نارسایی مادرزادی قلب و عروق CHD،congenital cardio vascular anomalies

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Congenital Cardiovascular Anomalies Behnam Masmoui , CCNS , MSN Hamadan University of Medical Sciences 

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بیماری های مادر زادی قلب در گذشته به 0 نوع سیانوتیک و غير سيانوتيك دسته بندى ميشد اما با توجه به علائم بالینی اين دسته بندی اعتبار نداشت زیرا ممکن بود کودکی با نقص .سیانوتیک دارای پوست صورتی رنگ باشد در تقسیم بندی دیگری که بر مبنای خصوصیات همودینامیکی و :جابجایی گردش خون صورت میگیرد گروه ها شامل .افزایش جریان خون ریوی .کاهش جریان خون ریوی 9.انسداد جریان خروجی خون از قلب اختلاط جریان خون بیش از 060 نوع ناهنجاری مادرزادی قلب تا کنون شناخته شده که بعضی مثل قلب تک بطنی نادر هستند. 

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0 ۴ مش اجان ‎Structural heart defects —due to‏ .1 abnormal development of the heart during the first 2 months after conception 1. Functional heart defects- ex: congenital heart block 3. Positional heart defects — ex: dextrocardia 

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How Situs Inversus Totalis Works “65008 tons ۳6۲5۵۱ ۷۵ Normal Organ Placement 01000 1 are L Atrium و | و 

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Ghurts vs. to lef t: un-oxygenated blood is shunted from the right side of the heart to the left side, and then enters the systemic circulation. : a portion of the oxygenated blood is shunted from the left side of the heart to the right side and enters the pulmonary circulation, Increasing the work load for the right heart 

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(usually left to right shunts): - PDA, ASD, VSD ic (right to left shunts): - TOF, Transposition of the Great Arteries, Hypoplastic Left Heart - O, Sat less than 95% - Child may have chronic hypoxia - Caused by: ۶ Decreased pulmonary blood flow -and/or- left side of the heart tothe sys 

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Cticlogy ‏خام‎ CWO ¢ Unknown in most cases ¢ Incidence of CHD in children is slightly increased if a sibling or parent has CHD * Gender Factors ¢ Environmental Factors * Genetic Factors 

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(ewer ۳۷۶ * Occur equally among males and females, but— - More common in males: aortic stenosis, coarctation of the aorta - More common in females: PDAs, ASDs 

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جسرصاصه ”ا أدج دوو مره 2 ۰ ‏تشه وت(‎ - ‏,هه ممطلم ,۳۵ :ملسل؟)‎ )208), 00 * Octerced Orcs: ‏مارا‎ Pricuspid vdve oboorcwotiies, Ebates Barwa - ‏م۳‎ ‎— Possibly related 7 OWOs: ‏سول 6 وا‎ - ‏اصامصاة)‎ abuse: 00 * Ostercd Oiseuse: — Onubetes! iroesportaiioe oP the yeu vessels, — OGO, sits iwerss, stage vedic, hypoplastic ‏تست‎ لا وب امین :)را - 

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(Beuetico (Puciors (roca 0 (Oueae ‏ا‎ ‎@-O cacd dePevs, OSD Meare] Cri): تساه اه ,وه وا خن توت Ostevyeursis ‘woperPevta: 0 م6 م(۵() وود هک وه ولا ‎(ort‏ 

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(Preveaioa pe OWO * Not possible in most cases * But -- there are actions a woman can take to reduce her risk of having a child with CHD: - Abstain from alcohol during pregnancy - Be immunized against rubella before conception - If diabetic, maintain tight control of blood sugars - Folic acid 400 mcg/daily before conception ma help to prevent CHD (unproven) - If there is a family history of CHD seek gg counseling prior to conception 

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Murmurs Cyanosis -worsens with crying or other exertion Respiratory distress Signs of poor perfusion, such as slow capillary refill, diminished peripheral pulses Fatigue - commonly observed during fe in newborns or during play in childre Failure to thrive 

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(Per Dirk * bugs disterd wits oir cod pukverary vosukor resistrave Puls. Putooary blood Plow 525 ٠ Phe ‏له ره موم و‎ ductus veweur usury dose duriay the Piret day oP fhe ٠ Dhe ductus oteripss usudly closes ‏رسد‎ the First SP — PC hours oP Pe 

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Common Congenital Heart Anomalies 

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Atrial Septal Defect * ASD is an opening in the atrial septum permitting free communication of blood between the at © is < of all CHD. SEPTUM. SECUNOUM 

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Atrial Septal Defect * There are 3 major types: * Secundum ASD - at the Fossa Ovalis, most common. Primum ASD - lower in position & is a form of ASVD, MV cleft. * Sinus Venosus ASD - high in the atrial septum, , associated w/partial anomalous venous retur! the least common. 

