Guide for Advanced Nursing Care of the Adult with Congenital Heart Disease

Description

The aim of this book is to provide one central resource for nurses within the adult spectrum of life-long congenital heart disease care who are seeking expert guidance for their practice, regardless of clinical setting. Over the past 50 years, advances in surgical techniques and medical therapies have drastically improved the number of congenital heart disease patients surviving into adulthood, with the result being that there are now more adults then children living with congenital heart disease. In the past three decades, recognition of this new cardiology subspecialty has given way to formalized programs, standards of care, and multidisciplinary expertise. Indeed Nursing care of adult patients with congenital heart disease (ACHD) is a relatively new medical subspecialty with limited knowledge and guidance available and also an important component of the multidisciplinary care team. Nursing care of the ACHD encompasses a holistic approach to the physical, psychological, social, and spiritual wellbeing of these unique individuals across their lifespan. Understanding the intricacies for the various heterogeneous defect types, the transition from pediatric to adult care, the unique educational and self-care needs, life-events such as pregnancy/reproduction, advanced heart failure, and end-of-life care helps prepare the nurse caring for the ACHD patient. Nurses as a first point of care for the ACHD patients play a pivotal role in the education and empowerment of the ACHD patient population and provide an invaluable role in the multidisciplinary team and with this guide nurses can feel confident in the quality of the care they provide.  This book aims to introduce nursing focused care to wider audiences, nurses, medical technicians, and physicians who are involved in the management and treatment of ACHD patients. Improving care and the quality of life for adult congenital heart disease patients with a multidisciplinary team-based approach, including nursing care, should be a central goal for all ACHD programs.  Foreword Contents Part I: Epidemiology, Anatomy and Pathophysiology 1: Incidence of Congenital Heart Disease and Relevance in Adulthood 1.1 Epidemiology of Congenital Heart Disease References 2: Cardiac Defects 2.1 Atrial Septal Defect 2.1.1 Clinical Presentation 2.1.2 Management 2.1.3 Complications 2.2 Ventricular Septal Defect 2.2.1 Clinical Presentation 2.2.2 Management 2.3 Atrioventricular Septal Defects 2.3.1 Clinical Presentation 2.3.2 Management 2.4 Left Ventricle Outflow Tract Obstructions 2.4.1 Clinical Presentation 2.4.2 Management 2.5 Coarctation of the Aorta 2.5.1 Clinical Presentation 2.5.2 Management 2.5.3 Complications 2.6 Patent Ductus Arteriosus 2.7 Clinical Presentation 2.7.1 Management 2.7.2 Complications 2.8 Transposition of the Great Arteries (D-TGA) 2.8.1 Clinical Presentations 2.8.2 Management 2.8.3 Complications 2.9 Tetralogy of Fallot and Right Ventricular Outflow Tract Disorders 2.9.1 Clinical Presentation 2.9.2 Management 2.9.2.1 Palliative Surgery 2.9.2.2 Corrective Surgery 2.9.3 Complications 2.10 Residual Right Ventricular Outflow Tract Obstruction 2.11 Endocarditis 2.11.1 Sudden Cardiac Death 2.12 Pulmonary Atresia 2.12.1 Clinical Presentation 2.12.2 Management 2.12.3 Complications 2.13 Single Ventricle and Fontan Circulation 2.13.1 Clinical Presentation 2.13.2 Surgical Procedure 2.13.3 Complications References Part II: Transition from Pediatric to Adult Care 3: Transitional Care for Young People 3.1 Background 3.2 What Is Transition? 