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

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.

scholarly journals Advanced therapies in pulmonary arterial hypertension and congenital heart disease in people with Down syndrome

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Zoya Rashid ◽  
Robert Tulloh
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Response To Therapy ◽  
Therapeutic Decisions ◽  
Advanced Therapies ◽  
Pulmonary Arterial

Abstract Background Management of patients with pulmonary arterial hypertension associated with congenital heart disease and trisomy 21 is complex due to uncertainty over the best first-line agent to use for treatment, and the outcome measures for assessing response to therapy. There are also ethical issues around therapeutic decisions which may require ‘best interests’ meetings of carers and health care professionals. Case presentation We present a case of a 35-year-old man with Down syndrome, with un-operated atrioventricular septal defect, right ventricular hypertrophy and moderate left atrioventricular valve regurgitation. In the clinic he was visibly cyanosed with peripheral oxygen saturations of 78% at rest. He had very limited walk distance and was in WHO class III. Conclusions We discuss the decisions made around the best therapy for his Eisenmenger syndrome taking into account patient and family views on therapy and considering the psychosocial burden of treatment.

Pulmonary Arterial Hypertension Complicating Congenital Heart Disease: Advances in Therapy

2017 ◽  
Vol 38 (05) ◽  
pp. 636-650 ◽  
Author(s):  
Margarita Brida ◽  
Laura Price ◽  
Colm McCabe ◽  
Rafael Alonso-Gonzalez ◽  
Stephen Wort ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Term Survival ◽  
Pulmonary Arterial

AbstractCongenital heart disease (CHD) is the most common inborn defect, affecting approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to dramatic improvements in patients' quality of life and long-term survival. Nevertheless, despite these innovations many patients require life-long follow-up and are at risk of numerous complications, namely, residual or progressive hemodynamic lesions, arrhythmia, sudden cardiac death, and the development of heart failure and pulmonary arterial hypertension (PAH), despite timely surgical intervention. Ill advisedly, some patients are also lost to follow-up, assuming that their early life surgical or catheter-based intervention was curative, hence missing out on invaluable lifetime screening and timely interventions that may be necessary. Additionally, there are many patients with undiagnosed or unoperated CHD in the developing world presenting later in life with irreversible complications. Even in this modern era of diagnosis and screening, PAH-CHD remains a burden in patients with CHD, with approximations of up to 10% in this heterogeneous group and is associated with an adverse impact on quality of life and survival.

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