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Fetal circulation EMBRYONIC PERIOD: Placenta serves as the exchange organ for respiration and metabolismus. Oxygenated blood (80%) passes from the placenta through the umbilical vein to the heart. As the flows toward the heart, it mixes with blood from the inferior vena cava and from hepatic veins, so that blood entering the right atrium is approximately 65% saturated. A considerable amount of this blood is shunted across the foramen ovale into the left atrium. The venous blood from upper part is much less saturated - 30% and most of it enters the right ventricule through tricuspid valve. Thus, the blood in right ventricle is a mixture of both relatively highly saturated blood from the umbilical vein and desaturated blood from venae cavae. This mixture result in saturation 50% in the right ventricle. The blood in the left atrium is derived from the blood shunting across the foramen ovale and the blood returning from pulmonary veins. Saturation is 60%. A great deal of the left ventricular output goes to the head, the lower portion of the body is supplied by blood both from right ventricle, through the patent ductus arterious, and from the left ventricle. - till 8 weeks of gestation - foundation of all organs - all have developed - the most vulnerable by exogenic influences viral, chemical, physical ... FETAL PERIOD: - since week 9th of gestation till birth - growth - remodeling - functional development Transient neonatal circulation Basic Principles Cardiovascular diseases are a significant cause of death and chronic illness in pediatric population. Frequency, incidency: about 1% of newborns have congenital heart disease. Note ! The most important clues to the presence of heart disease requiring prompt attention, diagnosis and managment is congestive heart failure and/or cyanosis. (Cyanosis = more than 5g of reduced Hb/l) The cardiologist and neotologist use for this situation a therm: „critical heart disease“. The presence of a heart murmur is not a significant sign of heart disease. The murmur may suggest the possibility of heart disease in an infant, or the murmur can be a functional or innocent murmur. On ohter hand not all serious heart diseases are accompanied by a murmur. Etiology of Congenital Heart Defects With incidence about 1% the CHD are the most common structural malformation. - cca 8% of all CHD are know to be associated with gene or chromosome abnormalities - M.Down, Turner syndrome, DiGeorge syndrome. - multiple enviromental factors: diabetes mellitus, alcohol consumption, progesteron use, and other teratogenes. - congenital - embryo and fetus infections, certain viruses. Typicall example is rubella syndrom (Gregg syndrom - CHD, pulmonary stenosis + cataracta, microphtalmia + deafness, hearing loss) In dealing with families of children with CHD the physician must often answer the question of risk for feature pregnancies. For siblings is cca 2-5%. More higher is the risk for child of affected mother - 1015%. The division of CHD: (and incidence, % of all CHD) a) with left-right shunt - NONCYANOTIC - ventricular septal defect 20% - patent ductus arteriosus 12% - atrial septal defect 10% - atrioventricular septal defect - AV canal 4% The division of CHD: (and incidence, % of all CHD) b) with right-left shunt - CYANOTIC - tetralogy of Fallot 10-15% - complete transposition of the great arteries 10-12% - pulmonary atresia 3 % - tricuspid atresia 1% - hypoplastic left heart 1% - persistent truncus arteriosus 1% - origin of both great vessels from the right ventricule rare - total anomalous pulmonary venous return rare The division of CHD: (and incidence, % of all CHD) c) without shunt 1) Malformations associated with obstruction to blood flow on the right side of the heart: - valvular pulmonary stenosis with intact vent. septum 10% - infundibular pulmonary stenosis with intact v.s. - distal pulmonary stenosis 2) Malformations associated with obstruction to blood flow on the left side of the heart: - coarctation of the aorta 6% - aortic stenosis 0.2% - mitral valve prolaps rare - congenital mitral stenosis rare - cor triatriatum extrem rare - Ebstein´s malformation of the tricuspid valve rare - congenital mitral regurgitation rare - congenital aortic regurgitation - bicuspidal aortic valve 3) Congenital myocardial diseases: all are very rare - glycogen storage disease - glykogenosis type II. Pompe - anomalous origin of the left coronary artery - endocardial fibroelastosis LR shunt Ventricular septal defect: VSD most common CHD - 25% Essential for dg.: - signs depandent on the size of the defect - „ hemodynamic significance“ LR shunt VSD Clinic: depands