Clinical bottom line
Patients with pulmonary arterial hypertension (PAH) undergoing cardiac surgery face significantly increased perioperative risks, including right ventricular failure, hemodynamic instability, and mortality. Careful preoperative risk stratification, multidisciplinary planning, and tailored anesthetic management strategies are essential to optimize outcomes. Current evidence supports the use of invasive hemodynamic monitoring, judicious fluid management, avoidance of factors that increase pulmonary vascular resistance, and consideration of pulmonary vasodilator therapies perioperatively. Despite advances, the evidence base remains limited, and anesthesiologists should approach these cases with heightened vigilance and individualized care plans.
What the evidence shows
Pulmonary arterial hypertension imposes a substantial burden on perioperative management due to the risk of right ventricular (RV) failure triggered by increased pulmonary vascular resistance (PVR) during cardiac surgery. Cortes et al. (2010) provided one of the foundational analyses of preoperative pulmonary risk assessment in PAH patients undergoing cardiac surgery, highlighting that elevated pulmonary artery pressures and compromised RV function correlate with worse postoperative outcomes. Their findings underscore the importance of comprehensive preoperative evaluation including echocardiography and right heart catheterization when feasible.
Bateman et al. (2016) emphasized the critical role of invasive monitoring and goal-directed therapy in managing PAH patients in the perioperative setting, advocating for continuous assessment of pulmonary pressures and cardiac output to guide fluid and vasoactive drug administration. Avoidance of hypoxia, hypercarbia, acidosis, and hypothermia—known triggers of pulmonary vasoconstriction—is repeatedly stressed in the literature (Yang et al., 2015).
Bhatia et al. (2023) argued that patients with PAH undergoing non-cardiac surgery benefit from anesthetic care by cardiothoracic fellowship-trained anesthesiologists due to the complexity of hemodynamic management and familiarity with pulmonary vasodilator therapies. Although this study focused on non-cardiac surgery, the principles apply to cardiac surgery where the stakes are higher.
Pediatric case reports by Xue et al. (2025) and Hou et al. (2025) illustrate the challenges of anesthetic management in children with PAH undergoing cardiac catheterization, highlighting the need for individualized anesthetic plans that minimize increases in PVR and support RV function.
Wardle et al. (2022) demonstrated that elective orthopedic surgery in PAH patients can be performed safely with meticulous perioperative planning, suggesting that similar principles may extend to cardiac surgery, albeit with greater complexity.
Overall, the evidence supports a multidisciplinary approach involving cardiology, pulmonology, and anesthesiology teams, with perioperative optimization of PAH-specific therapies and vigilant intraoperative monitoring.
Caveats and uncertainty
The evidence base for anesthetic management of PAH patients undergoing cardiac surgery is limited by small cohort sizes, retrospective designs, and heterogeneity of patient populations and surgical procedures. Most data derive from observational studies, expert opinion, and case series rather than randomized controlled trials. Pediatric data, while informative, may not fully extrapolate to adult populations.
There is no consensus on standardized anesthetic protocols or pulmonary vasodilator regimens perioperatively, and the optimal timing and dosing of these agents remain uncertain. The impact of newer PAH therapies on surgical outcomes has not been extensively studied.
Furthermore, risk stratification tools specific to PAH patients in cardiac surgery are underdeveloped, limiting precise prognostication. The interplay between PAH severity, RV function, and surgical complexity complicates outcome prediction.
How this may change practice
This evidence digest reinforces the necessity for anesthesiologists to recognize PAH as a high-risk comorbidity in cardiac surgery and to engage in thorough preoperative evaluation including right heart catheterization when indicated. Collaboration with pulmonary hypertension specialists to optimize medical therapy preoperatively is advisable.
Intraoperative management should prioritize invasive hemodynamic monitoring, avoidance of factors that exacerbate pulmonary vasoconstriction, and readiness to initiate or continue pulmonary vasodilators. Anesthetic techniques that preserve RV function and minimize sympathetic stimulation may improve outcomes.
Institutions may consider developing protocols and care pathways for PAH patients undergoing cardiac surgery, including involvement of cardiothoracic anesthesia specialists. Further research is warranted to establish evidence-based guidelines and to evaluate novel therapeutic strategies in this vulnerable population.