Pulmonary hypertensive crisis in children with pulmonary arterial hypertension undergoing cardiac catheterization

Abstract

Pediatric patients with pulmonary arterial hypertension (PAH) are considered to be at risk for pulmonary hypertensive crisis (PHC) or even death during right heart catheterization (RHC). This retrospective study was designed to identify the risks and clinical characteristics associated with PHC in pediatric PAH patients. We included 163 consecutive procedures from 147 pediatric patients diagnosed with PAH who underwent diagnostic RHC in Beijing Anzhen Hospital between January 2007 and December 2020. The average patient age was 9.0 ± 4.7 years and 84 (51.5%) were females. Before RHC, over 20% of patients were in New York Heart Association (NYHA) class III-IV. Sedation or general intravenous anesthesia was used in 103 procedures (63.2%), with spontaneous breathing in 93.2%. PHC occurred in 19 patients (11.7%), 5 (3.1%) required cardiac compression, and 1 died (0.6%). Compared to patients without PHC, those who experienced PHC were more likely to be in NYHA class III-IV (p = 0.012) before RHC, require sedation (p = 0.011), had echocardiographic indices of higher peak tricuspid regurgitation velocity (p = 0.018), and right ventricle (RV) to left ventricle (LV) ratio (p < 0.001). Multivariate logistic regression for PHC identified the need for sedation and a higher RV/LV ratio as independent predictors. In conclusion, the risk of RHC remains significant in children with PAH, particularly in those with severe RV dilation who require sedation during cardiac catheterization. Comprehensive evaluation, close monitoring, and appropriate treatment before and during the procedure are essential for reducing mortality.

A single institution anesthetic experience with catheterization of pediatric pulmonary hypertension patients

“…Safety issues related to the use of invasive airways include hypoxemia and hemodynamic compromise during induction with endotracheal intubation 17,40 . For example, acute pulmonary hypertensive episodes have been reported with induction and endotracheal intubation 15,19,41,42 . Conversely, the lack of having a controlled airway in the setting of acute decompensation is a potential important safety issue related to using a noninvasive airway.…”

“…17,40 For example, acute pulmonary hypertensive episodes have been reported with induction and endotracheal intubation. 15,19,41,42 Conversely, the lack of having a controlled airway in the setting of acute decompensation is a potential important safety issue related to using a noninvasive airway. In some reports, anesthesia with the use of invasive airways has been shown to be a risk factor for adverse events.…”

“…[6][7][8][9] The reported risk of serious adverse events during cardiac catheterization for pediatric pulmonary hypertension (PH) patients is variable, ranging from 2% to 6%. [6][7][8][9][10][11][15][16][17][18][19][20][21][22][23] Identified risk factors during cardiac catheterization include younger age, longer duration of procedure, suprasystemic pulmonary arterial pressures, right ventricular dysfunction, advanced functional class (III/IV), and treatment naiveté. [6][7][8][9][10][11][15][16][17][18][19][20][21]23 However, the type of anesthesia or respiratory support utilized has not been clearly identified as a risk factor.…”

“…[6][7][8][9][10][11][15][16][17][18][19][20][21][22][23] Identified risk factors during cardiac catheterization include younger age, longer duration of procedure, suprasystemic pulmonary arterial pressures, right ventricular dysfunction, advanced functional class (III/IV), and treatment naiveté. [6][7][8][9][10][11][15][16][17][18][19][20][21]23 However, the type of anesthesia or respiratory support utilized has not been clearly identified as a risk factor. 11,17,[19][20][21][22][23][24][25] Thus, there is a lack of consensus regarding the optimal anesthetic regimen and respiratory support for pediatric pulmonary vascular disease cardiac catheterization.…”

Outcomes of pregnancy in women with different types of pulmonary hypertension

“…The cardiac functional classification is I-IV based on the degree of physical activity, with reference to the WHO revised cardiac functional classification based on the NYHA cardiac functional classification. A diagnosis of pulmonary hypertensive crisis was defined based on PH, a rapid sharp increase in pulmonary artery pressure within a short period, and approaching the limit of or exceeding the baseline systemic circulation pressure and aortic pressure because of various factors, resulting in severe hypocardiac output (cardiac output < 3.5 L/min), hypoxemia (arterial oxygen partial pressure < 60 mmHg), hypotension (blood pressure < 90/60 mmHg), and acidosis (pH < 7.35) [ 10 , 11 ].…”