3.3 Process and Stage of Transition 3.4 Patient Education and Empowerment 3.5 Pregnancy, Contraception and Family Planning 3.6 Exercise and Participation in Sports 3.7 Preventing the Loss to Follow-up 3.8 Transition Information Day 3.9 Charities Involvement 3.10 Psychological Aspect of Transition and Challenges 3.11 Established Relationships with Paediatric Teams/Involving Parents and Carers 3.12 Role of Family/Carers in the Transition 3.13 Role of the ACHD CNS in Supporting Young Adults with CHD 3.14 Executive Summary References 4: Advancing High-Value Transitional Care: The Central Role of Nursing and Its Leadership 4.1 Introduction 4.2 Focus on Holistic Care in Nursing 4.3 Holistic Needs of Transitional Care 4.4 Nursing Leadership in Transitional Care 4.5 Conclusions References 5: Building a Transition Program 5.1 Factors That Impact the Structure of a Transitional Program 5.2 Models of Transitional Programs 5.2.1 Methods of Transitional Education Delivery 5.2.2 Timing of Transitional Care and Transfer of Care 5.3 Structure of a Transition Clinic 5.4 Domains of Assessment 5.4.1 Transition Readiness 5.4.1.1 Mental Health 5.4.1.2 Health Literacy 5.4.1.3 Educational Preferences 5.4.1.4 Family and Social Support 5.4.1.5 Health-Related Beliefs 5.4.2 Health Knowledge 5.4.3 Self-Care Assessment 5.4.4 Care Preferences 5.4.5 Future-Oriented Assessment 5.4.6 Diagnosis and Planning 5.4.7 Intervention Planning 5.4.8 Transitional APRN Interventions 5.4.9 Implementation 5.4.10 Evaluation 5.4.11 Future Nursing Research Opportunities 5.5 Conclusion References Part III: The Role of the Advance Nurse Practitioner in the Care of ACHD Patients in Different Care Setting 6: Nursing Care in the Outpatient and Hospital Setting 6.1 Specialist Role 6.2 Outpatient Setting 6.3 Specific Lesions 6.3.1 Valve and Outflow Tract Lesions 6.3.1.1 Left Heart 6.3.1.2 Right Heart 6.3.2 Septal Defects 6.3.3 Eisenmenger Syndrome 6.3.4 Aortic Lesions 6.3.5 Transposition Complexes 6.3.6 Tetralogy of Fallot/Pulmonary Atresia with VSD and MAPCA 6.3.7 Univentricular Circulation/Fontan 6.4 Inpatient Setting 6.4.1 Pre-assessment 6.4.2 Admission 6.4.3 Assessment 6.4.4 Oxygen 6.4.5 Medical Admission 6.4.6 Surgical Intervention 6.4.7 Catheter Intervention 6.4.8 Psychological Support 6.4.9 Discharge References 7: Nursing Care in the Cath Lab 7.1 Cath Lab 7.1.1 Indication to Heart Catheterization 7.2 Role of a Cath Lab Nurse 7.3 Operative Room Characteristics 7.4 Room Preparation 7.5 Procedure’s Management 7.5.1 Patient Preparation 7.5.2 Sterile Trolley Preparation (Fig. 7.3) 7.5.3 Cath Lab Tools 7.5.4 Interventional Procedures 7.5.5 Nurses During the Procedure 7.5.6 Post-procedural Monitoring 7.6 Tools Management References 8: Nursing Care in the Critical Setting 8.1 Pediatric vs. Adult ICU 8.2 Hemodynamic Monitoring 8.2.1 Non-invasive Monitoring 8.2.2 Invasive Monitoring 8.2.2.1 Types of Invasive Monitors 8.3 Fluid Resuscitation 8.3.1 Special Considerations 8.4 Ventilation and Sedation 8.5 Arrhythmia Management 8.6 Anticoagulation 8.6.1 Endocarditis 8.6.2 Pain Management 8.6.3 Cardiac Rehabilitation 8.6.4 Nutrition References