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Congenital cyanotic heart disease

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2 Congenital cyanotic heart disease
Under supervision of Prof.Dr/ Mariam Abu-Shady Professor of pediatrics and neonatology Faculty of medicine for girls Al-Azhar University Dr.Marwa El-Hady Abd Elmoneim Assisted lecturer of pediatric By: Amira Mohammad Ahmed Amira farag biomy Omnia shams godaa

3 CONTENT Definition of Congenital cyanotic heart disease
Evaluation of Criticlly Ill Neonate with Cyanosis Classification of Congenital cyanotic heart disease Tetralogy of Fallot Pulmonary atresia with VSD Double outlet right ventricle with PS Pulmonary atresia Tricuspid atresia Ebstein's Anomaly

4 Congenital cyanotic heart disease
It is abnormality in cardio circulatory structure that present at birth. CHD occurs in about 8/1000 live births, and those with critical CHD are 2/1000 live births. Prompt diagnosis and treatment of CHD significantly decrease mortality and prevent secondary damage to other organ systems.

5 Evaluation of Criticlly Ill Neonate with Cyanosis
hyperoxia ABG ECG CXR clinical cause pass low po2 High pco2 normal diagnostic distress respiratory fail Low po2 Normal or low pco2 May be abnormal Abnormal heart size and vascular marking. Distress less common Other cardiac fiding. cardiac Slow breathing . Cyanosis improve on stimulation neurologic

6 Classification Cyanotic CHD Decreased pulmonary blood flow
TOF Pulmonary atresia with VSD Double-Outlet Right Ventricle Pulmonary atresia without VSD Tricuspid atresia Ebstein anomaly Increased pulmonary blood flow TGA Truncus arteriosus DORV without PS TAPVR Single ventricale

7 Criteria of Cyanotic CHD with decrease pulmonary blood flow
Central cyanosis clubbing Growth retardation Decreased Pulmonary component of S2 Chest x ray show lung oligaemia ECG show right ventricular hypertrophy or left ventricular hypertrophy Echocardiography : Diagnostic cardiac catheterization; Diagnostic, done preoperative

8 Tetralogy of Fallot It is a condition caused by a combination of four heart defects that are present at birth. These defects are: Pulmonary Infundibular Stenosis Overriding aorta ventricular septal defect (VSD) Right ventricular hypertrophy,

9 Tetralogy of Fallot.

10 Tetralogy of Fallot hypercyanotic spells:
Sometimes, babies with tetralogy of Fallot will suddenly develop worsen of central cyanosis after crying, feeding or having a bowel movement. It is treated by placing child in the knee-to-chest position, oxygen,morphine and propranol. systolic thrill over left sternal border Single second sound Ejection systolic murmur(PS) Treated by: Total correction or The Black-Taussig operation

11 Tetralogy of Fallot boot-shaped heart

12 Pulmonary atresia with VSD
Pulmonary atresia with ventricular septal defect (PA-VSD) is a cyanotic congenital heart disease characterized by underdevelopment of the right ventricular (RV) outflow tract with atresia of the pulmonary valve and a large ventricular septal defect (VSD),

13 Pulmonary atresia with VSD

14 Pulmonary atresia with VSD
A prominent a wave in the jugular pulse may be found. auscultation: systolic murmur usually is audible along the lower left sternal border. A continuous murmur is best heard over the upper chest in the presence of a PDA. If systemic-to-pulmonary collateral arteries are present, continuous murmurs may be diffusely audible over the entire chest and back. Treatment: prostaglandin E2 is often required to keep the ductus arteriosus open Most babies will need a 'Shunt' operation during infancy

15 Double outlet right ventricle with PS
Double outlet right ventricle (DORV) is a congenital heart disease in which the aorta rises from the right ventricle instead of from the left ventricle Both the pulmonary artery and aorta come from the same pumping chamber. No arteries arise from the left ventricle .

16 Double outlet right ventricle with PS

17 Double outlet right ventricle with PS
initial medical management consists of increasing the fraction of inspired oxygen (FIO2), which may be up to 100%. This decreases pulmonary vascular resistance, thereby increasing the amount of blood flow in the lungs Surgical treatment create a tunnel through the VSD. The tunnel connects to the aorta.

