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Pulmonary Hypertension, An Issue of Cardiology Clinics, E-Book

Pulmonary Hypertension, An Issue of Cardiology Clinics, E-Book

Ronald J. Oudiz

(2016)

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Book Details

Abstract

This issue of the Cardiology Clinics entitled "Pulmonary Hypertension" is an in-depth review on the subject edited by Dr. Ronald J. Oudiz. The topics covered will include: classification of pulmonary hypertension, epidemiology and pathophysiology, diagnosis, and management in Groups I-V. There are also sections devoted to issues faced in pediatrics and pregnancy, as well as a section on patient support, among other topics.


Table of Contents

Section Title Page Action Price
Front Cover Cover
Pulmonary Hypertension\r i
Copyright\r ii
Contributors iii
EDITORIAL BOARD iii
EDITOR iii
AUTHORS iii
Contents v
Preface: Pulmonary Hypertension\r v
Classification of Pulmonary Hypertension\r v
World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology\r v
Pulmonary Arterial Hypertension: Diagnosis and Treatment\r v
Pulmonary Hypertension and Congenital Heart Disease\r v
Group 2 Pulmonary Hypertension: Pulmonary Venous Hypertension: Epidemiology and Pathophysiology\r vi
Group III Pulmonary Hypertension: Pulmonary Hypertension Associated with\rLung Disease: Epidemiology, Pathophysiology, and Treatments\r vi
Group 4 Pulmonary Hypertension: Chronic Thromboembolic Pulmonary Hypertension: Epidemiology, Pathophysiology, and Treatment\r vi
Group 5 Pulmonary Hypertension: The Orphan’s Orphan Disease\r vi
Pulmonary Hypertension in Children\r vii
Special Situations in Pulmonary Hypertension: Pregnancy and Right Ventricular Failure\r vii
Managing the Patient with Pulmonary Hypertension: Specialty Care Centers, Coordinated Care, and Patient Support\r vii
CARDIOLOGY CLINICS\r viii
FORTHCOMING ISSUES viii
November 2016 viii
February 2017 viii
May 2017 viii
RECENT ISSUES viii
May 2016 viii
February 2016 viii
November 2015 viii
Preface: Pulmonary Hypertension\r ix
Classification of Pulmonary Hypertension 359
Key points 359
REFERENCES 361
World Health Organization Group I Pulmonary Hypertension 363
Key points 363
DEFINITION AND CLASSIFICATION 363
EPIDEMIOLOGY 363
The National Institutes of Health Registry 363
Contemporary Pulmonary Arterial Hypertension Registries 365
Epidemiology of Pulmonary Arterial Hypertension in the Modern Era 365
PATHOLOGY 367
The Pulmonary Vasculature 367
The Right Ventricle 367
PATHOGENESIS 369
Pulmonary Vascular Dysfunction 369
Pulmonary Vascular Proliferation 369
Extracellular Matrix Remodeling 369
Inflammation 369
RIGHT VENTRICULAR PATHOLOGIC CHANGES 370
Right Ventricular Metabolic Derangements 370
Right Ventricular Ischemia 371
Right Ventricular Calcium Mishandling 371
Sarcomere Remodeling 371
Cytoskeletal Remodeling 371
GENETIC CAUSES OF PULMONARY ARTERIAL HYPERTENSION 372
SUMMARY 372
REFERENCES 372
Pulmonary Arterial Hypertension 375
Key points 375
INTRODUCTION 375
COLLABORATIVE CARE WITH A PULMONARY HYPERTENSION REFERRAL CENTER 376
DIAGNOSIS 376
Screening 376
General Approach 376
Echocardiography 376
Right Heart Catheterization 378
Vasoreactivity testing 380
Provocative testing 380
Pulmonary Function Tests 380
Overnight Pulse Oximetry and Polysomnography 380
