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Cardiovascular Disorders Lecturer Xin Yue TIANJIN MEDICAL UNIVERSITY PEDIATRIC DPT. GENERAL HOSPITAL

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Cardiovascular Disorders in Children <ul><li>Congenital heart disease (CHD) </li></ul><ul><li>Rheumatic heart disease </li></ul><ul><li>Kawasaki disease </li></ul><ul><li>Cardiomyopathy </li></ul><ul><li>Viral myocarditis </li></ul><ul><li>Arrhythmia </li></ul>

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Basic Knowledge about Cardiovascular system (CVS)

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Heart, front view

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Heart, internal view

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The components of the first heart sound : The closures of the tricuspid valve and the mitral valve during the systolic phase of the ventricles.

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The components of the second heart sound: The closures of the pulmonic valve and the aortic valve during the diastolic phase of the ventricles .

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Summary of blood circulation

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Anatomic and Physiologic Characteristics of CVS in Children

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1. Fetal circulation and circulatory changes after birth Special structures in the fetal CVS A patent foramen ovale A patent ductus arteriosus A ductus venosus The placenta

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The placenta (nourishing the developing fetus) and the umbilcal cord (connecting a fetus with the placenta of mother)

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Higher pulmonic resistance Lower systemic resistance

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The process of the fetal circulation: Oxygenated blood (placenta ) Liver Inferior vena cava Right atrium Left atrium Left ventricle Ascending aorta Upper part of the body Deoxygenated blood(upper part) Superior vena cava Right ventricle Puomonary artery Descending aorta Lower part of the body Lung Foramen ovale Righ atrium Ductus ateriosus Placenta Ductus venosus

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Right-left blood shunts through the foramen ovale and ductus arteriousus occur because of the higher pulmonic resistance and lower systemic resistance Two ventricles work in parallel rather than in series The concentration of oxygen is highest in the liver, next is the upper part of the body and the last is the lower part of the body. Distinct features of the fetal circulation:

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The changes of circulation after birth Establishment of pulmonary circulation Cessation of the umbilicus-placenta circulation Systemic resistance > pulmonic resistance Right-left blood shunt through the foramen ovale is limited and eventually eliminated, the foramen ovale anatomically closes within 5-7m after birth. Blood shunt through the ductus arteriosus is reduced. Higher oxygen content of the blood and loss of prostaglandins leads to gradual closure of the ductus arteriosus Functional closure within 10-15hs after birth and anatomically within 3m (80%)-1y(95%).

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2. Heart rate The younger the child is , the faster the heart rate is. Age Heart rate Neonate 120-140/min Infancy 110-130/min Preschool age 80-100/min School age 70-90/min

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3. Blood pressure Arterial pressure gradually increases with advancing age. A mature infant averages 10/6.67kPa(75/50mmHg) (1mmHg  0.133kPa, 1kPa  7.5mmHg) The formula for older children(>2y): Systolic pressure=age ×2+80mmHg Diastolic pressure=2/3 systolic pressure Hypertension: Systolic pressure is 20mmHg higher than the normal value Hypotension: Systolic pressure is 20mmHg lower than the normal value The blood pressure in the lower limbs is 20mmHg higher than that in the upper limbs

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Congenital Heart Diseases (CHD) Diagnostic Techniques for CHD VSD Tetralogy of Fallot ASD PDA Introduction of CHD

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Introduction of CHD

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Definition of CHD <ul><li>Congenital heart diseases are abnormalities of the heart's structure and function caused by abnormal or disordered heart development before birth. </li></ul>

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Incidence of CHD <ul><li>About 7-8 per 1000 alive births in developing countries and 2.2 in developed countries </li></ul><ul><li>Eight common lesions, which account for 85 percent of all cases: </li></ul><ul><li>[i] ventricular septal defect (VSD) </li></ul><ul><li>[ii] patent ductus arteriosus(PDA) </li></ul><ul><li>[iii] atrial septal defect (ASD) </li></ul><ul><li>[iv] pulmonary stenosis </li></ul><ul><li>[v] aortic valve stenosis </li></ul><ul><li>[vi] coarctation of the aorta </li></ul><ul><li>[vii] tetralogy of Fallot </li></ul><ul><li>[viii] transposition of great arteries. </li></ul>

