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[[Image:Diagram of the human heart (cropped).svg|thumb|250px|Cross-section diagram of a normal human heart.]]
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{{SI}}
{{Congenital heart disease}}
{{WikiDoc Cardiology Network Infobox}}
{{CMG}}


'''Associate Editor-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
'''For patient information, click [[Congenital heart disease(patient information)|here]]'''


'''Associate Editor-In-Chief:''':Atif Mohammad, M.D.
{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com] '''Assistant Editor(s)-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


{{Editor Join}}
{{SK}} CHD; cardiac malformation


==Overview==
==[[Congenital heart disease overview|Overview]]==
'''Congenital heart disease''' (CHD) is heart disease in the newborn, and includes [[congenital heart defect|structural defects]], congenital arrythmias, and [[cardiomyopathy|cardiomyopathies]]. CHD is a defect of the heart that exists primarily at birth, and can describe a wide variety of different abnormalities affecting the heart. CHD occurs when the heart or blood vessels near the heart does not develop properly before birth. Therefore, the heart does not pump because it is not completely developed. Also the blood flow is obstructed in the heart of the vessels nearby, causing an abnormal flow of blood through the heart. Blood flow obstructions put a strain on the heart muscle causing the heart to work harder and beat faster. Abnormal blood flow usually occurs when there is a hole in the walls of the heart and may be an abnormal connection between two arteries outside the heart.
Congenital heart disease (CHD) is the most common type of birth defect, accounting for about 1% of all cases [https://pubmed.ncbi.nlm.nih.gov/17051527/ <nowiki>[4]</nowiki>]. Although mild cases of CHD are not detected until after discharge, most of the critical CHD cases are identified soon after birth requiring surgery or catheter-based intervention in the first year of life. CHD is broadly classified into three major groups, namely, cyanotic CHD, ductal-dependent CHD and critical CHD. Cyanotic CHD involves defects that lead to mixing of deoxygenated blood into the systemic circulation. Ductal-dependent CHD relies on the patency of the ductus arteriosus for supply of blood to the pulmonary or systemic outflow which allows adequate mixing between the parallel circulations. Lesions requiring surgery or catheter-based intervention in the first year of life are referred to as critical CHD which includes ductal-dependent and cyanotic lesions, as well as forms of CHD that, although not requiring surgery in the neonatal period, do necessitate intervention in the first year of life, such as a big ventricular septal defect or an atrioventricular canal defect (or atrioventricular septal defect).  


==Causes==
==[[Congenital heart disease congenital heart disease anatomy |Anatomy]]==
Current knowledge about the causes of congenital heart disease is scanty and largely based on small studies(<1,000 patients) .  Potential identified factors include  environmental factors, such as chemicals, drugs, or infection, and genetic factors.  Many genetic conditions and syndromes are associated with congenital heart disease, such as  [[DiGeorge syndrome]] (22q11 deletion syndrome), [[Holt-Oram syndrome]], and [[Alagille syndrome]]. However, the large majority of those born with congenital heart disease do not have genetic syndromes.  Existing research suggests that the genetics of congenital heart disease may vary by defect, with reported recurrences rates varying from 0% in transposition of the great arteries to 7.8% in atrioventricular septal defects.  Overall total risk has been estimated at approximately 5%, but it is clear that more research is needed to further illuminate causation in congenital heart disease.


==Differential Diagnosis of Causes of Congenital Heart Disease==
==[[Congenital heart disease classification|Classification]]==
* [[Alcohol abuse|Alcohol use of the mother]]
* [[Chemotherapeutic]]s
* [[Down's Syndrome]]
* [[Ellis-van Creveld Syndrome]]
* [[Genetic disorders]]
* [[Immunosuppressive]]s
* [[Hypoxia]] ([[Hypoxia|Lack of oxygen]])
* [[Marfan's Syndrome]]
* [[Noonan Syndrome]]
* [[Radiation]]
* [[Retinoic acid]]
* [[Rubella]]
* [[Thalidomide]]
* [[Trisomy 13]]
* [[Turner's Syndrome]]