on size of the defect. History of frequent respiratory infections, pansystolic murmur, children are early sick in infancy. Failure to thrive. Dyspnea, exercise intolerance. Congestive heart failure may develop between 1-6 months of age. Danger: In hemodynamic significant defect can develop pulmonary hypertension and the shunt can revert to rightleft with cyanosis = Eisenmenger syndrome. a. Pharmac. treatment: When signs of congestive heart failure treatment this (Digoxin, Furosemid). b. Surgical treatment: patch 1) 30-50% of all ventricular septal defects close spontaneously and half of the defects that not close become functionally and anatomically smaller. So asymptomatic patients with hearts normal size and without pulmonary hypertension are not indicated for surgical patch. 2) Patients with the ratio of pulmonary to systemic blood flow more than 2:1 or with congestive heart failure and without pulmonary hypertension are indicated for surgical repair in age 2-5 years. 3) In age less than 2 years, when pulmonary hypertension is begining to develop (before reaching of inoperable levels !!!) is surgical repair - patch indicated or in centers which are not capability of doing total correction a palliative procedure pulmonary artery bandage. LR shunt Patent ductus arteriosus: Essential of diagnosis: - variable murmur with active precordium and full pulses in newborns - continuous murmur and full pulses in older infants. DAP LR shunt DAP PDA is the persistence of the normal fetal vessel that joint pulmonary arteria to the descendet aorta. It closes normally during first 4 days of life. Very common problem in intensive care premature nurseries. The frequency in newborns weighing less than 1500 g is to 60%. Clinics: The pulses are bounding and pulse pressure is widened (Psys-Pdia > 1/2 of Psys). Very rough „machinery“ continuous murmur at the 2.intercostal space left, inferioir the clavicule. If the shunt is large, congestive heart failure becomes important. Danger: pulmonary hypertension, subacuta bacterial endocarditis, congestive heart failure. Treatment: Surgical ligature or resection of ductus. Premature newborns are a specific problem. Ligature is rarely done. The pharmacology closure be using of indomethacin the most frequent intervation. Atrial septal defect of the ostium secundum: 10% CHD Essential for dg.: - visualisation of defect by Echo - S2 widely split and fixed - ejection systolic murmur 1-3/6 at the left sternal border pulmonary area, widely radiating - can mimick peripheral pulmonary artery stenosis - diastolic flow murmur at lower left sternal border if shunt is significant in size - ECG with rsR´ in lead V1, right axis deviation. LR shunt ASD LR shunt ASD Clinic: Children with this defect often have no problems. Some patient remain asymptomatic throughtout life, but others can failure to thrive and may be with congestive heart failure and pulmonary hypertension late in life, typically in third decade. When pulmonary hypertension develops the previously left-right shunt revers to right-left shunt and cyanosis appears. Treatment: Surgical closure (patch) is recommended when the ratio of pulmonary to systemic blood flow is greater than 2:1. Operation is performed in age 2-4 years. Early operation is indicated in infants presenting with congestive heart failure and/or pulmonary hypertension. LR shunt AVC Atrioventricular septal defect - AV canal: Essential of diagnosis: - murmur often inaudible in neonates, - loud pulmonary component of S2 - common in infants with Down´s syndrome - ECG with left axis deviation, first-degree heart block in 50% of cases, R, L or combined ventricular hypertrophy High ventricular septal defect, low atrium septal defect of ostium primum and cleft of both valves - septal leaflet of the tricuspid and the arterior leaflet of the mitral valve. Marked left to right shunt at atrial and ventricular levels and regurgitation of both valves. Marked pulmonary hypertension. Incomplete forms are . possible. Clinics: Incomplete form may be indistinguishable from atrial septal defect type ostium secundum. On other hand infants with complete form are severe sick with recurent pneumonia and early congestive heart failure. Treatment: complete anatomic correction as soon as possible RL shunt Tetralogy of Fallot: 1.Ventricular septal defect + 2.Severe infundibular pulm. stenosis - obstruction to right ventricular outflow + 3.Overriding of the aorta (dextroposition) + 4.Right ventricul. hypertrophy ... + other: right sided aortic arch (25%), atrial septal defect (15%) Essential of diagnosis: - cyanosis after the neonatal peridod, - hypoxemic spells during infancy, - systolic ejection murmur at