Acute pulmonary hypertension and pulmonary hypertensive crisis may result in adverse clinical outcomes if unsuccessfully treated. Inhaled nitric oxide has long been considered as the standard pharmacotherapy for acute pulmonary hypertension, but lack of feasibility in some settings and evidences challenging its benefits lead to the use of alternative treatment, amongst which is nitroglycerin inhalation. The purpose of this review article is to discuss available data on the use of nitroglycerin inhalation for acute treatment of pulmonary hypertension in children with CHD and its potential benefit in post-operative setting.

Literatures included in this review were acquired by searching in PubMed online database. Keywords used were “Pulmonary Hypertension”, “Congenital heart defects”, “Pediatrics”, “Inhaled nitroglycerin”, and its synonyms.

Title and abstract were screened to select relevant literatures including the three paediatric clinical trials on nitroglycerin inhalation. Critical appraisal of the clinical trials was then done using the University of Oxford Centre of Evidence-Based Medicine Critical Appraisal Tools.

Paediatric studies showed the benefit of nitroglycerin inhalation in uncorrected cases of CHD during catheterisation procedures. Until recently, there have been no studies conducted in paediatric post-operative CHD cases. Further study is required to provide evidence for inhaled nitroglycerin use in this setting including the appropriate dosing and potential side effects with repeated administration,

The policy of early repair of patients with tetralogy of Fallot, irrespective of age, as opposed to initial palliation with a shunt, remains controversial. The aim of our study was to analyze the midterm outcome of primary early correction of tetralogy of Fallot.

Between 1996 and 2005, a total of 61 consecutive patients less than 6 months of age underwent primary correction of tetralogy of Fallot in two institutions. The median age at surgery was 3.3 months, and 27 patients (44%) were younger than 3 months of age, including 12 (20%) newborns. We analyzed the patients in 2 groups: those younger than 3 months of age, and those aged between 3 and 6 months.

There was one early (1.6%), and one late death. Actuarial survival was 98.4%, 96.7%, 96.7% at 1, 5, and 10 years respectively, with a median follow up of 4.5 years. There was no difference in survival, bypass time, lengths of ventilation, and hospital stay between the groups. A transjunctional patch was placed significantly more often in the patients younger than 3 months (p = 0.039), with no adverse effect on survival and morbidity during the follow-up. Freedom from reoperation was 98.2%, 92.2%, and 83% at 1, 5, and 10 years respectively, with no difference between the groups.

Elective primary repair of tetralogy of Fallot in asymptomatic patients is delayed beyond 3 months of age. In symptomatic patients, primary repair of tetralogy of Fallot is performed irrespective of age, weight and preoperative state. This approach is safe, and provides an excellent midterm outcome with acceptable morbidity and rates of reintervention. The long-term benefits of this approach must be established by careful follow-up, with particular emphasis on arrhythmias, right ventricular function, and exercise performance.