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

Pulmonary Arterial Hypertension Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT05439460
• Other study ID #: 22554
• Status: Completed
• Phase: Phase 4
• Start date: January 2012
• Est. completion date: June 2014

Study information

• Verified date: July 2022
• Source: Stanford University
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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. --------------------------------------------------------------------------------

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 information / eligibility

• Status: Completed
• Enrollment: 15
• Est. completion date: June 2014
• Est. primary completion date: June 2014
• Accepts healthy volunteers: No
• Gender: All
• Age group: 1 Year to 18 Years

Eligibility

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

Related Conditions & MeSH terms

• Familial Primary Pulmonary Hypertension
• Hypertension
• Pulmonary Arterial Hypertension

Intervention

Drug:
• Phenylephrine
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.
• Epinephrine
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.
• Arginine Vasopressin
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.

Locations

Country	Name	City	State
United States	Cardiac Catheterization Lab,Stanford University Medical Center	Stanford	California

Sponsors (1)

Lead Sponsor	Collaborator
Stanford University

Country where clinical trial is conducted

United States, 

References & Publications (2)

Friesen RH, Williams GD. Anesthetic management of children with pulmonary arterial hypertension. Paediatr Anaesth. 2008 Mar;18(3):208-16. doi: 10.1111/j.1460-9592.2008.02419.x. Review. — View Citation

Siehr SL, Feinstein JA, Yang W, Peng LF, Ogawa MT, Ramamoorthy C. Hemodynamic Effects of Phenylephrine, Vasopressin, and Epinephrine in Children With Pulmonary Hypertension: A Pilot Study. Pediatr Crit Care Med. 2016 May;17(5):428-37. doi: 10.1097/PCC.000 — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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?)