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[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]
[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]
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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
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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)
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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)
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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
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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
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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.
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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.
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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
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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)
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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
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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
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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)
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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
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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).
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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)
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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
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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
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Revision as of 16:49, 29 June 2011

Congenital heart disease
Cross-section diagram of a normal human heart
ICD-10 I01.0, I09.2, I30-I32
ICD-9 420.90
DiseasesDB 9820
MedlinePlus 000182
eMedicine med/1781  emerg/412
MeSH heart disease&field=entry#TreeC14.280.720 C14.280.720

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

Congenital heart disease On the Web

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Risk calculators and risk factors for Congenital heart disease

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editors-In-Chief: Keri Shafer, M.D. [2]; Atif Mohammad, M.D.

Overview

Causes

Differential diagnosis

Congenital heart disease classification

Congenital heart disease septal |Congenital heart disease obstructive|Congenital heart disease cyanotic


Obstruction defects

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.


Cyanotic defects

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

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. 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

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.

ACC / AHA Guidelines-Recommendations for Permanent Pacing in Children, Adolescents, and Patients With Congenital (DO NOT EDIT) [1]

Class I

1. Permanent pacemaker implantation is indicated for advanced 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)

3. Permanent pacemaker implantation is indicated for postoperative advanced 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[3] 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. 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

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology









External links

Sources

  • The ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities [1]

References

1. “The Heart Chest.” Non-profit Organization.

2. “Congenital Heart Disease.” Clinical Reference Systems. McKesson Health Solutions LLC , 2004. pg 783. Health Reference Center-Academic. Accessed: 20 Feb. 2006.

3. Jacob, Dawn A. “Patent Ductus Arteriosus.” Gale Encyclopedia of Medicine. 2001. Health Reference Center-Academic . Accessed: 20 Feb. 2006.

4. Knopper, Melissa, and Teresa G Odle. “Congenital Heart Disease.” Gale Encyclopedia of Medicine. 2004. Health Reference Center-Academic . 20 Feb. 2006.

5. 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.

  1. 1.0 1.1 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


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