Selexipag as Add-on Therapy for Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: A Single-Center Retrospective Study

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

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10. Preliminary study of composite clinical worsening definitions in chinese people with pulmonary arterial hypertension associated with congenital heart disease – A single center experience

International Journal of Cardiology Congenital Heart Disease ◽

10.1016/j.ijcchd.2020.100038 ◽

2021 ◽

Vol 2 ◽

pp. 100038

Author(s):

Zhuoyuan Xu ◽

Qiangqiang Li ◽

Chen Zhang ◽

Hongsheng Zhang ◽

Hong Gu

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Congenital Heart ◽

Chinese People ◽

Single Center ◽

Single Center Experience ◽

Pulmonary Arterial ◽

Preliminary Study ◽

Clinical Worsening

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Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

Journal of Congenital Cardiology ◽

10.1186/s40949-020-00052-w ◽

2020 ◽

Vol 4 (S1) ◽

Author(s):

Nathalie Liew ◽

Zoya Rashid ◽

Robert Tulloh

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Standard Management ◽

Advanced Therapies ◽

Pulmonary Arterial ◽

The Uk

Abstract Background Pulmonary hypertension (PH) is commonly seen in adults who have congenital heart disease (CHD). Therapy is available for pulmonary arterial hypertension (PAH) and has greatly benefitted many patients with PAH related to CHD (PAH-CHD) over the last 15 years, with evidence of improved quality of life and prognosis in those with Eisenmenger syndrome and repaired PAH-CHD. In this review, we describe the standard management and advanced therapies for PAH, which are available in specialist PH centres around the UK and Ireland, and how these are used in PAH-CHD. Decisions around the choice of therapy are governed by commissioning and available evidence. Conclusion We explain the different pathways for action and the variety of medications now at our disposal to help this important group of patients.

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Predictive role of perioperative neutrophil to lymphocyte ratio in pediatric congenital heart disease associated with pulmonary arterial hypertension

BMC Surgery ◽

10.1186/s12893-020-01009-x ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Xiaoqiang Yin ◽

Mei Xin ◽

Sheng Ding ◽

Feng Gao ◽

Fan Wu ◽

...

Keyword(s):

Mechanical Ventilation ◽

Congenital Heart Disease ◽

Cardiac Surgery ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Prolonged Mechanical Ventilation ◽

Pulmonary Arterial ◽

Mechanical Ventilation Time

Abstract Background We aimed to explore the relationship between the neutrophil to lymphocyte ratio (NLR) and the early clinical outcomes in children with congenital heart disease (CHD) associated with pulmonary arterial hypertension (PAH) after cardiac surgery. Methods A retrospective observational study involving 190 children from January 2013 to August 2019 was conducted. Perioperative clinical and biochemical data were collected. Results We found that pre-operative NLR was significantly correlated with AST, STB, CR and UA (P < 0.05), while post-operative NLR was significantly correlated with ALT, AST, BUN (P < 0.05). Increased post-operative neutrophil count and NLR as well as decreased lymphocyte count could be observed after cardiac surgery (P < 0.05). Level of pre-operative NLR was significantly correlated with mechanical ventilation time, ICU stay time and total length of stay (P < 0.05), while level of post-operative NLR was only significantly correlated to the first two (P < 0.05). By using ROC curve analysis, relevant areas under the curve for predicting prolonged mechanical ventilation time beyond 24 h, 48 h and 72 h by NLR were statistically significant (P < 0.05). Conclusion For patients with CHD-PAH, NLR was closely related to early post-operative complications and clinical outcomes, and could act as a novel marker to predict the occurrence of prolonged mechanical ventilation.

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