Congenital Heart Diseases
Professor Mohammed Jalal pediatrics department Al-Kindy medical collegeCongenital Heart Diseases
It is common congenital anomaly in children. It is incidence about 8/1000. It is either cyanotic CHD with dominant RT to LF shunt or Acyanotic with dominant LF to RT shunt. Cyanotic CHD includes TOF,TGA,TAPVR,TA, TAr, single Ventricle, Hypoplastic left heart syndrome, Ebstien anomaly, Eisenmenger complex. Acyanotic CHD includes VSD, ASD, PDA, COA, and others. Commonest CHD is VSD (25-30%), ASD (6-8%), PDA (6-8%), TOF(5-7%), TGA(3-5%).Congenital Heart Diseases
Etiology Adverse maternal diseases, teratogens & drugs. Genetic factors &single gene defect (AD). Chromosomal anomalies. Multifactorial ( genetic & environmental). Gender difference. Racial factor. Unknown following steps required to diagnose any cardiac problems, these includes proper history, proper physical examination, CXR, echocardiography, cardiac catheterization & angiography. Others like MRI & exercise testing according the case.Ventricular Septal Defect (VSD)
Commonest CHD. The defect is in 2 types. Membranous Muscular Clinical manifestations. It Depends on the size of defect, pulmonary flow & pulmonary pressure. Small VSD with little LF to RT shunt & normal pulmonary pressure are the most commonest type. Clinically those are asymptomatic. Cardiac lesion is usually found during routine physical examination. Loud harsh pansystolic murmur best heard over lower LF sternal border frequently accompanied by thrill.
VSD
Clinical presentation CXR usually normal or minimal cardiomegaly with some increase in pulmonary vascularity (plethoric lungs). ECG is normal but may shows LF ventricular hypertrophy. While in patients with large VSD, excessive pulmonary flow & pulmonary hypertension clinically present with. Dyspnea, feeding difficulty, poor growth, profuse perspiration, recurrent chest infection, heart failure in early infancy. On examination there is prominent sternum& left pericardium, palpable apical thrust & systolic thrill. Pansystolic murmur at LF sternal border, loud & single second heart sound. Some time short mid diastolic murmur ( increase flow across mitral valve).
VSD
Clinical presentation. CXR shows gross cardiomegaly, increase pulmonary vascularity ( plethoric lungs). ECG shows biventricular hypertrophy, P wave may be notched or peacked. Echocardiography confirm the diagnosis & shows the size & position of defect. Cardiac catheterization demonstrate the effect of VSD on circulation. Gives information about O2 saturation & pressure in the heart chambers
VSD
Prognosis & complications. Significant number 30-50% of small defects closes spontaneously during first year of life, while moderate & large defects are less common to close spontaneously. A large number of children remain asymptomatic. Failure to thrive. Repeated chest infection specially in infants with large defect. Congestive heart failure in infants with large defect. Infective endocarditis which is more common in adolescent & uncommon in children less than 2 years. Pulmonary hypertension as a result of increase pulmonary blood flow. Eisenmeneger complex, due to increase pulmonary vascular resistance. Acquired infandibular pulmonary stenosis.
VSD
Treatment In small size defect. Encouraged to live normal life Protection against infective endocarditis by. A-careful maintenance of permanent teeth B-antibiotic prophylaxis before dental, genitourinary & lower intestinal procedures, tonsillectomy & other oropharyngeal surgery.