Part IV: Advanced Heart Failure in ACHD 9: Management of Acute Heart Failure 9.1 Introduction 9.2 Epidemiology of Heart Failure in CHD 9.3 Pathophysiology 9.3.1 General Concepts and Circulatory Models 9.3.2 Phenotypes of HF in CHD 9.3.3 Main Differences of HF in Acquired Vs Congenital Disease 9.4 Management 9.4.1 General Principles 9.4.2 Practical Management Multistep Algorithm of Acute HF in CHD 9.5 Key Learning Points References 10: Mechanical Circulatory Support Strategies 10.1 Introduction and Definition 10.2 Indications and Classification 10.3 MCS Modalities 10.3.1 ECMO 10.3.2 VAD 10.3.3 TAH 10.3.4 Impella and Tandem Heart 10.4 MCS Reports and Outcomes in ACHD Patients with Biventricular Physiology 10.5 MCS in IN ACHD Patients with Single-Ventricle Physiology References 11: Heart Transplantation: The Challenging Journey of an ACHD 11.1 Congenital Heart Disease and Heart Failure 11.2 Cardiac Surgery for End-Stage Heart Failure in ACHD 11.3 Heart Transplant and Listing 11.4 Transplant Eligibility and Listing 11.4.1 When Is a Heart Transplant Performed? 11.4.2 Crossmatch 11.5 Heart Transplant Surgery: Nursing Management 11.5.1 Preoperative Phase 11.5.2 Heart Transplant 11.6 Postoperative Phase in the Intensive Care Unit 11.6.1 Vasoactive Medications 11.6.2 Nitric Oxide 11.6.3 Extracorporeal Membrane Oxygenation 11.7 Postoperative Phase in Transplant Unit Ward 11.8 Complications during Hospital Stay 11.9 Clinical and Home Rehabilitation 11.9.1 Posttransplantation Management 11.9.2 Follow-Up References Part V: Adult Congenital Heart Disease and Pregnancy 12: Congenital Heart Disease During Pregnancy 12.1 Introduction 12.2 Hemodynamic Changes During Pregnancy and Delivery 12.3 Assessment of Maternal and Fetal Risk and Prediction of Outcome 12.3.1 Pulmonary Hypertension 12.3.2 Fontan Circulation (Total Cavopulmonary Connection) 12.3.3 Hereditary Aortopathies 12.3.4 Congenital Heart Diseases with Systemic Right Ventricle 12.3.5 Cyanotic Congenital Heart Diseases 12.4 The “Pregnancy Heart Team” 12.5 Medications 12.6 Cardiovascular Complications Pregnancy-Related 12.7 Delivery Planning 12.8 Follow-Up after Delivery (Postpartum and Long-Term Follow-Up) References 13: Contraception, Family Planning, and Pregnancy Management: The Nurse Specialist Role 13.1 Family Planning and Contraception Counseling 13.1.1 Contraception Counseling 13.1.2 Barriers to Contraceptive Use 13.1.3 Contraceptive Options 13.2 Tier 1: Most Effective (1% or Less Chance of Failure with Typical Use) 13.2.1 Intrauterine Devices (IUDs) 13.2.2 Progestin Subdermal Implant 13.2.3 Female and Male Sterilization 13.3 Tier 2: Less Effective (6–12% Chance of Failure per Year with Typical Use) 13.3.1 Progestin-Only Injectables 13.3.2 Progestin-Only Pills 13.3.3 Combined Hormonal Contraceptives 13.3.4 Emergency Contraception 13.4 Tier 3: Least Effective (18–28% Failure Rate per Year with Typical Use) 13.4.1 Barrier Methods, Spermicide, Diaphragm, Sponge, Fertility Awareness, and Withdrawal 13.5 Prepregnancy Counseling 13.5.1 Fetal Risk 13.6 Genetic Counseling 13.7 The Clinical Assessment 13.7.1 Medical and Cardiac History 13.7.2 Physical Assessment 13.7.3 Normal Signs and Symptoms in Pregnancy 13.8 Diagnostic Testing 13.9 Pregnancy Management: The Nurse Specialist Role 13.9.1 Antepartum Care 13.9.2 Delivery Plan References Part VI: Physical Activity and Sport 14: