PH treatment aids children having congenital heart disease surgery

Revatio reduced length of cardiopulmonary bypass, time in ICU, ventilation

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by Andrea Lobo |

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Treating children with Revatio (sildenafil) reduced pulmonary arterial pressure after surgery for congenital heart disease (CHD), according to a new analysis.

The pulmonary hypertension (PH) treatment, given between one and two weeks before surgery, reduced how long cardiopulmonary bypass — a procedure that takes over the functions of the heart and lungs — was needed during surgery, the time spent at the intensive care unit, and the duration of ventilation support for breathing.

“Sildenafil offers distinct advantages over other pulmonary vasodilators … in the management of pediatric patients undergoing congenital heart surgeries. One notable benefit of sildenafil is its [oral] route of administration,” the investigators wrote in “Preoperative Sildenafil in Pediatric Patients Undergoing Congenital Heart Surgeries: A Systematic Review and Meta-Analysis,” which was published in Seminars in Cardiothoracic and Vascular Anesthesia.

PH is a common complication of CHD, a group of birth defects that affect the normal way the heart works. Surgical repair of CHD generally seeks to restore normal heart function. Some patients see a temporary increase in pulmonary arterial pressure immediately after surgery, however.

“The probable underlying cause is a sudden constriction of the pulmonary blood vessels, leading to right heart failure and low blood pressure throughout the body, ultimately culminating in severe tissue oxygen deficiency and potential death,” the scientists wrote.

Revatio, a phosphodiesterase-5 (PDE-5) inhibitor, reduces blood pressure by promoting lung blood vessel widening. This increases blood circulation through the lungs, which helps transport oxygen around the body and reduces the burden on the heart.

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More than 50% of preterm babies with PH respond to Revatio: Study

Taking Revatio before surgery to control PH symptoms

Researchers in Indonesia conducted a meta-analysis, which is a type of study that pools data from multiple studies, to better understand whether taking Revatio before surgery helps control PH after the procedure, and identified 133 studies in scientific databases, four of which were deemed eligible for inclusion.

The studies included 233 patients who had ventricular septal defect, a CHD that happens if the wall between the two ventricles doesn’t fully develop, leaving a hole. All four studies were clinical trials that assigned patients to groups randomly.The patients’ median age ranged between 5.4 and 15.8 months across groups.

The dose of Revatio ranged from 0.3 to 0.5 mg/kg every four to six hours, given orally or via a tube from the nose to the stomach, called nasogastric administration.

Compared to controls, using Revatio before surgery was associated with a greater reduction of mean pulmonary arterial pressure after the procedure (a decrease of 5.02 mmHg), and of the ratio of pulmonary artery pressure relative to pressure in the aorta artery (median decrease of 0.11) — a common way to assess PH.

In three studies with 203 participants, PH crises — an acute increase in pulmonary arterial pressure — were less frequent in those who received Revatio compared to controls (2.94% vs. 6.93%), but this difference was not statistically significant.

Revatio treatment before surgery was also associated with a shorter cardiopulmonary bypass time, a shorter stay in the intensive care unit (a decrease of 14.59 hours), and a shorter duration of mechanical ventilation — a reduction of 3.79 hours.

The effect of pre-surgery Revatio on mortality was reported in one study, with rates being 4% among Revatio-treated patients and 6% in controls.

“To our knowledge, our study was the first systematic review to analyze the benefit of preoperative sildenafil in pediatric patients undergoing congenital heart surgeries,” wrote the researchers, who said more studies would help identify the optimal dosage and the timing of administration of the therapy in CHD patients before surgery. The low number of studies and not analyzing side effects were noted as limitations of the analysis.

A Conversation With Rare Disease Advocates