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| '''Associate Editor(s)-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu], Atif Mohammad, M.D. | | '''Associate Editor(s)-In-Chief:''' [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu], Atif Mohammad, M.D. |
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| ==Overview== | | ==[[Congenital heart disease cyanotic overview|Overview]]== |
| 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.
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| ==Pathophysiology== | | ==[[Congenital heart disease cyanotic classification|Classification]]== |
| ===Associated Conditions===
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| Some of the defects that are associated with cyanotic congenital heart disease are as follows:
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| *[[Truncus arteriosus]],
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| *[[Total anomalous pulmonary venous connection]]
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| *[[Tetralogy of Fallot]]
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| *[[Transposition of the great vessels]]
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| *[[Tricuspid atresia]]
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| ==Causes== | | ==[[Congenital heart disease cyanotic pathophysiology|Pathophysiology]]== |
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| ===Causes in Alphabetical Order=== | | ==[[Congenital heart disease cyanotic causes|Causes]]== |
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| *[[Aortic valve]] is unable to open wide enough.
| | ==[[Congenital heart disease cyanotic differential diagnosis|Differentiating Congenital heart disease cyanotic from other Diseases]]== |
| *Chemical exposure
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| *[[Coarctation]] or complete interruption of the aorta
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| *[[Ebstein's anomaly]]
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| *Genetic and chromosomal syndromes, such as:
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| **[[Down syndrome]]
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| **[[Trisomy 13]]
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| **[[Turner syndrome]]
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| **[[Marfan syndrome]]
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| **[[Noonan syndrome]]
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| *[[Hypoplastic left heart syndrome]]
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| *Infections (such as [[rubella]]) during pregnancy
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| *Medications prescribed by your doctor or bought on your own and used during pregnancy
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| *Poorly controlled blood sugar levels in women who have [[diabetes]] during pregnancy
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| *[[Pulmonary valve]] may be absent or unable to open wide enough.
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| *Street drugs used during pregnancy
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| *[[Tetralogy of Fallot]]
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| *[[Total anomalous pulmonary venous connection]]
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| *[[Transposition of the great arteries]]
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| *[[Tricuspid valve]] may be absent or unable to open wide enough.
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| *[[Truncus arteriosus]]
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| ==Natural History, Complications, and Prognosis== | | ==[[Congenital heart disease cyanotic risk factors|Risk Factors]]== |
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| The outlook depends on the specific disorder.
| | ==[[Congenital heart disease cyanotic screening|Screening]]== |
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| Complications of cyanotic heart disease include:
| | ==[[Congenital heart disease cyanotic epidemiology and demographics|Epidemiology and Demographics]]== |
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| *Abnormal heart rhythms and [[sudden death]]
| | ==[[Congenital heart disease cyanotic natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| *Chronic high blood pressure in the blood vessels of the lung
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| *[[Heart failure]]
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| *Infection in the heart
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| *[[Stroke]]
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| ==Diagnosis== | | ==Diagnosis== |
| | | [[Congenital heart disease cyanotic history and symptoms| History and Symptoms]] | [[Congenital heart disease cyanotic physical examination | Physical Examination]] | [[Congenital heart disease cyanotic laboratory findings|Laboratory Findings]] | [[Congenital heart disease cyanotic ultrasound|Ultrasound]] | [[Congenital heart disease cyanotic CT|CT]] | [[Congenital heart disease cyanotic MRI|MRI]] | [[Congenital heart disease cyanotic chest x ray|Chest X Ray]] | [[Congenital heart disease cyanotic other imaging findings|Other Imaging Findings]] | [[Congenital heart disease cyanotic other diagnostic studies|Other Diagnostic Studies]] |
| ===Symptoms===
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| Some heart defects cause major problems right after birth. Others cause few, if any, problems until adulthood.
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| One symptom is cyanosis, which usually seen as a bluish color of the lips, fingers, and toes. It may occur while the child is resting, or only when the child is active.
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| Some children have breathing problems ([[dyspnea]]). They may get into a squatting position after physical activity to relieve breathlessness.