Rehabilitation’s Point Break: Changing for Improving 14.1 Introduction 14.2 Phases 1 and 2 14.3 Exercise Prescription 14.4 Phases 3 and 4 References 15: Promotion of Daily Physical Activity and Exercise for Adults with Congenital Heart Disease 15.1 Background 15.2 Physical Aspect 15.3 Social Aspect 15.4 Psychological Aspect/Quality of Life (QoL) 15.5 Musculoskeletal Benefit 15.6 Respiratory Function Benefit 15.7 The Fontan Circulation 15.8 Impact of COVID-19 15.9 Impact of Technology 15.10 Nursing Assessment 15.11 Role of the ACHD CNSs 15.12 Executive Summary References Part VII: Patient Self-Care and Nursing Role for Patients’ Education 16: Connecting Dots for Framing Health: The Self-Care Process 16.1 Introduction 16.2 What Is Self-Care? 16.3 Why Self-Care Is Important? 16.4 What Is Known About Self-Care in Adults with Congenital Heart Defects? 16.5 Conclusion References 17: Patient Education to Self-Management 17.1 Patient Education to Self-Management: Education to Pharmacological Therapy 17.2 Patient Education to Self-Management: Education to Lifestyle Habits 17.2.1 Heart-Healthy Nutrition 17.2.2 Physical Activity 17.2.3 Smoking and Tobacco Use 17.2.4 Substance Use, Including Alcohol, Cannabis, and Illegal Drugs 17.2.5 Body Art (Tattooing and Piercing) 17.3 Patient Education to Self-Management: Infective Endocarditis 17.3.1 Major Criteria 17.3.2 Minor Criteria 17.3.3 Definite Infective Endocarditis References 18: The Health Engagement of Adult Congenital Heart Disease Patients 18.1 Introduction 18.2 What Is Health Engagement? 18.3 Health Engagement of Adults with Congenital Heart Disease References 19: The Essential Impact of Counseling in Improvement of Quality of Life in ACHD Patients 19.1 Introduction 19.2 Health-Related Quality of Life 19.3 CHD Complexity and Functional Status 19.4 Psychological Distress 19.5 Social Challenges 19.6 Sense of Coherence 19.7 Counseling in ACHD 19.8 Counseling in Transitional Age References Part VIII: End-of-Life Care: Nursing Care in the Final Stage of Life 20: Advance Care Planning and Advance Directives 20.1 Part 1: Developing a Comprehensive Advance Care Planning Program 20.1.1 Introduction 20.1.2 When to Have these Conversations with Patients 20.1.3 What Is the APP/RN Role 20.1.4 Model of Delivery of Discussions in Outpatient Clinic 20.1.5 The Serious Illness Conversation Guide 20.1.6 Patient Selection 20.1.7 Having the Conversations 20.1.8 Post Conversation Nursing Assessment and Follow-Up 20.1.9 Tracking 20.1.10 Referral to Palliative Care and Hospice 20.2 Part 2: Advance Directives 20.2.1 What Is an Advance Directive 20.2.2 Who Should Complete an Advance Directive 20.2.3 Why Complete an Advance Directive? Why Is It Important? 20.2.4 When to Complete an Advance Directive 20.2.5 How Is an Advance Directive Completed/Initiated by the ACHD Provider 20.2.6 Nursing Assessment 20.2.7 Future Nursing Research Opportunities 20.3 Conclusion References Part IX: Coronavirus Disease 2019 (COVID‐19) Pandemic Implications in ACHD 21: COVID-19 and Adult Congenital Heart Disease 21.1 Introduction 21.2 Basic Characteristics of SARS-COV-2 Infection and Its Management 21.3 Potential Impact of SARS-COV-2 on the Heart 21.4 Resilience and Response of Adult Patients with Congenital Heart Disease During the COVID-19 Pandemic 21.5 Nursing Role During the COVID-19 Pandemic 21.6 Conclusion References