1. Edgard A Bendaly, MD, FAAP Congenital Interventional Cardiology
Congenital Heart Disease: Case Studies for Congenital Interventional Procedures
Edgard A Bendaly, MD, FAAP
Congenital Interventional Cardiology

2. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

3. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

4. Glimmers of Knowledge: Ancient Times
20,000 YRS AGO: Hunters in Southern Spain recognized importance of heart, in thorax.
(Reproduction of cave fresco in Benchmark Papers in Human Physiology: Congenital Heart Disease, edited by W. J. Rashkind)
20,000 yrs ago. Fresco from Stone Age man.
The heart was marked with red ochre, a natural earth red pigment, or pointed to by arrows.
Rashkind in HBT lecture: In Northern Spain, not far from Altamira with its magnificent polychrome frescoes and stone age animals is the cave of Pindal. At first site the cave is unprepossessing, leaving one unprepared to find what may well be man’s first anatomic picture; some yrs ago drew the outline of a mammoth and in red ochre marked the heart. Of course, we can only speculate whether her really meant the heart, but as a hunter, he certainly must have known that to strike the heart was the fastest and easiest way to achieve a kill. Thus it is almost certain that prehistoric man know of the heart, and probably something of its function.

5. Glimmers of Knowledge: Ancient Civilizations
3000 BC, Egypt. First illustration/ description of blue skinned individuals
2600 BC, China. Yellow Emperor’s Book of Medicine: “...whenever one feels the pulse, one feels the heart. This means the heart speaks through the vessels of the limbs”
BC, Greece. Hippocrates: Direct auscultation (Hippocratic succession)
BC, Greece. Aristotle: noted the embryonic beating chick heart: the heart is center and origin of life. Greeks thought blood had inherent powers of movement.
China; just cause pulse related to heart, they did not recognize fluid was being moved along by heart
Succession splash or Hippocratic succession was the noise of fluid and water when shaken together in chest. Succession splash or Hippocratic succession was the noise of fluid and water when shaken together in chest.
“You shall know the chest contains water and not pus….if you perceive a noise like boiling vinegar”
Pleural friction rubs also appreciated.
Hippocrates also believed that a certain force was derived from the air , somehow entering the heart, and then distributed to rest of body

6. Renaissance - 1935: The Anatomists
The Renaissance
Diego Rivera Anatomist, detail from The History of Cardiology, Original painting in Mexico.Photograph from Detroit Institute of Art
In this fresco, actually one of two panels in which many of the famous contributors to cardiology are depicted. We’ll talk about Harvey, Laenec, Malphigi, Vesalius, etc. Note the flames on the side: is this showing heretics going up in flames? Not so far off, as you will see. The Inquisition brooked no change from official past dogma
Early 16th century, the bans on human dissection were lifted gradually. Artists studied the body to improve their work, the first was Donatello.

7. Rene Laennec Stethoscope innovation: 1819
Identified each cardiac cycle had 2 heart sounds
Emphasized inspection, palpation, percussion, auscultation
Point number 2: S1 = beginning of systole. S2= atrial contraction.

8. Willem Einthoven ( )
String galvanometer for recording ECG: 1903

9. Maude Abbott,1936: Atlas of Congenital Heart Disease
Comprehensive collation and analysis of > 400 cases of CHD
Anatomy, physical signs, and clinical-path-physiology correlation
Enabled those who came later to devise treatment
International scope of study of CHD
Coined “Eisenmenger’s complex”, used “TOF”, noted Downs/CHD correlation

10. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

11. History
The word catheter is derived from the Greek word katheter, meaning to send down.
While commonly thought to have its origin in urology, the first catheter was associated with the use of clysters (enemas).
Used as both purgatives to restore the balance of the four humors and as a means of administering nutrients, clysters date from ancient Egyptian times.

12. Old “Catheters”
The enema bag, usually an animal bladder, was affixed to quill or metal tube, and substances were injected through the rectum by squeezing the tube.

13. Sones’ Cardiac Catheterization

14. Cardiac Catheterization Laboratory

15. New Catheters

16. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

17. PDA Closure

18. PDA: Prevalence and Physiology
Represent 5-10% of congenital cardiac disease in full term babies
Female:male ratio is 2:1
PDA causes excess blood flow to the lungs and can cause symptoms like fatigue, difficult breathing, failure to thrive or chronic respiratory infections. Large defects can lead to heart failure and death.

19. Presentation Small: Asymptomatic
Large: lower respiratory tract infection, CHF, exertional dyspnea, failure to thrive

20. Physical Examination Tachycardia and tachypnea if CHF
Bounding peripheral pulses with wide pulse pressure with moderate or larger defect.
With moderate to large defect, the precordium is hyperactive. Systolic thrill may be present at the upper left sternal border. A continuous (“ machinery”) murmur at the left infraclavicular area.

