PULMONARY HYPERTENSION
PULMONARY HYPERTENSION
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Rajesh Shenoy
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Management of PH
Management of PH: PH should preferably be managed by specialized centers that have the multidisciplinary team and logistics to ensure appropriate testing and ongoing surveillance.

Pharmacotherapy:
Diuretics
Digoxin
Calcium channel blockers: Amlodipine, Nifedipine, Diltiazem. These agents are preferably used in children older than 1 year of age, as they are poorly tolerated in infancy.
Endothelin receptor antagonists: Bosentan, Ambrisentan.
Phosphodiesterase-5 inhibitors: Sildenafil, Tadalafil.
Prostacyclin analogs: Iloprost (inhaled), Treprostinil (subcutaneous), Epoprostenol (intravenous), Beraprost (oral).
Interventional therapy:
ASD creation, Palliative Potts shunt: These interventions aim to increase cardiac output at the expense of cyanosis.
Lung transplantation
Supportive measures:
Oxygen
Anticoagulation
Approach to treating a patient with PH:
The 2013 World Symposium on Pulmonary Hypertension arrived at a consensus regarding the algorithm towards management of idiopathic or familial pulmonary artery hypertension. After the diagnosis of PH is confirmed, the patient is stabilized with diuretics, digoxin, anticoagulation, and oxygen.
Acute vasoreactivity testing (AVT) is the next step in deciding on optimal medical therapy. Patients who show a positive response to AVT should be treated with calcium channel blockers. If the response is sustained, calcium channel blockers should be continued. If there is no response to calcium channel blockers, therapy should be escalated to endothelin receptor antagonists or phosphodiesterase inhibitors.
Patients who show a negative response to AVT should be stratified into low and high-risk categories. Clinical evidence of right ventricular failure, progression of symptoms on therapy, syncope, growth failure, WHO functional class III or IV, significant elevation of BNP, echocardiographic evidence of right ventricular enlargement, dysfunction or pericardial effusion are high-risk factors. On cardiac catheterization, a systemic cardiac index less than 2.5 L/min/m2, mPAP to mSAP ratio > 0.75, RA pressure > 10 mm Hg, and PVRi > 20 Woods units are high-risk factors. Patients who do not have high-risk criteria should be initially treated with endothelin receptor antagonists or phosphodiesterase inhibitors. Those with high-risk markers should be initially treated with prostacyclin analogs, with a low threshold for considering combination therapy with endothelin receptor antagonists or phosphodiesterase inhibitors.
All patients should be reassessed at 3-month intervals to assess efficacy of therapy and sustained improvement. In cases of clinical deterioration, combination therapy should be considered. In patients that are unresponsive to medical therapy, palliative procedures such as atrial septostomy or a Potts shunt should be considered. These patients should be evaluated for candidacy for a lung transplant.
Special clinical scenarios involving PH:
Persistent pulmonary hypertension of the newborn:
Use of inhaled nitric oxide, sildenafil and prostacyclin analogs.
Supportive care with ventilation, and intravenous milrinone.
PH in the setting of structural heart disease:
Cardiac catheterization to measure PVRi.
If PVRi < 6 Woods units, consider surgical repair.
If PVRi > 6 Woods units, and positive response to AVT – consider surgical repair.
If PVRi > 6 Woods units, and negative response to AVT – surgical repair is not beneficial. Medical treatment of PH, with reassessment in 4 – 6 months.

References

Contributor Information and Disclosures

Rajesh Shenoy
MD, DCH, DNB, FAAP,
FACC Icahn School of Medicine at
Mount Sinai 1 Gustave Levy Place,
Box 1201 New York, NY 10029


First Created : 3/18/2016

References

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