Clinical and Surgical Aspects of Congenital Heart Diseases : Text and Study Guide

This text and guide discusses the surgical and medical management of congenital heart diseases in both adult and children. It describes the disease, pathology, treatment, complications and follow-up with extensive use of didactic material to educate the reader to the practicalities of the subject. It details the novel research via an extensive literature review, while covering all aspects of the surgical and medical treatment of congenital heart disease. It includes review of the laparoscopic techniques and epidemiology of each disease involved and their prevalence to provide the reader with the full clinical picture. Clinical and Surgical Aspects of Congenital Heart Diseases: Text and Study Guide provides a thorough practical reference for the discipline with the use of illustrations and charts to facilitate understanding. It is essential reading for all trainee and practicing cardiac surgeons, cardiologists and internists, and all interested healthcare professionals. Overview:✓ Structured using simple practical features for all readers to build their understanding of the topic✓ Includes chapter-based MCQs to test the reader into a number of challenging clinical cases to test them✓ Considers each congenital heart defect and disease within the focus of genetics, medicine and surgery Preface Contents Genetic Basis of Congenital Heart Disease Introduction Aneuploidy Trisomy 21 (Down Syndrome) Monosomy X (Turner Syndrome) Copy Number Variations 22q11.2 Deletion Syndrome Other Common CNVs Single-Gene Variations Syndromic Single-Gene Variations Alagille Syndrome Holt–Oram Syndrome Noonan Syndrome Nonsyndromic Single-Gene Variations References Epidemiology of Congenital Heart Diseases Introduction Prevalence Etiology Mortality and Survival Rate Classification of Congenital Heart Disease Acyanotic Congenital Heart Disease Ventricular Septal Defects Atrial Septal Defects Patent Ductus Arteriosus Cyanotic Congenital Heart Disease Tetralogy of Fallot Transposition of the Great Arteries Adult Congenital Heart Disease References Diagnosis of CHD During Perinatal Life Introduction General Considerations for a Fetal Cardiac Scan Views of a Fetal Cardiac Scan Basic Fetal Cardiac Scan Four-Chamber View of the Heart Left Ventricular Outflow Tract View Right Ventricular Outflow Tract View Fetal Congenital Heart Diseases Conotruncal Anomalies Right Heart Defects Anomalies of the Left Heart Septal Defects References Ventricular Septal Defect Introduction Pathophysiology Clinical Features Presentation Diagnosis Differential Diagnosis Management Overview Neonates Small VSD Moderate to Large VSD Closure Interventions References Atrial Septal Defect Introduction Pathophysiology Diagnosis Clinical Manifestations and Physical Findings Electrocardiogram Chest Radiograph Imaging-Based Diagnosis Transthoracic Echocardiography Transesophageal Echocardiography Cardiovascular Magnetic Resonance, Computed Tomography and Cardiac Catheterization Management Conclusion References Patent Ductus Arteriosus Introduction Anatomy Pathophysiology Functional Closure Epidemiology Prognosis Morbidity Mortality Signs and Symptoms Diagnosis of PDA Echocardiography Chest Radiography Electrocardiography Angiography and Catheterization Managements Indomethacin Ibuprofen Diuretics Catheterization Amplatzer Duct Occluder Rashkind Ductus Occlusion Device Surgical Ligation Hospitalization References The Coarctation of Aorta Introduction Pathophysiology Diagnosis History Physical Examination Arteril Pulse Ausculation ECG X-Ray Echocardiogram Management Early Management Surgical Treatment Late Management Medical Treatment Procedure Follow-Up References Truncus Arteriosus Introduction Pathophysiology Etiology Diagnosis Prenatal Diagnosis Postnatal Diagnosis Investigations Differential Diagnosis Management Palliative Treatment Corrective Treatment Postoperative Period Prognosis Follow-Up References Tricuspid Atresia Introduction Epidemiology Embryology and Genetic Background Pathogenesis Classification Clinical Presentation Diagnosis Prenatal Diagnosis Postnatal Diagnosis Management and Treatment Outcome and Follow-Up Conclusion References Teratology of Fallot (TOF) Introduction Epidemiology Pathophysiology Embryonic Aspect of TOF Anatomical Aspects of TOF Hemodynamic Aspect of TOF Genetic Aspect of TOF Embryological Aspect of TOF Associated Anomalies Clinical Features Physical Examination Hypercyanotic Spells Diagnosis Differential Diagnosis Management Treatment of Hypercyanotic Spells Medical Treatment of TOF Surgical Treatment of TOF Surgery of TOF in Adults Time of Surgery Procedure of Surgery Choosing the Type of Valve (Bioprosthetic or Mechanical Valve) Using of AICD (Automatic Implantable Cardioverter-Defibrillator) Radiofrequency Ablation Postoperative Monitoring