Clinical trial NCT00543933: Pilot Study of Inhaled Nitric Oxide to Treat Pulmonary Insufficiency in Congenital Heart Disease
Official title: Acute Effect of Inhaled Nitric Oxide on Pulmonary Insufficiency in Congenital Heart Disease
Study type: INTERVENTIONAL
Overall status: TERMINATED
Phase: EARLY_PHASE1
Conditions: Pulmonary Insufficiency
Lead sponsor: The Cleveland Clinic
Sponsor class: OTHER
First posted: 2007-10-15
Enrollment: 18
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: TREATMENT

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.

Eligibility criteria: 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

Primary outcomes:
measure: pulmonary regurgitant volume and fraction
timeFrame: Single time point
Secondary outcomes:
Other outcomes:
Locations:
city: Cleveland
country: United States
facility: The Cleveland Clinic
state: Ohio
Study officials:
affiliation: The Cleveland Clinic
name: Richard Krasuski, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Hart SA, Devendra GP, Kim YY, Flamm SD, Kalahasti V, Arruda J, Walker E, Boonyasirinant T, Bolen M, Setser R, Krasuski RA. PINOT NOIR: pulmonic insufficiency improvement with nitric oxide inhalational response. J Cardiovasc Magn Reson. 2013 Sep 4;15(1):75. doi: 10.1186/1532-429X-15-75.