Outcomes and occurrence of post-operative pulmonary hypertension crisis after late referral truncus arteriosus repair

Abstract

BackgroundTruncus arteriosus (TA) is a rare congenital heart disease with a high rate of early mortality. The occurrence of post-operative pulmonary hypertension crisis (PHC), known to be a common and life-threatening complication, increases due to the irreversible development of pulmonary vascular resistance with age. We sought to figure out the risk factors for PHC and describe the surgical outcomes of TA patients with late referral (repair <1 month excluded).Materials and methodsWe retrospectively reviewed patients after TA repair between 2009 and 2021 at Shanghai Children’s Medical Center. The occurrence of PHC was defined according to post-operative Pp/Ps ≥ 1 and clinical manifestations. Risk factors for PHC and mortality were conducted by multivariable analysis.ResultsA total of 98 patients were treated, including 55 males and 43 females. The median age at repair was 121 (69, 245) days. Post-operative PHC occurred in 22 (22.4%) patients with a median age of 186 (122, 293) days. By multivariable analysis, patients with the sum of Z-score of pre-operative bilateral pulmonary artery (PA) diameters (OR: 1.6, 95% CI: 1.2–2.3, P = 0.01) was more likely to experience PHC. Longer CPB duration contributed to early death (OR: 1.0, 95% CI: 1.0–1.0, P = 0.01). Total survival at 10 years was 81.4%. In 4.5 (2.9, 7.5) years of follow-up, twenty-six patients received 30 reinterventions. Valved reconstruction of RVOT most predicted reinterventions (OR: 4.2, 95% CI: 1.4–13.0, P = 0.01).ConclusionSurgical repair of TA patients with late referral has resulted in comparatively favorable early and mid-term outcomes. PHC occurred more commonly in patients with overextended bilateral PA pre-operatively. Meanwhile, valved reconstruction of RVOT would more likely lead to early reintervention.

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 ].…”

Preoperative Sildenafil in Pediatric Patients Undergoing Congenital Heart Surgeries: A Systematic Review and Meta-Analysis

“…3 Perioperative PH crisis occurs in 2-22% of patients, which may lead to perioperative mortality. 4,5 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 development of this pulmonary hypertension crisis involves various factors, including the systemic inflammatory response syndrome, damage to endothelial cells, reduced production of nitric oxide (NO), and elevated endothelin levels.…”