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Localized cystic lung lesions in pediatric patients encompass a spectrum of benign and rare malignant conditions that are quite distinct from cystic lung disease arising in adulthood. The majority have historically fallen under the diagnostic category of "congenital pulmonary airway malformation," a term that has been used to denote a diverse group of diseases ranging in etiology from ectopia to bronchial atresia to mosaic oncogenic mutation or neoplasia. This article reviews the clinical characteristics, gross and histologic features, and pathogenetic underpinnings of congenital pulmonary airway malformation as well as lesions that enter its histologic differential diagnosis.

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Fetal Characteristics and Perinatal Outcomes in Tetralogy of Fallot Without a Ductus Arteriosus.

Pediatr Cardiol

December 2024

Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Lucile Packard Children's Hospital, Palo Alto, CA, USA.

Absence of the ductus arteriosus (DA) is common in tetralogy of Fallot (TOF), occurring in up to 30% of cases. Yet, the clinical course and fetal echocardiographic features are not well described, limiting prenatal counseling. This study examines the fetal echocardiographic characteristics and perinatal outcomes in children with TOF absent DA (TOF/ADA), comparing them to those with a DA (TOF/DA).

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Extended Rhythm Monitoring to Assess for Ventricular Arrhythmias After Transcatheter Pulmonary Valve Replacement With the Harmony Valve.

Circ Cardiovasc Interv

December 2024

Department of Pediatrics, Pediatric Cardiology, Stanford University, Palo Alto, CA. (J.K.Y., L.W., A.C.T., H.C., A.W.R., L.F.P., S.R.C., A.M.D., D.B.M.).

Background: Varying rates of nonsustained ventricular tachycardia (NSVT) have been reported early after transcatheter pulmonary valve replacement (TPVR) with the Harmony valve, but data regarding rhythm outcomes beyond hospital discharge are limited. This study aims to characterize ventricular arrhythmias after Harmony TPVR from implant through mid-term follow-up.

Methods: Ventricular arrhythmia data from postimplant telemetry and follow-up extended rhythm monitoring (ERM) were analyzed after Harmony TPVR.

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Background: In patients with adult congenital heart disease (ACHD), significant atrioventricular valve regurgitation is an important risk factor for poor outcomes, such as heart failure. However, in many cases, transcatheter intervention may reduce the risk profile to avoid a high surgical risk.

Case Summary: A 44-year-old man with complex ACHD in the form of a double-inlet left ventricle, congenitally corrected transposition of the great arteries, pulmonary atresia, atrial septal defect, and patent ductus arteriosus was referred for the treatment of severe tricuspid regurgitation.

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Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly characterized by rudimentary PV tissue with variable degrees of PV stenosis and regurgitant pulmonary blood flow. In most cases, it is associated with tetralogy of Fallot. In a minority of APVS cases, with an unknown frequency, intact ventricular septum (IVS), patent ductus arteriosus, and possible tricuspid atresia are present.

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