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۸ ‘ATRIAL SEPTAL DEFECT (stim Secunaum | Sinus Venosus 

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Atrial Septal Defect Clinical Signs & Symptoms ٠ Rarely presents with signs of CHF or other cardiovascular symptoms. ۰ Most are asymptomatic but may have easy fatigability or mild growth failure. ۰ Cyanosis does not occur unless pulmonary HTN is present. 1 

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Atrial Septal Defect Clinical Signs & Symptoms ¢ Hyperactive precordium, RV heave, fixed widely split S2. ¢ II-II/VI systolic ejection murmur @ LSB. ¢ Mid-diastolic murmur heard over 

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trad Gepra OePent Treatment: * Surgical or catherization laboratory closure is generally recommended for secundum ASD w/a Qp:Qs ratio >2:1. ۰ Closure is performed electively between ages 2 & 5 yrs to avoid late complications. ۱ ۰ Surgical correction is done ea in children w/ CHF or significant P HIN. 

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* Usually closes within 24 to 72 hours after birth * Closure of the ductus may be delayed, or not occur at all in preterm infants * Patent PDA causes increased pulmonary blood flow, pulmonary congestion, increases the workload of the right ventricle; causes increased pulmo venous return and increases wor the right ventricle 

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Patent Ductus Arteriosus (PDA) Vessel connecting Aorta 1 I Oe?) and Pulmonary Artery AO = Aorta PA = Pulmonary Artery Ml oxygen-rich Blood UA = Left Atrium RA = Right Atrium ‏ع مموبوت‎ LV = Left Ventricie ‎Bi000 RV = Right Ventricie‏ لا 

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Pakophysicioy - 08 * With a drop in pulmonary arterial pressure (reduction in hypoxic pulmonary vascular constriction), blood will flow through PDA. - LEFT TO RIGHT SHUNT * Increased pulmonary blood flow may lead to pulmonary edema. - Reduced blood flow to all postductal organs ¢ If pulmonary artery pressure rises above Aortic pressure, blood will move in the other direction. - RIGHT TO LEFT SHUNT 

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0: ‏دوه‎ - POO ¢ Loud grade I to grade III systolic murmur at left sternal border. ¢ Echocardiography 

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Preakvedt - POOP ۰ Restrict fluids. ¢ Diuretics ¢ Prostaglandin Inhibitors - Indomethacin ¢ Surgical closure (ligation). 

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Oountativa oP the (Borta Locdized carrpw tay oF the ‏نت‎ Oore cower in woles that Pewutes Ossvuvidted wit Turcer’s Gyadowe تمه امه موی ‎Ovst‏ ‏قوس 6 ونم ابر blood pressure ic thy Coarctation of the Aorta 

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Oountativa oP the (Borta * Coarctation- is narrowing of the aorta at varying points anywhere from the transverse arch to the iliac bifurcation. * Male: Female ratio 3:1. ¢ Accounts for 7 % of all CHD. 

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Oountativa oP the (Borta Hemodynamics * Obstruction of left ventricular outflow = pressure hypertrophy of the LV. 4) Coarctation the aorta MS { Ayo aN 6 ۱ ne | 

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Oventativa oP the (porta Clinical Signs & Symptoms * Classic signs of coarctation are diminution or absence of femoral pulses. * Higher BP in the upper extremities as compared to the lower extremities. * 90% have systolic hypertension of the upper. extremities. Pulse discrepancy between rt & It arms. 

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Oountativa oP the (Borta Clinical Signs & Symptoms ¢ With severe coarc, acidosis, HF and shock. * Differential cyanosis if ductus is still open * II/VI systolic ejection murmur @ LSB * Cardiomegaly, rib notching on X- 

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‎the @orta‏ اه وونل ‎ 

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Oountativa oP the (Borta Treatment * With severe coarctation maintaining the ductus with prostaglandin E is essential. * Surgical intervention, to prevent LV(Left Ventricular) dysfunction. ¢ Angioplasty is used by some centers. * Re-coarctation can occur, balloon an is the procedure of choice. 

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OGO Ventricular Septal Defect (VSD) * Oost www0e vooyedital heat dePevt * Ou veo dove, or wi 

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Oruriodur Geprl ‏ون‎ ¢ VSD - is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles. Accents far IROL af CUIN ‎Ventricular‏ 5 يمد ‎tal‏ 1 ‎Defect‏ ‎ ‎ ‎ ‎1 ‎Ventricle ۹ ‎© 1007 HeartPoint 

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Oruriodur Geprl ‏ون‎ Hemodynamics * The left to right shunt occurs secondary to PVR(Pulmonary vascular resistance) being < SVR(Systemic vascular resistance), not the higher pressure in the LV. * This leads to elevated RV & pulmona: Pressures & volume hypertrophy of the LA & LV. 

