BOOK
Heart Failure in Adult Congenital Heart Disease, An Issue of Heart Failure Clinics, E-Book
Andrew R Opotowsky | Michael Job Landzberg
(2014)
Additional Information
Book Details
Abstract
This issue of Heart Failure Clinics focuses on Heart Failure in Adult Congenital Heart Disease. As more children with congenital heart disease survive into adulthood, their care is becoming an increasingly important aspect of practice for Cardiologists. In this issue, expert authors review the most current information available about the work-up, diagnosis, and treatment of adult congenital heart disease, including medical therapy and percutaneous and surgical options. Keep up-to-the-minute with the latest developments in the management of heart failure in adult congenital heart disease.
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Heart Failure in AdultCongenital Heart Disease | i | ||
| Copyright\r | ii | ||
| Contributors | iii | ||
| Contents | vii | ||
| Forthcoming Issues | xii | ||
| Adults with Congenital Heart Disease: Growing Pains | xiii | ||
| Evolving Perspectives on Heart Failure in Adults with Congenital Heart Disease | xv | ||
| References | xvi | ||
| The Epidemiology of Heart Failure in Adults with Congenital Heart Disease | 1 | ||
| Key points | 1 | ||
| Introduction | 1 | ||
| Adults with congenital heart disease: how fast are the numbers growing? | 1 | ||
| Heart failure definitions and populations: who is at risk? | 2 | ||
| Scope of the Problem | 2 | ||
| The numbers: what do we know? | 3 | ||
| Obtaining Data | 3 | ||
| Reporting Data | 4 | ||
| The future: how do we get there? | 6 | ||
| Summary | 6 | ||
| References | 6 | ||
| Imaging for the Assessment of Heart Failure in Congenital Heart Disease | 9 | ||
| Key points | 9 | ||
| Introduction | 9 | ||
| Ventricular function and clinical outcomes in ACHD | 11 | ||
| Echocardiography | 12 | ||
| Geometric Versus Nongeometric Assessment of Ventricular Function | 12 | ||
| Speckle-Tracking Echocardiography | 13 | ||
| Diastolic Function | 15 | ||
| Cardiac magnetic resonance imaging | 16 | ||
| Myocardial Deformation Analysis | 17 | ||
| Fibrosis Detection | 18 | ||
| Computed tomography | 18 | ||
| Summary | 19 | ||
| References | 19 | ||
| Exercise Physiology and Testing in Adult Patients with Congenital Heart Disease | 23 | ||
| Key points | 23 | ||
| Impaired exercise tolerance in ACHD: prevalence and special considerations | 24 | ||
| Potential contributors to impaired exercise capacity | 25 | ||
| Exercise testing methods | 25 | ||
| CPET | 25 | ||
| Peak VO2 | 25 | ||
| Heart rate during exercise | 26 | ||
| Oxygen pulse | 26 | ||
| Ventilatory anaerobic threshold | 27 | ||
| VE/VCO2 slope | 27 | ||
| Implications in ACHD patients | 27 | ||
| Exercise Testing with Electrocardiographic Monitoring Without Gas Exchange | 28 | ||
| Implications in ACHD patients | 28 | ||
| The Six-minute Walk Test | 29 | ||
| The prognostic implications of exercise testing in ACHD | 30 | ||
| Summary | 31 | ||
| References | 31 | ||
| Psychosocial Functioning and Quality of Life in Adults with Congenital Heart Disease and Heart Failure | 35 | ||
| Key points | 35 | ||
| Introduction | 35 | ||
| Adults with CHD: psychosocial and QOL considerations | 36 | ||
| Psychosocial Functioning of Adults with CHD | 36 | ||
| QOL of Adults with CHD | 37 | ||
| Adults with heart failure: psychosocial and QOL considerations | 37 | ||
