Overexpression of endothelin-1 and endothelin receptors in the pulmonary arteries of failed Fontan patients

Ishida, Hidekazu; Kogaki, Shigetoyo; Ichimori, Hiroaki; Narita, Jun; Nawa, Nobutoshi; Ueno, Takayoshi; Takahashi, Kyoko; Kayatani, Futoshi; Kishimoto, H.; Nakayama, Masahiro; Sawa, Yoshiki; Beghetti, Maurice; Ozono, Keiichi

doi:10.1016/j.ijcard.2011.02.021

Cited by 40 publications

(27 citation statements)

References 26 publications

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“…The distal pulmonary arteries and micro‐circulation in Fontan connections may be strongly influenced by chronically decreased flow, absence of pulsatility, endothelial dysfunction, and absence of episodic high flow and pressure seen during normal exercise. The chronic nonpulsatile, low flow state increases vascular tone, and resistance, resulting in mildly elevated PVR at rest in many patients. Failing Fontan circuits often have high pulmonary vascular resistance.…”

Section: The Ventriclementioning

confidence: 99%

Cardiovascular adaptation to the Fontan circulation

Veldtman

Opotowsky

Wittekind

et al. 2017

Congenital Heart Disease

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Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide insight into the integrative pathophysiology that governs the Fontan circulation and stimulate thoughtful approaches to advance research.

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Section: The Ventriclementioning

confidence: 99%

Cardiovascular adaptation to the Fontan circulation

Veldtman

Opotowsky

Wittekind

et al. 2017

Congenital Heart Disease

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“…Although repeated catheter examinations indicated only mildly elevated increased pressure of the pulmonary artery, thickened endothelium of pulmonary capillary artery, to some extent, resembles that in pulmonary hypertension. Overexpression of endothelin‐1 and endothelin receptors in the pulmonary arteries has been observed in failed Fontan patients; and subclinical pulmonary pathophysiologic conditions with high plasma levels of endothelin‐1 in Fontan patients may play a role in the histologic finding …”

Section: Discussionmentioning

confidence: 99%

Diverse multi‐organ histopathologic changes in a failed Fontan patient

Mizuno

Ohuchi

Matsuyama

et al. 2016

Pediatrics International

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We report multi-organ histopathological changes in a patient with protein-losing enteropathy (PLE) over 12 years after Fontan operation. A 14-year-old boy with right isomerism heart and single ventricle had undergone Fontan procedure at 19 months of age, and PLE was diagnosed at 28 months. He had several episodes of intestinal bleeding and pre-renal failure with elevated creatine, and eventually died of pneumonia. The intrapulmonary small arteries showed medial and intimal thickening resembling pulmonary hypertension. No major ulcerative lesions were found in the small or large intestines. Dilated lymph ducts, one of the characteristic features of PLE, were not seen in mucosal and submucosal areas. Liver cirrhosis was obvious despite little increase in liver enzymes. Histological changes in bilateral kidneys were subtle despite repeated episodes of renal failure. Thus, there may be significant discrepancies between clinical manifestations and multi-organ histological changes in failed Fontan patients.

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“…Unfortunately, studies have also shown decrease in survival in those that undergo the procedure . Pulmonary arterial histology is abnormal, with biopsy studies showing increased wall thickness, smooth muscle proliferation, and intimal thickening with fibrosis . Given these findings, pulmonary vasodilator therapy has been used with increasing frequency but studies have shown mixed results .…”

Section: Fontan Physiology (Maan Jokhadar Md)mentioning

confidence: 99%

“…29 Pulmonary arterial histology is abnormal, with biopsy studies showing increased wall thickness, smooth muscle proliferation, and intimal thickening with fibrosis. 30,31 Given these findings, pulmonary vasodilator therapy has been used with increasing frequency but studies have shown mixed results. [32][33][34][35] The lack of efficacy is explained in part due to intrinsic problems with the lungs rather than the vasculature alone.…”

Section: Lon G-te Rm Out Comes (Rob Er T El De R Md)mentioning

confidence: 99%

“Frontiers in Fontan failure: A summary of conference proceedings”

Hebson

Book

Elder

et al. 2016

Congenital Heart Disease

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"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions.

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Overexpression of endothelin-1 and endothelin receptors in the pulmonary arteries of failed Fontan patients

Cited by 40 publications

References 26 publications

Cardiovascular adaptation to the Fontan circulation

Cardiovascular adaptation to the Fontan circulation

Diverse multi‐organ histopathologic changes in a failed Fontan patient

“Frontiers in Fontan failure: A summary of conference proceedings”

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