Patients who are diagnosed with dextrocardia, a rare congenital heart condition in which the heart points toward the right side of the chest, need their specific situs classification (eg, solitus, inversus, ambiguus) ascertained to optimize their care and outcomes. In this report, we discuss the perioperative anesthetic management of a patient presenting with dextrocardia. A 44-year-old African American female with a history of hypertension, hyperlipidemia, gastroesophageal reflux disease, and diabetes mellitus type 2 was admitted for shortness of breath, dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. The patient had been diagnosed with dextrocardia in 2003 at an outside hospital and was asymptomatic prior to this presentation. Chest x-ray revealed bilateral perihilar vascular congestion with bibasilar atelectasis and suspected small bilateral pleural effusions consistent with new-onset congestive heart failure. Preoperative 2-dimensional transthoracic echocardiography revealed an ostium secundum-type atrial septal defect (ASD) with mild left-to-right atrial shunting. The patient's ASD was repaired using a pericardial patch. The anesthetic management of patients presenting with dextrocardia is complex. Preoperative cardiac transthoracic echocardiography can identify cardiac lesions or aberrant anatomy associated with dextrocardia. Proper placement of electrocardiogram electrodes is necessary to avoid false-positive results for perioperative ischemia. Central line access must be adjusted to anatomic variations. Clinicians should have high suspicion for associated pulmonary hypertension and should limit sedatives preoperatively to minimize the cardiovascular effects of hypoxia and/or hypercarbia on the pulmonary vasculature. Finally, high clinical suspicion for respiratory complications should be maintained, as dextrocardia has been associated with respiratory complications secondary to primary ciliary dyskinesia in approximately 25% of patients.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7993426 | PMC |
http://dx.doi.org/10.31486/toj.19.0119 | DOI Listing |
Mol Genet Genomic Med
January 2025
The State Key Laboratory for Complex Severe and Rare Diseases, the State Key Sci-Tech Infrastructure for Translational Medicine, Peking Union Medical College Hospital, Beijing, China.
Background: Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder characterized by dysfunction of motile cilia. While approximately 50 genes have been identified, around 25% of PCD patients remain genetically unexplained; elucidating the pathogenicity of specific variants remains a challenge.
Methods: Whole exome sequencing (WES) and Sanger sequencing were conducted to identify potential pathogenic variants of PCD.
Birth Defects Res
December 2024
National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
Background: Reports from China describe an increase in the frequency of fetal situs inversus in 2023 after the country's "zero-Covid" policy was lifted, suggesting an association with maternal SARS-CoV-2 infection. However, a report of birth defects surveillance data from Scandinavia observed no sustained increase during the SARS-CoV-2 pandemic (2020-2022 vs. 2018-2019).
View Article and Find Full Text PDFMedicina (Kaunas)
November 2024
Department of Nucleic Acid Biochemistry, Medical University of Lodz, 92-213 Lodz, Poland.
Primary ciliary dyskinesia (PCD) is a rare genetic disorder that affects the structure and function of cilia, primarily impacting the respiratory system. Kartagener syndrome, a subset of PCD, is characterized by situs inversus, bronchiectasis, and chronic sinusitis. Patients with PCD are prone to recurrent respiratory infections due to impaired ciliary function, which hinders effective mucus clearance and promotes pathogen colonization.
View Article and Find Full Text PDFJ Intern Med
January 2025
Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Background: Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous disease. Due to difficulty accessing diagnostic services and a lack of awareness of the syndrome, clinicians often fail to recognize the classic phenotype, leading to missed diagnoses.
Methods: Relevant medical records were accessed through The BIG DATA QUERY AND ANALYSIS SYSTEM of Peking Union Medical College Hospital from September 1, 2012 to March 31, 2024.
Sci Rep
November 2024
Pediatrics and Rare Diseases Group, Sanford Research, 2301 E. 60th St. N., Sioux Falls, Sioux Falls, SD, 57104, USA.
Dysfunction of motile cilia can impair mucociliary clearance in the airway and result in primary ciliary dyskinesia (PCD). We previously showed that mutations in central pair apparatus (CPA) genes perturb ciliary motility and result in PCD in mouse models. However, little is known about how epithelial cell types in the ciliary microenvironment of the upper airway respond to defects in ciliary motility and mucociliary clearance.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!