs) in a large international sample of adults with congenital heart disease, (ii) whether the individual level of importance of religion/spirituality is an independent predictor for PROs, and (iii) if these relationships are moderated by the degree to which the respective countries are religious or secular. METHODS AND RESULTS: APPROACH-IS was a cross-sectional study, in which 4028 patients from 15 countries were enrolled. Patients completed questionnaires to measure perceived health status; psychological functioning; health behaviors; and quality of life. Religion/spirituality was measured using three questions: Do you consider yourself religious or spiritual?; How important is religion, spirituality, or faith in your life?; and If religious, to what religion do you belong?. The country level of religiosity/secularity was appraised using data from the Gallup Poll 2005-2009. General linear mixed models, adjusting for patient characteristics and country differences were applied. Overall, 49.2% of patients considered themselves to be religious/spiritual. Being religious/spiritual and considering religion/spirituality as important in one's life was positively associated with quality of life, satisfaction with life and health behaviors. However, among patients living in more secular countries, religion/spirituality was negatively associated with physical and mental health. CONCLUSION: Religiosity/spirituality is an independent predictor for some PROs, but has differential impact across countries.

Education as important predictor for successful employment in adults with congenital heart disease worldwide.

BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort. METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models. RESULTS: Median age was 32 years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62). CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD.

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases.

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation.

OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents. PATIENTS: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. OUTCOME MEASURES: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. RESULTS: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αß = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. CONCLUSIONS: The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

Physical Activity-Related Drivers of Perceived Health Status in Adults With Congenital Heart Disease.

Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients.

Patient-reported outcomes in adults with congenital heart disease: Inter-country variation, standard of living and healthcare system factors.

AIMS: Geographical differences in patient-reported outcomes (PROs) of adults with congenital heart disease (ConHD) have been observed, but are poorly understood. We aimed to: (1) investigate inter-country variation in PROs in adults with ConHD; (2) identify patient-related predictors of PROs; and (3) explore standard of living and healthcare system characteristics as predictors of PROs. METHODS AND RESULTS: Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS) was a cross-sectional, observational study, in which 4028 patients from 15 countries in 5 continents were enrolled. Self-report questionnaires were administered: patient-reported health (12-item Short Form Health Survey; EuroQOL-5D Visual Analog Scale); psychological functioning (Hospital Anxiety and Depression Scale); health behaviors (Health Behavior Scale-Congenital Heart Disease) and quality of life (Linear Analog Scale for quality of life; Satisfaction With Life Scale). A composite PRO score was calculated. Standard of living was expressed as Gross Domestic Product per capita and Human Development Index. Healthcare systems were operationalized as the total health expenditure per capita and the overall health system performance. Substantial inter-country variation in PROs was observed, with Switzerland having the highest composite PRO score (81.0) and India the lowest (71.3). Functional class, age, and unemployment status were patient-related factors that independently and consistently predicted PROs. Standard of living and healthcare system characteristics predicted PROs above and beyond patient characteristics. CONCLUSIONS: This international collaboration allowed us to determine that PROs in ConHD vary as a function of patient-related factors as well as the countries in which patients live.

Percutaneous closure of symptomatic large tricuspid paravalvular regurgitation using two muscular VSD occluders.

Paravalvular leaks are common following valve replacement surgery. Majority are benign and do not require any active intervention. However, occasionally severe paravalvular regurgitation can produce heart failure and/or hemolysis, needing closure of the defect. It is more commonly associated with aortic and mitral prosthesis, symptomatic tricuspid paravalvular regurgitation being a rare entity. In this report we present the successful percutaneous transcatheter closure of a large paravalvular tricuspid regurgitation in a 59-year old lady with history of multiple previous operations. The elongated crescent-shaped defect was closed using two muscular VSD devices without any residual leak and without hampering the bio-prosthetic tricuspid valve function. Patient had transient complete heart block during the procedure which recovered later. There was marked improvement in the symptomatic status of the patient at discharge (NYHA IV to NYHA II), which was sustained at follow-up.

Illness perceptions in adult congenital heart disease: A multi-center international study.

BACKGROUND: Illness perceptions are cognitive frameworks that patients construct to make sense of their illness. Although the importance of these perceptions has been demonstrated in other chronic illness populations, few studies have focused on the illness perceptions of adults with congenital heart disease (CHD). This study examined (1) inter-country variation in illness perceptions, (2) associations between patient characteristics and illness perceptions, and (3) associations between illness perceptions and patient-reported outcomes. METHODS: Our sample, taken from APPROACH-IS, consisted of 3258 adults with CHD from 15 different countries. Patients completed questionnaires on illness perceptions and patient-reported outcomes (i.e., quality of life, perceived health status, and symptoms of depression and anxiety). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, functional class, and ethnicity. Linear mixed models were applied. RESULTS: The inter-country variation in illness perceptions was generally small, yet patients from different countries differed in the extent to which they perceived their illness as chronic and worried about their illness. Patient characteristics that were linked to illness perceptions were sex, age, employment status, CHD complexity, functional class, and ethnicity. Higher scores on consequences, identity, and emotional representation, as well as lower scores on illness coherence and personal and treatment control, were associated with poorer patient-reported outcomes. CONCLUSIONS: This study emphasizes that, in order to gain a deeper understanding of patients' functioning, health-care providers should focus not only on objective indicators of illness severity such as the complexity of the heart defect, but also on subjective illness experiences.