Outcomes of Surgery Complications of Surgery Prophylaxis for Infective Endocarditis Secondary prevention Patient Education Reoperation Prognosis Follow-Up Complications of TOF References Total Anomalous Pulmonary Venous Return Introduction Etiology Darlings Classification Supracardiac Total Anomalous Pulmonary Venous Return Cardiac Total Anomalous Pulmonary Venous Return Infracardiac Total Anomalous Pulmonary Venous Return Mixed-Type TAPVC Signs and Symptoms Diagnosis Treatment for Total Anomalous Pulmonary Venous Return Follow-Up Care for TAPVR References Multiple Malformations Introduction Cantrell Pentalogy Classification Etiology and Incidence Pathogenesis Diagnosis Clinical Characteristics Associated Anomalies Treatment Shone Syndrome Etiology and Prevalence Diagnosis Clinical Features Cor Triatriatum Supramitral Ring Parachute Bicuspid Valve Aortic Coarctation Hypoplastic Aortic Arch Subaortic Stenosis Bicuspid Aortic Valve Hypoplastic Left Ventricle Scimitar Syndrome Epidemiology Classification Etiology Clinical Presentation Diagnosis Differential Diagnosis Treatment Complications References Ectopia Cordis Introduction Associated Anomalies Etiology Classification Genetic Basis of EC Prognosis of EC Differential Diagnosis of EC Diagnosis of EC Management of EC Surgery The Procedure of Surgery Outcomes of Surgery References Aortic Stenosis (AS) Introduction Epidemiology Anatomy of the Aortic Valve Etiology Valvular Aortic Stenosis Supravalvular Subvalvular Pathophysiology Adult Presentation Syncope Neonatal Presentation Diagnosis Physical Examination ECG Echocardiography Stress Test Findings on Auscultation Management Medical Management Surgical Management References Atrioventricular Septal Defect Introduction Pathophysiology Types Anatomy Embryology Clinical Presentation Diagnosis Prenatal Diagnosis Postnatal Diagnosis Imaging Studies Chest Radiography Doppler Echocardiography Electrocardiography Diagnostic Procedures Cardiac Catheterization Treatment Medical Therapy Surgical Therapy Follow-Up Postoperative Complications, Outcome, and Prognosis Conclusion References Bicuspid Aortic Valve Introduction Background Embryology Bicuspid Aortic Valve Morphology Epidemiology Clinical Presentation and Diagnosis Imaging Studies Chest X-Ray Echocardiography Angiography Transesophageal Echocardiography Treatment Plans (Medical, Surgical, Diet, Activity) Associated Pathologies and Their Outcome Aortic Stenosis Aortic Regurgitation Patent Ductus Arteriosus Supravalvular AS and William’s Syndrome Ventricular Septal Defect Bacterial (Infective) Endocarditis The Bicuspid Aortic Valve and Aortic Wall Abnormalities Coarctation of the Aorta (COA) Aortic Root Dissection Aortopathy Survival Rate Conclusion References Cardiomyopathies Introduction Dilated Cardiomyopathy Epidemiology Etiology (Table 1) Genetic Causes of DCM Clinical Manifestations Diagnosis ECG Echocardiography Cardiac Magnetic Resonance Imaging Myocarditis and DCM Peripartum Cardiomyopathy (PPCM) Stress-Induced Cardiomyopathy Drug-Induced Cardiomyopathy Management Restrictive Cardiomyopathy “RSC” Etiology (Table 2) Loeffler Endocarditis Treatment and Management Amyloidosis Diagnostics ECG Echocardiography Cardiovascular Magnetic Resonance (CMR) Treatment Hypertrophic Cardiomyopathy (HOCM) Pathogenesis Clinical Manifestations Diagnosis Treatment Mitral Valve Replacement Pacemaker Implantation Catheter Septal Ablation Alcohol Septal Ablation Implantable Cardioverter Defibrillator (ICD) Cardiac Transplantation References Complete Heart Block (CHB) Introduction Physiology Epidemiology Etiology History and Physical Examinations Diagnoses Differential Diagnosis Complications and Prognosis Treatment/Management References Dextrocardia Introduction Definitions Epidemiology Etiology Pathophysiology Clinical Presentation Symptoms Sign Evaluation of Physical Examination Assessment on Investigations Treatment and Management Medical Surgical Complications Prognosis Counseling and Educating Parents Differential Diagnosis References Double Inlet Left Ventricle Introduction Causes Pathophysiology Prognosis Complications Atrial Tachyarrhythmias Thromboembolism Malformations of the Pulmonary Arteriovenous System Plastic Bronchitis Systemic-to-Pulmonary Arterial Collaterals Are Formed Clinical Features History Physical Examination Differential Diagnosis DD of Double Inlet Left Ventricle Classification DILV (Fig. 1) Diagnosis Laboratory Investigations Imaging Two-Dimensional Echocardiography and Doppler Analysis Chest X-Ray ECG Holter Placement MRI Catheterization Treatment Surgical Procedures Complications of the Fontan Procedure Include Aortic Arch Blockage or Stenosis of the Pulmonary Outflow Tract The Physiologic Sensitivity of Infants Treated with a Norwood-Type Operation Was Reduced Creating a Cavopulmonary Circulatory System Transplantation of the Heart