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| {{Infobox_Disease |
| | __NOTOC__ |
| Name = {{PAGENAME}} |
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| Image = Diagram of the human heart (cropped).svg|thumb|250px|
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| Caption = Cross-section diagram of a normal human heart|
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| DiseasesDB = 9820 |
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| ICD10 = {{ICD10|I|01|0|i|00}}, {{ICD10|I|09|2|i|05}}, {{ICD10|I|30||i|30}}-{{ICD10|I|32||i|30}} |
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| ICD9 = {{ICD9|420.90}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = 000182 |
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| eMedicineSubj = med |
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| eMedicineTopic = 1781 |
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| eMedicine_mult = {{eMedicine2|emerg|412}} |
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| MeshName = Congenital heart disease |
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| MeshNumber = C14.280.720 |
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| }}
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| {{Congenital heart disease}} | | {{Congenital heart disease}} |
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| '''For patient information click [[Congenital heart disease(patient information)|here]]''' | | '''For patient information, click [[Congenital heart disease(patient information)|here]]''' |
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| {{CMG}}
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| '''Associate Editors-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]; Atif Mohammad, M.D.
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| ===[[Congenital heart disease overview | Overview]]===
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| ===[[Congenital heart disease causes | Causes]]===
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| ===[[Congenital heart disease differential diagnosis|Differential diagnosis]]===
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| ===Congenital heart disease classification===
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| [[Congenital heart disease septal]] |[[Congenital heart disease obstructive]]|[[Congenital heart disease cyanotic ]]
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| ===Obstruction defects===
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| 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.
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| ===Cyanotic defects===
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| 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.
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| ==Antenatal Detection and Diagnosis==
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| 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.
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| 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.
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| 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.
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| ==Postnatal Detection and Diagnosis==
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| 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.
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| 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).
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| ==Detection and Diagnosis in Adulthood==
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| 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.
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| ==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>==
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| {{cquote|
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| ===Class I===
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| 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)''
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| 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)''
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| 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)''
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| 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)''
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| 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)''
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| ===Class IIa===
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| 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)''
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| 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)''
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| 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)''
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| 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)''
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| 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)''
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| ===Class IIb===
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| 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)''
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| 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)''
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| 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)''
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| ===Class III===
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| 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)''
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| 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)''
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| 3. Permanent [[pacemaker]] implantation is not indicated for asymptomatic type I [[second-degree AV block]]. ''(Level of Evidence: C)''
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| 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)''}}
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| ==Outcomes==
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| 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.
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| ==Pathological Findings==
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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]
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| <div align="left">
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| <gallery heights="175" widths="175">
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| Image:Congenital heart defect 0052.jpg|Ventricular septal defect, view from left ventricle
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| Image:Congenital heart defect 0053.jpg|Atrial Septal Defect, Septum Primum; View from Right Atrium (a 4 month old baby)
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| Image:Congenital heart defect 0054.jpg|Atrial Septal Defect, Septum Primum; Also Cleft in Anterior Cusp of Mitral Valve
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| </gallery>
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| </div>
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| | {{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] |
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| <div align="left">
| | {{SK}} CHD; cardiac malformation |
| <gallery heights="175" widths="175">
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| Image:Hypoplastic left ventricle 1.jpg|Hypoplastic left ventricle
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| Image:Hypoplastic left ventricle 2.jpg|Hypoplastic left ventricle
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| Image:Hypoplastic left ventricle 3.jpg|Hypoplastic left ventricle
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| </gallery>
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| </div>
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| | ==[[Congenital heart disease overview|Overview]]== |
| | 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). |
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| <div align="left">
| | ==[[Congenital heart disease congenital heart disease anatomy |Anatomy]]== |
| <gallery heights="175" widths="175">
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| Image:Hypoplastic left ventricle 4.jpg|Hypoplastic left ventricle
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| Image:Hypoplastic left ventricle 5.jpg|Hypoplastic left ventricle
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| Image:Hypoplastic left ventricle.jpg|Hypoplastic left ventricle
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| </gallery>
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| </div>
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| | ==[[Congenital heart disease classification|Classification]]== |
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| <div align="left">
| | [[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) |
| <gallery heights="175" widths="175">
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| Image:3240.jpg|Right Ventricle Hypoplasia: Gross natural color good example showing tiny tricuspid inlet and very small but quite thick right ventricle
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| Image:3241.jpg|Right Ventricle Hypoplasia: Gross natural color view from right atrium showing patent foramen ovale and very small tricuspid valve
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| 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
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| </gallery>
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| </div>
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| ==External links== | | ==[[Congenital heart disease pathophysiology|Pathophysiology]]== |
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| *[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.]
