Pulmonary arterial hypertension in congenital heart disease

Frogoudaki, Alexandra

doi:10.1002/cce2.74

Cited by 9 publications

(4 citation statements)

References 38 publications

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“…Similar to the PHIS study, a majority of PH cases in this study were associated with CHD, followed by BPD; similar results have been reported by other studies [ 15 ]. However, there has been an improvement in morbidity and mortality of these patients in the last few decades due to the advent of newer treatment modalities and early surgical repair of CHD [ 16 ].…”

Section: Discussionsupporting

confidence: 92%

Demographics and Risk Factors of Pediatric Pulmonary Hypertension Readmissions

Sehgal¹,

Amritphale²,

Vadayla³

et al. 2021

Cureus

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Section: Discussionsupporting

confidence: 92%

Demographics and Risk Factors of Pediatric Pulmonary Hypertension Readmissions

Sehgal¹,

Amritphale²,

Vadayla³

et al. 2021

Cureus

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“…At that point, the therapeutic approach for adults with PAH and CHD differs from the approach for those with idiopathic PAH, as there are only few data to support routine up-front/early sequential oral combination therapy for Eisenmenger syndrome [1,6,26,[39][40][41][42]. In adults with PAH and CHD, sequential combination therapy is frequently initiated only upon symptomatic deterioration or if predefined, and often center-specific treatment goals are not achieved [43].…”

Section: Demographics Hemodynamics and Treatmentmentioning

confidence: 99%

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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“…24 One pending task for the health systems would be to identify this population in an early reversible stage to avoid the transition into irreversible complex vascular lesions. 31 Based on a high incidence of endovascular and surgery advances, 32 governmental measures and national health system programs are needed to prevent PAH, reducing the economic impact on health care systems. 24 Currently, standard and specific treatments are the foundation of PAH patients' care.…”

Section: Discussionmentioning

confidence: 99%

Demographic, hemodynamic characteristics, and therapeutic trends of pulmonary hypertension patients: The Pulmonary Hypertension Mexican registry (REMEHIP)

Jerjes‐Sánchez,

Ramírez‐Rivera,

Hernandez

et al. 2024

Pulm. circ.

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Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in‐hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6‐min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.

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Pulmonary arterial hypertension in congenital heart disease

Cited by 9 publications

References 38 publications

Demographics and Risk Factors of Pediatric Pulmonary Hypertension Readmissions

Demographics and Risk Factors of Pediatric Pulmonary Hypertension Readmissions

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

Demographic, hemodynamic characteristics, and therapeutic trends of pulmonary hypertension patients: The Pulmonary Hypertension Mexican registry (REMEHIP)

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