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
  • cyanide poisoning
  • Pneumothorax
  • Foreign body aspiration
    • Pertussis / Croup
    • Epiglottitis
    • Bacterial tracheitis
    • Choanal atresia
    • Laryngotracheomalacia
    • Myasthenia gravis
    • Asthma
    • Pneumonia
    • Bronchiolitis
    • Respiratory distress syndrome
    • Cardiac tamponade

• • congestive heart failure
  • Atrial septal defect
  • Pulmonary hypertension
  • Pulmonary edema
  • Pulmonary hemorrhage
  • Pulmonary embolism
  • pulmonary arteriovenous malformation
  • Shock
  • Sepsis
  • Amniotic fluid embolism
  • Methemoglobinemia
  • Severe hypoglycemia

Initial management of neonantal 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.

Hypoglycemia is common in critically

ill infants, therefore glucose levels should be monitored and glucose infusions provided to

maintain a blood glucose > 55 mg/dL. An airway and assisted ventilation should be considered

for infants with respiratory distress, but may be deferred for the comfortable infant.

Severe

acidosis should be corrected with infusions of sodium bicarbonate, but only after adequate gas

exchange has been established. If the infant is <10 days old and the umbilical stump is still

attached, umbilical venous and arterial lines can frequently be placed by experienced

Steinhorn

Hypocalcemia is often associated with cardiac disease

and critical illness, and should be corrected based on the ionized calcium.

Oxygen should be provided, although there are increasing concerns about the potential risks

associated with this therapy (6). Even brief (30 minute) exposures to extreme hyperoxia are

increasingly recognized to increase oxidative stress and potentially damage lung parenchymal

and vascular function, even in term infants (7. 8).

the use of 100% O2 should

generally be avoided at the outset. Initiating oxygen therapy with 40–60% O2 will allow the

caregiver to provide support, assess for improvement, and seek advice from a cardiologist.

This

point is particularly important if an infant has only a minimal response to oxygen, as this may

indicate potential cardiac disease and need for PGE1. In addition, it is important to remember

that oxygen may promote ductal closure. This may not be a major concern for lesions that limit

pulmonary blood flow, as the pulmonary venous PO2 would not be expected to rise.

However,

admixture lesions such as hypoplastic left heart syndrome may present with moderate cyanosis.

These conditions are dependent on a patent ductus to maintain systemic blood flow. Oxygen

may not only promote ductal closure, but may increase pulmonary and decrease systemic blood

flow.

In the infant who does not require assisted ventilation, oxygen may be delivered via a head

hood or nasal cannula (9). A head hood is the only method that allows the FiO2 to be determined

precisely.

The oxygen concentration should be measured by an oxygen analyser placed near

the baby’s mouth. Relatively high flows are needed achieve adequate concentrations of oxygen

and avoid carbon dioxide accumulation, although humidification is generally not necessary.

While head box oxygen is generally well tolerated, this method limits the infant’s mobility,

and oxygen concentrations fall quickly when the hood is lifted to provide care to the infant.

Oxygen is frequently delivered by a nasal cannula. The disadvantage of this method is that the

infant entrains variable amounts of room air around the nasal cannula. Therefore, it cannot

provide 100% oxygen, and the oxygen concentration in the hypopharynx (a good proxy for the

tracheal concentration) will be much lower than the concentration of oxygen at the cannula

inlet. Both the oxygen concentration and the cannula flow rate will be the major factors that

will determine the fraction of oxygen actually delivered. Therefore, it is generally better to

titrate delivery to achieve the desired oxygen saturation levels, generally 90% to 95% by pulse

oximetry.

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].

• • • Tetralogy of fallot

• • • Tricuspid atresia
    • Tricuspid stenosis
    • Ebstein's anomaly
  • Pulmonary stenosis
  • Pulmonary atresia 
  • TGA
  • Truncus arteriosus
  • TAPVC
  • Left sided obstructive lesion
  • Coarctation of aorta
  • Critical valvular aortic stenosis
  • Eisenmenger syndrome