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| Others have spells, in which their bodies are suddenly starved of oxygen. During these spells, symptoms may include:
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| *[[Anxiety]]
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| *Breathing too quickly ([[hyperventilation]])
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| *Sudden increase in bluish color to the skin
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| Infants may get tired or sweat while feeding and may not gain as much weight as they should.
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| Fainting ([[syncope]]) and chest pain may occur.
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| Other symptoms depend on the type of cyanotic heart disease, and may include:
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| *Feeding problems or reduced appetite, leading to poor growth
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| *Grayish skin
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| *Puffy eyes or face
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| *Tiredness all the time
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| ===Physical Examination===
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| Physical examination confirms [[cyanosis]]. The child may have [[clubbing|clubbed fingers]]. Abnormal heart sounds, a [[heart murmur]], and lung crackles may be heard.
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| ====Extremities====
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| The child may have [[clubbing|clubbed fingers]].
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| ===Laboratory Findings===
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| ====Electrolyte and Biomarker Studies====
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| The following tests could be performed to help diagnose cyanotic congenital heart disease:
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| *Checking oxygen levels in the blood using an arterial blood gas test or by checking it through the skin with a [[pulse oximeter]]
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| *[[Complete blood count]] (CBC)
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| ====Chest X Ray====
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| A chest X ray may be performed to help diagnose cyanotic congenital heart disease.
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| ====Echocardiography or Ultrasound====
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| The following can be used to help diagnose cyanotic congenital heart disease:
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| *[[ECG]]
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| *Looking at the heart structure and blood vessels using [[echocardiogram]] or MRI of the heart
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| ===Other Diagnostic Studies===
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| The following can be used to help diagnose cyanotic congenital heart disease:
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| *Passing a thin flexible tube ([[catheter]]) into the right or left side of the heart, usually from the groin ([[cardiac catheterization]])
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| *Transcutaneous oxygen monitor (pulse oximeter)
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| *[[Echo-Doppler]]
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| *Testing the heart's electrical system
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| ==Treatment== | | ==Treatment== |
| | [[Congenital heart disease cyanotic medical therapy|Medical Therapy]] | [[Congenital heart disease cyanotic surgery|Surgery]] | [[Congenital heart disease cyanotic primary prevention|Primary Prevention]] | [[Congenital heart disease cyanotic cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Congenital heart disease cyanotic future or investigational therapies|Future or Investigational Therapies]] |
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| ===Pharmacotherapy=== | | ==Case Studies== |
| | | [[Congenital heart disease cyanotic case study one|Case#1]] |
| ====Acute Pharmacotherapies====
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| Infants that remain in the hospital may receive medicines to:
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| *Get rid of extra fluids
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| *Help the heart pump harder
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| *Keep certain blood vessels open
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| *Treat abnormal heartbeats or rhythms
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| ===Surgery and Device Based Therapy===
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| Some infants may need to stay in the hospital after birth so they can receive oxygen or be put on a breathing machine.
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| The treatment of choice for most congenital heart diseases is surgery to repair the defect. There are many types of surgery, depending on the kind of birth defect. Surgery may be needed soon after birth, or it may be delayed for months or even years.
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| ====Pre-Operative Assessment====
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| The infant may be put on [[diuretics]].
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| ====Post-Operative Management====
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| The infant may be put on [[diuretics]].
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| ===Primary Prevention===
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| Women who are pregnant should get good prenatal care.
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| *Avoid using alcohol and drugs during pregnancy.
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| *Tell your doctor that you are pregnant before taking any prescribed medications.
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| *Get a blood test early in the pregnancy to see if you are immune to [[rubella]]. If you are not immune, you must avoid any exposure to rubella and should get immunized right after delivery.
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| *Pregnant women with [[diabetes]] should try to get good control over their blood sugar levels.
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| Some inherited factors may play a role in [[congenital heart disease]]. Many family members may be affected.
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| ==References== | | ==References== |
| {{Reflist|2}} | | {{Reflist|2}} |
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| {{Congenital malformations and deformations of circulatory system}} | | {{Congenital malformations and deformations of circulatory system}} |