| Psychosocial Functioning of Adults with Heart Failure | 37 | ||
| QOL of Adults with Heart Failure | 38 | ||
| The psychosocial and QOL implications of managing heart failure associated with CHD | 38 | ||
| Clinical recommendations | 38 | ||
| Future research directions | 39 | ||
| Summary | 40 | ||
| References | 40 | ||
| Biomarkers in Adult Congenital Heart Disease Heart Failure | 43 | ||
| Key points | 43 | ||
| Specific pathophysiology in ACHD | 43 | ||
| Assessment of pathophysiology in ACHD | 43 | ||
| Criteria for biomarkers | 44 | ||
| Evidence of biomarkers in ACHD | 44 | ||
| Mixed Group | 44 | ||
| Patients’ characteristics | 44 | ||
| Biomarkers | 44 | ||
| Note | 49 | ||
| PRV Group | 49 | ||
| Patients’ characteristics | 49 | ||
| Biomarkers | 49 | ||
| Note | 52 | ||
| SRV Group | 52 | ||
| Patients’ characteristics | 52 | ||
| Biomarkers | 52 | ||
| Note | 53 | ||
| Fontan Group | 53 | ||
| Diagnostic Tools for Arrhythmia Detection in Adults with Congenital Heart Disease and Heart Failure | 57 | ||
| Key points | 57 | ||
| Introduction | 57 | ||
| Noninvasive electrophysiologic tools | 58 | ||
| The 12-lead Electrocardiogram | 58 | ||
| External Loop Recorders | 58 | ||
| Signal-averaged ECG | 59 | ||
| Heart Rate Variability | 59 | ||
| Microvolt T-wave Alternans | 62 | ||
| Invasive electrophysiologic tools | 62 | ||
| Implantable Loop Recorders | 62 | ||
| Electrophysiologic Testing | 63 | ||
| Pacemakers and ICDs | 64 | ||
| References | 65 | ||
| Electrophysiologic Therapeutics in Heart Failure in Adult Congenital Heart Disease | 69 | ||
| Key points | 69 | ||
| Heart failure and arrhythmias in adult congenital heart disease: scope of the problem | 69 | ||
| Bradyarrhythmias | 70 | ||
| Sinus Node Dysfunction | 70 | ||
| AV Node Dysfunction | 70 | ||
| Atrial tachyarrhythmias | 70 | ||
| Intra-atrial Reentrant Tachycardia | 70 | ||
| Atrial Fibrillation | 71 | ||
| Pharmacologic Therapy for Atrial Arrhythmias | 71 | ||
| Class II and class IV antiarrhythmic agents (AV nodal blocking agents) | 71 | ||
| Class I antiarrhythmic agents | 71 | ||
| Class III antiarrhythmic agents | 72 | ||
| Catheter Ablation for Atrial Arrhythmias | 72 | ||
| Device Therapy for Atrial Arrhythmias | 73 | ||
| Surgical Therapy for Atrial Arrhythmias | 73 | ||
| Ventricular tachyarrhythmias | 73 | ||
| Pharmacologic Therapy for Ventricular Arrhythmias | 73 | ||
| Class II antiarrhythmic agents | 73 | ||
| Class III antiarrhythmic agents | 74 | ||
| Catheter Ablation for Ventricular Arrhythmias | 74 | ||
| Cardiac Rhythm Management Device Therapy for Ventricular Arrhythmias | 74 | ||
| Surgical Therapy for Ventricular Arrhythmias | 78 | ||
| Hemodynamic augmentation with cardiac resynchronization | 78 | ||
| CRT for the Failing LV | 79 | ||
| CRT for the Failing RV | 79 | ||
| CRT for the Failing Single Ventricle | 80 | ||
| Summary | 80 | ||
| References | 80 | ||
| The Exceptional and Far-Flung Manifestations of Heart Failure in Eisenmenger Syndrome | 91 | ||
| Key points | 91 | ||
| Introduction | 91 | ||
| Epidemiology and classification | 92 | ||
| Epidemiology | 92 | ||
| Classification | 92 | ||
| Pathophysiology of the Eisenmenger Syndrome: Cardiac Breathlessness Without Congestive Heart Failure | 93 | ||
| Consequences of chronic hypoxemia | 96 | ||
| Secondary Erythrocytosis and Hematologic Abnormalities | 96 | ||
| Vascular Function | 97 | ||
| Pulmonary and Ventilatory Effects | 97 | ||