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Orctdoutar Geptal DePevt Clinical Signs & Symptoms « Small - moderate VSD, 3-6mm, are usually asymptomatic and 50% will close spontaneously by age 2yrs. * Moderate - large VSD, almost always Tea symptoms and will require surgical #ép% 

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Orctdoutar Geptal DePevt Clinical Signs & Symptoms + IL-II/VI harsh holosystolic murmur heard along the(left sternal border) LSB, more prominent with small VSD, maybe absent with a very Large VSD. g + Diastolic murmur. * CHF, (Failure to thrive)FTT, Respiratory infect exercise ۹ intolerance hyperactive precordium. Sympto: develop between 1 - 6 months 

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Oruriodur Geprl ‏ون‎ Treatment * Small VSD - no surgical intervention, no physical restrictions, just reassurance and periodic follow-up and endocarditis prophylaxis. * Symptomatic VSD - Medical treatment initially with afterload reducers & diuretics. 

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Orctdoutar Geptal DePevt Treatment ° Indications for Surgical Closure: ¢ Large VSD w/ medically uncontrolled symptomatology & continued FTT. ٠ VSD of any size, secondary to risk of developing AV insufficiency. 

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Panos Crteripsus Truncus Arteriosus. . 4 Pals to ch 

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rns Orterivsus * Defect in which one large vessel arises from right and left heart over a large VSD. * Cyanosis is often present. * CHF common. * Diagnosed with echocardiography and cardiac catheterization. * Surgery: - Separate pulmonary arteries from t - Closure of VSD 7 - Create valved connection between RV afid 

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Head ۱ and ۰ سم Right Lung ~ Heart muscle (myocardium) — Baily and logs ‏اسه‎ 

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Petraioypy 2۳ ‏اصاه۳)‎ Tetralogy of Fallot (TOF or “Tet") TOP = — Orctriouhar septa dePent — ont postive is shiPted to the right gad puer-rides the ‏مه مر‎ 060 ‎Gteapsis oP the pukeocary‏ = رت ‎ ‎ ‎ ‎ ‎ ‎ ‎pening ‏| مسيم ‎0 ‎ution Otstncton 

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MORPHOLOGY ۰ Four morphological defects - Ventricular septal defect - Right ventricular outflow tract obstruction * Subvalvar * Valvar ¢ Supravalvar - Overriding of the aorta - Right ventricular hypertrophy 

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CLINICAL PRESENTATION * Cyanosis not usually noticed at birth * Causes - Child less active in the initial few months - Foetal hemoglobin has more affinity ۳۰ ‏ار‎ ‎for oxygen than adult hemoglobinag 

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CLINICAL PRESENTATION * Cyanosis manifests more as child becomes more active ٠ Physical growth is usually good ¢ Mental development may be dela in severe cases due to chronic me hypoxia of the brain i 

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TREBTOEODT ۳08 ¢ Only surgical - Palliative surgery - Definitive surgery 

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PALLIATIVE SURGERY * Aimed to divert systemic blood into the pulmonary circulation and thus enhance pulmonary flow and oxygenation Standard operation is the modified blalock-taussig shunt or other systemic pulmonary shunts such as pott’s shunt and waterston-cooley shunt 

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)0۶0۳/۱//۱۳۲۳۹۵۶ 207 * Relief of right ventricular outflow tract obstruction ¢ Separation of systemic and pulmonary circulations by closure of the vsd 

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*]۳)30۶ 0۰/۳/۶۲ 02 * Palliative surgery in early childhood followed by definitive surgery in the later years, usually after 3 - 4 years of age * Definitive surgery in the neonatal period or early childhood 

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T.O.F 10 00/۲ 0 6006001006 1۸۵۳/۵ * Delayed definitive repair following shunt in early childhood ¢ Delayed presentation, for definitive repair * Re-operation for delayed complications after definitve repair 

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۳30۵/۳۳/۲۹ ) 8۶ "005 ٠ Most will have a normal circulation postoperatively * C.V.P may run high due to stiff right ventricle and high rvedp * Gas exchange is usually normal * May have heart blocks or bradyarryhthmias 

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Proaspousiiod ve the Creat Orssels ‘Transposition of Great Arteries © Dhe aorte oriqivates Prow the 