In large size defect Medical treatment include. Antibiotic for chest infection. Treatment of congestive heart failure by antifailure drugs& diuretics. Prophylaxis prior any surgical procedures
VSD
Treatment Surgical treatment which indicated in following conditions. Failure of medical treatment of large VSD associated with failure to thrive & repeated chest infection Intractable heart failure not responding to medical treatment Pulmonary hypertension Associated aortic insufficiency with VSD
VSD
Atrial Septal Defect ( ASD)
Its second common CHD. Its in 3 types Ostium secondum Ostium primum Sinus venosus Ostium secondum ASD The defect is in the region of fossa ovalis Female to male ratio is 3:1
ASD
Clinical manifestations The patient often asymptomatic & lesion may be discovered during routine physical examination Even an extremely large ASD rarely produce heart failure in childhood In older children exercise intolerance may be noticed Pulse is normal Wide & fixed splitting of second heart sound Ejection systolic murmur medium pitched rarely associated with thrill best heard at LF mid& upper sternal border Short early diastolic murmur at lower LF sternal border due to high flow across tricuspid valve
ASD
CXR shows varying degree of enlargement of RT ventricle & atrium pulmonary artery is prominent with increase pulmonary vascularity (plethoric) ECG shows normal axis, RT axis deviation RT ventricular coduction delay with rSR pattern, RT ventricular hypertrophy, prolong PR Echocardiography shows location & size of defect & characteristic RT ventricle volume overload
ASD
Prognosis & complications Usually well tolerated during childhood, usually appear in the third decade or later Pulmonary hypertension, atrial arrhythmia, tricuspid& mitral incompetence, heart failure is late complication, infective endocarditis is very rare. Treatment Medical treatment 1-prophylaxis to protect patients against infective endocarditis 2-treatment of respiratory infection 3-treatment of heart failure Surgical treatment its advised even for asymptomatic patient prior to school entry.
ASD
Ostium primum defect The defect is situated at lower portion of atrial septum & overlies the mitral & tricuspid valve. in the majority of cases there is a cleft in the anterior leaflet of mitral valve. Clinical manifestation Many children are asymptomatic. Patient is normal size, or tall & thin with long fingers & toes Exercise intolerance, easy fatigability, recurrent chest infection in patient with large defect& sever mitral incompetence Wide fixed splitting of second heart sound, ejection systolic murmur at pulmonary area, early diastolic murmur at lower LT sternal border If there is mitral incompetence-apical pansystolic murmur that radiate to axilla
Ostium primum ASD
CXR shows cardiomegaly & increase pulmonary vascularity ( plethoric lungs) ECG is distinctive & shows LF axis deviation biventricular hypertrophy, RT ventricle conduction delay Echocardiography confirm the diagnosis Treatment Medical treatment 1-treatment of heart failure 2-treatment of chest infection 3-prophylaxis against infective endocarditis Surgical treatment the defect is closed by insertion of prosthetic patch.ASD
Patent Ductus Arteriosus( PDA)
Its commonest CHD associated with maternal rubella during early pregnancy Female to male ratio is 2:1 Clinical manifestation Depends on size of ductus, shunt & pulmonary vascular resistance Patient with small shunt usually asymptomatic, heart size is normal Patient with large shunt may present with LF ventricular failure Growth retardation in large shunt Dyspnea Bounding arterial pulsation & wide pulse pressure Apical impulse is prominent with heave Systolic thrill maximum in second left intercostal space which radiate to LF clavicle, LF sternal border or toward apexPAD
Classical murmur is contineous machinery murmur best heard at second LF intercostal space Some patient with large defect- mitral diastolic murmur may be audible CXR in small shunt is normal while in large shunt it shows prominent pulmonary artery, increase pulmonary vascularity & cardiomegaly ECG with large shunt shows LF ventricle or biventricle hypertrophy Echocardiography confirm the diagnosis
PDA
Prognosis& complications Patient with small PDA may live normal life span, spontaneous closure after infancy rare. With large PDA. Congestive heart failure occur commonly in infancy. other complications are infective endocarditis, pulmonary or systolic embolism, pulmonary hypertension, calcification of ductus, Eisenmenger complex. Treatment Medical treatment: which includes antibiotic for chest infection, prophylaxis against infective endocarditis, treatment of heart failure Surgical treatment irrespective of age patient with large PDA require surgical ligation of the duct preferably at 1 or 2 years of age
PDA
Coarctation Of Aorta (COA)
Coarctation of aorta of varying degree may occur at any point from transverse arch to iliac bifurcation, but 98% occur just below the origin of the left subclavian artery at the origin of ductus Arteriosus ( juxtaductal Coarctation). Male to female ratio is 2:1 It associated with bicuspid aortic valve in 70%. Clinical Features Isolated sever COA may occasionally cause heart failure with pulmonary edema in young infants. The classical sign of COA is a disparity in pulsation & blood pressure in the arms & legs. The femoral, popliteal, posterior tibias & dorsalis pedis pulses are weak or absent in 40% of patients.COA
While the pulses in upper arms & carotid artery are bounding, there radio femoral delay. The BP in the legs is lower than that in the arms. This difference in BP is common in patient with COA who are older than 1 year. Majority of patients are well developed & symptom less. the murmur is discovered during routine examination, its systolic in time best heard at RT second intercostal space ( aortic area), in the suprasternal notch & in both interscapular area The apex beat is forcible due to LF ventricular hypertrophy but it may not displaced for many years. In older patients with well developed collateral blood flow, systolic or contineous machinery may be heard over the LF & RT sides of chest laterally & post.