| Renal Function and Uric Acid Metabolism | 97 | ||
| Other Selected Miscellaneous Findings | 97 | ||
| Immune and Infectious Issues | 98 | ||
| Coronary Circulation | 98 | ||
| The right ventricle in Eisenmenger syndrome | 98 | ||
| Treatment | 98 | ||
| Future Aspects | 99 | ||
| Summary | 100 | ||
| References | 100 | ||
| Failure of the Fontan Circulation | 105 | ||
| Key points | 105 | ||
| The “Fontan” concept | 105 | ||
| Cardiac output in the Fontan circulation | 106 | ||
| Functional impairment after the Fontan operation | 108 | ||
| The heart and pulmonary vasculature in the Fontan circulation | 109 | ||
| The Heart | 109 | ||
| The Pulmonary Vasculature | 111 | ||
| Secondary complications of the Fontan circulation | 111 | ||
| Liver Fibrosis/Cirrhosis | 111 | ||
| Protein-Losing Enteropathy | 112 | ||
| Plastic Bronchitis | 112 | ||
| Treatment of circulatory failure in Fontan circulation | 113 | ||
| Elevated Systemic Venous Pressure (Increasing Pressure Before the Dam) | 113 | ||
| Impedance and Pulmonary Vascular Resistance (Lowering the Height of the Dam) | 113 | ||
| Ventricular Suction (Enhancing Runoff After the Dam) | 114 | ||
| Fenestration (Bypassing the Dam) | 114 | ||
| Heart Rate, Contractility, and Afterload | 114 | ||
| Mechanical support and heart transplantation | 114 | ||
| Summary | 115 | ||
| References | 115 | ||
| Pregnancy in Women with Heart Disease | 117 | ||
| Key points | 117 | ||
| Introduction | 117 | ||
| Cardiovascular changes during normal pregnancy that contribute to the risk of heart failure | 118 | ||
| Challenges of diagnosing heart failure in pregnancy | 119 | ||
| Maternal global risk assessment | 120 | ||
| Management strategies | 120 | ||
| Multidisciplinary Team | 120 | ||
| Antenatal Care | 121 | ||
| Chronic heart failure | 121 | ||
| Acute heart failure | 122 | ||
| Labor and Delivery | 122 | ||
| Postpartum | 122 | ||
| Lesion-specific risk assessment and principles of management | 122 | ||
| Cardiomyopathy | 122 | ||
| Transposition of the Great Arteries | 123 | ||
| Tetralogy of Fallot | 123 | ||
| Fontan Circulation | 124 | ||
| Eisenmenger Syndrome | 124 | ||
| Aortic Stenosis | 124 | ||
| Idiopathic dilated cardiomyopathy | 117 | ||
| Peripartum cardiomyopathy | 117 | ||
| Hypertrophic Cardiomyopathy | 118 | ||
| Mitral Stenosis | 126 | ||
| References | 127 | ||
| The Tricuspid Valve in Adult Congenital Heart Disease | 131 | ||
| Key points | 131 | ||
| Introduction | 131 | ||
| Embryology, anatomy, and relationships of the tricuspid valve | 132 | ||
| Embryology | 132 | ||
| Anatomy | 132 | ||
| Relationships | 132 | ||
| Clinical assessment | 132 | ||
| History | 132 | ||
| Examination | 134 | ||
| General | 134 | ||
| Vitals | 135 | ||
| Face | 135 | ||
| Neck | 135 | ||
| Precordium | 135 | ||
| Lungs | 135 | ||
| Heart sounds | 135 | ||
| Abdomen | 136 | ||
| Lower limbs | 136 | ||
| Key pathologic entities causing tricuspid disease in ACHD | 136 | ||
| Functional TR | 136 | ||
| ASD | 137 | ||
| VSD | 138 | ||
| AV Canal Defect | 138 | ||
| Ebstein Anomaly | 138 | ||
| Tricuspid Valve Dysplasia | 138 | ||
| Tricuspid Atresia/Hypoplasia | 138 | ||
| Repaired Tetralogy of Fallot/Pulmonary Stenosis | 139 | ||
| Pulmonary Atresia with Intact Ventricluar Septum | 139 | ||
| Systemic RV in Congenitally Corrected Transposition of the Great Arteries, D Transposition of the Great Arteries After Must ... | 139 | ||
| Pulmonary Hypertension | 139 | ||