==Classification==
[[Aortic stenosis]] | [[Atrial septal defect]] (ASD) | [[Atrial septal defect sinus venosus]] | [[Atrioventricular canal]] | [[Atrioventricular septal defect]] (AVSD) | [[Bicuspid aortic valve]] | [[Brugada syndrome]] | [[Cardiomyopathy]] | [[Coarctation of the aorta]] (CoA) | [[dextro-Transposition of the great arteries]] (d-TGA) | [[Dextrocardia]] | [[Ebstein's anomaly]] | [[Hypoplastic left heart syndrome]] (HLHS) | [[Hypoplastic right heart syndrome]] | [[Interrupted aortic arch]] (IAA) | [[levo-Transposition of the great arteries]] (l-TGA) | [[Lutembacher's syndrome]] | [[Mitral stenosis]] | [[Atrial septal defect ostium primum|Ostium primum]] | [[Atrial septal defect ostium secundum|Ostium secundum]] | [[Partial anomalous pulmonary venous connection]] (PAPVC) | [[Patent ductus arteriosus]] (PDA) | [[Pulmonary atresia]] | [[Pulmonary stenosis]] | [[Septum primum]] | [[Subaortic stenosis]] | [[Tetralogy of Fallot]] (ToF) | [[Total anomalous pulmonary venous connection]] (TAPVC) | [[Tricuspid atresia]] | [[Truncus arteriosus]] | [[Ventricular septal defect]] (VSD)


Congenital heart diseases are classified into 3 basic types
==[[Congenital heart disease pathophysiology|Pathophysiology]]==
#Septal
#Obstructive
#Cyanotic


===Obstruction defects===
==[[Congenital heart disease causes | Causes]]==


Obstruction defects occur when heart valves, arteries, or veins are abnormally narrow or blocked. Common obstruction defects include pulmonary valve stenosis, aortic valve stenosis, and coarctation of the aorta, with other types such as bicuspid aortic valve stenosis and subaortic stenosis being comparatively rare. Any narrowing or blockage can cause heart enlargement or hypertension.
==[[Congenital heart disease differential diagnosis|Differentiating Congenital heart disease from other Disorders]]==


===Septal defects===
==[[Congenital heart disease epidemiology and demographics |Epidemiology and Demographics]]==


The septum is a wall of tissue which separates the left heart from the right heart. It is comparatively common for defects to exist in the interatrial septum or the interventricular septum, allowing blood to flow from the left side of the heart to the right, reducing the heart's efficiency. Ventricular septal defects are collectively the most common type of CHD, although approximately 30% of adults have a type of atrial septal defect called probe patent foramen ovale. Septal defects may or may not cause cyanosis depending on the severity of the defect.
==[[Congenital heart disease risk factors |Risk Factors]]==


===Cyanotic defects===
==[[Congenital heart disease natural history, complications and prognosis | Natural History, Complications and Prognosis]]==
Cyanotic heart defects are called such because they result in cyanosis, a bluish-grey discoloration of the skin due to a lack of oxygen in the body. Such defects include persistent truncus arteriosus, total anomalous pulmonary venous connection, tetralogy of Fallot, transposition of the great vessels, and tricuspid atresia.


==Antenatal Detection and Diagnosis==
==Diagnosis==
Before birth, an obstetric ultrasound scan may be used to screen pregnant women for signs of CHD in their unborn babies. This screening scan is often performed around 20 weeks of pregnancy when the fast moving structures of the fetal heart are large enough to be more easily imaged. 
[[Congenital heart disease diagnosis|Diagnostic Criteria]] | [[Congenital heart disease history and symptoms|History and Symptoms]] | [[Congenital heart disease physical examination|Physical Examination]] | [[Congenital heart disease laboratory findings|Laboratory Findings]] | [[Congenital heart disease electrocardiogram|Electrocardiogram]] | [[Congenital heart disease chest x ray|Chest X Ray]] | [[Congenital heart disease MRI|MRI]] | [[Congenital heart disease CT|CT]] | [[Congenital heart disease echocardiography|Echocardiography]] | [[Congenital heart disease prenatal ultrasound|Prenatal Ultrasound]] | [[Congenital heart disease other imaging findings|Other Imaging Findings]]
If CHD is suspected, a mother will be referred for a fetal echocardiograph, which is a more detailed, diagnostic ultrasound scan by a specialist cardiologist.
It is increasingly possible for specialists to screen for CHD as early as 14 weeks, if CHD is suspected from other factors, such as a family history.
==Postnatal Detection and Diagnosis==
After delivery, if congenital heart disease is present but has not been detected, then a newborn baby may appear blue or breathless.  Signs of CHD are sometimes mistaken for an infection or illness, so it is important to rule this out.
Blueness and/or breathlessness may take some time to present, depending on the type of congenital heart disease and whether there is a duct-dependent lesion (i.e. one relying on an open ductus arteriosis for blood flow).  This duct usually closes within the first three days of life in babies born at term (i.e. at nine months gestation).