COA
CXR: the findings depends on the age of the patient, on the effect of hypertension& collateral circulation. Cardiomegaly & pulmonary congestion are noted in infants with sever COA. During childhood mild to moderate cardiomegaly occur due to LF hypertrophy A notch in aortic shadow at a site of Coarctation & aortic arch above with some dilatation of the aorta below, this notch give picture similar to figure 3 shape in CXR ( characteristic), there is also notch at inferior border of the ribs by enlarged intercostal arteries. ECG is usually normal in young children but it reveals LF ventricle hypertrophy in older children. Echocardiography confirm the diagnosis by showing the defect & other associated anomalies like bicuspid aortic valve
COA
Prognosis & complications Some patients survive into late middle age, some die suddenly from cerebro-vascular accident ( CVA). The most common serious complications are related to systemic hypertension, which may result in premature coronary artery disease, heart failure, hypertensive encephalopathy, or intracranial hemorrhage. Infective endocarditis is a significant complication in adult, aneurysm of descending aorta or enlarged collateral vessels may develops. Treatment. Medical treatment like prophylactic antibiotic & antifailure for heat failure Surgical treatment Surgical correction of the narrowed segment of aorta with direct end to end anastomosis is procedure of choice & should be performed before 4 years of age. Or by insertion of graft.
COA
Tetralogy Of Fallots ( TOF)
The anatomical defect classically consists Obstruction of RT ventricle outflow ( pulmonary stenosis) VSD Overriding of aorta RT ventricle hypertrophy Clinical Features Depends on the degree of RT ventricle outflow obstruction Cyanosis, clubbing of fingers Dyspnea on exertion. Characteristically assume squatting position for relieve of dyspnea due to physical effort Paroxysmal hyper cyanotic attacks ( hypoxic or blue spells ) is particular problem during first 2 years of life. clinically the infant become hyperapneic, restless, cyanosis increases, gasping respiration, syncope.
TOF
The spells may last few minutes to few hours. Sever episodes may progress to unconsciousness & to convulsion or hemi paresis. spells is caused by increased shunting of unoxygenated blood via VSD as a result of either increased pulmonary vascular resistance or decreased systemic vascular resistance or combination of both Growth & development delay in sever untreated TOF. normal pulse, normal BP. Substernal RT ventricle pulsation in 50% of cases systolic thrill is felt along LF sternal border Loud harsh systolic ejection murmur result from pulmonary stenosis best heard at LF sternal border. Or may hear pansystolic murmur Single second heart sound
TOF
CXR characteristically may show a boot shape heart as a result of decrease pulmonary artery shadow & RT ventricle hypertrophy. Heart size is normal ECG shows RT axis deviation & RT ventricle hypertrophy, p wave is tall & peak Echocardiography confirm the diagnosis Cardiac catheterization reveals systolic hypertension in the RT ventricle equal to systemic pressure, O2 saturation decreased
TOF
Complications Cerebral thrombosis- usually involve cerebral veins or dural sinuses Cerebral ischemia- commonly in patients under 2 years Brain abscess- commonly in patients over 2 years Infective endocarditis FTT, growth & development delay Congestive heart failure is not usual complication & it is rare .it may occur in young infant with pulmonary atresia & large collateral blood flow
TOF
Treatment Medical treatment Avoid dehydration Iron supplementation because patient with TOF prone to IDA Prophylaxis against infective endocarditis Treatment of cyanotic spells Knee-chest position 100% O2 Morphine subcutaneously in a dose of 0.2 mg\kg Correction of acidosis by IV sodium bicarbonate & correction of any dehydration B-adrenergic blocker-propranolol (inderal ) 0.1-0.2 mg\kg IV Surgical treatment Correction of abnormal anatomy can be done at any time when condition of patient permit. If not possible then palliative surgery can be done by shunt & anastomosis between subclavian artery & pulmonary artery .this palliative operation is called blalock taussig operation
TOF
TOF
TOF
