Pulmonary Hypertension in Congenital Heart Disease: A Scientific Statement From the American Heart Association

Jone, Pei‐Ni; Ivy, D. Dunbar; Hauck, Amanda; Karamlou, Tara; Truong, Uyen; Coleman, Ryan; Sandoval, Juan Pablo; Marín, M.J. del Cerro; Eghtesady, Pirooz; Tillman, Kathryn; Krishnan, Usha

doi:10.1161/hhf.0000000000000080

Cited by 28 publications

(16 citation statements)

References 132 publications

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“…Our results revealed that the occurrence of LV underfilling pattern was associated with a higher risk of adverse cardiac events in PAH patients, irrespective of RV changes. Moreover, although CHD-PAH patients tend to have better outcomes compared to IPAH and CTD-PAH patients in previous studies, 28,29 in PAH patients without LV underfilling pattern, they presented with similar outcome.…”

Section: Discussionmentioning

confidence: 67%

Left ventricular underfilling in PAH: A potential indicator for adaptive‐to‐maladaptive transition

Guo,

Wang,

Wang

et al. 2023

Pulm. circ.

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Pulmonary arterial hypertension (PAH) still remains a life‐threatening disorder with poor prognosis. The right ventricle (RV) adapts to the increased afterload by a series of prognostically significant morphological and functional changes, the adaptive nature should also be understood in the context of ventricular interdependence. We hypothesized that left ventricle (LV) underfilling could serve as an important imaging marker for identifying maladaptive changes and predicting clinical outcomes in PAH patients. We prospectively enrolled patients with PAH who underwent both cardiac magnetic resonance and right heart catheterization between October 2013 and December 2020. Patients were categorized into four groups based on their LV and RV mass/volume ratio (M/V). LV M/V was stratified using the normal value (0.7 g/mL for males and 0.6 g/mL for females) to identify patients with LV underfilling (M/V ≥ normal value), while RV M/V was stratified based on the median value. The primary endpoint was all‐cause mortality, and the composite endpoints included all‐cause mortality and heart failure‐related readmissions. A total of 190 PAH patients (53 male, mean age 37 years) were included in this study. Patients with LV underfilling exhibited higher NT‐proBNP levels, increased RV mass, larger RV but smaller LV, lower right ventricular ejection fraction, and shorter 6‐min walking distance. Patients with LV underfilling had a 2.7‐fold higher risk of mortality than those without and LV M/V (hazard ratio [per 0.1 g/mL increase]: 1.271, 95% confidence interval: 1.082–1.494, p = 0.004) was also independent predictors of all‐cause mortality. Moreover, patients with low LV M/V had a better prognosis regardless of the level of RV M/V. Thus, LV underfilling is an independent predictor of adverse clinical outcomes in patients with PAH, and it could be an important imaging marker for identifying maladaptive changes in these patients.

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Section: Discussionmentioning

confidence: 67%

Left ventricular underfilling in PAH: A potential indicator for adaptive‐to‐maladaptive transition

Guo,

Wang,

Wang

et al. 2023

Pulm. circ.

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“…Management of portopulmonary hypertension (PoPH) and PAH associated with congenital heart disease (PAH-CHD) requires a multidisciplinary management approach [46]. Larger clinical trials have either underrepresented or excluded patients with these conditions, leading to treatment strategies often based on the experience of clinical experts or findings from retrospective studies [47].…”

Section: Treating Portopulmonary Hypertension and Pulmonary Arterial ...mentioning

confidence: 99%

“…PDE-5 inhibitors like sildenafil and tadalafil are cheaper options and work by increasing intracellular cGMP levels [48]. These agents have been shown to improve hemodynamics and exercise capacity [47]. Agents exerting their action through the endothelin pathway include bosentan, macitentan, and ambrisentan; they have achieved favorable results in patients with PAH-CHD.…”

Section: Treating Portopulmonary Hypertension and Pulmonary Arterial ...mentioning

confidence: 99%

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Classification, Diagnosis, and Medical Treatment of Pulmonary Hypertension

Tenpas,

Panahi,

Udeani

et al. 2024

New Insights on Pulmonary Hypertension

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Pulmonary hypertension is a condition characterized by elevated blood pressure in pulmonary arteries due to increased muscle mass of vessel walls, leading to arterial constriction and reduced blood oxygenation. Commonly classified into five major groups, pulmonary hypertension is often viewed as quite rare when, in fact, it is far more common than traditionally advertised. It is also an extremely debilitating disease with far-reaching economic, societal, personal, and psychosocial impacts, especially in underserved populations. Though 10 FDA-approved medications—targeting four different biological pathways—have come to market over the last 20 years, more recent research has focused on complex signaling pathways regulating hypoxic and metabolic signaling, proliferation, apoptosis, senescence, and inflammation. In this chapter, we provide an overview of pulmonary hypertension’s prevalence and widespread impact, its underlying pathophysiology and clinical presentations, currently recognized treatment strategies, recommended regimens in special populations, and emerging therapeutic options and fields of research.

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“…During fetal development, the pulmonary vasculature undergoes intricate changes in preparation for the transition from intrauterine to extrauterine life [1]. However, certain factors, such as prematurity, congenital heart defects, or prenatal insults, can disrupt this process, leading to pulmonary vascular disease (PVD) in newborns [2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%

Clinical Phenotype of Pulmonary Vascular Disease Requiring Treatment in Extremely Preterm Infants

Hong,

Shin,

Kim

et al. 2024

Preprint

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Background Pulmonary vascular disease (PVD) and pulmonary hypertension (PH) is a significant disorder affecting prognosis of extremely preterm infants. However, there is still a lack of a consensus on the definition and optimal treatments of PH, and there is also a lack of research comparing these conditions with persistent pulmonary hypertension of newborn (PPHN), early PH, and late PH. To investigate pulmonary hypertension (PH) in extremely preterm infants, this study compared the basal characteristics, short-term outcomes, and treatment duration, categorized by the timing of requiring PH treatment. Methods This study retrospectively analyzed extremely preterm infants admitted to a single tertiary center. Between 2018 and 2022, infants with clinical symptoms or echocardiographic evidence of PH who required treatment were divided into three groups based on the timing of treatment initiation: from birth to day 2 (extremely early-period), from day 3 to day 27 (early-period), and after day 28 (late-period). The study compared the outcomes, including mortality rates, bronchopulmonary dysplasia (BPD) severity, PH treatment duration, and oxygen therapy duration, among the three groups. Results Among the 157 infants, 67 (42.7%) were treated for PH during their stay. Of these, 39 (57.3%) were treatment in extremely early, 21 (31.3%) in early, and seven (11.4%) in late periods. No significant differences were observed in maternal factors, neonatal factors, or morbidity between the three groups. However, infants who received extremely early-period treatment had a higher mortality rate, but shorter duration of noninvasive respiratory support, oxygen therapy, and PH medication use. On the other hand, the late-period treatment group received longer durations of respiratory support and treatment. Conclusions This study revealed differences in mortality rates, respiratory outcomes, and treatment duration between the three groups, suggesting varying pathophysiologies over time in extremely preterm infants.

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Pulmonary Hypertension in Congenital Heart Disease: A Scientific Statement From the American Heart Association

Cited by 28 publications

References 132 publications

Left ventricular underfilling in PAH: A potential indicator for adaptive‐to‐maladaptive transition

Left ventricular underfilling in PAH: A potential indicator for adaptive‐to‐maladaptive transition

Classification, Diagnosis, and Medical Treatment of Pulmonary Hypertension

Clinical Phenotype of Pulmonary Vascular Disease Requiring Treatment in Extremely Preterm Infants

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