==Detection and Diagnosis in Adulthood==
==Treatment==
Although the majority of congenital heart disease diagnoses are made in childhood, there are significant congenital heart defects which may be go undetected until adulthood.  These typically include defects that do not cause cyanosis ("blueness") in childhood but may cause problems over time, such as certain kinds of valve problems, transposition disorders, holes in the heart, and abnormalities of the heart's major veins and arteries.  Congenital heart defects are most commonly diagnosed through an echocardiogram - an ultrasound of the heart which shows the heart's structure.  Cardiac magnetic resonance(MRI) are used to confirm CHD when signs or symptoms occur in the physical examination. An echocardiograph displays images of the might also be used to confirm the problem, particularly in complex defects in which anatomy is hard to determine with echocardiography.    It also finds abnormal rhythms or defects of the heart present with CHD. A chest x-ray may also be issued to look at the anatomical position of the heart and lungs. A Cat Scan(CT) can also be used to visualize CHD. All of these tests are ways to diagnose CHD by a physician.
[[Congenital heart disease medical therapy|Medical Therapy]] | [[Congenital heart disease surgery|Surgery]] | [[Congenital heart disease prevention|Prevention]] | [[Congenital heart disease outcomes|Outcomes]] | [[Congenital heart disease reproduction|Reproduction]]


==ACC / AHA Guidelines-Recommendations for Permanent Pacing in Children, Adolescents, and Patients With Congenital (DO NOT EDIT) <ref name="Epstein"> Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207 </ref>==
==Case Studies==
{{cquote| 
[[Congenital heart disease case study one|Case #1]]
===Class I===
1. Permanent [[pacemaker]] implantation is indicated for advanced [[second-degree AV block|second-]] or [[third-degree AV block]] associated with symptomatic [[bradycardia]], [[ventricular dysfunction]], or low cardiac output. ''(Level of Evidence: C)''


2. Permanent [[pacemaker]] implantation is indicated for [[SND]] with correlation of symptoms during age-inappropriate [[bradycardia]]. The definition of [[bradycardia]] varies with the patient’s age and expected heart rate. ''(Level of Evidence: B)''
==Related Chapters==


