Markers as predictors of clinical worsening in pulmonary arterial hypertension

Current understanding and perioperative management of pediatric pulmonary hypertension

“…Right ventricular diastolic dysfunction and RV failure play a central role in morbidity and mortality of pediatric PH and are thus a key prognostic factor. Due to effective remodeling and perhaps improved metabolic efficiency of the pediatric RV under chronic strain, it is not infrequent for children, especially compared to adults, to have high PAP, nearsyncope, and exertional dyspnea yet remain in functional class II for a prolonged period due to preserved RV systolic and diastolic function . The mechanism of syncope is not understood but likely relates to a transient elevation of the PAP to aortic pressure ratio, resulting in an acute reduction in cerebral blood flow .…”

“…Recall that the presence of syncope or near syncope is an ominous sign, regardless of the listed WHO functional class. A BNP value >59.5 pg/mL has been shown to be an indicator of high risk for future clinical deterioration in pediatric PAH, as it reflects the severity of RV dysfunction . The most recent echocardiogram should be reviewed, while appreciating that the test is a snapshot of the child at one moment in time and may not represent the cardiopulmonary status on the day of surgery.…”

“…The major classes of PAH-targeted therapies used in children are listed in Table 2 in functional class II for a prolonged period due to preserved RV systolic and diastolic function. 39,63 The mechanism of syncope is not understood but likely relates to a transient elevation of the PAP to aortic pressure ratio, resulting in an acute reduction in cerebral blood flow. 64 The preoperative evaluation should include case discussion between the anesthesiologist and the child's cardiologist.…”

“…A BNP value >59.5 pg/mL has been shown to be an indicator of high risk for future clinical deterioration in pediatric PAH, as it reflects the severity of RV dysfunction. 63 The most recent echocardiogram should be reviewed, while appreciating that the test is a snapshot of the child at one moment in time and may not represent the cardiopulmonary status on the day of surgery. In most cases, an echo should be available within the past 3-6 months, as that is the recommended surveillance interval in children with active disease.…”

“…The etiology of PH is inconsistently associated with risk 1,39,[67][68][69][70][71][72]. Anecdotally, newly diagnosed children presenting for cardiac catheterization are also at significant risk, perhaps because most have yet to be medically optimized until after the catheterization results.Adapted from Refs ( 1,39,51,52,63,67. ).…”