Respiratory Failure in Congenital Heart Disease

DiCarlo, Joseph; Steven, James M.

doi:10.1016/s0031-3955(16)38768-5

Cited by 23 publications

(5 citation statements)

References 61 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Concurrent with this decrease in blood flow around e14.5 are heart malformations including DORV and VSD as well as the presence of pericardial edema. Pulmonary edema is often seen as a classic sign of fetal cardiac abnormalities, often developing secondary to the effect of the malformation itself (31).…”

Section: Discussionmentioning

confidence: 99%

Deletion of the Aryl Hydrocarbon Receptor-associated Protein 9 Leads to Cardiac Malformation and Embryonic Lethality

Lin

Sullivan²,

Lee

et al. 2007

Journal of Biological Chemistry

View full text Add to dashboard Cite

The aryl hydrocarbon receptor-associated protein 9, ARA9 (also known as XAP2 or AIP1), is a chaperone that is found in complexes with certain xenobiotic receptors, such as the aryl hydrocarbon receptor (AHR) and the peroxisome proliferatoractivated receptor ␣ (PPAR␣). In an effort to better understand the physiological role of ARA9 outside of its role in xenobiotic signal transduction, we generated a null allele at the Ara9 locus in mice. Mice with a homozygous deletion of this gene die at various time points throughout embryonic development. Embryonic lethality is accompanied by decreased blood flow to head and limbs, as well as a range of heart deformations, including double outlet right ventricle, ventricular-septal defects, and pericardial edema. The early cardiovascular defects observed in Ara9-null mice suggest an essential role for the ARA9 protein in cardiac development. The observation that the developmental aberrations in Ara9-null mice are distinct from those observed for disrupted alleles at Ahr or Ppar␣ indicates that the role of ARA9 in cardiac development is independent of its interactions with its known xenobiotic receptor partners.The aryl hydrocarbon receptor-associated protein 9 (ARA9, 2 also known as AIP1) is found in association with two mammalian client proteins, the aryl hydrocarbon receptor (AHR) and the peroxisome proliferator-activated receptor ␣ (PPAR␣) (1-4). The ARA9 protein has also been characterized in association with the hepatitis-B virus X-protein, HBVx. As the result of its interaction with this viral protein, it is also known by the name hepatitis B virus X-associated protein or XAP2 (5).The ARA9 protein is structurally related to the immunophilin family of proteins, harboring an N-terminal FK506 binding (FKBP) domain and C-terminal tetratricopeptide repeats (TPRs) (1,3,6). The FKBP domains of many immunophilin proteins possess peptidyl-prolyl cis-trans isomerase (PPIase) activity that can be blocked by the binding of immunosuppressants such as FK506 (7,8). Despite the sequence similarity between the N terminus of ARA9 and the FKBP domains of other immunophilins, ARA9 does not appear to possess isomerase activity or affinity for FK506 (1). We postulate that the structural similarity of ARA9 to the immunophilin family is a reflection of a shared capacity to act as a cellular chaperone, and play a role in the folding and localization of client proteins.The ARA9 protein has been extensively studied in association with the AHR. As a chaperone, ARA9 maintains the AHR in a cytosolic localization, decreases AHR degradation, and increases its ligand binding capacity (9 -11). Although little is known regarding the functional role of ARA9 in PPAR␣ and HBVx function, initial data are consistent with ARA9 acting as a chaperone for these proteins as well (4, 5). We hypothesize that in addition to xenobiotic signaling, the ARA9 protein is also a common chaperone to many proteins involved in a variety of essential cellular functions. This idea is supported by results from whole mount in ...

show abstract

Section: Discussionmentioning

confidence: 99%

Deletion of the Aryl Hydrocarbon Receptor-associated Protein 9 Leads to Cardiac Malformation and Embryonic Lethality