Comprehensive training for the future pediatric cardiologist.

India faces a huge burden of pediatric and adult congenital heart diseases (CHDs). Many acquired valvar, myocardial, and vascular diseases also need treatment in childhood and adolescence. The emergence of pediatric cardiology as an independent specialty has been a relatively recent development. A few centers of excellence in pediatric cardiology have developed. However, the requirement of pediatric cardiac care and pediatric cardiologists is far in excess of what is available. There are no guidelines at present in India for uniform training in pediatric cardiology. Many training programs are nonstructured and do not focus on the regional needs. Both core training and advanced training programs are essential to provide adequate numbers of community-level pediatric cardiologists and academic leaders respectively. This article proposes a detailed plan and curriculum for comprehensive training of future pediatric cardiologists in India.

Coarctation of aorta presenting as acute abdominal pain.

Coarctation of aorta is the most common cardiovascular cause of secondary hypertension. The condition is correctable although early diagnosis is often not made. We report the case of a 9-year-old child who was admitted with severe intractable abdominal pain as the presenting symptom of post-subclavian coarctation of aorta with hypertension. His pain resolved after control of hypertension with parenteral antihypertensives and the narcotic analgesics. He subsequently underwent transcatheter balloon dilation of the coarctation of aorta and remains well with normal blood pressure on follow-up.

Vascular rings: an important cause of persistent respiratory symptoms in infants and children.

OBJECTIVE: To study the pattern of symptomatic vascular rings in infants and children with particular reference to clinical features, investigations and management. SETTING: Tertiary Care Paediatric Cardiology Division at the Royal Hospital, Muscat, Sultanate of Oman. DESIGN: Retrospective study. METHODS: Data on all patients aged less than 13 years diagnosed to have symptomatic vascular rings at the Royal Hospital, Muscat, Oman from 1992 to 2001 were retrieved from hospital records. RESULTS: Sixteen patients, 8 males and 8 females, aged 15 days to 36 months were identified, and included 12 with double aortic arch and 4 with right aortic arch, aberrant left subclavian artery and ligamentum arteriosum. Associated intracardiac defects were not found in any. Noisy breathing (7/16), stridor (6/16), respiratory distress (5/16) and recurrent respiratory infections (5/16) were the common modes of presentation. The duration from onset of symptoms to diagnosis ranged from 3 weeks to 24 months. Besides suggestive clinical features, the diagnosis was suspected from the chest radiograph (presence of right aortic arch) in 7 patients and from 2-Dimensional echocardiography and Doppler studies in 5 patients. Barium swallow studies, however, documented the abnormal esophageal indentation in all the 16 patients. Confirmatory imaging techniques included digital subtraction angiography in 7/16, cardiac catheterization and angiography in 5/16, and CT and MRI of the chest in 2 each. All 16 patients underwent successful surgical repair, and 13/16 became asymptomatic. 3 patients continued to suffer from occasional respiratory infections. CONCLUSIONS: A high index of clinical suspicion coupled with the use of barium oesophagraphy enabled early diagnosis in patients with symptomatic vascular rings, and surgical repair could be performed successfully in all patients.

Malignant familial long QT syndrome.

We report a family with congenital long QT syndrome, an inherited disorder of myocardial repolarization in which affected individuals have prolongation of corrected QT interval on the electrocardiogram and a tendency to develop ventricular arrhythmia, leading to syncope, convulsion or sudden death. Our family is characterized by several affected members (11/16), early onset of symptoms, malignant course prior to diagnosis and good response to beta-blocker therapy. The genetic basis of long QT syndrome has been traced to defective proteins encoding cardiac ion channels. Diagnosis is based on an unexplained prolongation of QT interval >0.45 second in the presence of suggestive symptoms or evidence, or both of a familial pattern. Beta-adrenergic blocker therapy gives symptomatic relief in 80%-85% of patients. Precipitating factors like vigorous exercise especially swimming and exposure to significant emotional or auditory stimuli should be avoided. Occasional patients require in addition, a demand cardiac pacemaker, left cardiac sympathectomy or an implantable cardioverter-defibrillator, or both. Regular follow up is mandatory even after subsidence of symptoms.