Congenital Heart Disease
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Congenital Heart Disease

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Pathology of congenital heart disease

Pathology of congenital heart disease

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Congenital Heart Disease Congenital Heart Disease Presentation Transcript

  • PEDIATRICS CONFERENCE PATHOLOGY OF CONGENITAL HEART DISEASE Boonyathee Sorawit MD. Mar 25th , 2014
  • Epidemiology  Congenital heart disease occurs in 0.5–0.8% of live births.  Incidence is higher in stillborns (3–4%), spontaneous abortuses (10–25%) and premature infants (about 2% excluding patent ductus arteriosus [PDA])  About 2–3 in 1,000 newborn infants will be symptomatic with heart disease in the 1st year of life.  The diagnosis is established by 1 week of age in 40–50% of patients with congenital heart disease and by 1 month of age in 50–60% of patients.
  • Etiology  Caused by developmental abnormalities  Genetic factors  chromosomal abnormalities  outflow tract defect may cause by abnormal development of neural crest- derived cell (region of chromosome 22)  Environmental factors, such as congenital infection or teratogens  Multifactorial: genetic, environmental, and maternal factors  The risk of recurrence of congenital heart disease increases if a 1st-degree relative (parent or sibling) is affected.
  • Chromosome Abnormalities  Down Syndrome (40%) AVSD, VSD  Trisomy 13 (80%) VSD, PDA  Trisomy 18 (100%) VSD, PDA  Turner’s Syndrome (35%) Coarctation of aorta  Marfan’s Syndrome Aortic aneurysm, MVP
  • Teratogen  Infection  Rubella PDA, peripheral PS  Drug  Dilantin PS, AS  Lithium Ebstein anomaly  Alcohol VSD, ASD  Retinoic acid Interrupted aortic arch, TGA, TOF  Maternal disease  Diabetes TGA, VSD, cardiomyopathy  SLE Complete AV block
  • Frequencies of CHD  VSD  ASD  Pulmonary stenosis  PDA  TOF  AVSD 42 % 10 % 8 % 7 % 5 % 5 %
  • How to Approach Congenital Heart Disease
  • History Taking  History of cardiology related  Heart Failure  Usually present in first year old  Tachypnea, Prolong feeding, Poor weight gain, Diaphoresis, pale (sympathetic activity increased), Exercise intolerance  Cyanosis  Hypoxic Spell  Cyanosis, Tachypnea, Flaccid or loss of consciousness  Arrhythmia  Most common -> Supraventricular tachycardia  Neurologic Symptoms  Cerebral thrombosis, Brain Abscess
  • History Taking  Maternal and Perinatal History  History of drug used (Teratogen)  Birth weight  Family History  Usually prevalence 8 in 1,000  1st degree related increased in prevalence during 10 – 15 percent
  • Physical Examination  General Appearance  Finding dysmorphic feature  Vital Signs  Blood pressure -> Leg > Arm SBP 10-20 mmHg  Pulse  Weak Leg > Arm -> Coarctation of Aorta  Diminish pulse -> Cardiogenic shock or low cardiac output  Respiratory Rate (Upper limit)  < 1 month 60 /min  1 month – 1 year 50 /min  1 year – 5 years 40 /min  > 5 years 30 /mins
  • Physical Examination  Jugular Venous Pressure  Suspected right-side heart failure  Cardiac Exam  Inspection -> Precordial bulging (RV volume overload)  Palpation  Apex : 4th Lt intercostal space midclavicular line  Thrill for grading murmur  Auscultation
  • Physical Examination  Pulmonary Exam  Crepitation or Rales -> Pulmonary congestion  Complication : Pneumonia -> Left to Right Shunt  Liver and Spleen  Right-side heart failure -> Hepatomegaly or splenomegaly  Extremities  Edema -> Systemic venous congestion -> Right-side heart failure  Clubbing of finger -> Cyanotic heart
  • Chest X-Ray  Cardiovascular Structure  Location of Heart, Stomach and Liver  Levocardia, Mesocardia, Dextrocardia (Left, Central, Right)  Situs Solitus or Situs inversus  Chamber and Heart Enlargement  Cardiothoracic Ratio 50 – 60 %  Identified what chamber enlargement  Aorta  Right or Left Aortic Arch  Size of aorta
  • Chest X-ray  Pulmonary Vasculature  Increased pulmonary vasculature (Left to Right shunt)  Decreased pulmonary vasculature (Right to Left shunt, PS, PA)  Normal pulmonary vasculature (PS, AS)  Extracardiac Structure  Ribs and Vertebra  Diaphragm  Phrenic nerve paralysis, Diaphragmatic hernia  Lung parenchyma  Pneumonia or Atelectasis
