Cyanosis medical therapy
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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
- Conditions associated with decreased concentration of inspired oxygen:
- Smoke inhalation most commonly from house fires
- Carbon monoxide poisoning
- Hydrogen cyanide poisoning
- Intentional or unintensional exposure to asphyxiating gases (eg, Propane, methane, butane, hydrogen sulphide)
- Impairment of chest wall or lung expansion:
- External compression
- Pneumothorax
- Hemothorax
- Flail chest
- Hypoventilation:
- Upper airway obstruction:
- Foreign body aspiration
- Pertussis / Croup
- Epiglottitis
- Bacterial tracheitis
- Traumatic disruption (Thermal injury, fractures)
- Acute chest syndrome
- Congenital airway abnormalities:
- Choanal atresia
- Laryngotracheomalacia
- Macroglossia
- Micrognathia or retrognathia (eg, Pierre-Robin syndrome)
- Neurologic abnormalities:
- CNS depression
- Birth asphyxia
- Severe head trauma
- Apnea of prematurity
- Obstructive sleep apnea
- Infections (eg, meningitis, encephalitis)
- Intraventricular hemorrhage
- Seizures
- Cyanotic breath holding spells
- Coma
- Neuromuscular disorders:
- Myasthenia gravis
- Injury to the phrenic nerve
- Type 1 spinal muscular dystrophy (Wernig-Hoffman disease)
- Upper airway obstruction:
- Intrinsic lung diseases:
- Vascular causes:
Cyanotic congenital heart diseases:
- Decreased pulmonary flow:
- Tetralogy of fallot
- Tricuspid valve anomalies:
- Pulmonary stenosis (critical valvular)
- Pulmonary atresia with intact ventricular septum
- Increased pulmonary flow:
- TGA (Transposition of great arteries, most common dextro type)
- Truncus arteriosus
- TAPVC (Total anamalous pulmonary venous connection)
- Heart failure: Condition that present with cyanosis and severe heart failure include:
- Left sided obstructive lesion (HLHS)
- Coarctation of aorta
- Critical valvular aortic stenosis
- Eisenmenger syndrome
- congestive heart failure
- Atrial septal defect
- Pulmonary hypertension
- Pulmonary edema
- Pulmonary hemorrhage
- Pulmonary embolism
- pulmonary arteriovenous malformation
- Multiple small intrapulmonary shunts
- Shock
- Sepsis
- Amniotic fluid embolism
- Hematologic abnormalities:
- Methemoglobinemia (congenital or acquired)
- Sulfhemoglobinemia (acquired)
- Hemoglobin mutations with low oxygen affinity:
- Hb Kansas
- Hb Beth israel
- Hb Saint Mande
- Hb Bruxells
- Polycythemia vera
- Disseminated intravascular coagulation
- Metabolic disorders:
Peripheral Cyanosis
There following are some of the common causes of peripheral cyanosis:
- Various conditions that cause central cyanosis can also present with peripheral cyanosis.
- Common conditions where peripheral cyanosis is more specific:
- Cold exposure
- Acrocyanosis
- Erythrocyanosis
- Raynaud's phenomenon
- Raynaud's disease
- Arterial obstruction:
- Venous obstruction:
- Decreased cardiac output:
- 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].