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WLypoplstiz bePt Weut 5 Hypoplastic Left Heart Syndrome Pata ‏بات توت‎ ‏تیصو او‎ Vessel Conecting Aorta Very Smal Aorta, 4 0 — and Pulmonary Artery Opening Between — aoa LUnderdeveloped ‘Lek Ventre 

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¢ Several anomalies: - Coarctation of the aorta - Hypoplastic left ventricle - Aortic and mitral valve stenosis or atresia. * Cyanotic defect. ٠ Right heart pumps blood to body through PDA. * Closure of PDA results in hypoten shock, and death. 3 - Maintain hypoxemia with normaliz CO, leve 

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1 Patent foramen ovale 2 Coarctation of the aorta 3 Patent ductus arteriosus 4 Narrowed aorta 5 Hypoplastic left ventricle 6 Aortic atresia 

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عمسلمرظ) ‎Three separate‏ ¢ ‎surgeries.‏ - Norwood procedure ¢ First few days after birth. - Glenn Shunt (Cavo Pulmonary Connection) ¢ 3-9 months of age - Fontan Procedure °2 years of age - Less wait because of damage from pulmonary 

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Gtage 1 - Oonwo0d ‏لحم‎ (b) Shunt is formed (a) Trunk of the pulmonary artery is attached to the aorta (c) Enlarge the atrial septal defect oe 

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Groge 19 - Glew Ghuat Superior vena cava Right pulmonary artery 

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Gtrage 111 — Coote Procedure Pulmonary artery Right atrium connected to the pulmonary artery Fenestration — Baffle created in the right atrium ۴ 

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به محض تشخیص آغاز می گردد. کمک به خانواده جهت سازگاری با اختلال فرصتی برای ارتباط مناسب والد و نوزاد نیست این بیماری بر همه اعضاء اثر سوء می گذارد مادر احساس عدم کفایت می کند. خواهر و برادر احساس اهمال و خشم می ‎SUS‏ از طرفی حمایت بیش از حد از کودک به ضرر همه است نیاز به فرصت برای تکامل اجتماعی دارند. استرس های زیادی دارند. آموزش به خانواده در مورد اختلال لازم است در آموزش از تصاویر استفاده کنیم در مورد پیش آگهی و جراحی نیاز به آگاهی دارند. صداقت داشتن و دادن اطلاعات از تغییرات ایجاد شده مهم است 

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کمک به خانواده جهت تطبیق با اختلال بايد بدانند که چه موقع به پزشک مراجعه کنند. بايد بتوانند در تمام اوقات شبانه روز با یک متخصص قلب دسترسی داشته باشند. باید در مورد میزان فعالیت مجاز بدانند. تامین تغذیه مناسب لازم است اکثرا بی اشتها هستند. در مورد مصرف داروهها آموزش ببینند. آمادگی جهت پرسیجر ها و جراحی مهم است آشنایی با محیط » وسایل و پرسیجر ها مهم است روز پیش از جراحی زمان مناسبی است زمانی از روز باشد که کمترین سرو صدا باشد. بیشتر روی نکات مثبت تاکید کنیم وسایل را باید بشناسند و طرز کار را روی عروسک بینند. در مورد برش جراحی صحبت شود و اینکه باز نمی شود. در مورد اینکه در زمان وجود 97) نمی توانند صحبت کنند. دی و رفتن به خانه صحبت شود. 

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کنترل علائم حیاتی و فشارهای شریانی و وریدی ضربان قلب و تنفس ) دقيقه کامل چک شود. کنترل اسید و باز » الکترولیت ها ,دیسترس های تنفسی صداهای ریوی را بررسی کنیم » تغییرات درجه حرارت مهم است . بلافاصله بعد از عمل هیپوترمی رخ می دهد طی 630-06 ساعت به دلیل پاسخ التهابی افزایش درجه حرارت داریم اما پس از آن نشانه عفونت است چک پانسمان محل مهم است . نیاز به تهویه مکانیکی دارند. پس از آن کودک تشویق به تتفس عمیق و تغییر وضعیت دارند.البته ضد درد مهم است . ساکشن ترشحات در صورت لزوم مراقبت از سوند های سینه ای تامین حداکی استر ات بررسی از نظر عوارض : عفونت کم خونی اختلالات قلبی و ات ریوی 

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(ewer OL , curstoy more oP ‏الم موق‎ 60008 ©- درسنامه پرستاری کودکان ونگ ترجمه مهناز سنجری تحت نظارت سونيا أرزومانيانس سال انتشار 49870 پرستاری نوزادان با تالیف سونیا آرزومانیانس انتشارات بشری ‎GOOF‏