Pulmonary Hypertension

Rajesh Shenoy
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.

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:
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.

Pulmonary Hypertension Pulmonary Hypertension 03/18/2016
<< Work-up of Pulmonary Hypertension
ask a doctor
Ask a Doctor
Disclaimer: The information given by www.pediatriconcall.com is provided by medical and paramedical & Health providers voluntarily for display & is meant only for informational purpose. The site does not guarantee the accuracy or authenticity of the information. Use of any information is solely at the user's own risk. The appearance of advertisement or product information in the various section in the website does not constitute an endorsement or approval by Pediatric Oncall of the quality or value of the said product or of claims made by its manufacturer.
0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0