  • Electrocardiography  Ventricular Hypertrophy (Right or Left)  Axis Right Ventricular hypertrophy • R wave >98th percentile in lead V1 • S wave >98th percentile in lead V6 • R wave in V1 + S wave in V6 >98th percentile • R/S ratio >98 percentile in lead V1 • Right axis deviation • qR pattern in V1 • Upright T wave in V1 (1 week old to 8 years old) • RSR’ pattern in lead V1, where R’ >15 mm (<1 year old) or R’ >10 mm (>1 year old) • Pure R wave in V1 >10 mm (newborn) • RVH (by voltage criteria) with strain pattern Left Ventricular Hypertrophy • R wave >98th percentile in lead V6 • S wave >98th percentile in lead V1 • R wave in V6 + S wave in V1 >98th percentile • Q wave >98th percentile in lead lll or V6 • R/S ratio >98th percentile in lead V6 • LVH (by voltage criteria) with strain pattern
  • Congenital heart disease  Cyanotic / Acyanotic  Three major categories:  left-to-right shunt  right-to-left shunt  obstruction  A shunt is an abnormal communication between chambers or blood vessels  Direction and magnitude of shunt depends on  Size of defect  Relative pulmonary and systemic pressures  Relative pulmonary and systemic resistance
  • Congenital heart disease Cyanosis Acyanosis Increase pulmonary blood flow Decrease pulmonary blood flow Normal pulmonary blood flow Increase pulmonary blood flow How to Approach Congenital Heart Disease
  • Acyanotic Congenital Heart Disease
  • Acyanotic Heart Disease  Left to Right Shunt  Increased pulmonary blood flow  Clinical -> Heart Failure  VSD, ASD, PDA, AVSD  Obstructive Lesions  Normal pulmonary blood flow  Clinical -> Exercise intolerance  PS, AS, Coarctation of aorta
  • Acyanosis Increase pulmonary blood flow Normal pulmonary blood flow RVH LVHRVHLVH ASD PAPVR PS MS VSD PDA AVSD AS CoA
  • Increased pulmonary blood flow Acyanotic Congenital Heart Disease
  • Atrial Septal Defect (ASD)  5 – 10 % of Total congenital heart diseases  2-fold of Female than male  Three types exist : primum, secundum and sinus venosus  The most common is the secundum type (Patent Foramen Ovale; PFO)
  • Type of ASD The Cardiovascular System Cochard, Larry R., PhD, Netter's Atlas of Human Embryology, Updated Edition, Chapter 4, 83-111
  • Pathophysiology
  • Clinical Presentation  Usually incidental finding heart murmur without symptoms  1% of first year -> Developed heart failure  Clinical heart failure depended on shunt  Physical examination  Increased right ventricular impulse  Systolic ejection murmur at left upper sternal border (Relative PS)  Widely fixed split second heart sound -> prolong ejection time
  • Investigation for Diagnosis  Plain film (CXR)  can be normal is early stages +/- when the ASD is small  signs of increased pulmonary flow (shunt vascularity)  enlarged pulmonary vessels  upper zone vascular prominence  vessels visible to the periphery of the film  eventual signs of pulmonary arterial hypertension  chamber enlargement  right atrium  right ventricle  note : left atrium is normal in size & aortic arch is small to normal
  • Investigation for Diagnosis
  • Chest X-ray
  • Investigation for Diagnosis  Electrocardiography  Right axis deviation  Right atrial enlargement  Right ventricular hypertrophy (rsR’ in V1 80-90 %)
  • Complications  Right-side heart failure  Right side of the heart to work harder (Volume overload)  Arrhythmias  Atrial enlargement -> Atrial Fibrillation  Stroke  Clot pass Right to Left to Systemic circulation  Pulmonary Hypertension
  • Treatment
  • Ventricular Septal Defect (VSD)  Most common of congenital heart disease (20 – 30 %)  Type of VSD  Subpulmonic VSD  Perimembranous VSD  Inlet VSD  Muscular VSD
  • Pathophysiology
  • Clinical Presentation  Depended on VSD size and Pulmonary vascular resistant  Small VSD  Usually asymptomatic  Can detect murmur in 1st or 2nd weeks after birth  Pansystolic murmur at Left lower parasternal border  Moderate to Large VSD  Developed heart failure in 1 – 2 months  Murmur same as small VSD and loud P2  Eisenmenger’s Complex
  • Eisenmenger’s Complex  Consequence of a large preexisting left-to-right shunt  Pulmonary artery pressures approach systemic levels and the direction of the flow becomes bidirectional or right to left.  The high pulmonary vascular resistance is usually established in infancy (by age 2 years, except in ASD) and is sometimes present from birth.
