Residual Pulmonary Hypertension More than 20 Years after Repair of Shunt Lesions

Abstract

Background and Objectives: After successful surgical repair of a congenital shunt lesion, pulmonary hypertension (PH) often disappears. However, PH can persist long-term after the closure. This study aimed to assess the prevalence of PH long-term after surgical repair of congenital heart disease (CHD), and to evaluate the outcomes and preoperative factors related to residual PH. Materials and Methods: In this retrospective cohort study, we reviewed patients who underwent right heart catheterisation in Vilnius University Hospital Santaros Klinikos during the period of 1985–2007. Among 4118 right heart catheterisations performed, 160 patients underwent congenital systemic-to-pulmonary shunt repair at a young age (<18 years) and had pre-operative PH. Half of the patients were foreigners whose follow-up data were unavailable. Eventually, 88 patients with available follow-up data were included in this study. Results: The median age at diagnosis of CHD with PH was 0.8 (0.6–3.0) and 1.1 (0.6–3.9) years at surgery (50% females). Residual PH was assessed 9.5 years after surgery and observed in 30.7% (n = 27) of the patients. It was associated with having more than one shunt (44.4% (n = 12), p = 0.016) and higher median pulmonary vascular resistance (3.4 (2.5–6.5) vs. 2.2 (1.0–3.7), p = 0.035) at baseline. After a median follow-up of 21 (15–24) years, 9.1% of the patients were deceased. Kaplan–Meier survival analysis revealed significantly higher mortality in the residual PH group (p = 0.035). Conclusions: Residual PH affects a significant proportion of patients after surgical repair of a shunt lesion and is associated with worse long-term outcome.

Inhaled Milrinone via HFNC as a Postextubation Cardiopulmonary Elixir: Case Series and Review of Literature

“…1 Notably so, PH after surgically repaired CHD is associated with worse outcomes compared with other etiologies of PH. 2 Moreover, the present day perioperative cardiac surgical challenges have only been intensified considering increasing number of patients presenting for surgical intervention postcoronavirus disease 2019 (COVID-19) infection because of its long-term ill effects and multiorgan involvement. In this context, COVID-19 infection may exacerbate and escalate PH secondary to CHD.…”

“…1 Notably so, PH after surgically repaired CHD is associated with worse outcomes compared with other etiologies of PH. 2…”

“…Perioperative PH can peculiarly lead to a protracted intensive care unit (ICU) stay with enhanced morbidity and mortality owing to its perilous cardiopulmonary effects. 2 Therefore, the current strategies to treat PH focus on pharmacological agents that may unload right ventricle while maintaining systemic pressures as well as applying cardiopulmonary protective ventilatory approach. 4…”

“…3 Perioperative PH can peculiarly lead to a protracted intensive care unit (ICU) stay with enhanced morbidity and mortality owing to its perilous cardiopulmonary effects. 2 Therefore, the current strategies to treat PH focus on pharmacological agents that may unload right ventricle while maintaining systemic pressures as well as applying cardiopulmonary protective ventilatory approach. 4 In this background, we report a series of six pediatric PH predisposed cardiac surgical cohort who were administered inhaled milrinone (iMil), a pulmonary vasodilator via highfrequency nasal cannula (HFNC) postextubation, with an aim to avert residual PH-associated cardiopulmonary repercussion, thereby expediting ICU recovery and discharge.…”

Elementos hemodinâmicos e de resposta inflamatória e seu papel prognóstico no contexto do tratamento cirúrgico em pacientes pediátricos com cardiopatia congênita e hipertensão pulmonar

“…No momento do tratamento, alguns pacientes submetidos à intervenção cirúrgica terão curso pós-operatório tormentoso com alterações hemodinâmicas e eventos clínicos relevantes 3 . Após a alta hospitalar, no seguimento a médio e longo prazo, determinados pacientes persistirão com pressão pulmonar elevada após ter sido corrigido o defeito cardíaco, seja de forma percutânea, seja por meio de cirurgia 4,5 .…”