3. Permanent [[pacemaker]] implantation is indicated for postoperative advanced [[second-degree AV block|second-]] or [[third-degree AV block]] that is not expected to resolve or that persists at least 7 days after [[cardiac surgery]]. ''(Level of Evidence: B)''
4. Permanent [[pacemaker]] implantation is indicated for congenital [[third-degree AV block]] with a wide QRS escape rhythm, complex [[ventricular ectopy]], or [[ventricular dysfunction]]. ''(Level of Evidence: B)''
5. Permanent [[pacemaker]] implantation is indicated for congenital [[third-degree AV block]] in the infant with a ventricular rate less than 55 bpm or with [[congenital heart disease]] and a ventricular rate less than 70 bpm. ''(Level of Evidence: C)''
===Class IIa===
1. Permanent [[pacemaker]] implantation is reasonable for patients with [[congenital heart disease]] and [[sinus bradycardia]] for the prevention of recurrent episodes of intra-atrial reentrant [[tachycardia]]; [[SND]] may be intrinsic or secondary to antiarrhythmic treatment. ''(Level of Evidence: C)''
2. Permanent [[pacemaker]] implantation is reasonable for congenital [[third-degree AV block]] beyond the first year of life with an average heart rate less than 50 bpm, abrupt pauses in ventricular rate that are 2 or 3 times the basic cycle length, or associated with symptoms due to chronotropic incompetence. ''(Level of Evidence: B)''
3. Permanent [[pacemaker]] implantation is reasonable for [[sinus bradycardia]] with complex [[congenital heart disease]] with a resting heart rate less than 40 bpm or pauses in ventricular rate longer than 3 seconds. ''(Level of Evidence: C)''
4. Permanent [[pacemaker]] implantation is reasonable for patients with [[congenital heart disease]] and impaired hemodynamics due to [[sinus bradycardia]] or loss of AV synchrony. ''(Level of Evidence: C)''
5. Permanent [[pacemaker]] implantation is reasonable for unexplained [[syncope]] in the patient with prior congenital [[heart surgery]] complicated by transient [[complete heart block]] with residual [[fascicular block]] after a careful evaluation to exclude other causes of [[syncope]]. ''(Level of Evidence: B)''
===Class IIb===
1. Permanent [[pacemaker]] implantation may be considered for transient postoperative [[third-degree AV block]] that reverts to sinus rhythm with residual [[bifascicular block]]. ''(Level of Evidence: C)''
2. Permanent [[pacemaker]] implantation may be considered for congenital [[third-degree AV block]] in asymptomatic children or adolescents with an acceptable rate, a narrow QRS complex, and normal ventricular function. ''(Level of Evidence: B)''
3. Permanent [[pacemaker]] implantation may be considered for asymptomatic [[sinus bradycardia]] after biventricular repair of [[congenital heart disease]] with a resting heart rate less than 40 bpm or pauses in ventricular rate longer than 3 seconds. ''(Level of Evidence: C)''
===Class III===
1. Permanent [[pacemaker]] implantation is not indicated for transient postoperative [[AV block]] with return of normal AV conduction in the otherwise asymptomatic patient. ''(Level of Evidence: B)''
2. Permanent [[pacemaker]] implantation is not indicated for asymptomatic [[bifascicular block]] with or without [[first-degree AV block]] after surgery for [[congenital heart disease]] in the absence of prior transient complete [[AV block]]. ''(Level of Evidence: C)''
3. Permanent [[pacemaker]] implantation is not indicated for asymptomatic type I [[second-degree AV block]]. ''(Level of Evidence: C)''
4. Permanent [[pacemaker]] implantation is not indicated for asymptomatic [[sinus bradycardia]] with the longest relative risk interval less than 3 seconds and a minimum heart rate more than 40 bpm. ''(Level of Evidence: C)''}}
==Outcomes==
It is now estimated that the number of adults in the United States who have congenital heart disease is approaching one million. Because of advances in [[cardiac surgery]], many who would not previously have survived childhood, now lead normal or relatively normal lives. However, some increase in complications has been observed in adults who were previously thought to have had successful repair of [[heart defects]]. These complications include [[cardiac arrhythmia]], disorders of [[heart valves]], and [[heart failure]]. Regular check-ups by cardiologists are now recommended for patients with histories of congenital heart disease, including those who may have previously been told that their defects were successfully repaired. Since most adult cardiologists have little experience with congenital heart disease, congenital heart disease centers[http://www.achaheart.org/for_members/clinicdirectory/index.php] have been developed to care for adult patients with more severe congenital heart disease. It is thought that some patients, especially those with more complex disorders, and women who are pregnant or considering pregnancy, would likely do better if they are followed in specialty centers. [http://www.acc.org/qualityandscience/clinical/bethesda/beth32/dirIndex.htm Guidelines] have been developed regarding which patients may be successfully followed in non-specialized cardiology practices, and which should be seen in adult congenital heart disease centers.