  • Eisenmenger’s Complex Left to Right Shut Increased pulmonary blood flow (Shear stress / Circumferential stretch) Endothelial dysfunction and vascular remodeling (Smooth muscle cell proliferation, Increased in extracellular matrix, Intravascular thrombosis) Increase in PVR Inverted shunt : Right to Left Cyanosis (Irreversible Process)
  • Investigation for Diagnosis  Plain Film  The chest radiograph can be normal with a small VSD.  Larger VSDs may show cardiomegaly  Left atrial enlargement  Right and left ventricle can be enlarged  A large VSD may also show features of pulmonary oedema, pleural effusion or / and increased pulmonary vascular markings.
  • Investigation for Diagnosis
  • Chest X-ray
  • Investigation for Diagnosis  Electrocardiography  Small VSD : Normal EKG  Moderate VSD : LVH (R in Lead V6 high and can be found deep Q)  Large VSD : RVH and LVH
  • Treatment  Spontaneous Closure (35 – 40 % within 2 years)  Below 6 months that can’t control heart failure or recurrence pneumonia  6 – 24 months should surgery in pulmonary hypertension case
  • Atrioventricular Septal Defect (AVSD)  Endocardial cushion defect  4 – 5 % of Total congenital heart disease  40 % of Down Syndrome -> CHD  40 % of CHD in Down Syndrome -> AVSD  Type of AVSD  Complete AVSD  Primum ASD + Inlet VSD + Common AV valve  Partial AVSD  Primum ASD +/- Cleft of Mitral valve  Transitional (Intermediate) AVSD  Primum ASD + Small VSD but Separate Mitral and Tricuspid valve
  • Pathophysiology
  • Clinical Presentation  Partial and Intermediate AVSD  Can be developed heart failure in infant  Cardiac murmur  Systolic ejection murmur (Relative PS)  Widely fixed split S2  Pansystolic murmur at Apex (MS)  Complete AVSD  Within 4-8 weeks after birth can be developed heart failure  Cardiac murmur same as above and Loud P2
  • Investigation for Diagnosis  Plain Film  Not specific can be found chamber enlargement  Increased Pulmonary Vasculature
  • Investigation for Diagnosis
  • Chest X-ray
  • Investigation for Diagnosis  Electrocardiography  Left superior QRS axis (Lead I : positive and aVF : negative)  Right ventricular hypertrophy or combine ventricular hypertrophy
  • Treatment
  • Patent ductus arteriosus (PDA)  9 – 12 % of Total congenital heart disease  2 fold of prevalence in female than male  80 % of Low birth weight (1,200 gm)
  • Ductus arteriosus  Lung expansion  PVR↓ , PBF↑, PO2↑  PGE2↓  Ductus arteriosus constriction  Functionally closed; immediately after birth (within 10-15hr)  Anatomical closure; a week to 10 days → Ligamentum arteriosum
  • Pathophysiology
  • Clinical Presentation  Small duct with no LV volume overload (normal LV) and normal PAP (generally asymptomatic)  Moderate PDA with predominant LV volume overload:  large LV with normal or reduced function (may present with left heart failure)  Moderate PDA with predominant PAH:  pressure-overloaded RV (may present with right heart failure)  Large PDA:  Eisenmenger physiology with differential hypoxaemia and differential cyanosis (lower extremities cyanotic, sometimes left arm, too)
  • Investigation for Diagnosis  Plain Film  may vary depending on  isolated or associated with other cardiac anomalies  direction of shunt flow (right to left or left to right).  Can have cardiomegaly  predominantly left atrial and left ventricular enlargement
  • Investigation for Diagnosis
  • Chest X-ray
  • Investigation for Diagnosis  Electrocardiography  Small PDA : normal  Medium to Large PDA :  LA enlargement (wide P wave > 2.5 mm)  LV hypertrophy (R wave in lead V6 > 98th percentile)  +/- ST elevation (decreased diastolic filling time -> myocardial ischemia)
  • Treatment  Preterm with congestive heart failure  Restrict fluid with corrected anemia (Keep Hct > 45%)  Indomethacin should be used within 10 days  Low birth weight : 0.2 mg/kg in first dose and 2nd – 3rd dose q 12-24 hrs  < 2 days : 0.1 mg/kg  2-7 days : 0.2 mg/kg  > 7 days : 0.25 mg/kg  Small PDA  should perform intervention if more than 1 year old  Medium to Large PDA with clinical heart failure  should perform intervention as soon as possible
