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Congenital Heart Disease: Case Studies for Congenital Interventional Procedures Edgard A Bendaly, MD, FAAP Congenital Interventional Cardiology Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary 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) 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” • 460-370 BC, Greece. Hippocrates: Direct auscultation (Hippocratic succession) • 400-320 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. Renaissance - 1935: The Anatomists Rene Laennec • Stethoscope innovation: 1819 • Identified each cardiac cycle had 2 heart sounds • Emphasized inspection, palpation, percussion, auscultation Willem Einthoven (1860-1927) • String galvanometer for recording ECG: 1903 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 Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary 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. 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. Sones’ Cardiac Catheterization Cardiac Catheterization Laboratory New Catheters Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary PDA Closure 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. Presentation • Small: Asymptomatic • Large: lower respiratory tract infection, CHF, exertional dyspnea, failure to thrive 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. EKG • Small-moderate: normal or LVH • Large: BVH CXR • Small: normal • Moderate-large: cardiomegaly PDA Types Based on Shape Krichenko Type A Krichenko Type B Krichenko Type D Krichenko Type C Krichenko Type E Indications for Closure • Children – Endocarditis/endarteritis risk – Left ventricular volume overload • Infants – Congestive heart failure – Failure to thrive Historical Methods Primary Device Options Coils Amplatzer Ductal Occluder Procedure Overview PDA Closure PDA Closure PDA Closure Results • Acute closure rates with the ADO are 70% • Complete occlusion in 90-95% within 24 hours • More than 97% by 1 year Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary Atrial Septal Defect 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 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 Physical Examination • • • • A relatively slender body build Widely split and fixed S2 Grade 2-3/6 SEM Mid diastolic rumble in large shunts 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 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 ASD Device ASD ASD Evaluation ASD Evaluation ASD Closure Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary 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 Balloon Atrial Septostomy Balloon Atrial Septostomy Balloon Atrial Septostomy Balloon Atrial Septostomy Balloon Atrial Septostomy Aortic Stenosis Natural History of AS Aortic Valvuloplasty Results • Palliation comparable to the palliation achieved by a surgical aortic valvotomy Pulmonary Valve Stenosis Indications • • • • Gradient > 40 mmHg Right ventricular hypertrophy Right ventricle failure Symptoms Pulmonary Valvuloplasty Pulmonary Valvuloplasty Pulmonary Valvuloplasty Pulmonary Valvuloplasty Pulmonary Valvuloplasty 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 Coarctation of the Aorta Indications for angioplasty/stent • Gradient > 20 mmHg • Narrowing > 50% • Signs and symptoms – Hypertension – Exercise induced hypertension – Congestive heart failure – Severe acidosis Stents Coarctation Stenting Coarctation Stenting Coarctation Stenting Coarctation Stenting Results • Depends on the underlying physiology • Depends on the age the procedure is performed • Stents maintain long term vessel patency Outline • • • • • • History of cardiology History of cardiac catheterization Patent ductus arteriosus Atrial septal defect Other interventions Summary Summary • Cardiac cath in patients with CHD is a safe and standard procedure when done by a congenital interventional cardiologist • Hospital stay is between 24-36 hours • Interventional procedures allow either palliation or treatment of cardiac disease replacing or delaying open heart surgery 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 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 Summary • Interventional procedures are increasing in number and complexity • New procedures and techniques are constantly developed