Lin

Sullivan²,

Lee

et al. 2007

Journal of Biological Chemistry

View full text Add to dashboard Cite

show abstract

“…A left-to-right shunt can adversely affect lung function, 2 and superimposed lower respiratory tract infections cause additional compromise and might lead to respiratory failure, necessitating mechanical ventilation. 3 Malnutrition and delayed presentation in the developing world further compound disease severity. 4 Once mechanical ventilation is initiated, it is often difficult to wean and extubate these From the Departments of Pediatric Cardiology, a Anesthesiology,…”

mentioning

confidence: 99%

Management of infants with large, unrepaired ventricular septal defects and respiratory infection requiring mechanical ventilation

Bhatt

Roth

Kumar

et al. 2004

The Journal of Thoracic and Cardiovascular Surgery

View full text Add to dashboard Cite

show abstract

“…When CHD children underwent one or two cardiac surgery procedures, the risk of having an impaired functional residual capacity of the lungs was increased of 5.4 and 12.9 folds, respectively. In addition, high Qp lungs were stiffer than normal, while the elasticity of the low Qp lungs was increased [ 8 , 10 , 30 ]. Lanteri et al observed an improvement in lung compliances and resistances among children with increased Qp after cardiac surgery that were not detected in children with normal or decreased Qp [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Effect of preoperative pulmonary hemodynamic and cardiopulmonary bypass on lung function in children with congenital heart disease

et al. 2023

View full text Add to dashboard Cite

In children with congenital heart disease (CHD), pulmonary blood flow (Qp) contributes to alterations of pulmonary mechanics and gas exchange, while cardiopulmonary bypass (CPB) induces lung edema. We aimed to determine the effect of hemodynamics on lung function and lung epithelial lining fluid (ELF) biomarkers in biventricular CHD children undergoing CPB. CHD children were classified as high Qp (n = 43) and low Qp (n = 17), according to preoperative cardiac morphology and arterial oxygen saturation. We measured ELF surfactant protein B (SP-B) and myeloperoxidase activity (MPO) as indexes of lung inflammation and ELF albumin as index of alveolar capillary leak in tracheal aspirate (TA) samples collected before surgery and in 6 hourly intervals within 24 h after surgery. At the same time points, we recorded dynamic compliance and oxygenation index (OI). The same biomarkers were measured in TA samples collected from 16 infants with no cardiorespiratory diseases at the time of endotracheal intubation for elective surgery. Preoperative ELF biomarkers in CHD children were significantly increased than those found in controls. In the high Qp, ELF MPO and SP-B peaked 6 h after surgery and tended to decrease afterward, while they tended to increase within the first 24 h in the low Qp. ELF albumin peaked 6 h after surgery and decreased afterwards in both CHD groups. Dynamic compliance/kg and OI significantly improved after surgery only in the High Qp. Conclusion: In CHD children, lung mechanics, OI, and ELF biomarkers were significantly affected by CPB, according to the preoperative pulmonary hemodynamics. What is Known:• Congenital heart disease children, before cardiopulmonary run, exhibit changes in respiratory mechanics, gas exchange, and lung inflammatory biomarkers that are related to the preoperative pulmonary hemodynamics.• Cardiopulmonary bypass induces alteration of lung function and epithelial lining fluid biomarkers according to preoperative hemodynamics. What is New:• Our findings can help to identify children with congenital heart disease at high risk of postoperative lung injury who may benefit of tailored intensive care strategies, such as non-invasive ventilation techniques, fluid management, and anti-inflammatory drugs that can improve cardiopulmonary interaction in the perioperative period.

show abstract

Respiratory Failure in Congenital Heart Disease

Cited by 23 publications

References 61 publications

Deletion of the Aryl Hydrocarbon Receptor-associated Protein 9 Leads to Cardiac Malformation and Embryonic Lethality

Deletion of the Aryl Hydrocarbon Receptor-associated Protein 9 Leads to Cardiac Malformation and Embryonic Lethality

Management of infants with large, unrepaired ventricular septal defects and respiratory infection requiring mechanical ventilation

Effect of preoperative pulmonary hemodynamic and cardiopulmonary bypass on lung function in children with congenital heart disease

Contact Info

Product

Resources

About