  • Treatment
  • Normal pulmonary blood flow Acyanotic Congenital Heart Disease
  • Coarctation of aorta (CoA)  6 – 8 % of Total congenital heart disease  2-fold of prevalence in male than female  35 % associated with Turner syndrome  Type of Coarctation of aorta  Simple CoA -> +/- PDA  Complex CoA -> Associated with other congenital heart disease
  • Pathophysiology
  • Clinical Presentation  Depended on severity of stenosis and other congenital heart disease  critical CoA  Shock, Hypotension, Congestive heart failure  Ductal Dependent systemic blood flow  Anemia, Poor perfusion, Tachypnea  Weak pulse and BP Leg > Arm  Differential cyanosis Leg < Arm (Except Large PDA)  Adulthood may be present hypertension unknown caused  Systolic ejection murmur at left upper sternal border
  • Investigation for Diagnosis  Plain Film  rib notching  Compression of intercostal collateral arteries below rib  3 sign  Proximal to stenosis -> enlargement  Distal to stenosis -> narrow  pulmonary vascular marking normal
  • Chest X-ray
  • Investigation for Diagnosis  Electrocardiography  critical CoA  Right ventricular hypertrophy  Pumping of right ventricle -> ductus arteriosus -> descending aorta
  • Treatment  Critical CoA  Maintain Airway  Prostaglandin E 1 for opening ductus arteriosus  Coarctation repair
  • Treatment
  • Pulmonary Stenosis (PS)  25 – 30 % of total congenital heart disease  Type of pulmonary stenosis  Pulmonary valve stenosis  Infundibular stenosis  Pulmonary artery stenosis
  • Clinical Presentation  Subinfundibular/infundibular:  asymptomatic or they may present with angina, dyspnoea, dizziness, or syncope  Valvular:  Mild to moderate valvular PS (Usually asymptomatic)  Moderate PS can progress at the valvular level (calcification) or at the subvalvular level, due to reactive myocardial hypertrophy  Severe stenosis  dyspnoea and reduced exercise capacity, and have a worse prognosis  Supravalvular:  asymptomatic or have symptom of dyspnoea and reduced exercise capacity
  • Investigation for Diagnosis  Plain film  Non specific  normal heart size  Right ventricular hypertrophy  Dilated pulmonary trunk or a main pulmonary artery  Electrocardiography  Right axis deviation  Right atrial enlargement  Right ventricle hypertrophy
  • Chest X-ray
  • Treatment  Mild PS  Follow up and aware bacterial endocarditis prophylaxis  Moderate to Severe PS  Balloon pulmonary valvuloplasty  Valvulotomy in case fail balloon valvuloplasty or dysplastic pulmonic valve  Critical PS  Need ductus arteriosus should give prostaglandin E1 and corrected anemia
  • Treatment
  • Aortic Stenosis (AS)  3-8 % of total congenital heart disease  4-fold of prevalence in male than female  Type of aortic stenosis  Aortic valve stenosis  Subaortic stenosis  Supravalvular aortic stenosis
  • Clinical Presentation  90 % Asymptomatic  10 % developed heart failure in first year  2/3 occur in 1-2 months  Tachypnea, low birth weight, hepatomegaly  Critical AS  Cardiogenic shcok, poor perfusion  Ductal dependent systemic blood flow  +/- Difference cyanosis (Due to PA pass to Aorta)
  • Investigation for Diagnosis  Plain Film  Usually normal cardiac size  Critical AS -> Cardiomegaly  Electrocardiography  Critical AS  Right atrial enlargement  Right ventricle hypertrophy  In adulthood  Left ventricular hypertrophy  +/- inverted T wave or ST depression in lead V5 and V6 due to myocardial ischemia in severe stenosis
  • Treatment  Mild AS  No specific treatment -> F/U clinical and echocardiogram q 1-2 year  Moderate AS (PPSG 50–75 mmHg) or EKG change or have symptom  Balloon valvuloplasty or valvulotomy  Severe AS (PPSG > 75 mmHg)  Suggest Balloon valvuloplasty or valvulotomy even though no clinical symptom  Critical AS  Prostaglandin E1  Inotropic drug
  • Treatment
  • Cyanotic Congenital Heart Disease
  • Acyanotic Heart Disease  Decreased pulmonary blood flow  Right ventricular outflow tract obstruction -> right–to–left shunt  Increased pulmonary blood flow  Can be developed to congestive heart failure and cyanosis