==Pathological Findings==
[http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0001.jpg|Tetralogy of Fallot: Gross, a good example of repaired perimembranous septal defect
Image:Congenital heart defect 0002.jpg|Interventricular Septal Defect (Muscular Septum): Gross, natural color, muscular septal defect in newborn
Image:Congenital heart defect 0003.jpg|Subvalvular Ventricular Septal Defect: Gross, good view of defect with overriding aorta
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0004.jpg|Ventricular Septal Defect: Gross, infant heart, pulmonary outlet, muscular septal defect
Image:Congenital heart defect 0005.jpg|Atrioventricular Canal: Gross, right ventricular view of canal defect
Image:Congenital heart defect 0006.jpg|Atrioventricular Canal: Gross, left ventricle view of canal defect (very good example)
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0007.jpg|Perimembranous Ventricular Septal Defect: Gross, an excellent example
Image:Congenital heart defect 0008.jpg|Ventricular Septal Defect: Gross, subvalvular defect, left ventricle view of tetralogy of Fallot (very good example)
Image:Congenital heart defect 0009.jpg|Tetralogy of Fallot: Gross, close-up of aortic valve with subvalvular septal defect with Dacron patch (very good example)
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0010.jpg|Subpulmonic Ventricular Septal Defect: Gross, a well shown lesion.
Image:Congenital heart defect 0011.jpg|Subvalvular Ventricular Septal Defect
Image:Congenital heart defect 0012.jpg|Subvalvular Ventricular Septal Defect
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0013.jpg|Ventricular Septal Defect: Gross, natural color, view of opened heart with lungs attached shows rather well a subvalvular VSD
Image:Congenital heart defect 0014.jpg|Atrioventricular Canal: Gross, patch repair of defect seen from left side showing left atrial portion extending into a cleft mitral valve
Image:Congenital heart defect 0015.jpg|Atrioventricular Canal: Gross, corrected defect with patch viewed from left side atrium and cleft mitral valve
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0016.jpg|Atrial Septal Defect: Gross, (an excellent example) foramen ovale defect with right ventricular hypertrophy and fatty infiltration of the right ventricular wall, enlarged right atrium
Image:Congenital heart defect 0017.jpg|Ventricular Septal Defect: Gross close-up adult heart, small perimembranous septal defect (very good example)
Image:Congenital heart defect 0018.jpg|Interventricular Septal Defect (Muscular Septum): Gross, natural color, low septal defect shown from aortic outlet. The same defect (with a probe in hole) shown from right ventricle.
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0019.jpg|Interventricular Septal Defect (Muscular Septum): Gross natural color right ventricular outlet (probe in defect) view from left ventricular side
Image:Congenital heart defect 0020.jpg|Atrial Septal Defect: Gross natural color infant heart foramen ovale defect, septum secundum
Image:Congenital heart defect 0021.jpg|Aortic Subvalvular Ventricular Septal Defect: Gross, natural color, septal defect has patch repair. Aortic valve is myxomatous. A complex case of truncus with interrupted arch.
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0022.jpg|Interventricular Septal Defect Membranous Septum: Gross natural color close-up (an excellent demonstration)
Image:Congenital heart defect 0023.jpg|Interventricular Septal Defect Membranous Septum: Gross natural color small defect well shown. Aortic cusps are scarred and one is perforated
Image:Congenital heart defect 0024.jpg|Subvalvular Ventricular Septal Defect: Gross, natural color, close-up view of aortic outflow tract with a large subvalvular defect
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0025.jpg|Membranous Interventricular Septal Defect: Gross natural color subvalvular defect with probe immediately inferior to membranous septum
Image:Congenital heart defect 0026.jpg|Subvalvular Ventricular Septal Defect: Gross, fixed tissue, large subpulmonic defect apparently represent left displacement of the pulmonary artery
Image:Congenital heart defect 0027.jpg|Interventricular Septal Defect: Gross, fixed tissue, opened right ventricular outflow tract positioned to show perimembranous septal defect (as surgeon would see it during repair)
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0028.jpg|Ventricular Septal Defect Muscular: Gross, natural color, view from right ventricle with probe in defect right ventricular hypertrophy is evident
Image:Congenital heart defect 0029.jpg|Ventricular Septal Defect Muscular: Gross, natural color, view from left ventricle with probe in defect
Image:Congenital heart defect 0030.jpg|Interventricular Septal Defect Subvalvular with Patch Repair: Gross natural color 19yo with Tetralogy of Fallot also shows overriding aorta
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0031.jpg|Interventricular Septal Defect Subvalvular with Patch Repair: Gross, natural color, close-up
Image:Congenital heart defect 0032.jpg|Interventricular Septal Defect (Perimembranous) with Patch Repair: Gross, natural color, view from right ventricle. A case of inverted ventricles