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Etiology of CHD <ul><li>Environmental factors existing during first trimester of pregnancy: </li></ul><ul><li>Maternal Infections viral infections Rubella, cytomegalovirus, herpes virus, coxsackievirus. </li></ul><ul><li>Maternal Drugs </li></ul><ul><li>Maternal exposure of radiation </li></ul><ul><li>Maternal metabolic diseases </li></ul><ul><li>Genetic factors </li></ul><ul><li>Chromosomal abnormalities (trisomy-21) </li></ul>

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Classification of CHD <ul><li>With a left to right shunt – occult cyanotic </li></ul><ul><li>VSD, ASD, PDA </li></ul><ul><li>With a right to left shunt- cyanotic </li></ul><ul><li>Tetralogy of Fallot </li></ul><ul><li>Transposition of the great arteries. </li></ul><ul><li>With no shunt - acyanotic obstructive </li></ul><ul><li>Pulmonic stenosis (PS) </li></ul><ul><li>Coarctation of the aorta </li></ul>

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Presenting features of CHD <ul><li>Abnormal Heart Murmurs </li></ul><ul><li>Congestive Heart Failure: rapid and labored breathing </li></ul><ul><li>pallor with peripheral cyanosis </li></ul><ul><li>tachycardia and excessive sweating </li></ul><ul><li>Recurrent Respiratory Tract Infections </li></ul><ul><li>Central cyanosis </li></ul><ul><li>Growth Failure </li></ul><ul><li>hypercyanotic spells </li></ul>

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Complications of CHD <ul><li>Left to right shunt </li></ul><ul><li>Recurrent respiratory infections (pneumonia) </li></ul><ul><li>Infective endocarditis </li></ul><ul><li>Congestive heart failure </li></ul><ul><li>Right to left shunt </li></ul><ul><li>Cerebral infarction </li></ul><ul><li>Brain abscess </li></ul>

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Treatment of CHD <ul><li>Control infection </li></ul><ul><li>Control heart failure </li></ul><ul><li>Control hypoxemic spells </li></ul><ul><li>Surgical treatment </li></ul>

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Diagnostic Techniques for CHD <ul><li>History taking </li></ul><ul><li>Physical examination </li></ul><ul><li>Special examination </li></ul><ul><li>Radiography </li></ul><ul><li>Electrocardiography(ECG, EKG) </li></ul><ul><li>Echocardiography (UCG) </li></ul><ul><li>Catheterization </li></ul>

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History taking Pregnant history of the mother: rubella infection, medications , radiation exposure Family history: family members with CHD Present ill history: all possible cardiac symptoms failure to thrive, feeding difficulty, cyanosis, squatting, respiratory distress, sweating, pallor, syncope.

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<ul><li>General Examination </li></ul><ul><li>Cardiac Examination </li></ul><ul><li>Inspection </li></ul><ul><li>Palpation </li></ul><ul><li>Percussion </li></ul><ul><li>Auscultation </li></ul>Physical examination

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General Examination Poor development Central cyanosis arterial deoxygenation (right - left shunt) Respiratory distress poor systemic output, increased pulmonary blood flow, heart failure Clubbing of fingers or toes prolonged hypoxia

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Inspection Protrusion of the left precardium and sternum often indicates right ventricular enlargment. Position and range of the apex beat Cardiac Examination

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<ul><li>Apical Impulse </li></ul><ul><li>location (normal: 5 th intercostal space just </li></ul><ul><li>medial to the left mid-clavicular line) </li></ul><ul><li>size </li></ul><ul><li>duration </li></ul><ul><li>Character heaves ( systolic lift) </li></ul><ul><li>A thrill often is accompanied with a murmur </li></ul>Palpation

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Can roughly estimate the size and position of the heart Percussion

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<ul><li>First heart sound and second heart sound </li></ul><ul><li>P 2: earlier closure and accentuation; delay and softening </li></ul><ul><li>Murmur: loudness, frequency (pitch), form or shape, cardiac phase, position and radiation. </li></ul>Auscultation

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Radiography Cardiac size Cardiac contours Lung vasculature Pulmonary segment Aortic arch Individual cardiac chambers Special examination

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LV RV RA Aortic arch PS

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Electrocardiography(ECG,EKG) The utility of the ECG in the diagnosis of congenital heart disease is largely for the diagnosis of ventricular and atrial hypertrophy.