  • Cyanosis Decrease pulmonary blood flow Increase pulmonary blood flow RVH CVHRVHLVH TOF PA/VSD Critical PS DORV/PS TGA/VSD/PS TGA TAPVR DORV HLHS PA/IVS Tricuspid atresia Truncus arteriosus
  • Decreased pulmonary blood flow Cyanotic Congenital Heart Disease
  • Tetralogy of Follot (TOF)  Most common in cyanotic congenital heart disease  9 % of total congenital heart disease  4 Common of defect  Ventricular septal defect  Pulmonary stenosis  Overriding of aorta  Right ventricular hypertrophy
  • Pathophysiology
  • Clinical Presentation  Early clinical presentation  A heart murmur in infancy and progressive cyanosis (from right to left shunting at the ventricular level secondary to RVOTO).  Unoperated tetralogy carries a poor prognosis (.95% of patients used to die before 40 years of age).  Late clinical presentation  Late survival after tetralogy repair is excellent, with a 35-year survival of 85%.
  • Hypoxic Spell Increased Right to Left shunt Hypoxia Metabolic Acidosis Hyperpnoea Increased systemic venous return Infundibular Spasm Decreased Systemic vascular resistance
  • Investigation for Diagnosis  Plain Film  Boot shape heart  Apex shift with pulmonary trunk indentation  Decreased pulmonary marking  Electrocardiography  Usually found Right ventricular hypertrophy
  • Chest X-Ray
  • Treatment  Hypoxic Spells (Emergency condition)  Knee-Chest Position  Control Crying  Oxygen supplement  Morphine 0.1 mg/kg (Sedate and reduce infundibular spasm)  IV Hydration and correct metabolic acidosis  +/- drug for increased systemic vascular resistance  Propranolol 1-4 mg/kg/day q 6-8 hrs  Correct Anemia  Surgery consideration
  • Treatment
  • Tricuspid Atresia (TA)  3 % of total congenital heart disease  Hemodynamic  Blood can’t flow from right atrium to right ventricle  Right Atrium -> Left atrium (Via ASD) -> Left ventricle)
  • Clinical presentation  Developed cyanosis in 1 month  Large VSD and no pulmonary stenosis  Heart failure due to decreased pulmonary vascular resistance  Progressive cyanosis due to restrictive VSD  Progressive pulmonary stenosis -> Hypoxic Spell  Cardiac murmur  Pansystolic murmur at left lower sternal border (restrictive VSD)  Systolic ejection murmur (pulmonary stenosis)  Single second heart sound
  • Investigation for Diagnosis  Plain Film  Normal or minimal cardiomegaly  Decreased pulmonary vascular marking  Electrocardiography  Left superior QRS axis  R wave amplitude in lead V1 low (due to hypoplastic right ventricle)  +/- Left ventricular hypertrophy
  • Treatment  Newborn with severe cyanosis  Give PGE1 dose 0.05–0.1 mcg/kg/min for maintain ductus arteriosus  Consider modified Blalock-Taussig shunt  If restrictive ASD -> balloon atrial septostomy  Large VSD and no pulmonary stenosis  For prevent to develop heart failure -> pulmonary artery banding
  • Timing for Operation  6 – 12 months  Bidirectional Glenn shunt  Superior vena cava -> pulmonary artery  3 – 4 year  Fontan operation or total cavopulmonary shunt  Inferior vena cava -> pulmonary artery
  • Treatment
  • Increased pulmonary blood flow Cyanotic Congenital Heart Disease
  • Transposition of Great Arteries (TGA)  5 % of total congenital heart disease  Ventriculo-arterial discordance:  Left ventricle to pulmonary artery  Right ventricle to aorta
  • Pathophysiology
  • Clinical Presentation  Pure TGA with small PFO and PDA  Cyanosis after birth, metabolic acidosis  Weak murmur, single second heart sound  TGA with VSD  Minimal cyanosis  Developed heart failure in 2-6 months  TGA with VSD with PS  Similar tetralogy of Fallot  Cyanosis but no clinical heart failure
  • Investigation for Diagnosis  Plain film  Cardiomegaly with a cardiac contours classically described as appearing like an egg on a string.  Narrowing of the superior mediastinum as result of the aortic and pulmonary arterial configuration.  Electrocardiography  Right axis deviation  Right ventricular hypertrophy  TGA with VSD -> combined ventricular hypertrophy