Image:Congenital heart defect 0033.jpg|Interventricular Septal Defect (Perimembranous) with Patch Repair: Gross, natural color, view from left ventricular outflow tract
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0034.jpg|Ventricular Septal Defect (Subvalvular): Gross, fixed tissue, small heart with opened aorta and subvalvular defect shown. A case of pulmonary artery atresia
Image:Congenital heart defect 0035.jpg|Truncus Arteriosus with Subvalvular Ventricular Septal Defect: Gross, natural color, an excellent view of subvalvular defect. Quadricuspid truncus valve and type I origin of pulmonary arteries
Image:Congenital heart defect 0036.jpg|runcus Arteriosus with Subvalvular Interventricular Septal Defect: Gross, natural color, defect is shown from the right side (view toward right ventricular outlet)
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0037.jpg|Truncus Arteriosus with Subvalvular Interventricular Septal Defect: Gross natural color excellent view of lesion looking at opened aortic ring with quadricuspid aortic valve. A large subvalvular defect (origin of pulmonary arteries is at forceps)
Image:Congenital heart defect 0038.jpg|Av Canal with Left Side Bjork Shiley Prosthetic Valve: Gross, natural color, a close-up view of valve and the bridging defect
Image:Congenital heart defect 0039.jpg|Interventricular Septal Defect (Perimembranous) with Patch Repair: Gross, fixed tissue, a close-up view of patch repair from right ventricle
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0040.jpg|Conduit Right Ventricle to Pulmonary Artery: Gross, fixed tissue, opened conduit showing sutures into ventricle and patch closed perimembranous interventricular septal defect
Image:Congenital heart defect 0041.jpg|Ventricular Septal Defect (Perimembranous): Gross, natural color, (quite good photo - lesion before the operation)
Image:Congenital heart defect 0042.jpg|Ventricular Septal Defect (Subvalvular) Repaired: Tetralogy of Fallot; Gross, fixed tissue, close-up view of a large subvalvular defect repaired with a Dacron patch (overgrown with fibrous tissue prominent subaortic shelf with endocardial thickening).
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0043.jpg|Ventricular Septal Defect (Subvalvular) Repaired: Tetralogy of Fallot; Gross, fixed tissue, close-up view of a large subvalvular defect repaired with a Dacron patch
Image:Congenital heart defect 0044.jpg|Ventricular Septal Defect (Subvalvular) Repaired: Gross, fixed tissue, close-up view of Dacron patch. Nearly completely covered with fibrous tissue
Image:Congenital heart defect 0045.jpg|Transposition Great Vessels with Interventricular Septal Defect: Gross, fixed tissue, opened left ventricular outflow tract into a pulmonary artery (perimembranous defect)
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0046.jpg|Transposition Great Vessels with Interventricular Septal Defect: Gross, fixed tissue, close-up of interventricular septal defect and pulmonary valve
Image:Congenital heart defect 0047.jpg|Double Outlet Right Ventricle: Gross, fixed tissue, close-up view of left ventricular outflow tract and patched ventricular septal defect. The override is obvious in this (very good) close-up view
Image:Congenital heart defect 0048.jpg|Perimembranous Ventricular Septal Defect: Gross, fixed tissue, opened left ventricular outflow tract into aorta. Defect was patched 3 days prior to death
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0049.jpg|Perimembranous Ventricular Septal Defect: Gross, fixed tissue, lesion seen from right ventricle (with patch)
Image:Congenital heart defect 0050.jpg|Perimembranous Interventricular Septal Defect: Gross, fixed tissue, view from right atrium and ventricle with patch placed three days prior to death.
Image:Congenital heart defect 0051.jpg|Ventricular septal defect
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Congenital heart defect 0052.jpg|Ventricular septal defect, view from left ventricle
Image:Congenital heart defect 0053.jpg|Atrial Septal Defect, Septum Primum; View from Right Atrium (a 4 month old baby)
Image:Congenital heart defect 0054.jpg|Atrial Septal Defect, Septum Primum; Also Cleft in Anterior Cusp of Mitral Valve
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:Hypoplastic left ventricle 1.jpg|Hypoplastic left ventricle
Image:Hypoplastic left ventricle 2.jpg|Hypoplastic left ventricle
Image:Hypoplastic left ventricle 3.jpg|Hypoplastic left ventricle
</gallery>
</div>
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<gallery heights="175" widths="175">
Image:Hypoplastic left ventricle 4.jpg|Hypoplastic left ventricle
Image:Hypoplastic left ventricle 5.jpg|Hypoplastic left ventricle
Image:Hypoplastic left ventricle.jpg|Hypoplastic left ventricle
</gallery>
</div>
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<gallery heights="175" widths="175">
Image:821.jpg|Patent Ductus Arteriosus: Gross example in an infant heart
Image:4433.jpg|Patent Ductus Arteriosus: Gross fixed tissue probe in ductus
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:5320.jpg|Patent Ductus Arteriosus: Gross fixed tissue view of ductus opened from pulmonary artery into aorta with edematous appearing intimal surface