Transposition of Great Arteries (TGA)
In this congenital HD the aorta arises from RT ventricle & pulmonary artery arises from LF ventricle. The pulmonary veins drain normally into RT & LF ventricle respectively. Life could not be maintained in this situation unless there is associated LF to RT shunt through VSD or ASD or PDA or combination of these. It is more common in infants of diabetic mothers Male to female ratio 3:1
TGA
Clinical features Affected infants fail to thrive & usually markedly cyanosed from birth Dyspnea may present at rest & finger clubbing may develop early in life The precordium is prominent & palpation reveals forceful cardiac impulse in LF parasternal area Thrill is absent There may be no murmur on examination or a loud systolic murmur at LF sternal border Cardiac enlargement develops rapidly CXR the characteristic cardiac shadow shows enlarged heart with narrow supracardiac shadow like an egg lying on it is side ( egg on shell) ECG shows RT axis deviation with RT or biventricular hypertrophy, p pulmonale
TGA
Echocardiography confirm the diagnosis Prognosis & complications is poor most patient die in first year of life due to heart failure, hypoxia & pulmonary hypertension Treatment 1-When TGA is suspected an infusion of prostaglandin E1should be initiated immediately after birth to maintain patency of the ductus Arteriosus & improve oxygenation (0.05-0.2 microgram\kg\min). The patient should be kept warm. urgent correction of acidosis & hypoglycemia is essential
TGA
2- Infant who remain severely hypoxic or acidotic despite prostaglandin infusion should undergo Rash kind balloon atrial septostomy. 3- The arterial switch ( jantene ) procedure is surgical treatment of choice for the neonate with TGA and an intact VSD. It is usually performed within first 2 weeks of life. It consist of dividing the aorta & pulmonary artery just above the sinuses & re-anastomosing them into their correct position. 3- previous operation for TGA consisted of some form of atrial switch procedure ( mustard or senning operation) this procedure reverses blood flow at atrial level by surgical creation of an intra-atrial baffle that allows systemic venous blood to directed to the LF atrium, LF ventricle & then via pulmonary artery into lungs. The same baffle also permit oxygenated pulmonary venous blood flow cross over to RT atrium, RT ventricle & aorta.
TGA
TGA
Total Anomalous Pulmonary Vascular return ( TAPVR )
In this cyanotic congenital anomaly all the pulmonary veins, as well as venacava enters RT atrium. There must be an ASD for life to continue. There are several anatomical variation, most commonly pulmonary veins form a common vessel which empties into persistent LF superior venacava less commonly into RT SVC. The pulmonary veins may also unite to form a common trunk which drain directly to RT atrium or into coronary sinus. Rarely pulmonary veins drains to inferior VC or even to portal vein.TAPVR
Clinical FeaturesThe infants present with FTT, cyanosis, become Dyspnea on slight exertion.They extremely prone to repeated chest infection.Cardiac pulsation is forceful over LF parasternal area, pericardium may be bulging.Auscultation reveals triple or quadruple rhythm & there is usually a blowing systolic murmur.CXR shows RT atrium & RT ventricle hypertrophy with congested lungs. In the common type of anomaly with persistent LF SVC, the supracardiac shadow is very broad give rise to so called “ Cottage Loaf “ or figure of eight (8) or snow man.TAPVR
ECG shows RT axis deviation, sever RT ventricle hypertrophy & a P pulmonale. Echocardiography confirm the diagnosis & precisely shows any associated anomalies Catheterization reveals the same O2 concentration in RT atrium, RT ventricle, pulmonary artery & femoral artery. The pressure in RT ventricle & pulmonary artery are above normal. Prognosis Most affected infants develop RT ventricle failure early with progressive dyspnea, cardiomegaly & hepatomegaly. Treatment Medical treatment Surgical treatment ; the majority of the patients need surgical treatment, the most common type of operation is based upon an anastomosis of the common pulmonary venous trunk to the back of LF atrium, we close ASD if present & connection with RT atrium, VC or coronary sinus obliterated
