Julien Hoffman 
The Natural and Unnatural History of Congenital Heart Disease 

* Evaluates the natural history of congenital heart lesions as a
background to finding out if and how much treatment has improved
outcomes

* Introduces and defines lesions, providing general information
about its frequency, familial or syndromic associations, and
associated congenital heart lesions

* Provides sections on pathological anatomy and physiology
– important in determining outcomes

* Includes results of surgery, both in terms of survival and also
in terms of event-free survival, that is, survival free of
reoperation, cardiac failure, arrhythmias, and other late
complications that are often seen

* Helps cardiologists and cardiac surgeons understand what is
likely to happen to patients with or without treatment, and which
forms of treatment currently in use provide the best outcomes to
date

Table of Content

Foreword vii

Preface ix

Part 1 Introduction

1 Practical and Theoretical Considerations 3

2 Associated Noncardiac Problems 18

Part 2 Surgical Procedures

3 Aortopulmonary Shunts 25

4 Banding the Pulmonary Artery 33

5 Valves and Conduits 37

6 ‘Single Ventricle’ Repairs 57

7 Transplantation 71

Part 3 Left-to-Right Shunts

Section A Aortic Origin

8 Patent Ductus Arteriosus 79

9 Anomalous Origin of One Pulmonary Artery from the Aorta (Hemitruncus) 93

10 Aortopulmonary Window 97

11 Systemic Arteriovenous Fistula 103

12 Coronary Arterial Fistula 111

13 Congenital Aneurysms of the Sinus of Valsalva 121

Section B Ventricular or Atrial Origin

14 Atrial Septal Defect (Secundum) 133

15 Partial Anomalous Pulmonary Venous Connection with an Intact Atrial Septum 157

16 Scimitar Syndrome 161

17 Ostium Primum Defect 167

18 Complete Atrioventricular Septal Defect 175

19 Isolated Ventricular Septal Defect 183

20 Congenitally Corrected Transposition of the Great Arteries 206

Part 4 Obstructive Lesions

Section C Right Ventricular Outflow Tract

21 Pulmonary Stenosis 223

22 Double-Chambered Right Ventricle 234

23 Stenosis of the Main and Branch Pulmonary Arteries 237



Section D Left Ventricular Outflow Tract and Aorta

24 Valvar Aortic Stenosis 245

25 Bicuspid Aortic Valve without Stenosis 264

26 Supravalvar Aortic Stenosis 277

27 Discrete Subvalvar Aortic Stenosis 282

28 Coarctation of the Aorta 291

29 Interrupted Aortic Arch 318

Section E Left Ventricular Inflow Tract

30 Cor Triatriatum Sinister 329

31 Congenital Atresia or Stenosis of Pulmonary Veins 335

32 Congenital Mitral Valve Obstruction 341

Section F Regurgitant Lesions

33 Aortic Valve Regurgitation 353

34 Aortoventricular Tunnel 356

35 Mitral Valve Regurgitation 359

36 Tricuspid Valve Regurgitation 364

37 Idiopathic Pulmonary Regurgitation 368

Part 5 Miscellaneous Aortic Root Lesions

38 Anomalous Origin of a Coronary Artery from the Pulmonary Artery 373

39 Abnormal Origins of the Coronary Arteries from the Aortic Root 384

40 Vascular Rings and Slings 401

Part 6 Right-to-Left Shunts

41 Tetralogy of Fallot 413

42 Tetralogy of Fallot with Pulmonary Atresia 437

43 Tetralogy of Fallot with Absent Pulmonary Valve 446

44 Complete Transposition of the Great Arteries 451

45 Tricuspid Atresia 486

46 Pulmonary Atresia with Intact Ventricular Septum 494

47 Ebstein Anomaly 507

48 Truncus Arteriosus 519

49 Hypoplastic Left Heart Syndrome 531

50 Single (Double-Inlet) Ventricle 546

51 Double-Outlet Right or Left Ventricle 556

52 Total Anomalous Pulmonary Venous Connection 566

53 Heterotaxy Syndromes 579

54 Pulmonary Arteriovenous Fistula 587

55 Eisenmenger Syndrome 595

Index 605

About the author

Julien I.E. Hoffman, M.D., B.SC. HONS., F.R.C.P

Professor of Pediatrics (Emeritus)

Senior Member, Cardiovascular Research Institute

Department of Pediatrics

University of California at San Francisco

San Francisco, CA

USA