upper left sternal border TOF RL shunt TOF RL shunt TOF Clinics: The degree of R-L shunt and cyanosis is dependent upon the degree of right ventricular outflow obstruction. Failure to thrive. Clubbing of fingers. Quite all children with cyanosis have a mikrocytic, hypochromic polyglobulia with very high hematocrit. Hypoxemic spells: are characteristic signs for Fallot tetralogy.: - 1.sudden onset of cyanosis or deeping of preexisting cyanosis + 2. sudden onset of dyspnea + 3.alteration of consciousness (syncope) + 4.decrease in intesity of murmur. Treatment: Oxygen and placing in knee-chest position + propanolol i.v. - NO Digoxin. Treatment: Total surgical correction as soon as possible. Paliative op „Blalock-Taussig shunt“. RL shunt TGA Complete transposition of the great arteries: Essential of diagnosis:-cyan.newborn-more common in males. The aorta is located anterior to the pulmonary artery and is outgoing from right ventricule, pulmonary artery from left. In most cases associated intracardiac abnormalities are present: v. and a. septal def., pulm. stenosis, patent ductus. Life is possible thanks a shunt via one of these defects. RL shunt TGA Clinics: Deep cyanosis in newborns age. Without emergency treatment can only occasionaly live - cyanosis progress most severe, failure to thrive. Murmurs are not characteristic, dependent on associated defects. Treatment: First help: Continual infusion with prostaglandin E2 to decrease pulmonary hypertension and achieve opened ductus arteriousus. In newborns cathetrization with Rashkind baloon atrioseptostomy - to shunting at atria level. Previously was a paliative operation done - an insertion of an intra-atrial baffle to redirect systemic and pulmonary blood flow to the appropriate artery (Op Mustard, Senning). Now is an anatomic correction - switch of great artery avaible. RL shunt PA Pulmonary atresia: 1) With ventricular septal defect: it is an extreme form of Fallot tetralogy, the pulmonary blood flow is derived via patent ductus arteriousus from aorta. Suddenly death may occurs with postnatal closure of ductus. Treatment is an urgent systemic - pulmonary anastomosis (Blalock-Taussig). 2) With intact ventricular septum: Essential of diagnosis: - cyanosis at birth, - continuous murmur (PDA), - x-ray with concave pulmonary artery and apex tilted upward. Patient´s life depends upon patency of the ductus. Clinic: Severe cyanosis, severe dyspnea. Patient can died suddenly with ductus closure. Treatment: First: maintaining patency of ductus by use of prostaglandin E2. Paliative intervation to achieve the mixing of blood at level of atrias - Rashking baloon atrioseptostomy during cathetrization and paliative shunt. Anatomic correction - definitive reconstruction of the right ventricular outflow tract is in some cases possible at a later age. RL shunt Tricuspid atresia: Hemodynamic is very complex, depends upon concomitant defects: transposition of the great artery, ventricular septal defect, patent ductus arterious, pulmonary stenosis or atresia, hypoplastic pulmonary arteria. Atrial septum defect is life saving. Essential of diagnosis: - marked cyanosis present from birth, - ECG with left axis deviation, right atrial enlargment and left ventricular hypertrophy. Clinic: Because there is no direct communication between the right atrium and right ventricule, the systemic venous return must flow through the atrial septum defect. Filling of the right ventricle depends upon ventricular septal defect. Complete mixing of blood and the degree of the cyanosis - arterial desaturation depends upon other defects / transposition, PDA, pulmonary hypoplasia. Treatment: Fontan procedure - connection of right atrium to right ventricule or pulmonary artery (without valve). TA RL shunt Hypoplastic left heart syndrome: is present by mitral and aortic atresia. Prognosis poor. During the past years a palliative surgical approach is done with the aim of heart transplantation in the later age. RL shunt Total anomalous pulmonary venous return: Essential of diagnosis: - mild cyanosis, - systolic ejection murmur with left sternal border, - right atrial and right ventricular hypertrophy. The pulmonary venous blood does not drain into left atrium but entry into right atrium or into vena cava or portal vena. A right - left shunt is always present at the atrial level via a defect of the septum. Clinic: Complete mixing of venous and arterial blood. Patients are relatively good when the pulmonary resistance and flow are good. In case of increased pulmonary resistance and hypertension the patients are being worst and the cyanosis markedly and develop severe right heart failure. Frequent respiratory