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==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Disease Name===


* '''1 Stage 1 - Name of stage'''
* '''Conditions associated with decreased concentration of inspired oxygen:'''
** 1.1 '''Specific Organ system involved 1'''
** [[Smoke inhalation]] most commonly from house fires
*** 1.1.1 '''Adult'''
** [[Carbon monoxide poisoning]]
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''  
** Hydrogen [[cyanide poisoning]]
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
** Intentional or unintensional exposure to asphyxiating gases (eg, [[Propane]], [[methane]], [[Butane|butane,]] [[Hydrogen sulfide|hydrogen sulphide]])
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
* '''Impairment of chest wall or lung expansion:'''
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
** External compression
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
** [[Pneumothorax]]
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
** [[Hemothorax]]
*** 1.1.2 '''Pediatric'''
** [[Flail chest]]
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
* '''Hypoventilation:'''
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
** '''Upper airway obstruction:''' 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
*** [[Foreign body aspiration]]
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
*** [[Pertussis]] / [[Croup]]
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
*** [[Epiglottitis]]
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
*** [[Bacterial tracheitis]]
****1.1.2.2 (Specific population e.g. ''''''children < 8 years of age'''''')
*** Traumatic disruption (Thermal injury, fractures)
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
*** [[Acute chest syndrome]]
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
*** Congenital airway abnormalities:
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
**** [[Choanal atresia]]
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
**** [[Laryngotracheomalacia]]
** 1.2 '''Specific Organ system involved 2'''
**** [[Macroglossia]]
*** 1.2.1 '''Adult'''
**** [[Micrognathia]] or [[Retrognathism|retrognathia]] (eg, Pierre-Robin syndrome)
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
** '''Neurologic abnormalities:'''
*** 1.2.2  '''Pediatric'''
*** [[CNS depression]]
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
*** [[Birth asphyxia]]
*** Severe head trauma
*** [[Apnea of prematurity]]
*** [[Obstructive sleep apnea]]
*** Infections (eg, [[meningitis]], [[encephalitis]])
*** [[Intraventricular hemorrhage]]
*** [[Seizure|Seizures]]
*** Cyanotic breath holding spells
*** [[Coma]]
** '''Neuromuscular disorders:'''
*** [[Myasthenia gravis]]
*** Injury to the phrenic nerve
*** Type 1 spinal muscular dystrophy (Wernig-Hoffman disease)
* '''Intrinsic lung diseases''':
** [[Asthma]]
** [[COPD]]
** [[Pneumonia]]
** [[Bronchiolitis]]
** [[Acute respiratory distress syndrome|Respiratory distress syndrome]] (Hyaline membrane disease)
** [[Empyema]]
** [[Pleural effusion]]
** [[Cystic fibrosis]]
** [[Atelectasis]]
** [[Bronchopulmonary dysplasia]]
** [[Alveolar capillary dysplasia]]
* '''Vascular causes''':
** [[Cardiac tamponade]]


