Potential for PHC Necessitates Pediatric Cardiology Care for Children with PAH

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General anesthesia use and the potential for greater risk of adverse outcomes among pediatric patients who have idiopathic pulmonary arterial hypertension (IPAH) and undergo right heart catheterization (RHC) make it necessary for them to receive specialized pediatric cardiology care, according to new study findings published in Journal of Clinical Medicine.

In this new retrospective study, outcomes were compared between 2 population cohorts: 469 patients with congenital heart disease–PAH (CHD-PAH) and 122 patients with IPAH. Median (IQR) ages were 8.8 (3.3-13.3) and 9.0 (5.0-12.9) years, respectively, and 60.4% were female patients. All underwent diagnostic RHC between 2005 and 2020, and the most common New York Heart Association functional class prior to RHC was class III-IV in 7.9% of the CHD-PAH group and 27% of the IPAH group. Syncope was present in 1.5% and 27%, respectively.

“RHC is generally considered a low-risk procedure, with a rate of complications of around 1% in adult patients. However, pediatric patients tend to have higher risks due to low weight, respiratory depression, anesthetic sensitivity, and relatively unstable hemodynamic status,” the study investigators wrote. “This study therefore set out to investigate the clinical characteristics and hemodynamics of Chinese pediatric patients with PAH from one of the largest pediatric PAH referral centers, and further, to quantify the safety and efficiency of RHC in this vulnerable population.”

Overall, all of the patients in the CHD-PAH group underwent RHC, representing 79.4% of these procedures during the study. Among these patients, the median time from symptom onset to diagnosis was twice that of the IPAH cohort: 24 (6-68) months vs 12 (4-25.5) months.

General anesthesia was used in approximately two-thirds of the RHC procedures, with 18 patients experiencing an adverse event (AE) during their procedure; among these patients, 14 were from the IPAH study cohort and 4 were from the CHD-PAH study cohort, and 2 patients were under local anesthesia whereas 16 were under general anesthesia. Seventeen of the AEs were a pulmonary hypertensive crisis (PHC), and these developed during anesthesia induction (n = 5), during RHC (n = 10), and after pulmonary angiography (n = 2). One patient died almost 3 days after the procedure.

Ketamine, propofol, dexmedetomidine, or opioids were the anesthetics used, “at the anesthetist’s discretion,” the authors wrote.

Several factors were associated with a significantly higher risk of developing a PHC. These were the following:

  • Presence of IPAH: 1302% higher risk (odds ratio [OR], 14.02; 95% CI, 4.49-43.85; P < .001)
  • Atrial blood gas pH below 7.35: 1150.4% higher risk (OR, 12.504; 95% CI, 3.545-44.102; P < .001)
  • Right atrial pressure above 14 mm Hg: 963.6% higher risk (OR, 10.636; 95% CI, 3.668-30.847; P < .001)

The authors’ investigation also determined that the most common reason for a referral for RHC was made following an abnormal physical exam in 60.3% of the CHD-PAH group and decreased exercise tolerance in 44.3% of the IPAH cohort, as well as that PHC was seen more often in patients who did not have an intracardiac shunt.

“Overall, our data reveal that RHC is a safe procedure in pediatric patients with CHD-PAH, while the probability of AEs, especially PHC, was relatively higher in IPAH patients and correlated with poor cardiac functional class and poor hemodynamic condition, history of syncope, lower pH value, and necessity of general anesthesia,” the investigators concluded. “However, with timely aggressive treatments, most pediatric patients can complete the procedures safely.”

Their ultimate recommendation is for the procedure to be performed at a large referral pediatric cardiology center.

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