Ventilation Perfusion Lung Scan 380
Computed Tomography 381
Cardiac Magnetic Resonance 381
TREATMENT 381
General Measures 383
Pulmonary Arterial Hypertension Specific Treatments 384
Calcium channel blockers 384
Prostacyclin pathway 384
Prostacyclin analogues 384
Prostacyclin receptor agonist 385
Endothelin receptor antagonists 385
Nitric oxide pathway 385
Phosphodiesterase inhibitors 385
Guanylate cyclase agonists 385
Combination therapy 385
SUMMARY 388
SUPPLEMENTARY DATA 388
REFERENCES 388
Pulmonary Hypertension and Congenital Heart Disease 391
Key points 391
INTRODUCTION 391
UPDATED DEFINITIONS AND CLASSIFICATION 391
ANATOMIC AND PATHOPHYSIOLOGIC FEATURES 392
MEDICAL, CATHETER, AND SURGICAL-BASED STRATEGIES 394
SUMMARY 398
REFERENCES 398
Group 2 Pulmonary Hypertension 401
Key points 401
INTRODUCTION 401
DEFINITIONS 401
EPIDEMIOLOGY 402
COMMON PATHOPHYSIOLOGIC MECHANISMS IN PULMONARY HYPERTENSION INDUCED BY LEFT-SIDED HEART DISEASE 403
The Left Atrium 404
The Pulmonary Veins and Capillaries 404
The Pulmonary Arteries 405
The Right Ventricle 406
HEMODYNAMIC TESTING 407
SUMMARY 407
REFERENCES 408
Group III Pulmonary Hypertension 413
Key points 413
INTRODUCTION 413
EPIDEMIOLOGY 414
Prevalence of Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease 414
Prevalence of Pulmonary Hypertension in Patients with Interstitial Lung Disease 416
Prevalence of Pulmonary Hypertension in Sleep Disordered Breathing 416
Severe Pulmonary Hypertension in Chronic Lung Disease 416
Effect of Pulmonary Hypertension on Prognosis 417
PATHOPHYSIOLOGY 418
TREATMENT OF PULMONARY HYPERTENSION ASSOCIATED WITH LUNG DISEASE 421
Treatment of Pulmonary Hypertension Associated with Chronic Obstructive Pulmonary Disease 421
Endothelin receptor antagonist for pulmonary hypertension associated with chronic obstructive pulmonary disease 423
Phosphodiesterase inhibitors for pulmonary hypertension associated with chronic obstructive pulmonary disease 423
Prostacyclin analogues for treatment of pulmonary hypertension associated with chronic obstructive pulmonary disease 425
Soluble guanylate cyclase stimulators for pulmonary hypertension associated with chronic obstructive pulmonary disease 425
Treatment of pulmonary hypertension Associated with interstitial lung disease 425
Endothelin receptor antagonists for pulmonary hypertension in interstitial lung disease 425
Phosphodiesterase inhibitors for pulmonary hypertension associated with interstitial lung disease 426
Prostacyclin derivatives for pulmonary hypertension associated with interstitial lung disease 427
Soluble guanylate cyclase stimulators for pulmonary hypertension and interstitial lung disease 427
Treatment of Pulmonary Hypertension Associated with Sleep Disordered Breathing 428
SUMMARY 429
REFERENCES 429
Group 4 Pulmonary Hypertension 435
Key points 435
EPIDEMIOLOGY 435
PATHOPHYSIOLOGY OF CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION 437
TREATMENT 439
SUMMARY 440
REFERENCES 440
Group 5 Pulmonary Hypertension 443
Key points 443
INTRODUCTION 443
GROUP 5.1: HEMATOLOGIC DISORDERS 443
Chronic Myeloproliferative Diseases 443
Prevalence/etiology 444
Pulmonary Hypertension in Children 451
Key points 451
INTRODUCTION 451
DEFINITION 451
EPIDEMIOLOGY 452
A. Eisenmenger syndrome 453
B. Left-to-right shunts 453