Image:5338.jpg|Patent Ductus Arteriosus: Gross natural color opened ductus in infant shows apparent intimal edema in ductus.
</gallery>
</div>
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<gallery heights="175" widths="175">
Image:6818.jpg|Patent Ductus Arteriosus with Aneurysmal Dilation: Gross fixed tissue external photo of heart shows the lesion
Image:6819.jpg|Patent Ductus Arteriosus with Aneurysmal Dilation: Gross fixed tissue aorta and ductus have been cross sectioned showing arch of aorta and huge ductus in a 5 day old infant
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:6820.jpg|Patent Ductus Arteriosus with Aneurysmal Dilation: Gross fixed tissue opened aortic arch and descending thoracic showing very large opening of ductus into aorta
Image:249019.jpg|Patent Ductus Arteriosus
</gallery>
</div>
<div align="left">
<gallery heights="175" widths="175">
Image:3240.jpg|Right Ventricle Hypoplasia: Gross natural color good example showing tiny tricuspid inlet and very small but quite thick right ventricle
Image:3241.jpg|Right Ventricle Hypoplasia: Gross natural color view from right atrium showing patent foramen ovale and very small tricuspid valve
Image:3242.jpg|Right Ventricle Hypoplasia: Gross natural color external view of heart showing very large left ventricle and very small right ventricle delineated by anterior descending branch of left coronary artery
</gallery>
</div>
==External links==
*[http://my.clevelandclinic.org/heart/webchat/adult_congenital_heart_disease_webchat_transcript.aspx Cleveland Clinic Webchat - Adult Congenital Heart Disease Webchat with Dr. Richard Krasuski.]
*[http://my.clevelandclinic.org/heart/webchat/pettersson100307.aspx Cleveland Clinic Webchat - Adult Congenital Heart Disease Surgery Webchat with Dr. Gosta Pettersson.]
*[http://www.itsmyheart.org  It's My Heart Advocating for and Supporting those affected by Congenital Heart Defects - US Non-Profit under section 501(c)3.]
*[http://www.savinglittlehearts.com/ Saving Little Hearts]
*[http://www.card-ag.org Card-AG, The Cardiologycal Working Group of the University Pediatric Clinic Munster]
*[http://www.heartchest.org The Heart Chest]
*[http://www.americanheart.org/presenter.jhtml?identifier=1200000 American Heart Association]
*[http://www.marchofdimes.com/professionals/681_1212.asp Congenital Heart Defect]
*[http://www.nlm.nih.gov/medlineplus/congenitalheartdisease.html Treating Congenital Heart Disease]
*[http://www.bhf.org.uk/hearthealth/index.asp?secID=1&secondlevel=77&thirdlevel=362&artID=509 Coping with Congenital Heart Disease]
*[http://fetus.ucsfmedicalcenter.org/heart/ Fetal Treatment for Congenital Heart Disease (UCSF Fetal Treatment Center)]
*[http://www.eactscongenitaldb.org/ EACTS Congenital Database ― European database of cardiothoracic surgeries with publicly available reports]
*[http://www.achaheart.org/ Adult Congenital Heart Association]
*[http://www.tchin.org/  Congenital Heart Information Network ]
*[http://www.sciencemuseum.org.uk/antenna/tickers/ Fixing Tiny Tickers - Fetal heart surgery]
*[http://www.echocharity.org.uk/ Tiny Tickers - Antenatal congenital heart disease information]
*[http://www.cardiacdiseases.org Cardiacdiseases.org]
==Sources==
* The ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities <ref name="Epstein"> Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207 </ref>
==References==
1. [http://www.heartchest.org “The Heart Chest.” Non-profit Organization.]
2. [http://web7.infotrac.galegroup.com/itw/infomark/1/1/1/purl=rc6_HRCA “Congenital Heart Disease.” Clinical Reference Systems. McKesson Health Solutions LLC , 2004. pg 783. Health Reference Center-Academic. Accessed: 20 Feb. 2006.]
3. [http://web7.infotrac.galegroup.com/itw/infomark/1/1/1/purl=rc6_HRCA Jacob, Dawn A. “Patent Ductus Arteriosus.” Gale Encyclopedia of Medicine. 2001. Health Reference Center-Academic . Accessed: 20 Feb. 2006.]
4. [http://web7.infotrac.galegroup.com/itw/infomark/1/1/1/purl=rc6_HRCA Knopper, Melissa, and Teresa G Odle. “Congenital Heart Disease.” Gale Encyclopedia of Medicine. 2004. Health Reference Center-Academic . 20 Feb. 2006.]
5. [http://web7.infotrac.galegroup.com/itw/infomark/1/1/1/purl=rc6_HRCA Washington, Reginald L. “Hypoplastic Left Heart Syndrome.” Clinical Reference Systems. McKesson Health Solutions LLC , 2004. p 1724. Health Reference Center-Academic. Accessed: 20 Feb. 2006.]
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{{Congenital malformations and deformations of circulatory system}}
{{SIB}}
[[de:Herzfehler]]
[[es:Cardiopatía congénita]]
[[es:Cardiopatía congénita]]
[[fr:Cardiopathie congénitale]]
[[fr:Cardiopathie congénitale]]
[[lv:Iedzimtās sirds slimības]]
[[nn:Medfødd hjartefeil]]
[[pl:Wada serca]]
[[pl:Wada serca]]
[[pt:Cardiopatia congênita]]
[[pt:Cardiopatia congênita]]
[[sr:Урођене срчане мане]]
[[uk:Вроджені вади серця]]
[[wa:Maladeye des bleus påpåds]]
[[tr:Doğumsal kalp hastalıkları]]
[[tr:Doğumsal kalp hastalıkları]]
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[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Congenital heart disease]]
[[Category:Genetic Disease]]
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Latest revision as of 05:29, 21 October 2021

