Management of Adults With Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK: Survey of Current Practice, Unmet Needs and Expert Commentary

Tulloh, Robert; Dimopoulos, Konstantinos; Condliffe, Robin; Clift, Paul

doi:10.1016/j.hlc.2017.10.018

Cited by 8 publications

(12 citation statements)

References 31 publications

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“…The international COMPERA-CHD registry provides information on the characteristics, treatment patterns, and long-term outcomes in a large number of patients with PAH caused by CHD, in the modern treatment era. This is of paramount importance as there are relatively limited data on targeted PAH therapy and outcomes in adults with PAH resulting from CHD compared with idiopathic PAH [1,[23][24][25][26][27]. Furthermore, the existing data are mostly derived from small, uncontrolled studies with few patients, predominantly with simple congenital heart defects, and short monitoring periods [28].…”

Section: Discussionmentioning

confidence: 99%

“…Accordingly, there is a shortage of evidence about targeted PAH therapies for CHD with PAH, and it remains unclear whether data from current studies on other forms of PAH can be applied to PAH patients with CHD [23,33].…”

Section: Discussionmentioning

confidence: 99%

“…Although mortality has decreased in adults with CHD, morbidity is still high due to residua, sequelae, or complications, and many patients develop pathologies of the pulmonary vascular bed, complicating the natural history of the particular anomaly [4,23,47,48]. In adults with PAH from CHD, all-cause mortality is more than two-fold, and the rate of health service utilization is three-fold higher compared with CHD without PAH [38].…”

Section: Survivalmentioning

confidence: 99%

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Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Kaemmerer

Gorenflo

Huscher

et al. 2020

JCM

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Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients’ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan–Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Survivalmentioning

confidence: 99%

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Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Kaemmerer

Gorenflo

Huscher

et al. 2020

JCM

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“…The interpretation of invasive haemodynamic data should always be made in the context of the clinical history, presentation, and results from other noninvasive investigations. Haemodynamic assessment of patients with CHD should be performed in expert centres, ensuring adequate quality control with accurate acquisition and interpretation of data, especially in the estimation of PVR and shunt fraction [22].…”

Section: Right Heart Catheterisationmentioning

confidence: 99%

The investigation and diagnosis of pulmonary hypertension in adults with congenital heart disease

Nashat

Favoccia

Constantine

et al. 2020

J Congenit Heart Dis

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Pulmonary hypertension is not uncommon in adult patients with congenital heart disease and can significantly affect their exercise capacity, quality of life and prognosis. Timely identification and management of pulmonary hypertension in these patients is important. Patients must be allocated to the correct diagnostic group and treatment decided upon, including offering pulmonary arterial hypertension therapies to those who are most likely to benefit.In this paper, we provide an overview of the diagnostic modalities that are used to investigate and diagnose pulmonary hypertension in modern adult congenital heart disease practice. Emphasis is placed on echocardiography, which is the modality of choice for raising the suspicion of PH, and cardiac catheterisation, which is fundamental in establishing the diagnosis. Other modalities, such as cardiac magnetic resonance imaging and computed tomography can provide complementary information on anatomy and physiology.Combining all this information into a clinical diagnosis and management plan requires clinical expertise and a multidisciplinary approach, especially when managing this rare and complex group of patients.

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“…Tulloh and colleagues in the United Kingdom conducted a survey of physicians managing adults with PAH-CHD. 68 They report that although 97% of respondents have access to a specialist pediatric health center, patients are still being managed in nonspecialist settings. Shared care arrangements are widespread but only 41% have formal shared care protocols.…”

Section: Adult Congenital Heart Diseasementioning

confidence: 99%

Noteworthy Literature published in 2017 for Congenital Cardiac Anesthesiologists

Ing

Twite

2018

Semin Cardiothorac Vasc Anesth

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Management of Adults With Congenital Heart Disease and Pulmonary Arterial Hypertension in the UK: Survey of Current Practice, Unmet Needs and Expert Commentary

Abstract: The survey revealed gaps and inconsistencies in the management of patients with PAH-CHD therefore patient-specific guidance is needed for many of these aspects.

Cited by 8 publications

References 31 publications

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry

The investigation and diagnosis of pulmonary hypertension in adults with congenital heart disease

Noteworthy Literature published in 2017 for Congenital Cardiac Anesthesiologists

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