Tricuspid Atresia ( TA )
In TA no outlet from RT atrium to RT ventricle is present. The entire systemic venous return enters LF side of heart by either foramen ovale or an associated ASD. The LF ventricle blood usually flows into RT ventricle via a VSD. Clinical manifestations Cyanosis is usually evident at birth, with the extend depending on the degree of limitation in pulmonary blood flow. An increased LF ventricle impulses may be noticed. The pansystolic murmur audible along LF sternal border. Second sound is usually single.TA
In older patients, cyanosis, polycythemia, Easy fatigability, exertion dyspnea, and occasional hypoxic episodes occur as a result of decrease pulmonary blood flow. Diagnosis CXR shows decrease circulation in both lungs ( oligemic lungs ) ECG shows LF axis deviation & LF ventricle hypertrophy. These features distinguish TA from most other cyanotic heart diseases. Echocardiography confirms the diagnosis reveals the presence of a fibro muscular membrane in a place of tricuspid valve, small RT ventricle, VSD, and large LF ventricle & aorta
TA
Treatment It depend on adequacy of pulmonary blood flow. In severely cyanotic neonate life should be maintained by an infusion of prostaglandin E until a surgical aortopulmonary shunt procedure can be performed. The blalock-Taussig procedure by anastomosis of pulmonary artery to subclavian artery. Some patient with restrictive atrial level communication needs a Rash kind balloon septostomy or surgical septectomy. Another palliative operation is creation of anastomosis between SVC and the pulmonary arteries ( called Glenn shunt) The modified fontan operation is preferred approach. It performed between 1.5-3 yrs of age by anastomosis of RT atrium directly to pulmonary artery.
Truncus Arteriosus ( TAr )
In This defect, a single arterial trunk arises from the heart & supplies the systemic, pulmonary & coronary circulation. A VSD is always present with the truncus overriding the defect & receiving blood from both LF & RT ventricle. The number of truncal valve cusps varies from 2 to as many as 6. Both ventricles are at systemic pressure & both eject blood into truncus. It is a total mixing lesion with admixture of pulmonary & systemic venous return.
Truncus Arteriosus
Clinical manifestations Clinical picture vary with age & depend on pulmonary vascular resistance. In immediate newborn period, signs of heart failure usually absent, minimal cyanosis & a murmur are initial sign. In older infant, pulmonary blood flow is huge & the clinical picture is dominated by heart failure & cyanosis also minimal. Wide pulse pressure & bounding pulses. The heart usually enlarged & precordium is hyper dynamic. 2nd heart sound is loud & single. A systolic ejection murmur with thrill audible along LF sternal border. The murmur frequently accompanied by an early ejection click.Truncus Arteriosus
. An apical mid-diastolic murmur caused by increased flow through the mitral valve. In older children with decrease pulmonary vascular resistance, progressive cyanosis, polycythemia & clubbing developed. Diagnosis CXR shows cardiomegaly due to enlarged both ventricles, pulmonary vascularity increased. EEG shows RT, LF, or combined ventricle hypertrophy. Echocardiography demonstrates the large truncal artery overriding the VSD & the pattern of origin of the branch pulmonary artery. Angiography reveals the large T Arteriosus & more precisely define the origin of pulmonary arteries.Truncus Arteriosus
Prognosis & complications Without surgery majority of patients die during 1st or 2nd yr of life. If pulmonary blood flow is restricted by development of pulmonary vascular resistance, those patient may survive into early adulthood. Treatment In 1st few weeks of life, patients can be treated with anti-failure medication, as pulmonary vascular resistance falls, symptoms of heart failure worsen & surgery is indicated usually in first few weeks of life. VSD is closed & pulmonary arteries are separated from the truncus & continuity is established between RT ventricle & pulmonary arteries with a homograft ( rastelli repair ). In older children who have increase pulmonary vascular resistance routine surgical treatment is contraindicated & heart-lung transplantation is the only option.