C. PAH with coincidental congenital heart disease 453
D. Postoperative PAH 453
HIGHLIGHTED CAUSES OF PULMONARY HYPERTENSION 454
Heritable Pulmonary Arterial Hypertension 454
Single-Ventricle Circulation 454
Bronchopulmonary Dysplasia 454
EVALUATION 454
CONVENTIONAL THERAPY 460
VASOREACTIVITY TESTING 460
PHARMACOLOGIC THERAPY OF PULMONARY ARTERIAL HYPERTENSION 461
CALCIUM CHANNEL BLOCKERS 461
PROSTACYCLINS 462
ENDOTHELIN RECEPTOR ANTAGONISTS 463
PHOSPHODIESTERASE-5 INHIBITORS AND SOLUBLE GUANYLATE CYCLASE STIMULATORS 464
COMBINATION THERAPY 465
ATRIAL SEPTOSTOMY AND POTTS SHUNT FOR REFRACTORY PULMONARY ARTERIAL HYPERTENSION 466
TRANSPLANTATION 466
SUMMARY 466
REFERENCES 466
Special Situations in Pulmonary Hypertension 473
Key points 473
PREGNANCY 473
Introduction 473
Hemodynamic Effects of Pregnancy 473
Gestation: a hyperdynamic and hypercoagulable state 473
Peripartum and postpartum: a time of rapid hemodynamic shifts 474
Pulmonary Arterial Hypertension Outcomes in Pregnancy 474
Diagnosis of Pulmonary Arterial Hypertension in Pregnancy 476
Management of Pulmonary Arterial Hypertension in Pregnancy 476
Medical management 476
Calcium channel blockers 476
Phosphodiesterase-5 inhibitors 476
Endothelin receptor antagonists 476
Prostacyclin analogs 476
Soluble guanylate cyclase stimulator 477
Inhaled nitric oxide 477
Anticoagulation 477
Mode of delivery 478
Peripartum and postpartum management 478
Summary 478
RIGHT VENTRICULAR FAILURE 478
Introduction 478
Anatomy and Physiology of the Right Ventricle 479
Pathophysiology of Right Ventricular Failure in Pulmonary Arterial Hypertension 479
Diagnosis of Right Ventricular Failure 479
Physical examination 479
Transthoracic echocardiography 479
Hemodynamic monitoring 481
Management of Right Ventricular Failure in Pulmonary Arterial Hyptension 481
Chronic right ventricular failure management 481
Medical therapy 481
Atrial septostomy 481
Mechanical circulatory support 481
Lung and heart-lung transplantation 482
Acute right ventricular failure management 482
Correction of triggering factors 482
Restoration of oxygenation 482
Maintenance of cardiac output and systemic perfusion 482
Relief of congestion 484
Summary 484
REFERENCES 485
Managing the Patient with Pulmonary Hypertension 489
Key points 489
INTRODUCTION 489
CURRENT CHALLENGES IN THE MANAGEMENT OF PULMONARY HYPERTENSION 490
Centralized Versus Decentralized Care 490
Challenges in Evaluating Pulmonary Hypertension and Diagnostic Errors 490
Challenges with the Management of Pulmonary Arterial Hypertension 490
SPECIALTY CARE CENTERS 492
The Evidence for Team-based Care in Chronic Disease Management 492
Designing the Pulmonary Hypertension Care Center Program: Key Goals and Features 492
Improving Access to Expert Pulmonary Hypertension Care and Supporting Collaboration Among Clinical Programs 493
Promoting Adherence to Published Diagnosis and Management Guidelines, Optimizing the Clinical Management of Pulmonary Arter ... 493
Promoting Pulmonary Hypertension Disease Awareness and Growing the Pulmonary Hypertension Community 494
The Pulmonary Hypertension Care Center Accreditation Process 494
BEYOND THE SPECIALTY CARE CENTER 494
Long-term Comanagement of the Patient with Pulmonary Arterial Hypertension 494
External Organizations 496
SUMMARY 498
REFERENCES 499
Index 501