* 2 '''Stage 2 - Name of stage'''
'''Cyanotic congenital heart diseases:'''
** 2.1 '''Specific Organ system involved 1 '''
* '''Decreased pulmonary flow:'''
**: '''Note (1):'''  
** [[Tetralogy of Fallot|Tetralogy of fallot]]
**: '''Note (2)''':
** Tricuspid valve anomalies:
**: '''Note (3):'''
*** [[Tricuspid atresia]]
*** 2.1.1 '''Adult'''
*** [[Tricuspid stenosis]]
**** Parenteral regimen
*** [[Ebstein's anomaly of the tricuspid valve|Ebstein's anomaly]]
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
** [[Pulmonary valve stenosis|Pulmonary stenosis]] (critical valvular)
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
** [[Pulmonary atresia]] with intact ventricular septum
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
* '''Increased pulmonary flow:'''
**** Oral regimen
** [[TGA]] (Transposition of great arteries, most common dextro type)
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
** [[Truncus arteriosus]]
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
** [[Total anomalous pulmonary venous connection|TAPVC]] (Total anamalous pulmonary venous connection)
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
* '''Heart failure:''' Condition that present with cyanosis and severe heart failure include:
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
** Left sided obstructive lesion ([[Hypoplastic left heart syndrome|HLHS]])
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
** [[Aortic coarctation|Coarctation of aorta]]
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
** Critical valvular [[aortic stenosis]]
*** 2.1.2 '''Pediatric'''
** [[Eisenmenger's syndrome|Eisenmenger syndrome]]
**** Parenteral regimen
** [[congestive heart failure]]
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
** [[Atrial septal defect]]
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
** [[Pulmonary hypertension]]
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) ''''''(Contraindications/specific instructions)''''''
** [[Pulmonary edema]]
**** Oral regimen
** [[Pulmonary hemorrhage]]
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
** [[Pulmonary embolism]]
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
** [[pulmonary arteriovenous malformation]]
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
** Multiple small intrapulmonary shunts
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
** [[Shock]]
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
** [[Sepsis]]
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** [[Amniotic fluid embolism]]
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
* '''Hematologic abnormalities:'''
**: '''Note (1):'''  
** [[Methemoglobinemia]] (congenital or acquired)
**: '''Note (2)''':
** [[Sulfhemoglobinemia]] (acquired)
**: '''Note (3):'''
** Hemoglobin mutations with low oxygen affinity:
*** 2.2.1 '''Adult'''
*** Hb Kansas
**** Parenteral regimen
*** Hb Beth israel
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*** Hb Saint Mande
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
*** Hb Bruxells
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
** [[Polycythemia vera]]
**** Oral regimen
** [[Disseminated intravascular coagulation]]
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
* '''Metabolic disorders:'''
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
** Severe [[hypoglycemia]]
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
** [[Inborn error of metabolism|Inborn errors of metabolism]]
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
==== Peripheral Cyanosis ====
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
There following are some of the common causes of peripheral cyanosis: 
*** 2.2.2 '''Pediatric'''
* Various conditions that cause central cyanosis can also present with peripheral cyanosis.
**** Parenteral regimen
* Common conditions where peripheral cyanosis is more specific:
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
** [[Hypothermia|Cold exposure]]
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
** [[Acrocyanosis]]
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)  
** Erythrocyanosis
**** Oral regimen
** [[Raynaud's phenomenon]]
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
** [[Raynaud's disease]]
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
** Arterial obstruction:
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
*** [[Peripheral arterial disease|Peripheral vascular disease]]
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
*** [[Buergers disease]]
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
** Venous obstruction:
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
*** [[Thromboembolism]]
*** [[Deep vein thrombosis]]
*** [[Superior vena cava syndrome]]
** Decreased cardiac output:
*** [[Congestive heart failure|Left sided heart failure]]
*** [[Shock]]
*** [[Hypovolemia]]
** Redistribution of blood flow from extremities
 
Newborns with cyanosis require maintains adequate tissue perfusion and oxygenation.
 
Specific interventions for neonatal cyanotic congenital heart disease (CHD) include administration of prostaglandin E1 and cardiac catheter palliative or corrective procedures.
 
Initial management begins with general care that includes
 
cardiorespiratory support
 
monitoring to ensure sufficient organ/tissue perfusion and oxygenation
 
an adequate airway should be established immediately and supportive therapy (eg, supplemental oxygen and/or mechanical ventilation) instituted as needed.
 
Placement of secure intravenous and intraarterial catheters is most easily accomplished via the umbilical vessels.
 
This will enable efficient correction and monitoring of acid-base balance
 
metabolic derangements (eg, hypoglycemia, hypocalcemia), and blood pressure.
 
Inotropic agents such as dopamine or dobutamine may be necessary to correct hypotension.
 
In infants with severe polycythemia (>70 percent), an isovolumetric partial exchange transfusion should be performed with saline to reduce the hematocrit.
 
Antibiotics
 
broad spectrum antibiotics should be initiated (ampicillin and gentamicin) after obtaining blood and urine cultures.
 
Specific CHD measures
 
An infant who fails the hyperoxia test and does not have persistent pulmonary hypertension of the newborn or a chest radiograph consistent with lung disease is likely to have a cyanotic CHD. In most cases, cyanotic CHD is dependent upon a patent ductus arteriosus (PDA) for pulmonary or systemic blood flow. Closure of the ductus arteriosus can precipitate rapid clinical deterioration with significant life-threatening changes (ie, severe metabolic acidosis, seizures, cardiogenic shock, cardiac arrest, or end-organ injury).
 