Prevention Difference in Between the Double Outlet RV and the Double Inlet LV References Double Outlet Right Ventricle Introduction Pathophysiology Subaortic VSD Type Subpulmonary VSD Type Doubly Committed VSD Type Noncommitted VSD Type Clinical Features Diagnosis Echocardiography Cardiac Angiography Magnetic Resonance Imaging (MRI) Chest Radiography Computed Tomography (CT) Scanning Management Indications for Surgery Contraindications for the Surgery Preoperative Assessment Intraoperative Assessment Repair of DORV with Subaortic VSD Subtype Subpulmonary VSD Type Repair Doubly Committed VSD-Type Repair Noncommitted VSD Repairs Outcome and Prognosis References Ebstein’s Anomaly Introduction Associated Defects with EA Associated Defects Teratogens and the Role of Genetics in EA Syndromic EA Could Be Due To Pathophysiology of EA Clinical Manifestation Diagnosis Appearance and Physical Examination Show While Imaging Techniques Include Treatment Medical Management of Patients with EA Indication for Surgical Repair Surgical Procedures Biventricular Repair Procedures Technique of Knott–Craig Monocusp da Silva Cone Repair Procedure Prognosis References Hypoplastic Left Heart Syndrome Background Postnatal Anatomy of a Human Heart Functionalities of the Four Heart Chambers Anatomy of the Fetal Heart Heart of a Newborn Right Heart Left Heart What Is HLHS? Epidemiology of the HLHS Genecology and Genealogy of HLHS Genecology Genealogy Morphology and Pathogenesis of HLHS Management of the Disease Diagnosis of the HLHS Prenatal Diagnosis At Birth Diagnosis Postnatal Diagnosis Surgical Intervention Prenatal Surgeries Postnatal Surgeries Stage-1: Palliative Treatment: Norwood Operation Blalock Taussig Fistula Right Ventricle to Pulmonary Artery Conduit Reconstruction of the Aortic Arch Tricuspid Regurgitation and Right Ventricular Dysfunction Stage-2 Palliative Treatment: Glenn Procedure/Hemi-Fontan Stage-3: Modified Fontan Procedure Heart Transplant Hybrid Procedure Hybrid Interstage 1 Monitoring and Pharmacology Further Reading for Surgical Intervention References Hypoplastic Right Heart Syndrome Introduction Etiology Pathogenesis Clinical Manifestation Physical Examination Diagnosis Management Surgical Management Blalock-Taussig Shunt Glenn Procedure Fontan Procedure Conclusion References Mitral Stenosis Introduction Epidemiology Pathological Anatomy Pathophysiology Clinical Features Mitral Valve Classification of the Stenosis Severity [15] Physical Examination General Inspection Auscultation Diagnosis Transthoracic 2D Echocardiography Chest X-Ray Trans-Esophageal Echocardiogram (TEE) Laboratory Test Cardiac Catheterization Prognosis Complication Treatment Non-pharmacological Treatment Medical Treatment The Prevention of Rheumatic Fever Surgical Treatment Surgical Valvotomy and Valve Replacement Balloon-Expandable Valve Implantation References Rhabdomyoma Introduction Epidemiology Etiology Molecular/Genetics Pathologic Features Gross Features Microscopic Features Clinical Features Prognosis and Predictive Factors Presentation Radiological Features Management Complications References Transposition of the Great Artery Introduction Pathophysiology Diagnosis History Physical Appearance Arterial Pulse Jugular Venous Pulse Palpation Auscultation ECG Imaging X-Ray Echo Management Initial Management Surgery Arterial Switch Procedure Rastelli Procedure Atrial Switch Procedure Complication Follow-up References Wolff–Parkinson–White Syndrome Introduction Epidemiology Etiology Pathophysiology Clinical Appearance Diagnosis Differential Diagnosis Management of a Patient with the WPW Syndrome Acute Management of a Symptomatic Patient with the WPW Syndrome Chronic Treatment to Prevent Recurrent Arrhythmias Transcatheter Ablation (TCA) Management of Asymptomatic Patients with the WPW Syndrome Surgical Ablation and Perioperative Management Sports Eligibility Conclusion References Laparoscopic Surgery of Congenital Heart Introduction How Are Congenital Heart Defects Treated? Patent Ductus Arteriosus Repair Pulmonary Valve Stenosis Repair Coarctation of Aorta Repair Atrial Septal Defect (ASD) Repair Ventricular Septal Defect (VSD) Repair Tetralogy of Fallot Repair Transposition of the Great Vessels Repair Truncus Arteriosus Repair Tricuspid Atresia Repair Total Anomalous Pulmonary Venous Return (TAPVR) Correction Hypoplastic Left Heart Repair In Open-Heart Surgery the Surgeon May Topics Related to Surgery Robotically Assisted Heart Surgery: New Approaches to Minimally Invasive Heart Surgery What Are the Benefits of Robotically Assisted Surgery? References Perfusion in Congenital Heart Surgery Introduction The Parts of the Cardiopulmonary Bypass Circuit Pumping Heater-Cooler System Cannulae Primers for Congenital Surgery The Role of Heparin Blood and Gas Management Carbon Dioxide Management pH and Blood Gas Management References