Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction

Harden, Brandon W.; Tian, Xin; Giese, Rachel; Nakhleh, Nader; Kureshi, Safina; Francis, Richard; Hanumanthaiah, Sridhar; You, Li; Swisher, Matthew W; Kuehl, Karen S.; Sami, Iman; Olivier, Kenneth N.; Jonas, Richard A.; Lo, Cecilia; Leatherbury, Linda

doi:10.1016/j.jtcvs.2013.06.018

Cited by 53 publications

(54 citation statements)

References 19 publications

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“…59 Certain complex lesions such as heterotaxy syndrome have cilia dysmotility, increasing the risk of postoperative pneumonia and prolonged ventilation. [475][476][477] Many patients have had surgeries that intentionally sacrificed a subclavian artery (classic Blalock-ThomasTaussig shunt, subclavian flap repair of CoA). These patients often have spuriously low blood pressure in the affected arm.…”

Section: Clinical Statements and Guidelinesmentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

174

136

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ABSTRACT:Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

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Section: Clinical Statements and Guidelinesmentioning

confidence: 99%

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Lui¹,

Saïdi²,

Bhatt³

et al. 2017

Circulation

174

136

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“…Patients with situs anomalies should be referred for diagnostic testing if they have any additional features suggestive of PCD, for example, ear disease or wet cough. Respiratory symptoms in patients with complex cardiac disease associated with heterotaxy might erroneously be attributed to their cardiac condition; a very low threshold for referral for PCD diagnostic testing is important in this patient group, particularly as a diagnosis of PCD impacts on perioperative management and prognosis 15. The incidence of situs anomalies in the general population is approximately 1:10 000, but there have been no studies to determine how many of these people have PCD; a widely quoted estimate is 25%.…”

Section: Diagnosismentioning

confidence: 99%

Diagnosis and management of primary ciliary dyskinesia

Lucas

Burgess

Mitchison

et al. 2014

Archives of Disease in Childhood

217

249

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Primary ciliary dyskinesia (PCD) is an inherited autosomal-recessive disorder of motile cilia characterised by chronic lung disease, rhinosinusitis, hearing impairment and subfertility. Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable. Organ laterality defects, usually situs inversus, occur in ∼50% of cases. The estimated prevalence of PCD is up to ∼1 per 10 000 births, but it is more common in populations where consanguinity is common. This review examines who to refer for diagnostic testing. It describes the limitations surrounding diagnosis using currently available techniques and considers whether recent advances to genotype patients with PCD will lead to genetic testing and screening to aid diagnosis in the near future. It discusses the challenges of monitoring and treating respiratory and ENT disease in children with PCD.

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“…For example, PCD patients undergoing cardiac surgery are known to have an increased risk of post-operative pulmonary complications (pneumonia, atelectasis, failure to wean from a ventilator, etc). This risk can be minimized by ensuring optimization of pre-operative pulmonary function, aggressive post-operative pulmonary toilet and (when possible) early extubation following surgery (54). Similarly vaccination and the early use of appropriate antibiotics can lessen the severity of pulmonary infection in patients with PCD and potentially slow the decline in pulmonary function in these patients (3).…”

Section: Clinical Implications Of Genetic Causes Of Chdmentioning

confidence: 99%

The genetics of congenital heart disease… understanding and improving long-term outcomes in congenital heart disease: a review for the general cardiologist and primary care physician

Simmons¹,

Brueckner²

2017

Current Opinion in Pediatrics

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Purpose of the Review This review has two purposes: 1) To provide an updated review of the genetic causes of congenital heart disease (CHD) and the clinical implications of these genetic mutations; 2) To provide a clinical algorithm for clinicians considering a genetics evaluation of a CHD patient. Recent Findings A large portion of congenital heart disease is thought to have a significant genetic contribution, and at this time a genetic cause can be identified in approximately 35% of patients. Through the advances made possible by next generation sequencing, many of the comorbidities that are frequently seen in patients with genetic congenital heart disease patients can be attributed to the genetic mutation that caused the congenital heart disease. These comorbidities are both cardiac and non-cardiac and include: neurodevelopmental disability, pulmonary disease, heart failure, renal dysfunction, arrhythmia and an increased risk of malignancy. Identification of the genetic cause of congenital heart disease helps reduce patient morbidity and mortality by improving preventive and early intervention therapies to address these comorbidities. Summary Through an understanding of the clinical implications of the genetic underpinning of congenital heart disease, clinicians can provide care tailored to an individual patient and continue to improve the outcomes of congenital heart disease patients.

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Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction

Cited by 53 publications

References 19 publications

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

Diagnosis and management of primary ciliary dyskinesia

The genetics of congenital heart disease… understanding and improving long-term outcomes in congenital heart disease: a review for the general cardiologist and primary care physician

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