Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental abnormality of the lower respiratory tract, primarily diagnosed in the neonatal period. The most concerning sequelae for patients with CPAM are recurrent respiratory infections and malignancy. Rarely discovered in asymptomatic adults, CPAM presents challenging questions for management. We describe such a case and discuss the risks and benefits of resection.
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http://dx.doi.org/10.1016/j.rmcr.2018.10.009 | DOI Listing |
We present a clinical observation of an 18-year-old female patient with congenital bronchiectasis combined with congenital cystic degeneration of the upper lobes of both lungs, Williams-Campbell syndrome, long-COVID, severe course. The patient was treated in infectious disease department (three times), with subsequent transfer to pulmonology department of Kursk Regional Multi-Purpose Clinical Hospital from 31.01.
View Article and Find Full Text PDFEur J Hum Genet
December 2024
National EDS Service, London North West University Healthcare NHS Trust, London, UK.
Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder predominantly caused by pathogenic COL3A1 variants. Characteristic arterial and intestinal fragility and generalised severe tissue friability can lead to clinical events from childhood. We highlight a paucity of literature regarding children diagnosed with vEDS, possibly explained by a restraint in predictive testing, and present data on 63 individuals (23 index cases) with a clinical and genetic diagnosis of vEDS in childhood (<18 years) to address this.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
Department of Anesthesiology, University Children's Hospital, 30-663 Krakow, Poland.
Background: Major aortopulmonary collateral arteries (MAPCAs) are rare remnants of pulmonary circulation embryological development usually associated with complex congenital anomalies of the right ventricular outflow tract and pulmonary arteries. Effective management requires surgical unifocalization of MAPCAs and native pulmonary arteries (NPAs). Traditional imaging may lack the spatial clarity needed for precise surgical planning.
View Article and Find Full Text PDFJ Cardiovasc Dev Dis
December 2024
Laboratory of X-Ray Endovascular and Reconstructive Cardiovascular Surgery, Department of Cardiovascular Surgery, Federal State Budgetary Institution "Research Institute for Complex Issues of Cardiovascular Diseases", Blvd. Named After Academician L.S. Barbarasha, 6, 650002 Kemerovo, Russia.
The Purpose: Evaluation of the short-term and long-term results of a phased correction of the tetralogy of Fallot (ToF) with stenting of the right ventricular outflow tract (RVOT) in comparison with a one-stage total correction (TC) of the defect.
Materials And Methods: Two groups of patients with classical ToF were formed. Group 1 (n = 25; median age = 72 days) was initially represented by children with ToF with a more severe clinical status (median weight = 3.
J Cardiovasc Dev Dis
December 2024
Cardiology Department, Mother and Child Health Institute of Serbia, 11070 Belgrade, Serbia.
Background: The vertical vein (VV) ligation during the total anomalous pulmonary venous return (TAPVR) correction is still controversial. Our study aimed to define the potential risk factors for VV persistence and their percutaneous occlusion.
Methods: The retrospective cohort study included 40 patients (26 males) with TAPVR treated at the tertiary referral center from 2005 to 2024.
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