Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era

An, Haichao; Kim, Jun Seok; Song, Mi Kyoung; Bang, Ji Seok; Lee, Sang-Yun; Bae, Eun Jung; Noh, Chung Il

doi:10.1007/s00246-018-1956-y

Cited by 5 publications

(3 citation statements)

References 21 publications

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“…In human ES patients, desaturated blood circulates systemically due to R-L flow, and consequently, clinical signs develop (26). Morbidity and mortality in human ES patients are usually related to chronic hypoxemia, erythrocytosis, and coagulopathy (1,10,26).…”

Section: Discussionmentioning

confidence: 99%

Type 4 Tetralogy of Fallot with Pulmonary Hypertension in an American Shorthair Cat

Kim

et al. 2022

J Vet Clin

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A 2-year-old, spayed female, American shorthair cat presented with acute weight loss, tachypnea, and dyspnea. The cat had grade V holosystolic murmur and systemic hypotension. Echocardiography showed a 9 mm defect in the ventricular septum, left-to-right dominant bi-directional shunt, right ventricular hypertrophy, pulmonary stenosis, pulmonary hypertension, and overriding aorta. The cat was diagnosed with a Tetralogy of Fallot. The cat was treated with furosemide, pimobendan, ramipril, and sildenafil. Treatment reduced pulmonary infiltration, pulmonary vessel enlargement, and main pulmonary artery bulging. However, right-to-left flow increased over time and right ventricular outflow tract velocity was elevated. Currently, the patient has maintained an improved state for 1 year. This case report described a severe inherited feline Tetralogy of Fallot case that was successfully managed for a long time.

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Section: Discussionmentioning

confidence: 99%

Type 4 Tetralogy of Fallot with Pulmonary Hypertension in an American Shorthair Cat

Kim

et al. 2022

J Vet Clin

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“…Overall, the multidisciplinary approach to ES patients, including supportive management and treatment with targeted therapies, has improved quality of life, morbidity and outcomes, yet mortality is high in ES patients, especially in young patients with complex CHD [ 97 , 122 ].…”

Section: Group 1 Ph ( Table 1 )mentioning

confidence: 99%

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

2020

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Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.

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“…Although all unrestricted communications may be associated with PAH, early development of pulmonary vasculopathy is particularly common in patients with irregularities, such as truncus arteriosus, transposition of the great arteries with a ventricular septal defect (VSD), and complete atrioventricular septal defects. Nearly 100% of unoperated patients with volume overload develop pulmonary hypertension (PH) [ 2 , 3 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Remodeling Matrix Synthesis in a Rat Model of Aortocaval Fistula and the Cyclic Stretch: Impaction in Pulmonary Arterial Hypertension-Congenital Heart Disease

Chang

Huang

Chen

et al. 2020

IJMS

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Pulmonary arterial hypertension-congenital heart disease (PAH-CHD) is characterized by systemic to pulmonary arterial shunts and sensitively responds to volume overload and stretch of the vascular wall leading to pulmonary vascular remodeling. We hypothesized that the responses of pulmonary artery smooth muscle cells (PASMCs) to mechanical stress-associated volume overload may promote vascular remodeling in PAH-CHD. Here, we show that significantly increased collagen was in the PA adventitial layer by trichrome staining in PAH-CHD patients and an aortocaval fistula (ACF) rat model in which chronic vascular volume overload induced-PAH. We assessed the gene expression profiles of SMC markers, extracellular matrix, and collagen in isolated SMCs from pulmonary and thoracic vessels with cyclic stretch-triggered responses by real-time PCR analysis. The data corresponded to collagen deposition, which modulated pulmonary vascular remodeling in clinical and experimental PAH-ACF cases as well as in cyclic stretch-triggered SMCs in an in vitro model. We observe that collagen I A2 (COLIA2) is expressed in the control rat, but collagen I A1 (COLIA1) and Notchs remarkably increase in the lungs of ACF rats. Interestingly, closing the left-to-right shunt that leads to a reduced blood volume in the PA system of ACF rats (ACFRs) decreased the expression of COLIA1 and increased that of collagen I A2(COLIA2). This study contributes to the stretch-induced responses of SMCs and provides important future directions for therapies aimed at preventing abnormal matrix protein synthesis in volume overload-induced pulmonary hypertension (PH).

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Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era

Cited by 5 publications

References 21 publications

Type 4 Tetralogy of Fallot with Pulmonary Hypertension in an American Shorthair Cat

Type 4 Tetralogy of Fallot with Pulmonary Hypertension in an American Shorthair Cat

Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

Remodeling Matrix Synthesis in a Rat Model of Aortocaval Fistula and the Cyclic Stretch: Impaction in Pulmonary Arterial Hypertension-Congenital Heart Disease

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