Pulmonary arterial hypertension associated with congenital heart disease: classification and pathophysiology

Condliffe, Robin

doi:10.1186/s40949-020-00040-0

Cited by 5 publications

(4 citation statements)

References 33 publications

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“…PAH is a rare disease, with a reported prevalence of around 10 per million individuals in the United States, although this may be an underestimation [ 12 ] due to the generally non-specific symptoms. However, the combination of congenital heart defects with PAH is not uncommon, with PAH occurring in 4–28% of patients with congenital heart defects [ 13 ]. Given the rarity of PAH, neither patients nor their parents/guardians are likely to be aware of this chronic condition or, as in Haley’s case, have any family members with the disease.…”

Section: Clinician Perspectivementioning

confidence: 99%

Why Living with Pulmonary Arterial Hypertension Requires a Holistic Approach: A Patient and Clinician Perspective

Lynn

Wilson

2023

Pulm Ther

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Pulmonary arterial hypertension (PAH) is a rare disease caused by high pressure in the blood vessels leading from the heart to the lung. PAH affects many parts of a patient's life, which means that patients should be managed by a clinical team of different specialists, including doctors, advance practice providers, nurses, social workers, and therapists. This article is coauthored by a patient living with PAH and an acute care nurse practitioner specializing in the management of patients with pulmonary hypertension. In the first section of this commentary, the patient describes her experience of living with PAH. The specialist nurse practitioner then discusses the management of PAH, to provide a clinician perspective in the context of the patient's experiences.

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Section: Clinician Perspectivementioning

confidence: 99%

Why Living with Pulmonary Arterial Hypertension Requires a Holistic Approach: A Patient and Clinician Perspective

Lynn

Wilson

2023

Pulm Ther

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“…Patients with unrepaired, moderate to large, systemic-to-pulmonary shunts and mildly to moderately elevated PVR constitute the second group of PAH-CHD. These patients are in an earlier stage pathophysiologically compared ES but require more complex management strategies due to a lack of evidence and their limited inclusion in clinical trials [44,45]. The decision to close the shunt or to use medical therapy depends on several factors, including the severity of PAH, increased PVR, and the location and size of the defect [46].…”

Section: Pah Associated With Prevalent Left-to-right Shuntsmentioning

confidence: 99%

Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Mahmoud,

Abbas,

Kamel

et al. 2023

JPM

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Current management of patients with congenital heart disease has increased their survival into adulthood. This is accompanied by potential cardiac complications, including pulmonary hypertension associated with congenital heart disease (PAH-CHD). PAH-CHD constitutes a challenging subgroup of pulmonary hypertension and requires expert management to improve quality of life and prognosis. Novel agents have shown a significant improvement in morbidity and mortality in patients with pulmonary arterial hypertension. However, the long-term effects of these medications on PAH-CHD patients remain somewhat uncertain, necessitating treatment plans largely founded on the clinical experience of the healthcare providers. The aim of this review is to summarize the current evidence and future perspectives regarding treatment strategies for PAH-CHD to help better guide management of this complex disease.

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“…Pulmonary arterial hypertension (PAH) is prevalent among adults with congenital heart disease (CHD), affecting from 4.2 to 28% of CHD patients [1]. PAH associated with CHD is divided into four clinical and hemodynamic profiles: Eisenmenger syndrome, PAH associated with a predominant moderate to-large systemic-to-pulmonary shunt, PAH associated with a small defect, and PAH associated with a repaired defect [1,2]. Eisenmenger syndrome is characterized by the reversal of large unrepaired systemic-to-pulmonary shunt due to pulmonary vasculopathy and increased pulmonary vascular resistance (PVR) [1].…”

Section: Introductionmentioning

confidence: 99%

“…PAH associated with CHD is divided into four clinical and hemodynamic profiles: Eisenmenger syndrome, PAH associated with a predominant moderate to-large systemic-to-pulmonary shunt, PAH associated with a small defect, and PAH associated with a repaired defect [1,2]. Eisenmenger syndrome is characterized by the reversal of large unrepaired systemic-to-pulmonary shunt due to pulmonary vasculopathy and increased pulmonary vascular resistance (PVR) [1]. Patients with PAH associated with CHD have better outcomes compared to those with idiopathic PAH, and the survival of patients with Eisenmenger syndrome is superior compared to cases with unrepaired CHD without Eisenmenger syndrome or patients with persistently increased PVR after shunt closure [2,3].…”

Section: Introductionmentioning

confidence: 99%

Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report

Ereminienė,

Stuoka,

Ordienė

et al. 2024

Medicina

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Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently. Case presentation: A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24–48 h of admission. Conclusions: This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD.

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Pulmonary arterial hypertension associated with congenital heart disease: classification and pathophysiology

Cited by 5 publications

References 33 publications

Why Living with Pulmonary Arterial Hypertension Requires a Holistic Approach: A Patient and Clinician Perspective

Why Living with Pulmonary Arterial Hypertension Requires a Holistic Approach: A Patient and Clinician Perspective

Current Management and Future Directions for Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report

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