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

Phase 4

Completed

Interventions: Drug: Arginine Vasopressin
Drug: Phenylephrine
Drug: Epinephrine

Brief Summary: 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: 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.

Recruitment & Eligibility

Inclusion Criteria

Patients presenting for cardiac catheterization procedure with a diagnosis of PAH either by previous cardiac catheterization or echocardiography

Exclusion Criteria

Arm && Interventions

Group	Intervention	Description
Arginine Vasopressin	Arginine Vasopressin	Arginine Vasopressin will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery.
Phenylephrine	Phenylephrine	Phenylephrine will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery.
Epinephrine	Epinephrine	Epinephrine will be administered once the child is under anesthesia and the interventional cardiologist has measured the pressures in the pulmonary artery.

Primary Outcome Measures

Name	Time	Method
Change in Systemic Vascular Resistance Index (SVRI) to Pulmonary Vascular Resistance Index (PVRI) Ratio (Rp:Rs Ratio)	Day 1 (at baseline and up to 5 minutes following study drug administration) (Q: 2 minutes - 2 to 5 minutes?)	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.

Secondary Outcome Measures