Management of Acute Pulmonary Hypertensive Crisis in Children With Known Pulmonary Arterial Hypertension
Condition: Pulmonary Arterial Hypertension
MeSH terms: Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension
Study type: Interventional
Phase: Phase 4

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.

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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.
    

Intervention name(s): Phenylephrine; Epinephrine; Arginine Vasopressin
Intervention type(s): Drug; Drug; Drug
Intervention description(s): 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.; 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.; 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.
Other intervention name(s): Phenylephrine Hydrochloride; Epinephrine injection; Vasopressin injection
Intervention MeSH terms: Phenylephrine; Vasopressins; Arginine Vasopressin; Epinephrine; Racepinephrine; Oxymetazoline; Epinephryl borate
Allocation: Non-Randomized
Intervention model: Sequential Assignment
Masking: None (Open Label)
Primary purpose: Treatment