As a result, infants with ductal-dependent lesions are at increased risk for death and significant morbidity unless interventions are initiated to maintain patency of the ductus arteriosus for ductal-dependent lesions, ensure adequate mixing of deoxygenated and oxygenated blood, or relieve obstructed blood flow.
 
In infants with or who have a clinical suspicion for a ductal-dependent congenital heart defect, prostaglandin E1 (alprostadil) should be administered until a definitive diagnosis or treatment is established [7].
 
The initial dose is dependent on the clinical setting, as the risk of apnea, one of the major complications of prostaglandin E1 infusion, is dose dependent.
 
●If the ductus is known to be large in a patient with duct-dependent physiology, the initial dose is 0.01 mcg/kg per minute. This scenario typically is seen in patients with echocardiographic confirmation of a large PDA who are cared for in a tertiary center that provides treatment for neonates with cyanotic heart disease.
 
●If the ductus is restrictive or the status of the ductus is unknown, the initial dose is 0.05 mcg/kgper minute. This is the standard dose used in patients who require transport to a center with expertise in the care of neonates with cyanotic heart disease.
 
The dose of prostaglandin can be increased as needed to a maximum dose of 0.1 mcg/kg per minute.
 
Complications of prostaglandin E1 infusion include hypotension, tachycardia, and apnea [8]. As a result, a separate reliable intravenous catheter must be in place to provide fluids for resuscitation. Intubation equipment should be immediately available because apnea can occur at any time during infusion.
 
Deterioration of the clinical status after starting prostaglandin E1 usually indicates the presence of rare congenital cardiac defects associated with pulmonary venous or left atrial obstruction. These include obstructive (usually infradiaphragmatic) total anomalous pulmonary venous connection or various conditions associated with a restrictive atrial septum (eg, hypoplastic left heart syndrome, cor triatriatum, severe mitral stenosis or atresia, or D-transposition of the great arteries associated with restrictive atrial shunting). These patients require urgent echocardiography followed by interventional cardiac catheterization or surgery [9].
 
Cardiac catheterization
 
Cardiac catheter interventions can either be palliative by improving cyanosis or be corrective by relieving obstruction to flow.
 
●Balloon atrial septostomy can relieve marked cyanosis in patients with D-transposition of the great arteries associated with restrictive atrial shunting, and in patients with a restrictive atrial septum associated with left-sided obstructive disease. In patients with D-transposition of the great arteries, this procedure can be performed at the bedside under echocardiographic guidance. (See "Management and outcome of D-transposition of the great arteries", section on 'Balloon atrial septostomy' and "Hypoplastic left heart syndrome: Management and outcome", section on 'Initial medical management'.)
 
●Balloon valvuloplasty can be effective in patients with critical pulmonary stenosis or aortic stenosis. Selected patients with pulmonary atresia are also candidates for balloon valvuloplasty if the obstruction is membranous, the tricuspid annulus and right ventricular size are adequate to support a two ventricle repair, and the coronary circulation does not depend upon the right ventricle [10]. (See "Valvar aortic stenosis in children", section on 'First-line treatment'.)
 
●Transcatheter occlusion of pulmonary arteriovenous malformations can also be performed [11].


==References==
==References==

Revision as of 19:58, 6 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

Cyanotic congenital heart diseases:

Peripheral Cyanosis

There following are some of the common causes of peripheral cyanosis: 

Newborns with cyanosis require maintains adequate tissue perfusion and oxygenation.

Specific interventions for neonatal cyanotic congenital heart disease (CHD) include administration of prostaglandin E1 and cardiac catheter palliative or corrective procedures.

Initial management begins with general care that includes

cardiorespiratory support

monitoring to ensure sufficient organ/tissue perfusion and oxygenation

an adequate airway should be established immediately and supportive therapy (eg, supplemental oxygen and/or mechanical ventilation) instituted as needed.

Placement of secure intravenous and intraarterial catheters is most easily accomplished via the umbilical vessels.