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Echocardiography (UCG) The best way to diagnose CHD Safe, noninvasive and accurate. Demonstrate the structure of the components of the heart and the blood flow in the heart.

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Catheterization Need to diagnose has decreased dramatically An invasive, radioactive modality Obtaining pressure measurement and accurate shunt flows which is beyond the scope of Echocardiography.

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<ul><ul><li>Relative Frequency of Congenital Heart Lesions </li></ul></ul><ul><li>Lesions % of All Lesions </li></ul><ul><li>Ventricular septal defect(VSD) 25-30 </li></ul><ul><li>Atrial septal defect (ASD) 6-8 </li></ul><ul><li>Patent ductus arteriosus(PDA) 6-8 </li></ul><ul><li>Tetralogy of Fallot 5-7 </li></ul><ul><li>Pulmonary stenosis (PS) 5-7 </li></ul><ul><li>Transposition of the great arteries (TGA) 3-5 </li></ul><ul><li>The former four types are the focus of this chapter. </li></ul>

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Ventricular septal defect (VSD)

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(VSD) a hole in the septum between the right and left ventricles. The shunt occurs predominantly during ventricular systole. Blood passes from the left to right ventricle and is ejected directly into the pulmonary artery along with systemic venous blood.

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Pathophysiology <ul><li>Left-to-right shunting at the level of ventricles </li></ul><ul><li>Increased flow to RV -LA-LV dilatation or hypertrophy </li></ul><ul><li>Increased pulmonary flow Vasospasm Dynamic pulmonary hypertension intimal hyperplasia and lumen occlusion elevated pulmonary vascular resistance obstructive pulmonary hypertension shunt reversal and cyanosis Eisenmenger's syndrome </li></ul>

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Eisenmenger's syndrome Refers to patients with reversed or bi-directional shunt and sustained cyanosis as a result of a pulmonary vascular obstructive disease (PVOD) and pulmonary hypertension resulting from Long-term presence of the large left to right blood shunt.

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Clinical manifestations <ul><li>Small defect (Roger’s disease, <0.5cm): </li></ul><ul><li>No obvious symptoms </li></ul><ul><li>Characteristic murmur: a high-pitched, harsh, holosystolic murmur, well localized between the third and fourth intercostal spaces along the left sternal border . </li></ul>

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Large defect (>1cm): Manifestations of congestive heart failure: irritability, increased respiratory effort, poor feeding; tachycardia, tachypnea, dyspnea, pallor, diaphoresis, failure to thrive Recurrent respiratory infections Growth retardation Hoarseness in the voice: the recurrent laryngeal nerve being pressed by dilated pulmonary artery Symptoms

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Hyperactive precordial impulse , and a thrill is often palpable Accentuated P2 The murmur: loud, low-pitched, harsh, holosystolic, and loudest along the left sternal border, less well localized than a small VSD, radiate to the right of the sternum Older children with Eisenmenger's syndrome: resting cyanosis , nail-bed clubbing Signs

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Chest X-Ray <ul><ul><ul><li>Normal in small defects </li></ul></ul></ul><ul><ul><ul><li>Small or normal aortic knob in size. </li></ul></ul></ul><ul><ul><ul><li>Dilated main pulmonary artery segment. </li></ul></ul></ul><ul><ul><ul><li>Cardiomegaly (both the left and right </li></ul></ul></ul><ul><ul><ul><li>ventricles and the left atrium). </li></ul></ul></ul><ul><ul><ul><li>Increased pulmonary vascular markings </li></ul></ul></ul>

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VSD

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ECG The ECG suggests left ventricular hypertrophy (LVH), and can show RVH as the pulmonary resistance rises. V 1 V 1 Inverted T wave suggests no pulmonary hypertension Upright T wave indicates pulmonary hypertension