  • Chest X-ray
  • Treatment  Severe Cyanosis  PGE1: dose 0.05– 0.1 mcg/kg/min for maintain ductus arteriosus  Correct metabolic acidosis and electrolytes  If persistent cyanosis  Atrial balloon septostomy then consider arterial switch operation that should perform within 2 weeks  If more than 2 weeks -> Two-stage arterial switch operation  Pulmonary banding -> For Left ventricle hypertrophy  Arterial switch after 7-10 days of pulmonary banding
  • Treatment  TGA with VSD and PS -> Palliative operation  modified Blalock–Taussig shunt then considered definite surgery if body weight 10-15 kgs for Ratelli Operation  Closed VSD and used conduit bridging between right ventricle and pulmonary artery
  • Treatment
  • Truncus Arteriosus  < 1 % of total congenital heart disease  Common route between pulmonary artery and aorta
  • Type of Truncus arteriosus
  • Clinical Presentation  Minimal Cyanosis  Can be developed heart failure in 1 month  Cardiac murmur : pansystolic murmur at left lower sternal bonder  Single second heart sound
  • Investigation for Diagnosis  Plain Film  Cardiomegaly  Increased pulmonary marking  Electrocardiography  Left atrial enlargement  Combined ventricular hypertrophy
  • Treatment  Control heart failure during waiting for operation  Consider for Total repair (Should perform before 6 months)  Closed VSD  Connected conduit between right ventricle to pulmonary artery  If can’t do total repair  Pulmonary artery banding for prevent pulmonary vascular obstructive disease
  • Treatment
  • Total Anomalous Pulmonary Venous Return (TAPVR)  1.5 % of total congenital heart disease  Usually pulmonary vein must drainage into Left atrium but blood returning from the lungs drains back to the right side of the heart.
  • Pathophysiology
  • Type of TAPVR
  • Clinical Presentation  56 % Symptom presented in first month  Minimal cyanosis  Sign of Heart failure  Systolic ejection murmur at left upper sternal border (Relative PS)
  • Investigation for Diagnosis  Plain film  The right heart is prominent  because of the increased flow volume  The supracardiac variant (type I) can classically depict a snowman appearance on a frontal chest radiograph, also known as figure of 8 heart or cottage loaf heart .
  • Chest X-Ray
  • Investigation for Diagnosis  Electrocardiography  Right axis deviation  Right atrium enlargement  Right ventricular hypertrophy
  • Treatment  Should perform Total repair as soon as possible
  • Double-outlet right ventricular (DORV)  Two Great Arteries (Aorta and Pulmonary Artery) both originate from the right ventricle  Blood from the left ventricle passes across a VSD into the RV to reach the great arteries
  • Double-outlet right ventricular (DORV)
  • Hypoplastic left heart syndrome (HLHS)  Left side of the heart is very poorly formed  Cannot support the main circulation.  Left ventricle and aorta are abnormally small (hypoplastic)
  • Treatment
  • Thank you for your kind attention.
  • Reference  Moss and Adams’ heart disease in infants, children, and adolescents, 6 th ed. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ, eds. Philadelphia: Lippincott Williams & Wilkins, 2001:618-35.  The science and practice of pediatric cardiology, 2nd ed. In: Garson A Jr, Bricker JT, Fisher DJ, Neish SR, eds. Baltimore: Williams & Wilkins, 1998:1141–79.  Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am 1999;46:355–68.  Comprehensive surgical management of congenital heart disease. In: Jonas RA, ed. London: Arnold, 2004:386-401.
  • Reference  Waldman JD, Wernly JA. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am 1999;46:385-404.  Victorica BE. Cyanotic newborns. In: Gessner IH, Victorica BE, eds. Pediatric cardiology: a problem oriented approach. Philadelphia, W.B. Saunders 1993:97-109.  Pediatric cardiac surgery. In: Mavroudis C, Backer CL, eds. Philadelphia, Mosby 2003:383-97.
  • Picture Reference  http://radiopaedia.org  http://www.rch.org.au/cardiology/heart_defects/