Cyanosis medical therapy
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Newborns with cyanosis require adequate tissue perfusion and oxygenation. Initiating oxygen therapy with 40-60% O2 is sufficient. Exposures to hyperoxia increases oxidative stress and damage lung parenchyma and vascular function. Inotropic agents such as dopamine or dobutamine may be necessary to correct hypotension. In case of a minimal response to oxygen, cardiac disease should be suspected and need for PGE1 should be discussed. Complications of prostaglandin E1 infusion include hypotension, tachycardia, and apnea. In cases of respiratory distress syndrome, exogenous surfactant replacement therapy is effective in reducing RDS mortality and morbidity in preterm infants. In cases of Esinmenger syndrome, if surgical intervention is not available, treatment is mostly palliative. Anticoagulants, pulmonary vasodilators such as bosentan, prostacyclin may improve pulmonary artery pressure and may improve length of life. Antibiotic prophylaxis prevents endocarditis. For aortic stenosis, beta blockers are the treatment of choice. Caution should be taken as too much control of hypertension in upper limb can cause hypotension in lower limbs. Surgical treatment of the lesion should not be delayed for the correction of hypertension.
Initial management of neonantal cyanosis
- Newborns with cyanosis require adequate tissue perfusion and oxygenation.
- A hyperoxia test is a test that is performed to determine whether the patient's cyanosis is due to lung disease or a problem with blood circulation. It is performed by measuring the arterial blood gases of the patient while they breathe room air, then re-measuring the blood gases after the patient has breathed 100% oxygen for 10 minutes.
- An infant who fails the hyperoxia test and does not have persistent pulmonary hypertension of the newborn is likely to have a cyanotic congenital heart disease (CHD).
- Monitoring of oxygen level and tissue perfusion is necessary.
- An adequate airway should be established immediately, mechanical ventilation may be needed in case of failed spontaneous respiration.
- Initiating oxygen therapy with 40-60% O2 is sufficient. Exposures to hyperoxia increases oxidative stress and damage lung parenchyma and vascular function.
- Placement of secure intravenous and intraarterial catheters is most easily accomplished via the umbilical vessels.
- Inotropic agents such as dopamine or dobutamine may be necessary to correct hypotension.
- An isovolumetric partial exchange transfusion should be performed with saline to reduce the hematocrit in cases of severe polycythemia.
- Maintenance a blood glucose > 55 mg/dL should be considered.
- Acidosis should be corrected with infusions of sodium bicarbonate.
- Hypocalcemia should be corrected based on the ionized calcium.
- Broad spectrum antibiotics should be initiated (ampicillin and gentamicin).
- In case of a minimal response to oxygen, cardiac disease should be suspected and need for PGE1 should be discussed.
- Closure of the ductus arteriosus can precipitate rapid clinical deterioration with significant life-threatening changes. It may increase pulmonary blood flow and decrease systemic blood flow.
- Interventions are initiated to maintain patency of the ductus arteriosus for ductal-dependent lesions.
- The initial dose is dependent on the clinical setting, as the risk of apnea.
- 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.
- If the ductus is restrictive or the status of the ductus is unknown, the initial dose is 0.05 mcg/kgper minute.
- 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.
Respiratory distress syndrome
- Exogenous surfactant replacement therapy is effective in reducing RDS mortality and morbidity in preterm infants.
- It may be natural or synthetic surfactants.
- Natural surfactants have been shown to be more efficient with lower inspired oxygen concentration and ventilator pressures, decreased mortality, and lower rate of RDS complications in preterm infants.
- All patients with RDS, and intubate and administer surfactant to those with persistent severe respiratory distress (defined as requiring a fraction of inspired oxygen [[[FiO2]]] of 0.40 or higher to maintain oxygen saturation above 90 percent or who are apneic.
- If the infant maintains adequate respiratory efforts and has an FiO2 requirement less than 0.30, no additional doses of surfactant are needed.
- Endotracheal intubation has been the standard technique of surfactant administration.
- Control of the heart rhythm with antiarrhythmic drugs: Ebstein's anomaly may present with an AV nodal reentrant tachycardia with associated pre-excitation.
- Among these patients, the preferred pharmacological treatment agent is procainamide.
- Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers and digoxin are contraindicated. If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and slow the ventricular response.
- Inotropic agents and diuretics for heart failure.
- Anticoagulation in patients with atrial fibrillation and paradoxical embolization Tricuspid valve repair is indicated in patients in which there is symptoms or deteriorating exercise capacity, cyanosis (oxygen saturation less than 90%), paradoxical embolism, progressive cardiomegaly on chest x-ray or progressive right ventricular dilation or reduction of right ventricular systolic function.
- Repair is favored over replacement. Warfarin is recommended for patients with Ebstein’s anomaly with a history of paradoxical embolus or atrial fibrillation.
Coarctation of aorta
- Beta blockers are the treatment of choice.
- Caution should be taken as too much control of hypertension in upper limb can cause hypotension in lower limbs.
- Surgical treatment of the lesion should not be delayed for the correction of hypertension.
- Immediate post operative hypertension - use short-term vasodilators for e.g. sodium nitroprusside, or intravenous beta-blockers like esmolol.
- Long-term antihypertensive post surgery
- If surgical intervention is not available, treatment is mostly palliative
- Pulmonary vasodilators such as bosentan
- Prostacyclin may improve pulmonary artery pressure and may improve length of life
- Antibiotic prophylaxis to prevent endocarditis
- Phlebotomy to treat polycythemia
- Maintaining proper fluid balance
- These measures can prolong lifespan and improve quality of life
- Methemoglobinemia is treated with supplemental oxygen and methylene blue 1% solution (10mg/ml) 1-2mg/kg administered intravenously slowly over five minutes followed by IV flush with normal saline.
- Methylene blue restores the iron in hemoglobin to its normal (reduced) oxygen-carrying state. This is achieved through the enzyme inducing effect of methylene blue on levels of diaphorase II (NADPH methemoglobin reductase).
- Diaphorase II normally contributes only a small percentage of the red blood cells reducing capacity but is pharmacologically activated by exogenous cofactors, such as methylene blue, to 5 times its normal level of activity.
Peripheral cyanosis treatment
- Drug treatment is normally with a calcium channel blocker, frequently nifedipine to prevent artery constriction.
- It has the usual side effects of headache, flushing, and ankle edema, and patients often stop treatment, preferring the symptoms of Raynaud's to the symptoms of the drug.
- The extract of the Ginkgo biloba leaves reduces symptoms in two weeks.
- There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks.
- In intractable cases, sympathectomy and infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.
- Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's vasospasms under the supervision of a healthcare provider.
Peripheral vascular disease
- Urgent measures should be taken to ensure blood flow and protect the limb:
- If the limb is not immediately threatened:
- Continue treatment with thrombolytic therapy for 14 days.
- If the limb ischemia is critical:
- Consider percutaneous transluminal angioplasty
- Consider surgery: thromboembolectomy, bypass grafting
- Send sample for pathologic examination (myxoma may be present)
- Cilostazol is a phosphodiesterase III inhibitor.
- Cilostazol is not administered to all PAD cases but rather to selected cases where regular walking program has failed to improve the walking time and capacity.
- It is contraindicated in congestive heart failure.
- Side effects:
Endovascular Revascularization Modalities