| Pacemakers and Implantable Cardioverter-Defibrillator | 139 | ||
| Investigations | 140 | ||
| Transthoracic Echocardiography | 140 | ||
| Three-dimensional transthoracic echocardiogram | 141 | ||
| Transesophageal echocardiogram | 141 | ||
| Cardiac MRI | 141 | ||
| Cardiac catheterization | 141 | ||
| Management | 141 | ||
| Management Strategies are Presented in Outline Form, Reflecting Data-driven and Experience-driven Care Guidelines | 141 | ||
| Overall approach | 141 | ||
| Nonsurgical | 141 | ||
| Surgery | 142 | ||
| Indications for tricuspid surgery | 142 | ||
| Surgical approach to tricuspid disease | 143 | ||
| General | 143 | ||
| Techniques of tricuspid valve repair | 144 | ||
| Annuloplasty Techniques | 144 | ||
| Other Repair Techniques | 144 | ||
| Tricuspid Valve Replacement | 145 | ||
| Special situations | 145 | ||
| Ebstein Anomaly | 145 | ||
| Types of repair | 146 | ||
| Systemic RV | 146 | ||
| AV Canal Defect | 147 | ||
| Pacemaker Leads | 147 | ||
| Combined Arrhythmia Surgery | 147 | ||
| Postoperative management | 148 | ||
| Complications | 149 | ||
| Summary | 151 | ||
| Supplementary data | 151 | ||
| References | 151 | ||
| Heart Failure Caused by Congenital Left-Sided Lesions | 155 | ||
| Key points | 155 | ||
| Introduction | 155 | ||
| Aortic valve dysfunction | 155 | ||
| Bicuspid Aortic Valve | 155 | ||
| Other Causes of Congenital Aortic Stenosis | 156 | ||
| Aortic Regurgitation | 156 | ||
| Coarctation of the aorta | 157 | ||
| Anatomy and Associated Lesions | 157 | ||
| Arterial Dysfunction and Ventricular Interaction | 157 | ||
| Ventricular Dysfunction and Heart Failure in CoA | 158 | ||
| Shone complex (syndrome) | 158 | ||
| Anatomy | 158 | ||
| Impact on Left Ventricular Function | 159 | ||
| Left ventricular noncompaction | 159 | ||
| Anatomy and Physiology | 159 | ||
| Diagnosis | 159 | ||
| Clinical Manifestations | 159 | ||
| Treatment | 160 | ||
| Cor triatriatum | 160 | ||
| Anatomy and Definition | 160 | ||
| References | 161 | ||
| Medical Therapy in Adults with Congenital Heart Disease | 167 | ||
| Key points | 167 | ||
| Introduction | 167 | ||
| The HF guidelines: rationale for medical therapy | 168 | ||
| Preventing HF: risk-factor modification | 168 | ||
| Extrapolation from the HF guidelines: initial assessment of the symptomatic patient | 168 | ||
| Diuretics | 168 | ||
| Fluid Retention: Additional Considerations | 170 | ||
| Evaluation of the ACHD patient with HF | 170 | ||
| Medical therapy for HF: left-sided lesions and/or repaired septal defects | 170 | ||
| Use of β-blockers in regurgitant left-sided valvular lesions | 170 | ||
| Right-sided lesions with normal connections | 171 | ||
| HF in repaired tetralogy of Fallot | 171 | ||
| Medical therapy for rTOF with competent pulmonary valve and HFrEF | 171 | ||
| Subaortic (systemic) RV dysfunction in patients with two-ventricle circulation | 171 | ||
| Medical therapy: caution advised | 171 | ||
| β-Blockers | 171 | ||
| ACEI and angiotensin receptor blockers | 172 | ||
| CCTGA: Special Considerations | 172 | ||
| Future needs | 172 | ||
| Single-ventricle circulation | 172 | ||
| HF Caused by Reduced Systemic Ventricular Ejection Fraction | 172 | ||
| HF Caused by the Failing Fontan Circuit and Relatively Preserved Systemic Ventricular Ejection Fraction | 173 | ||
| Management of acute decompensated HF | 174 | ||
| Overcoming challenges: developing an evidence base for management of HF in ACHD | 174 | ||
