Catalina Vargas-Acevedo¹, Gareth J. Morgan¹, Rhynn Soderstrom², Richard Ing³, Nicholas Houska³, Jenny E. Zablah^1,*

Congenital Heart Disease, Vol.19, No.4, pp. 389-397, 2024, DOI:10.32604/chd.2024.055121 - 31 October 2024

Abstract Introduction: Pulmonary hemorrhage (PHm) is a life-threatening complication that can occur after catheter-based interventions in patients with pulmonary vein stenosis (PVS). Inhaled racemic epinephrine (iRE) and tranexamic acid (iTXA) have been used in other conditions, but a standardized approach in PVS has not been described. We aimed to describe the current management of PHm after PVS catheter-based interventions. Methods: We present a retrospective review of episodes of PHm from July 2022 to February 2024. PHm was defined as frank blood suctioned from the endotracheal tube including blood-tinged secretions and >3% decrease in saturations and/or ventilatory… More >

Samuel Menahem^1,2,*, Arvind Sehgal^3,4

Congenital Heart Disease, Vol.16, No.2, pp. 189-196, 2021, DOI:10.32604/CHD.2021.014903 - 26 January 2021

Abstract Most congenital heart disease (CHD) is readily recognisable in the newborn. Forewarned by previous fetal scanning, the presence of a murmur, tachypnoea, cyanosis and/or differential pulses and saturations all point to a cardiac abnormality. Yet serious heart disease may be missed on a fetal scan. There may be no murmur or clinical cyanosis, and tachypneoa may be attributed to non-cardiac causes. Tachypnoea on day 1 is usually non-cardiac except arising from ventricular failure or a large systemic arteriovenous fistula. A patent ductus arteriosus (PDA) may support either pulmonary or systemic duct dependent circulations. The initially… More >

Jason F. Goldberg¹, Craig L. Jensen², Rajesh Krishnamurthy³, Nidhy P. Varghese⁴, Henri Justino¹

Congenital Heart Disease, Vol.13, No.1, pp. 124-130, 2018, DOI:10.1111/chd.12537

Abstract Objective: We describe the long-term follow-up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices.
Design: Case report
Setting: Tertiary children’s hospital
Patient: Single child through ages 2- to 11-year old
Interventions: The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE-covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long-term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating More >

Aaron R. Prosnitz¹, Jane Leopold², Mira Irons³, Kathy Jenkins¹, Amy E. Roberts¹

Congenital Heart Disease, Vol.12, No.4, pp. 475-483, 2017, DOI:10.1111/chd.12471

Abstract Objective: To describe a group of children with co-incident pulmonary vein stenosis and SmithLemli-Opitz syndrome and to generate hypotheses as to the shared pathogenesis of these disorders.
Design: Retrospective case series.
Patients: Five subjects in a pulmonary vein stenosis cohort of 170 subjects were diagnosed with Smith-Lemli-Opitz syndrome soon after birth.
Results: All five cases were diagnosed with Smith-Lemli-Opitz syndrome within 6 weeks of life, with no family history of either disorder. All cases had pathologically elevated 7-dehydrocholesterol levels and two of the five cases had previously reported pathogenic 7-dehydrocholesterol reductase mutations. Smith-Lemli-Opitz syndrome severity scores ranged from mild… More >