Management of Acute Pulmonary Hypertensive Crisis in Children With Known Pulmonary Arterial Hypertension

Study Overview
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Brief Summary
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Pulmonary arterial hypertension (PAH) is a disease where the blood pressure in the pulmonary arteries (PAP) is high. PAH increases the risk of adverse events, including death, during and or after procedures. The severity of baseline PAH correlates with the incidence of major complications, such that those with PAP higher than their systemic blood pressure (SBP) had a 8 fold increased risk of complications. These children present for procedures where an acute exacerbation of their chronic illness-termed Pulmonary Hypertensive (PH)crisis, can occur, often resulting in death if not detected and managed expeditiously. Unfortunately there is little data and no consensus in the pediatric literature on how PH crisis should be managed. --------------------------------------------------------------------------------

Detailed Description
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Pulmonary arterial hypertension (PAH) is a disease where the blood pressure in the pulmonary arteries (PAP) is high. PAH increases the risk of adverse events, including death, during and or after procedures. The severity of baseline PAH correlates with the incidence of major complications, such that those with PAP higher than their systemic blood pressure (SBP) had a 8 fold increased risk of complications. These children present for procedures where an acute exacerbation of their chronic illness-termed PH crisis, can occur, often resulting in death if not detected and managed expeditiously. Unfortunately there is little data and no consensus in the pediatric literature on how PH crisis should be managed. Over the last 10 years we have developed considerable expertise in managing children with PAH and preventing and treating their acute crisis, using a medication called phenylephrine. This medication is routinely used to increase the blood pressure in patients (adults and children) to treat hypotension. Our theory has been that by increasing SBP, we can increase the blood flow to the coronary arteries and prevent the right ventricle from failing acutely. The latter results in catastrophic hypotension, heart arrythmias and death. There is no consensus or protocol guiding the management of the acute crisis. This purpose of this study is to close that gap.

Official Title
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Management of Acute Pulmonary Hypertensive Crisis in Children With Known Pulmonary Arterial Hypertension

Conditions
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Pulmonary Arterial Hypertension

Intervention / Treatment
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* Drug: Phenylephrine
* Drug: Epinephrine
* Drug: Arginine Vasopressin


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients presenting for cardiac catheterization procedure with a diagnosis of PAH either by previous cardiac catheterization or echocardiography Exclusion Criteria: Children presenting for cardiac catheterization who do not have PAH; Children with PAH but with intracardiac shunts

Ages Eligible for Study
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Minimum Age: 1 Year
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Sequential Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Phenylephrine<br>Phenylephrine will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery. | Drug: Phenylephrine<br>* 5 subjects will get Phenylephrine during cardiac catheterization in patient with known Pulmonary Arterial Hypertension.Dose will be 1ug/kg body weight. Pressures in the pulmonary artery will be measured before and after the drug administration.<br>* Other names: Phenylephrine Hydrochloride;|
| Experimental: Epinephrine<br>Epinephrine will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery. | Drug: Epinephrine<br>* 5 subjects will get Epinephrine during cardiac catheterization in patient with known Pulmonary Arterial Hypertension.Dose will be 0.5-1ug/kg body weight. Pressures in the pulmonary artery will be measured before and after the drug administration.<br>* Other names: Epinephrine injection;|
| Experimental: Arginine Vasopressin<br>Arginine Vasopressin will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery. | Drug: Arginine Vasopressin<br>* 5 subjects will get Arginine Vasopressin during cardiac catheterization in patient with known Pulmonary Arterial Hypertension.Dose will be 1ug/kg body weight. Pressures in the pulmonary artery will be measured before and after the drug administration.<br>* Other names: Vasopressin injection;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in Systemic Vascular Resistance Index (SVRI) to Pulmonary Vascular Resistance Index (PVRI) Ratio (Rp:Rs Ratio) | In patients with pulmonary hypertension (PH) one anticipates a greater increase in pulmonary vascular resistance as opposed to systemic vascular resistance when vasopressors are administered. | Day 1 (at baseline and up to 5 minutes following study drug administration) (Q: 2 minutes - 2 to 5 minutes?) |