| References | 175 | ||
| Percutaneous Options for Heart Failure in Adults with Congenital Heart Disease | 179 | ||
| Key points | 179 | ||
| Introduction | 179 | ||
| Lesions predominantly affecting the RV | 180 | ||
| ASDs | 180 | ||
| Transcatheter ASD Closure | 180 | ||
| Indications for transcatheter ASD closure | 180 | ||
| Transcatheter ASD closure devices | 181 | ||
| Safety, efficacy, and complications of transcatheter ASD closure | 181 | ||
| RV Outflow Tract Dysfunction: Pulmonary Outflow Obstruction and PR | 182 | ||
| RVOT Obstruction | 182 | ||
| Congenital pulmonary valve stenosis | 182 | ||
| Postoperative RVOT obstruction | 183 | ||
| PR | 184 | ||
| TPV replacement | 184 | ||
| Hemodynamic and cardiac functional outcomes | 185 | ||
| Functional outcomes | 186 | ||
| Expanded applications and the future of TPV replacement | 186 | ||
| Lesions that predominantly affect the LV | 186 | ||
| VSDs | 186 | ||
| Indications for VSD closure | 187 | ||
| Transcatheter VSD closure | 187 | ||
| Transcatheter VSD closure devices | 187 | ||
| Safety, efficacy, and complications of transcatheter VSD closure | 188 | ||
| PDA | 188 | ||
| Indications for PDA closure | 188 | ||
| Transcatheter PDA closure | 189 | ||
| Safety, efficacy, and complications of transcatheter PDA closure | 189 | ||
| Aortic Coarctation | 189 | ||
| Indication for coarctation repair | 189 | ||
| Transcatheter coarctation stenting | 189 | ||
| Safety, efficacy, and complications of transcatheter coarctation intervention | 190 | ||
| Summary | 191 | ||
| References | 191 | ||
| Surgical Device Therapy for Heart Failure in the Adult with Congenital Heart Disease | 197 | ||
| Key points | 197 | ||
| Incidence of heart failure in adults with congenital heart disease | 197 | ||
| Neurohormonal activation | 198 | ||
| Pharmacologic treatment | 199 | ||
| Device therapy | 200 | ||
| Balloon Pump | 200 | ||
| Impella (Abiomed) | 200 | ||
| TandemHeart Percutaneous Ventricular Assist Device | 200 | ||
| Berlin Heart EXCOR (Pediatric Ventricular Assist Device) | 200 | ||
| Left Ventricular Assist Devices | 201 | ||
| Use of LVADs in ACHD | 201 | ||
| Systemic right ventricles (CC-TGA and D-TGA) | 201 | ||
| Fontan | 202 | ||
| Right Ventricular Assist Devices | 204 | ||
| Antithrombotic therapy strategy | 204 | ||
| Summary | 204 | ||
| References | 204 | ||
| Heart Transplantation in Adults with Congenital Heart Disease | 207 | ||
| Key points | 207 | ||
| Introduction | 207 | ||
| Indications for transplant | 208 | ||
| Transplant evaluation | 209 | ||
| Pulmonary Hypertension | 209 | ||
| Allosensitization | 210 | ||
| Hepatic Function | 210 | ||
| Neuropsychiatric Status and Social Supports | 211 | ||
| Transplant waiting list | 211 | ||
| Transplant operation | 212 | ||
| Outcomes after heart transplant | 213 | ||
| Fontan Transplants | 213 | ||
| Heart-lung transplant | 214 | ||
| Retransplantation | 214 | ||
| Ethical considerations | 214 | ||
| Acknowledgments | 215 | ||
| References | 215 | ||
| Heart Failure in Congenital Heart Disease | 219 | ||
| Key points | 219 | ||
| Introduction | 219 | ||
| Routes to HF in patients with CHD | 220 | ||
| Monogenic causes of HF in cardiomyopathy and CHD | 221 | ||
| Overlapping pathways in CHD and HF genetics | 223 | ||
| The future of personalized treatment of HF in ACHD | 224 | ||
| Summary | 225 | ||
| References | 225 | ||
| Index | 229 |