21. EKG
Small-moderate: normal or LVH
Large: BVH

22. CXR
Small: normal
Moderate-large: cardiomegaly

23. PDA Types Based on Shape
Krichenko Type A
Krichenko Type B
Krichenko Type C
Type A: Most common (65%), funnel-shaped ductus with a localized narrowing at the pulmonary artery junction
Type B: Second most common (18%), funnel-shaped PDAs with an aortic ampulla
Type C: Tubular shape
Type D: Oval shape with both aortic and pulmonary ampullae
Type E: Other anomalous forms
Krichenko Type D
Krichenko Type E

24. Indications for Closure
Children
Endocarditis/endarteritis risk
Left ventricular volume overload
Infants
Congestive heart failure
Failure to thrive
Indications for transcatheter closure of a patent ductus arteriosus or PDA in “older” children include the prevention of endocarditis and the treatment of left ventricular volume overload.
The indications for PDA closure in “early infancy” are symptoms of congestive heart failure and/or failure to thrive.

25. Historical Methods

26. Primary Device Options
Amplatzer Ductal Occluder
Coils
ADO: FDA approved for PDA closure since 2003.
One cone-shaped lobe and a retention skirt
Polyester fabric sewn in to facilitate duct occlusion

27. Procedure Overview

28. PDA Closure

29. PDA Closure

30. PDA Closure

31. Results Acute closure rates with the ADO are 70%
Complete occlusion in 90-95% within 24 hours
More than 97% by 1 year
Pass RH, Hijazi Z, Hsu DT, Lewis V, Hellenbrand WE. Multicenter USA Amplatzer patent ductus arteriosus occlusion trial. J Am Coll Cardiol 2004;44:

32. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

33. Atrial Septal Defect

34. ASD: Prevalence and Physiology
Represent 6-10% of all cardiac anomalies
Female:male ratio 2:1
Occur in 1 child per 1,500 live births
Imposes a volume
overload on the right heart
and chronic lung disease

35. Presentation Usually asymptomatic Asthma that is difficult to control
Very subtle findings as mild shortness of breath at full exertion in an athletic patient
Failure to thrive

36. Physical Examination A relatively slender body build
Widely split and fixed S2
Grade 2-3/6 SEM
Mid diastolic rumble in large shunts
Less than 10th percentile

37. Natural History of ASD Symptoms early in life are rare.
CHF rare < 40yo
Atrial arrhythmias increase in frequency in older patients
13% in > 40 yo
52% in > 60 yo
PHTN in older untreated patients
Usually > 20yo
Predominantly in females
Up to 1/3 of patients in 3rd decade of life

38. Transcatheter Treatment of ASD
Transcatheter occlusion first described in 1976 using a double umbrella device
Currently the Amplatzer Septal Occluder and Helex Septal Occluder are the 2 devices with FDA approval for ASDs in the United States.
Elective repair recommended between 3-5 years of age
98.5% closure rate
US pivotal study

39. ASD Device

40. ASD

41. ASD Evaluation

42. ASD Evaluation

43. ASD Closure

44. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

45. Balloon Atrial Septostomy
Primarily used to palliate transposition of the great arteries
Newborns can survive several months after this procedure
Currently performed at the bedside under echocardiography

46. Balloon Atrial Septostomy

47. Balloon Atrial Septostomy

48. Balloon Atrial Septostomy

49. Balloon Atrial Septostomy

50. Balloon Atrial Septostomy

51. Aortic Stenosis

52. Natural History of AS

53. Aortic Valvuloplasty

54. Results
Palliation comparable to the palliation achieved by a surgical aortic valvotomy

55. Pulmonary Valve Stenosis

56. Indications Gradient > 40 mmHg Right ventricular hypertrophy
Right ventricle failure
Symptoms

57. Pulmonary Valvuloplasty

58. Pulmonary Valvuloplasty

59. Pulmonary Valvuloplasty

60. Pulmonary Valvuloplasty

61. Pulmonary Valvuloplasty

62. Results
Valvuloplasty is the gold standard for treatment and first line management
In non dysplastic valve it is usually curative
In dysplastic valve it is usually palliative until valve is replaced

63. Coarctation of the Aorta

64. Indications for angioplasty/stent
Gradient > 20 mmHg
Narrowing > 50%
Signs and symptoms
Hypertension
Exercise induced hypertension
Congestive heart failure
Severe acidosis

65. Stents

66. Coarctation Stenting

67. Coarctation Stenting

68. Coarctation Stenting

69. Coarctation Stenting

70. Results Depends on the underlying physiology
Depends on the age the procedure is performed
Stents maintain long term vessel patency

71. Outline History of cardiology History of cardiac catheterization
Patent ductus arteriosus
Atrial septal defect
Other interventions
Summary

72. Summary
Cardiac cath in patients with CHD is a safe and standard procedure when done by a congenital interventional cardiologist
Hospital stay is between hours
Interventional procedures allow either palliation or treatment of cardiac disease replacing or delaying open heart surgery

73. Summary PDA cause volume and pressure and overload
Presentation and physical findings can be subtle
Closure in the cath lab is safe, effective and considered the gold standard

74. Summary ASD cause volume overload on the heart
Presentation can be subtle
If untreated, late complications include a shorter lifespan
Closure in the cath lab is safe and effective

75. Summary
Interventional procedures are increasing in number and complexity
New procedures and techniques are constantly developed