18 Pulmonary atresia Pulmonary atresia with intact ventricular septum (PA/IVS) is a condition in which the valve that allows blood to flow from right ventricle of heart to lungs (pulmonary valve) hasn't formed properly or is closed (atresia). PA/IVS is a rare congenital heart disease present from the early stages of a baby's heart development

19 Pulmonary atresia.

20 Pulmonary atresia systolic thrill over pulmonary area
prostaglandin E1 is usually used to help the blood move into the lungs. Open heart surgery to repair or replace the valve

21 Tricuspid atresia Tricuspid atresia is the third most common form of cyanotic congenital heart disease, The deformity consists of a complete lack of formation of the tricuspid valve with absence of direct connection between the right atrium and right ventricle.

22 Tricuspid atresia 1) Atrial Septal Defect 2) Missing Tricuspid Valve 3) Hypoplastic (very small) Right Ventricle 4) Pulmonary Stenosis (narrowing of pulmonary valve) 5) Ventricular Septal Defect 6) Patent Ductus Arteriosus

23 Tricuspid atresia single second sound giant A wave murmer of VSD(+PDA)
prostaglandin E2 is often required to keep the ductus arteriosus open in the early neonatal period until surgery can be performed. Surgical treatment: The Fontan operation

24 Ebstein's Anomaly Ebstein's anomaly is a condition in which the tricuspid valve is malformed and sits lower than normal in the right ventricle. This condition is associated with right ventricle dysfunction, and commonly atrial septal defect or patent foramen ovale.

25 Ebstein's Anomaly =

26 Ebstein's Anomaly Jugular venous pulse
Large a and v waves late in the course of the disease, First heart sound is widely split with loud tricuspid component Third and fourthheart sounds are commonly present Medical Care Antibiotic prophylaxis for infective endocarditis Medical therapy for heart failure - Angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin Surgical Care Surgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities heart sounds are commonly present

27 Critaria CCHD with increased pulmonary blood flow
Poor feeding Recurrent chest infection Recurrent heart failure Increase P2 Chest x ray show plethora

28 Transposition of the great vessels
Def:Transposition of the great vessels is acongenital heart defect in which The two major vessels that carry blood away from the heart -- the aorta and the pulmonary artery -- are switched (transposed).

29 ()

30 Investigation . Chest x-ray

31 Total anomalous pulmonary venous return
Def:Total anomalous pulmonary venous return is acongenital heart disease) in which none of the four veins that take blood from the lungs to the heart is attached to the left atrium (left upper chamber of the heart)


33 Double outlet right ventricle with out pulmonary stenosis

34 Investigation Chest x-rays :no pulmonary stenosis will cause increase pulmonary blood flow resulting in cardiomegaly with increase pulmonary vascular markings.  The mediastinum may be narrow due to malposed great vessels .

35 . ;

36 Truncus arteriosus (TA)
is an uncommon congenital cardiovascular anomaly that is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve (ie, truncal valve).

37 .

38 Classification .Truncus arteriosus type I is characterized by origin of a single pulmonary trunk from the left lateral aspect of the common trunk, with branching of the left and right pulmonary arteries from the pulmonary trunk. .Truncus arteriosus type II is characterized by separate but proximate origins of the left and right pulmonary arterial branches from the posterolateral aspect of the common arterial trunk.

39 Type3,4 .In truncus arteriosus type III, the branch pulmonary arteries originate independently from the common arterial trunk or aortic arch, most often from the left and right lateral aspects of the trunk. This occasionally occurs with origin of one pulmonary artery from the underside of the aortic arch, usually from a ductus arteriosus. .Type IV truncus arteriosus, originally proposed by Collett and Edwards as a form of the lesion with neither pulmonary arterial branch arising from the common trunk, is now recognized to be a form of pulmonary atresia with ventricular septal defect rather than truncus arteriosus.