infects. Failure to thrive. Treatment: Rashkind. Surgical anatomic correction is possible in some cases. RL shunt without shunt Malformations associated with obstruction to blood flow on the right side of the heart: Valvular pulmonary stenosis with intact vent.septum: Essential of diagnosis: - NO SYMPTOMS with mild and moderately severe cases, - righ-sided congestive heart failure in very severe cases, - systolic ejection murmur, - widely split S2, - right ventricular lift, - dilated pulmonary artery „poststenotic“. Clinic: Cyanosis only in very severe cases - right-left shunt at the atrail level via a septal defect. Patients with a mild or moderate degree of valvular pulmonary stenosis are asymptomatic during childhood. On other hand severe stenosis may produce cyanosis and congestive right heart failure even in neonatal period. Treatment: Balloon valvuloplasty via cathetrization. Surgical valvotomy is possible too. Malformations associated with obstruction to blood flow on the left side of the heart: Coarctation of the aorta: occurs in thoracic portion of the descending aorta., usually in the juxtaductal portion, rather then pre- or postductal postition. Males 3times affected. Essential of diagnosis: - pulse lag in lower extremities, blood pressure of 20 mm Hg greater in upper than in lower extremities, - systolic murmur in the left axilla. without shunt without shunt Coarctation of aorta syndrome: + patent ductus, hypoplasia of aorta isthmus, ventricular septal defect and bicuspid aorta valve. Clinic: Is depended on the hemodynamic significance, mass of the stenosis. Patients may not have symptoms during whole childhood, or only a decreased exercise tolerance and fatigability. In severe cases may congestive left heart failure develop. The most important physical finding is a diminution or abscence of femoral pulses. The pathognomic murmur of coarctation is heard in the interscapular area of the back. Treatment: Resection of the coarctation and end to end anastomosis at the optimal age - as late as possible. Anticongestive measure when left sided congestive heart failure is present. In cases of systemic hypertension systemic resistance decreasing by using of nitroprussid, betablocators - propranolol, trimepranol ... Percutaneous baloon angioplasty is avaible. without shunt Aortic stenosis: is an obstruction to the outflow from the left ventricule at or near the aortic valve: - valvular (75%), - discrete membranous subvalvular (20%), - supravalvular, - idiopathic hypertrophic subaortic stenosis. Essential of diagnosis: - systolic ejection murmur at upper right sternal border, - thrill in carotid arteries, - systolic clic at the apex, - dilatation of the ascending aorta on chest X-ray, left ventricular hypertrophy on ECG Clinic: Most patients may be asymptomatic until the congestive left heart failure develop in the third-fifth decade of life. Although some patients have exercise intolerance and fatigability. Syncope and sudden death are uncommon but possible. Treatment: Surgical repair should be considered only in patient with great gradient (80 mmHg) across the stenosis. Percutanous baloon valvuloplasty is the first choice. without shunt without shunt Mitral valve prolaps: Essential of diagnosis: - midsystolic click best heard with patient in standing position, - late systolic murmur. Clinic: Asymptomatic. Chest pain, palpitation. It is a benign entity. Treatment: No. Betablocators may be usefull in chest pain. Rarely may be complicated by mitral insuficiency. without shunt Ebstein´s malformation of the tricuspid valve: consist of downward displacement of the tricuspid valve such that the greater portion of the valve is attached to the ventricular wall. The upper portion of right ventricule is in the right atrium. Functionally small capacity of right ventricule. Prophylactic Regimens for Dental, Oral, Respiratory Tract, or Esophageal Procedures. (Follow-up dose no longer recommended.) Total children's dose should not exceed adult dose. Standard general prophylaxis for patients at risk: Amoxicillin: Adults, 2.0 g (children, 50 mg/kg) given orally one hour before procedure. Unable to take oral medications: Ampicillin: Adults, 2.0 g (children 50 mg/kg) given IM or IV within 30 minutes before procedure. Prophylactic Regimens for Genitourinary/Gastrointestinal Procedures: High-risk patients: Ampicillin plus gentamicin: Ampicillin (adults, 2.0 g; children 50 mg/kg) plus gentamicin 1.5 mg/kg (for both adults and children, not to exceed 120 mg) IM or IV within 30 minutes before starting procedure. 6 hours later, ampicillin (adults, 1.0 g; children, 25 mg/kg) IM or IV, or amoxicillin (adults, 1.0 g; children, 25 mg/kg) orally.