Severe Bacterial Infection in Patients with Heterotaxy Syndrome

Chiu, Shuenn–Nan; Shao, Pei-Lan; Wang, Jou‐Kou; Chen, Hui‐Chi; Lin, Min; Chang, Luan‐Yin; Lu, Chun‐Yi; Lee, Ping–Ing; Huang, Li‐Min; Wu, Mei‐Hwan

doi:10.1016/j.jpeds.2013.08.051

Cited by 33 publications

(41 citation statements)

References 25 publications

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“…In our previous large cohort study, we demonstrated that community-acquired severe bacterial infection (SBI) is common among heterotaxy patients, and that the incidence is significantly higher than that among patients with other cause of complex CHD (6). We also found a patient experiencing multiple SBI episodes is common, indicating that some heterotaxy patients have a higher risk of infection than those of others (6). Therefore, developing diagnostic methods for identifying such highly susceptible patients is crucial.…”

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confidence: 97%

“…However, LAI patients also exhibit a higher rate of sepsis, which may be related to abnormal splenic function (8). In our previous large cohort study, we demonstrated that community-acquired severe bacterial infection (SBI) is common among heterotaxy patients, and that the incidence is significantly higher than that among patients with other cause of complex CHD (6). We also found a patient experiencing multiple SBI episodes is common, indicating that some heterotaxy patients have a higher risk of infection than those of others (6).…”

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confidence: 99%

“…Therefore, cardiac-and operation-related causes account for 60% of death in heterotaxy patients. Infection-related mortality is another major cause of death (6). Maldevelopment of the spleen is common in heterotaxy patients, and is an important cause of immunodeficiency in young children with heterotaxy (5)(6)(7)(8).…”

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confidence: 99%

“…Infection-related mortality is another major cause of death (6). Maldevelopment of the spleen is common in heterotaxy patients, and is an important cause of immunodeficiency in young children with heterotaxy (5)(6)(7)(8). The incidence of heterotaxy syndrome is 1-1.24% of CHD patients (9,10), and can be further divided into right atrial isomerisms (RAI) and left atrial isomerisms (LAI) according to the atrial appendage morphology.…”

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confidence: 99%

“…In RAI patients, agenesis of the spleen is common, occurring in approximately 75% of these patients (4). Fulminant-encapsulated bacterial infection (particularly pneumococcus and Hemophilus influenza) is a common complication in asplenic patients (5,6,8). For LAI, approximately 43% of patients present with polysplenia and 10% of them present with asplenia (4).…”

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confidence: 99%

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Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

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Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

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Multi-Systemic Consequences of CHD and the Impact on Perioperative Care

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Congenital Heart Disease in Pediatric and Adult Patients

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Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without

2016

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Isomerism, also known as heterotaxy, is a unique clinical entity in which there are mirror imaged findings in the thoracic. In the abdomen, the arrangement of organs can be random. The anatomic findings also have functional consequences. Isomerism has been known to increase morbidity and mortality in those with functionally univentricular hearts. The aim of this study was to determine the impact of isomerism on the Glenn hospitalization. Data from the 1997 to 2012 Kids' Inpatient Database were utilized for this cross-sectional study. Admissions during which a Glenn procedure was done were identified. Next, these admissions were separated into those with and without isomerism. Admission characteristics were then compared in a univariate fashion as well as by regression analysis. Length of hospitalization, cost of hospitalization, extracorporeal membrane oxygenation, and inpatient mortality were the outcomes of interest. A total of 4959 admissions with a Glenn procedure were identified. Of these, 450 were associated with isomerism. The median age at which the Glenn procedure was done was 10 and 13 months in those without and with isomerism, respectively. Neither univariate nor regression analysis demonstrated any significant difference in length of hospitalization, cost of hospitalization, need for extracorporeal membrane oxygenation, and inpatient mortality between those with and without isomerism. Isomerism does not impact the characteristics of the Glenn hospitalization. Those with isomerism did tend to be older when they underwent the Glenn procedure.

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Severe Bacterial Infection in Patients with Heterotaxy Syndrome

Cited by 33 publications

References 25 publications

Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

Low immunoglobulin M memory B-cell percentage in patients with heterotaxy syndrome correlates with the risk of severe bacterial infection

Multi-Systemic Consequences of CHD and the Impact on Perioperative Care

Characteristics of Hospitalizations for the Glenn Procedure in Those With Isomerism Compared to Those Without

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