40 Double inlet left ventricle

41 hypoplastic left heart syndrome (HLHS),
In hypoplastic left heart syndrome (HLHS), the heart’s left side — including the aorta, aortic valve, left ventricle and mitral valve — is underdeveloped.

42 Pathophysiology

43 Heterotaxy Syndrome Heterotaxy syndrome is a rare birth defect that involves the positionof the heart and other organs. nt

44 “right atrial isomerism” and “left atrial isomerism”
In a normal heart, the left atrial appendage looks different than the right. In heterotaxy syndrome, the two appendages look similar.

45 Asplenia Asplenia or right atrial isomerism: Children with this condition have multiple heart defects. They may have septal defects and problems with heart valves, particularly the pulmonary valve. The spleen may be absent (asplenia), and the liver and other organs may be on the wrong side of the body.

46 polysplenia Polysplenia or left atrial isomerism: Children with this condition may have septal defects as well as problems with heart valves and the heart’s electrical system. The spleen may be absent, or there may be several small spleens (polysplenia), instead of one spleen.

47 conclusions

48 References Ariane J. Marelli, Andrew S. Mackie, Raluca Ionescu-Ittu, Elham Rahme and Louise Pilote(2007): Congenital Heart Disease in the General Population : Changing Prevalence and Age Distribution. Circulation.;115: Attie F, Casanova JM, Zabal C, Buendía A, Miranda I, Rijlaarsdam M.(2009): Ebstein's anomaly. Clinical profile in 174 patients. Arch Inst Cardiol Mex;69(1):17-25. Brickner ME, Hillis LD, Lange RA( 2008): Congenital heart disease in adults. Second of two parts. N Engl J Med;342(5): Baba K, Ohtsuki S, Kamada M, Kataoka K, Ohno N, Okamoto Y,( 2009). Preoperative management for tricuspid regurgitation in hypoplastic left heart syndrome. Ped Internat;51: Bacha EA, Daves S, Hardin J, et al: 2006 Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thora e2. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID (2010):Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol;23(1):170-6. Charpie JR,Skinner J,Martin P,Castle.2010.transposition of great vessels inOrphant Journal of rare disease ,published at 13 october 2010 Chiu SN, Wu MH, Su MJ, Wang JK, Lin MT, Chang CC (2012):Coexisting mutations/polymorphisms of the long QT syndrome genes in patients with repaired Tetralogy of Fallot are associated with the risks of life-threatening events. Hum Genet ;77(11): 721-8 Collison SP, Dagar KS, Kaushal SK, Radhakrishanan S, Shrivastava S, Iyer KS (2008): Coronary artery fistulas in pulmonary atresia and ventricular septal defect. Asian Cardiovasc Thorac Ann;16(1):29-32.

49 References Devine WA, Webber SA, Anderson RH(2008): Congenitally malformed hearts from a population of children undergoing cardiac transplantation: comments on sequential segmental analysis and dissection. Pediatr Dev Pathol.;3(2): Durongpisitkul K, Saiviroonporn P, Soongswang J, Laohaprasitiporn D, Chanthong P, Nana A(2008): Pre-operative evaluation with magnetic resonance imaging in tetralogy of fallot and pulmonary atresia with ventricular septal defect. J Med Assoc Thai;91(3):350-5. Duro RP, Moura C, Leite-Moreira A (2010):Anatomophysiologic basis of tetralogy of Fallot and its clinical implications. Rev Port Cardiol;29(4): Elsevier; 2008 Park MK. Park: Pediatric Cardiology for Practitioner, 5th ed. Philadelphia, PA s :chap 14 Fricker FJ(2008). Hypoplastic Left Heart Syndrome – Diagnosis and early management. NeoReviews;9:253. Thiebaud B, Michelakis E, Wu XC, Harry G, Hashimoto K, Archer SL(2008). Fox D, Devendra GP, Hart SA, Krasuski RA (2010) : When 'blue babies' grow up: What you need to know about tetralogy of Fallot. Cleve Clin J Med;77(11):821-8. Freedom RM, Hamilton R, Yoo SJ(2010):The Fontan procedure: analysis of cohorts and late complications. Cardiol Young;10(4):307-31


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