This will enable efficient correction and monitoring of acid-base balance

metabolic derangements (eg, hypoglycemia, hypocalcemia), and blood pressure.

Inotropic agents such as dopamine or dobutamine may be necessary to correct hypotension.

In infants with severe polycythemia (>70 percent), an isovolumetric partial exchange transfusion should be performed with saline to reduce the hematocrit.

Antibiotics

broad spectrum antibiotics should be initiated (ampicillin and gentamicin) after obtaining blood and urine cultures.

Specific CHD measures

An infant who fails the hyperoxia test and does not have persistent pulmonary hypertension of the newborn or a chest radiograph consistent with lung disease is likely to have a cyanotic CHD. In most cases, cyanotic CHD is dependent upon a patent ductus arteriosus (PDA) for pulmonary or systemic blood flow. Closure of the ductus arteriosus can precipitate rapid clinical deterioration with significant life-threatening changes (ie, severe metabolic acidosis, seizures, cardiogenic shock, cardiac arrest, or end-organ injury).

As a result, infants with ductal-dependent lesions are at increased risk for death and significant morbidity unless interventions are initiated to maintain patency of the ductus arteriosus for ductal-dependent lesions, ensure adequate mixing of deoxygenated and oxygenated blood, or relieve obstructed blood flow.

In infants with or who have a clinical suspicion for a ductal-dependent congenital heart defect, prostaglandin E1 (alprostadil) should be administered until a definitive diagnosis or treatment is established [7].

The initial dose is dependent on the clinical setting, as the risk of apnea, one of the major complications of prostaglandin E1 infusion, is dose dependent.

●If the ductus is known to be large in a patient with duct-dependent physiology, the initial dose is 0.01 mcg/kg per minute. This scenario typically is seen in patients with echocardiographic confirmation of a large PDA who are cared for in a tertiary center that provides treatment for neonates with cyanotic heart disease.

●If the ductus is restrictive or the status of the ductus is unknown, the initial dose is 0.05 mcg/kgper minute. This is the standard dose used in patients who require transport to a center with expertise in the care of neonates with cyanotic heart disease.

The dose of prostaglandin can be increased as needed to a maximum dose of 0.1 mcg/kg per minute.

Complications of prostaglandin E1 infusion include hypotension, tachycardia, and apnea [8]. As a result, a separate reliable intravenous catheter must be in place to provide fluids for resuscitation. Intubation equipment should be immediately available because apnea can occur at any time during infusion.

Deterioration of the clinical status after starting prostaglandin E1 usually indicates the presence of rare congenital cardiac defects associated with pulmonary venous or left atrial obstruction. These include obstructive (usually infradiaphragmatic) total anomalous pulmonary venous connection or various conditions associated with a restrictive atrial septum (eg, hypoplastic left heart syndrome, cor triatriatum, severe mitral stenosis or atresia, or D-transposition of the great arteries associated with restrictive atrial shunting). These patients require urgent echocardiography followed by interventional cardiac catheterization or surgery [9].

Cardiac catheterization

Cardiac catheter interventions can either be palliative by improving cyanosis or be corrective by relieving obstruction to flow.

●Balloon atrial septostomy can relieve marked cyanosis in patients with D-transposition of the great arteries associated with restrictive atrial shunting, and in patients with a restrictive atrial septum associated with left-sided obstructive disease. In patients with D-transposition of the great arteries, this procedure can be performed at the bedside under echocardiographic guidance. (See "Management and outcome of D-transposition of the great arteries", section on 'Balloon atrial septostomy' and "Hypoplastic left heart syndrome: Management and outcome", section on 'Initial medical management'.)

●Balloon valvuloplasty can be effective in patients with critical pulmonary stenosis or aortic stenosis. Selected patients with pulmonary atresia are also candidates for balloon valvuloplasty if the obstruction is membranous, the tricuspid annulus and right ventricular size are adequate to support a two ventricle repair, and the coronary circulation does not depend upon the right ventricle [10]. (See "Valvar aortic stenosis in children", section on 'First-line treatment'.)

●Transcatheter occlusion of pulmonary arteriovenous malformations can also be performed [11].

References

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