| | ==[[Congenital heart disease causes | Causes]]== |
| *[http://my.clevelandclinic.org/heart/webchat/pettersson100307.aspx Cleveland Clinic Webchat - Adult Congenital Heart Disease Surgery Webchat with Dr. Gosta Pettersson.]
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| *[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.]
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| *[http://www.savinglittlehearts.com/ Saving Little Hearts]
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| *[http://www.card-ag.org Card-AG, The Cardiologycal Working Group of the University Pediatric Clinic Munster]
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| *[http://www.heartchest.org The Heart Chest]
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| *[http://www.americanheart.org/presenter.jhtml?identifier=1200000 American Heart Association]
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| *[http://www.marchofdimes.com/professionals/681_1212.asp Congenital Heart Defect]
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| *[http://www.nlm.nih.gov/medlineplus/congenitalheartdisease.html Treating Congenital Heart Disease]
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| *[http://www.bhf.org.uk/hearthealth/index.asp?secID=1&secondlevel=77&thirdlevel=362&artID=509 Coping with Congenital Heart Disease]
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| *[http://fetus.ucsfmedicalcenter.org/heart/ Fetal Treatment for Congenital Heart Disease (UCSF Fetal Treatment Center)]
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| *[http://www.eactscongenitaldb.org/ EACTS Congenital Database ― European database of cardiothoracic surgeries with publicly available reports]
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| *[http://www.achaheart.org/ Adult Congenital Heart Association]
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| *[http://www.tchin.org/ Congenital Heart Information Network ]
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| *[http://www.sciencemuseum.org.uk/antenna/tickers/ Fixing Tiny Tickers - Fetal heart surgery]
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| *[http://www.echocharity.org.uk/ Tiny Tickers - Antenatal congenital heart disease information]
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| *[http://www.cardiacdiseases.org Cardiacdiseases.org]
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| ==Sources== | | ==[[Congenital heart disease differential diagnosis|Differentiating Congenital heart disease from other Disorders]]== |
| * 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>
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| ==References== | | ==[[Congenital heart disease epidemiology and demographics |Epidemiology and Demographics]]== |
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| 1. [http://www.heartchest.org “The Heart Chest.” Non-profit Organization.]
| | ==[[Congenital heart disease risk factors |Risk Factors]]== |
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| 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.]
| | ==[[Congenital heart disease natural history, complications and prognosis | Natural History, Complications and Prognosis]]== |
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| 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.]
| | ==Diagnosis== |
| | [[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]] |
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| 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.]
| | ==Treatment== |
| | [[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]] |
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| 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.]
| | ==Case Studies== |
| | [[Congenital heart disease case study one|Case #1]] |
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| {{Reflist|2}}
| | ==Related Chapters== |
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| {{Congenital malformations and deformations of circulatory system}}
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| {{SIB}}
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| [[de:Herzfehler]]
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| [[es:Cardiopatía congénita]] | | [[es:Cardiopatía congénita]] |
| [[fr:Cardiopathie congénitale]] | | [[fr:Cardiopathie congénitale]] |
| [[lv:Iedzimtās sirds slimības]]
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| [[nn:Medfødd hjartefeil]]
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| [[pl:Wada serca]] | | [[pl:Wada serca]] |
| [[pt:Cardiopatia congênita]] | | [[pt:Cardiopatia congênita]] |
| [[sr:Урођене срчане мане]]
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| [[uk:Вроджені вади серця]]
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| [[wa:Maladeye des bleus påpåds]]
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| [[tr:Doğumsal kalp hastalıkları]] | | [[tr:Doğumsal kalp hastalıkları]] |
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