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UCG <ul><li>demonstrates the precise anatomy and physiology of the defect: </li></ul><ul><li>--the presence </li></ul><ul><li>--the location </li></ul><ul><li>--the size </li></ul><ul><li>--associated </li></ul><ul><li>lesions </li></ul>

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A medium-sized muscular ventricular septal defect

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A left-to-right shunt during systole

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A small right-to-left shunt during diastole

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Cardiac catheterization <ul><li>Increased oxygen saturation at the right ventricular level. </li></ul><ul><li>Direct measurement of pulmonary artery pressures and calculation of the pulmonary to systemic flow ratio. </li></ul>

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Treatment <ul><li>small, usually need no treatment. </li></ul><ul><li>large, needs medical management and then surgery to repair the VSD </li></ul><ul><li>----Early surgical repair of VSD is indicated if congestive heart failure appears in infancy and is difficult to control, or the pulmonary : systemic flow ratio>2:1. Otherwise, the operation is performed at preschool age. </li></ul><ul><li>Eisenmenger's syndrome, a heart- lung transplantation. </li></ul>

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Atrial septal defect (ASD)

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Anatomy <ul><li>Three types: </li></ul><ul><li>1. Patent ostium secundum (secundum ASD) : </li></ul><ul><li>a deficiency of the flap valve of the fossa ovalis </li></ul><ul><li>a defect in the growth of the septum secundum </li></ul><ul><li>2. Patent ostium primum (primum ASD): </li></ul><ul><li>locate at the atrioventricular junction </li></ul><ul><li>3. Patent foramen oval </li></ul>

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Pathophysiology <ul><li>The pressure in the right atrium is higher than that in the left atrium at birth and during early neonatal period </li></ul><ul><li>----right-to-left shunt, transient cyanosis. </li></ul><ul><li>The pressure in the left atrium is higher than that in the right atrium when the pulmonary blood flow increases </li></ul><ul><li>----left-to-right shunt. </li></ul><ul><li>Large left-to-right shunt at the level of atrial septum </li></ul><ul><li>----Increased blood flow and enlargement of the right atrium, right ventricle, and pulmonary arteries </li></ul><ul><li>----Decreased blood flow to aorta (systemic circulation ) </li></ul>

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Clinical manifestations Small ASDs usually go undiagnosed for years because children are asymptomatic and physical signs are subtle. Symptoms of congestive heart failure are rare compared to VSDs. However, a few patients do present with a history of recurrent respiratory tract infections . Symptoms

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Signs Protrusion of the precardium may be seen and the right ventricular tap (a systolic lift) is especially palpable from the left sternal border to the midclavicular line. Systolic murmur due to an increased right ventricular stroke volume and relative pulmonary stenosis is heard best in the second intercostal space just along the left sternal border and usually is grade 2 to 3.

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Signs Accentuated second heart sound with fixed and wide splitting the increased right ventricular volume prolonging the right ventricular systole the increased time interval between the aortic and pulmonic component of the second heart sound A soft mid-diastolic murmur the increased tricuspid flow and relative stenosis of the tricuspid is best appreciated along the lower left sternal border

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Chest x-Ray <ul><ul><ul><li>Normal in small defects </li></ul></ul></ul><ul><ul><ul><li>Small or normal aortic knob in size. </li></ul></ul></ul><ul><ul><ul><li>Dilated main pulmonary artery </li></ul></ul></ul><ul><ul><ul><li>segment. </li></ul></ul></ul><ul><ul><ul><li>Cardiomegaly (the right atrium and </li></ul></ul></ul><ul><ul><ul><li>right ventricle). </li></ul></ul></ul><ul><ul><ul><li>Increased pulmonary vascular </li></ul></ul></ul><ul><ul><ul><li>markings. </li></ul></ul></ul>

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ASD

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ECG <ul><ul><li>The electrocardiogram shows evidence of the right ventricular volume load. </li></ul></ul><ul><li>The QRS axis in frontal plane is shifted to the </li></ul><ul><li>right. </li></ul><ul><li>The characteristic electrocardiographic </li></ul><ul><li>pattern is an rsR’ pattern in AVR and the </li></ul><ul><li>right precordial leads (right bundle branch </li></ul><ul><li>block) </li></ul><ul><li>A part of the patients have right atrial and </li></ul><ul><li>ventricular hypertrophy. </li></ul>

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UCG Echocardiography can define the precise position of the atrial defect and demonstrate the volume-loaded right ventricle. And it allows definition of the entire cardiac structure and the exclusion of associated defects.

