Pulmonary hypertension after operations for congenital heart disease: Analysis of risk factors and management

Bando, Ko; Turrentine, Mark W.; Sharp, T. G.; Sekine, Yasuo; Aufiero, Thomas X.; Sun, Kyung; Sekine, Eri; Brown, John W.

doi:10.1016/s0022-5223(96)70019-3

Cited by 163 publications

(112 citation statements)

References 15 publications

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“…Concurrently with this better understanding of the development of pulmonary hypertension, there has been a trend toward early surgical repair in neonates and infants with congenital heart disease with significant left to right shunt, even among premature and low-birth-weight neonates (13,14,39,51). Despite improved outcomes compared with initial palliation and delayed repair, Bando et al (5) have found that preexisting pulmonary hypertension and "late repair" continued to be significant risk factors for perioperative morbidity and mortality. The aim of our study was to determine the effect of increased pulmonary blood flow on SP-A, -B, and -C gene expressions in a lamb model of congenital heart disease with significant left to right shunt within the first week of life.…”

Section: Discussionmentioning

confidence: 99%

Increased pulmonary blood flow does not alter surfactant protein gene expression in lambs within the first week of life

Lee¹,

Ovadia²,

Azakie³

et al. 2004

American Journal of Physiology-Lung Cellular and Molecular Phys

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Neonates and infants with congenital heart disease with increased pulmonary blood flow suffer morbidity from poor oxygenation and decreased lung compliance. In a previous experiment involving 4-wk-old lambs with pulmonary hypertension secondary to increased pulmonary blood flow following an in utero placement of an aortopulmonary vascular graft, we found a decrease in surfactant protein (SP)-A gene expression as well as a decrease in SP-A and SP-B protein contents. To determine the timing of these changes, the objective of the present study was to characterize the effect of increased pulmonary blood flow and pulmonary hypertension on SP-A, -B, and -C gene expressions and protein contents within the first week of life. Of eight fetal lambs that underwent the in utero placement of the shunt, there was no difference in the expression of SP-A, -B, and -C mRNA levels or SP-A and -B protein contents compared with age-matched controls. The results showed that, in this model of congenital heart disease with pulmonary hypertension and increased pulmonary blood flow, the effect of the shunt on SP gene expression and protein content was not apparent within the first week of life.congenital heart disease AMONG NEONATES AND INFANTS with congenital heart disease with increased pulmonary blood flow, the presence of the systemicto-pulmonary communication often leads to progressive structural and functional changes in the pulmonary vascular bed, leading to pulmonary hypertension. The structural changes include increased muscularity of small pulmonary arteries with early distal extension, intimal hyperplasia, scarring and thrombosis, and a reduction in the number of intra-acinar arteries, as well as the arterial-to-alveolar ratio. These vascular changes result in eventual obliteration of the pulmonary vascular bed with elevation in pulmonary vascular resistance and extreme sensitivity to vasoconstricting stimuli. If uncorrected, these children will suffer from morbidity from cyanosis and myocardial failure (12,34,49).Multiple studies have shown that increased pulmonary blood flow and/or pressure affect endothelial cell functions within the alveolar space. Grigg et al. (22) found elevated levels of endothelin-1 (ET-1) in bronchoalveolar lavage in children with congenital heart disease with increased pulmonary blood flow. Ishikawa et al. (36) found elevated plasma ET-1 levels in children with ventricular septal defects who had both volume and pressure overload to the pulmonary circulation but not in children with atrial septal defects who had only volume overload. In cultured human vascular endothelial cells, shear stress stimulated the synthesis of mRNA for platelet-derived growth factors-A and -B (48), tissue plasminogen activator (20), and intercellular adhesion molecule-1 (46).Recently, increased pulmonary blood flow and/or pressure has also been shown to affect pulmonary epithelial cell functions. Wang et al. (59) showed that, in an isolated, constantly inflated, blood-perfused rat lung, increased left atrial pressure increa...

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Section: Discussionmentioning

confidence: 99%

Increased pulmonary blood flow does not alter surfactant protein gene expression in lambs within the first week of life

Lee¹,

Ovadia²,

Azakie³

et al. 2004

American Journal of Physiology-Lung Cellular and Molecular Phys

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“…Older patients with elevated pulmonary arterial pressure preoperatively are at higher risk of developing postoperative PAH. 29,30 Advanced PAH therapies might have the potential to produce a reduction in pulmonary vascular resistance, lowering perioperative risks and possibly widening the range of cases amenable to repair.…”

Section: Corrective Interventionsmentioning

confidence: 99%

Eisenmenger Syndrome: Not Always Inoperable

Liang

Zhou

2012

Respir Care

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Recently, advanced therapies for pulmonary arterial hypertension have become available, and have been effective in reducing pulmonary vascular resistance and symptoms in patients with Eisenmenger syndrome, previously thought to be inoperable. This review summarizes the current knowledge on the pathophysiology and treatment of Eisenmenger syndrome. The recent introduction of targeted therapies in pulmonary arterial hypertension has led to a renewed insight in the pathophysiology and treatment of Eisenmenger syndrome. Patients with Eisenmenger syndrome using a diagnostic-treatment-and-repair strategy are amenable to surgery after successful treatment with advanced therapy. With continued improvements in the diagnosis, preoperative management, refinement of surgical techniques and intra-and postoperative management strategies, the patients with Eisenmenger syndrome selected using a diagnostic-treatment-and-repair strategy are operable with safety and efficacy in the current era with advanced pulmonary arterial hypertension therapy. Future directions of Eisenmenger syndrome may be the combination of reversal of pulmonary vascular remodeling and correction.

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“…Since inhaled nitric oxide (iNO) is established as an additional therapeutic option, the mortality rate of PAH has further declined. [4,18,19,20] Despite relatively high mortality rates reported in the literature, two deaths occur in our study population. However, this finding should be confirmed in further large-scale studies, before we conclude that the use of aerosolized iloprost reduces the mortality rates.…”

Section: Discussionmentioning

confidence: 56%

Long-term results of surgical repair of congenital heart defects in children with severe pulmonary arterial hypertension treated with perioperative inhaled iloprost

Paç¹

2017

Turk Gogus Kalp Dama

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Long-term results of surgical repair of congenital heart defects in children with severe pulmonary arterial hypertension treated with perioperative inhaled iloprostPerioperatif inhale iloprost ile tedavi edilen ciddi pulmoner arteriyel hipertansiyonlu çocuklarda doğuştan kalp hastalıklarının cerrahi onarımının uzun dönem sonuçları ABSTRACTBackground: This study aims to evaluate the long-term outcomes of patients with pulmonary arterial hypertension associated with congenital heart disease treated with inhaled iloprost perioperatively.

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Pulmonary hypertension after operations for congenital heart disease: Analysis of risk factors and management

Abstract: Mixed venous saturation monitoring and alpha-receptor blockade reduced the incidence of pulmonary hypertension after operations for congenital heart disease. Early definitive repair reduced morbidity and mortality from postoperative pulmonary hypertension.

Cited by 163 publications

References 15 publications

Increased pulmonary blood flow does not alter surfactant protein gene expression in lambs within the first week of life

Increased pulmonary blood flow does not alter surfactant protein gene expression in lambs within the first week of life

Eisenmenger Syndrome: Not Always Inoperable

Long-term results of surgical repair of congenital heart defects in children with severe pulmonary arterial hypertension treated with perioperative inhaled iloprost

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