Pilot Study of Inhaled Nitric Oxide to Treat Pulmonary Insufficiency in Congenital Heart Disease

Early Phase 1

Terminated

• Conditions: Pulmonary Insufficiency
• Interventions: Drug: iNO administered

• Registration Number: NCT00543933
• Lead Sponsor: The Cleveland Clinic

Brief Summary

Inhaled nitric oxide in patients with pulmonic valve insufficiency.

Detailed Description

Pulmonic valve insufficiency (PI) is a well-defined problem after primary surgical repair of Tetralogy of Fallot (TOF). Though well-tolerated for years, long-term PI can lead to structural changes in the right ventricle, the sequelae of which include right heart failure, arrhythmia, and sudden cardiac death. The only current treatment for severe symptomatic PI is pulmonic valve replacement. We hypothesize that inhaled nitric oxide (iNO), a selective pulmonary vasodilator, can acutely decrease PI as assessed by cardiac magnetic resonance imaging (CMR). Methods: 22 consecutive patients with PI in the setting of corrected TOF or post pulmonic valve balloon valvuloplasty will undergo a clinically indicated CMR. Nitric oxide gas will be delivered via facemask through a specialized delivery device at 40ppm. After 5 minutes, flow velocity mapping and gradient echo sequences will be repeated to assess pulmonary regurgitant fraction, right ventricular volumes, and ejection fraction. Nitric oxide will be discontinued after acquisition of the last picture. Wilcoxon rank-sum for paired data will be used to assess effect of intervention. Significance: If decreasing pulmonary vascular resistance decreases PI, medical therapy with long-acting pulmonary vasodilators may be an attractive therapeutic option with the goal of delaying or even obviating pulmonic valve replacement.

Study Timeline

• Study Start: 2007-10
• Study First Submitted: 2007-10-11
• Study First Submitted QC: 2007-10-11
• Study First Posted: 2007-10-15
• Primary Completion: 2010-06
• Study Completion: 2010-06
• Status Verified: 2017-01
• Last Update Submitted: 2017-01-26
• Last Update Posted: 2017-01-27

Recruitment & Eligibility

• Status: TERMINATED
• Sex: All
• Target Recruitment: 18

Inclusion Criteria

• Known pulmonary insufficiency status
• Previous Tetralogy of Fallot repair or balloon valvuloplasty/surgical valvotomy for pulmonary stenosis
• Clinically indicated cardiac magnetic resonance imaging study

Exclusion Criteria

• Enrollment in another clinical trial
• Age less then 18 years
• Inability to provide informed consent
• Institutionalized individual
• Pregnant or lactating
• Serious claustrophobia
• Pacemaker/ICD
• Aneurysm clips
• Internal hardware
• Severe obesity (>350lbs)
• Residual ventricular septal defect
• History of methemoglobinemia
• History of blood dyscrasias
• Acute pulmonary infection
• Pulmonary edema
• Hypersensitivity to nitric oxide or any of its components
• Left ventricle dysfunction (EF<40%)
• Concurrent use of nitroglycerin or prilocaine

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
iNO administered	iNO administered	iNO administered at 40 ppm via a non-rebreather mask

Primary Outcome Measures

Name	Time	Method
pulmonary regurgitant volume and fraction	Single time point	Aortic regurgitant fraction measured by CMR velocity flow mapping

Trial Locations

Locations (1)

The Cleveland Clinic; 🇺🇸 Cleveland, Ohio, United States