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Cardiac catheterization <ul><ul><ul><li>Higher oxygen saturation in the right atrium than the superior vena cava. </li></ul></ul></ul><ul><ul><ul><li>The catheter frequently enters the left atrium from the right atrium. </li></ul></ul></ul>

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Treatment Surgical correction is accomplished under direct vision through a right atriotomy while on cardiopulmonary bypass. Most defects are closed by direct suture and surgical mortality is less than 1%.

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Patent ductus arteriosus(PDA) An abnormal persistence of a normal fetal pathway between the main pulmonary artery and the descending aorta .

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Pathophysiology <ul><ul><ul><li>The ductal shunt is from the aorta to the </li></ul></ul></ul><ul><ul><ul><li>pulmonary artery throughout the cardiac </li></ul></ul></ul><ul><ul><ul><li>cycle left atrial and left ventricular </li></ul></ul></ul><ul><ul><ul><li>volume overload left atrial and left </li></ul></ul></ul><ul><ul><ul><li>ventricular enlargement and hypertrophy </li></ul></ul></ul><ul><li>With large and long-term shunts </li></ul><ul><li>pulmonary hypertension occurs right </li></ul><ul><li>ventricular hypertrophy. </li></ul><ul><li>If the pulmonary pressure > aortic pressure </li></ul><ul><li>a right-to-left shunt differential cyanosis </li></ul>

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Clinical manifestations Symptoms Children with small shunts will be asymptomatic. In the presence of a large shunt, signs and symptoms of congestive heart failure, and recurrent respiratory infections will exist.

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Signs <ul><li>Typical murmur: continuous machinery murmur heard best at the second intercostal space along the left sternal border and thrill can be palpable at the loudest area continuous systolic and diastolic turbulent flow from the aorta to the pulmonary artery. </li></ul><ul><li>A wide systemic pulse pressure that produces bounding pulses is the hallmark of a patent ductus arteriosus diastolic flow from the aorta to the pulmonary artery lowering aortic diastolic pressure. </li></ul><ul><li>Accentuated P2 </li></ul><ul><li>Differential cyanosis severe pulmonary hypertension </li></ul><ul><ul><li>. </li></ul></ul>

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Chest x-Ray <ul><ul><ul><li>Normal in small ductus </li></ul></ul></ul><ul><ul><ul><li>Normal or prominent aortic knob . </li></ul></ul></ul><ul><li>Prominent pulmonary arterial segment </li></ul><ul><li>and increased pulmonary vascular </li></ul><ul><li>markings . </li></ul><ul><li>Cardiomegaly resulting from left atrial and </li></ul><ul><li>ventricular enlargement. </li></ul>

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ECG Left ventricular hypertrophy may be present. Some cases also have left atrial hypertrophy. In patients with pulmonary hypertension due to increased blood flow, there is usually biventricular hypertrophy.

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UCG <ul><ul><ul><li>Increased left atrial and left ventricular dimensions are observed with a large ductus. </li></ul></ul></ul><ul><ul><ul><li>Continuous systolic and diastolic turbulent flow from the aorta to the pulmonary artery is diagnostic </li></ul></ul></ul>

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Cardiac catheterization <ul><ul><ul><li>There is evidence of increased oxygen content or saturation at the level of the pulmonary artery. </li></ul></ul></ul><ul><ul><ul><li>Catheter may pass through the ductus from the pulmonary artery to the descending aorta. </li></ul></ul></ul>

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Treatment Surgical ligation through a left thoracotomy is performed safely and at low risk after a noninvasive evaluation.