Congenital heart disease Microchapters

Home

Patient Information

Overview

Anatomy

Classification

Pathophysiology

Causes

Differentiating Congenital heart disease from other Disorders

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

MRI

CT

Echocardiography

Prenatal Ultrasound

Other Imaging Findings

Treatment

Medical Therapy

Surgery

Prevention

Outcomes

Reproduction

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, MBBS [2] Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [3]

Synonyms and keywords: CHD; cardiac malformation

Overview

Congenital heart disease (CHD) is the most common type of birth defect, accounting for about 1% of all cases [4]. Although mild cases of CHD are not detected until after discharge, most of the critical CHD cases are identified soon after birth requiring surgery or catheter-based intervention in the first year of life. CHD is broadly classified into three major groups, namely, cyanotic CHD, ductal-dependent CHD and critical CHD. Cyanotic CHD involves defects that lead to mixing of deoxygenated blood into the systemic circulation. Ductal-dependent CHD relies on the patency of the ductus arteriosus for supply of blood to the pulmonary or systemic outflow which allows adequate mixing between the parallel circulations. Lesions requiring surgery or catheter-based intervention in the first year of life are referred to as critical CHD which includes ductal-dependent and cyanotic lesions, as well as forms of CHD that, although not requiring surgery in the neonatal period, do necessitate intervention in the first year of life, such as a big ventricular septal defect or an atrioventricular canal defect (or atrioventricular septal defect).

Anatomy

Classification

Aortic stenosis | Atrial septal defect (ASD) | Atrial septal defect sinus venosus | Atrioventricular canal | Atrioventricular septal defect (AVSD) | Bicuspid aortic valve | Brugada syndrome | Cardiomyopathy | Coarctation of the aorta (CoA) | dextro-Transposition of the great arteries (d-TGA) | Dextrocardia | Ebstein's anomaly | Hypoplastic left heart syndrome (HLHS) | Hypoplastic right heart syndrome | Interrupted aortic arch (IAA) | levo-Transposition of the great arteries (l-TGA) | Lutembacher's syndrome | Mitral stenosis | Ostium primum | Ostium secundum | Partial anomalous pulmonary venous connection (PAPVC) | Patent ductus arteriosus (PDA) | Pulmonary atresia | Pulmonary stenosis | Septum primum | Subaortic stenosis | Tetralogy of Fallot (ToF) | Total anomalous pulmonary venous connection (TAPVC) | Tricuspid atresia | Truncus arteriosus | Ventricular septal defect (VSD)

Pathophysiology

Causes

Differentiating Congenital heart disease from other Disorders

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Prenatal Ultrasound | Other Imaging Findings

Treatment

Medical Therapy | Surgery | Prevention | Outcomes | Reproduction

Case Studies

Case #1

Related Chapters

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