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Tetralogy of Fallot (TOF) 1. Pulmonary stenosis 2. Ventricular septal defect 3. Overriding aorta 4. Right ventricular hypertrophy

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Pathophysiology Decrease in systemic arterial oxygen saturation and cyanosis is the main pathologic result caused by PS, VSD and overriding aorta oxygen-poor blood returning to the RV is shunted across the VSD to the LV, and/or pumped directly into the overriding aorta in the presence of marked PS Right ventricular outflow tract obstruction results in compensatory right ventricular hypertrophy.

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The hemodynamic consequences and severity of clinical manifestations depend on the degrees of PS: mild PS:Pulmonary outflow resistance<systemic left to right shunt acyanotic or pink TOF moderate-severe PS: right to left shunt decreased pulmonary blood flow causes cyanotic insufficient blood oxygenation TOF A systolic murmur caused by PS P 2 is either faint or absent because of low pulmonary pressure  

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Clinical manifestations Cyanosis and clubbing fingers and toes .

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Fatigability, dyspnea and a squatting position for the relief of dyspnea. <ul><li>squatting an increase in systemic vascular resistance that decreases the right-to-left shunting and increases pulmonary blood flow </li></ul><ul><li>decrease in systemic venous return that reduces a volume burden on the heart </li></ul>Hypoxemic spell Increasing cyanosis, restlessness, and increased rate and depth of respiration suddenly, even syncope. Disappearance or attenuation of the systolic murmur. Convulsions or hemiparesis, coma and death.  The physiologic change: suddenly further increase in resistance at the right ventricular outflow tract further increase in right-to-left shunt and a further decrease in the pulmonary blood flow 

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Growth and development may be delayed in severe untreated tetralogy of Fallot. Cardiac signs:  The left sternal bordor may bulge forward  A cardiac lift (right ventricular impulse) is palpable  A systolic thrill is palpable in 50% of cases Systolic murmur: is frequently loud and harsh, heard best from the second to fourth intercostal spaces along the left sternal border pulmonary stenosis  The pulmoanry component of the second heart sound is either faint or absent. 

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Chest x-Ray Concave main pulmonary arterial segment and remarkably clear lung fields owing to the diminished pulmonary blood flow. Boot-shaped heart The hypertrophied right ventricle makes the cardiac apex upturned The aortic knob is prominent or shiffted to the right .

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ECG <ul><ul><ul><li> Right axis deviation </li></ul></ul></ul><ul><li> Right ventricular hypertrophy . </li></ul>

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UCG Cardiac catheterization The anatomic features of TOF are identified by echocardiography <ul><ul><ul><li> The right ventricular pressure is elevated. </li></ul></ul></ul><ul><ul><ul><li> The pulmonary artery pressure is extremely low. </li></ul></ul></ul><ul><ul><ul><li> Catheter frequently passes from the right ventricle into the overriding ascending arota . </li></ul></ul></ul><ul><ul><ul><li> Injection of contrast material into the right ventricle reveals the right ventricular outflow obstruction and right to left shunt at the ventricular level . </li></ul></ul></ul>

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Laboratory findings  Polycythemia and high hematocrit and hemoglobin Complications  Cerebral thrombosis  Brain abscess  Bacterial endocarditis

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Treatment  Palliative surgery to increase pulmonary blood flow: Infants with symptomatic TOF in the first few months of age  Corrective surgery: at around 6 m  Hypercyanotic spells:  Knee-chest position  Oxygen inhalation  Sedation  Correct metabolic acidosis  Propranalol

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Keys to be remembered <ul><li>The components of the first and second heart sound </li></ul><ul><li>Special structures in the fetal CVS </li></ul><ul><li>Distinct features of the fetal circulation </li></ul><ul><li>The changes of circulation after birth </li></ul><ul><li>Normal Heart rate of different ages </li></ul><ul><li>Eisenmenger’s syndrome </li></ul>

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Keys to be remembered <ul><li>The etiology and classification of CHD </li></ul><ul><li>The pathophysiology, clinical manifestations and chest X-ray of VSD, ASD, PDA, TOF </li></ul><ul><li>The differentiation among VSD, ASD, PDA, TOF </li></ul>

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谢 谢！