Segmental Pulmonary Arterial Disease

Characterization of an induced pluripotent stem cell line NCHi011-A from a 23-year-old female with Alagille Syndrome harboring a heterozygous JAG1 pathogenic variant

Isaac Stanberry, David Cunningham, Shiqiao Yea, Matthew Alonzo, Ming-Tao Zhao, Vidu Garg, Brenda LillyNationwide Children’s Hospital and Ohio State University College of Medicine.United States Stem Cell ResearchStem Cell Res 2023; 72:DOI: 10.1016/j.scr.2023.103213 AbstractAlagille syndrome (ALGS) is a multisystem disease with high variability in clinical features. ALGS is predominantly caused by pathogenic variants in the Notch ligand […]

Characterization of an induced pluripotent stem cell line NCHi011-A from a 23-year-old female with Alagille Syndrome harboring a heterozygous JAG1 pathogenic variant Read More »

Novel use of the double kissing crush technique to stent complex pulmonary artery stenosis in a child with Alagille syndrome

Danielle D. Strah, Riley D. Hellinger, Kwan S. Lee, Michael D. SeckelerUniversity of Arizona. Rady Children’s Hospital.United States Annals of Pediatric CardiologyAnn Pediatr Cardiol 2023; 6: 290-292DOI: 10.4103/apc.apc_60_23 AbstractCoronary bifurcation lesions and treatment with two-stent techniques have been developed, including the double kissing (DK) crush technique. The use of this technique in children or noncoronary vessels,

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Successful hybrid management of hypertensive patent ductus arteriosus with unilateral absence of the right pulmonary artery – A case report

Jayitri Mazumder, Debasree Gangopadhyay, Mahua Roy, Anoop Singh, Somrita Laha, Joyeeta DuttaRabindranath Tagore International Institute of Cardiac Sciences.India Annals of Pediatric CardiologyAnn Pediatr Cardiol 2023; 16: 293-296DOI: 10.4103/apc.apc_54_23 AbstractUnilateral absence of pulmonary artery (UAPA) can be either isolated or associated with other congenital cardiac defects. It is a rare congenital lesion with a diverse clinical presentation.

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Effective control of refractory pulmonary hypertension with iloprost inhalation in an infant with congenital absence of the right pulmonary artery: a case report

Chu=Yuan Hsiao, Wen-Hsien LuKaohsiung Veterans General Hospital. Fooyin University. National Sun Yat-sen University.Taiwan Cardiology in the YoungCardiol Young 2024;DOI: 10.1017/S104795112300450X AbstractUnilateral absence of the pulmonary artery is a rare congenital cardiovascular anomaly that can lead to pulmonary hypertension and poor outcomes. We report the case of a 1-month-old infant with isolated unilateral absence of the pulmonary

Effective control of refractory pulmonary hypertension with iloprost inhalation in an infant with congenital absence of the right pulmonary artery: a case report Read More »

Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review

Lauren Marie Kucirka, Shivani Desai, Barbara LeVarge, Jamie L. Conklin, M. Elisabeth Leong, Johanna Quist-Nelsonniversity of North Carolina at Chapel Hill School of Medicine.United States American Journal of PerinatologyAm J Perinatol 2023; DOI: 10.1055/a-2217-0241 AbstractObjective: Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation associated with hemoptysis, pulmonary hypertension, and infection. Little is known about the impact on pregnancy

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The Hamman-Rich syndrome in childhood; report of a case with unilateral pulmonary arterial and venous stenosis and atriovenous occlusion

Israel DiamondChildren’s Hospital and University of Louisville School of MedicineUnited States PediatricsPediatrics 1958; 22: 279–288https://doi.org/10.1542/peds.22.2.279 AbstractThe Hamman-Rich syndrome is described in a 4-year-old Negro male. The clinical picture was that of persistent cough and progressive dyspnea beginning at 4 months of age. Diagnosis was made ante mortem by lung biopsy. The fibrotic process and arteriolosclerosis

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Intraoperative and percutaneous stenting of congenital pulmonary artery and vein stenosis

Alan M. Mendelsohn, Edward L. Bove, Flavian M. Lupinetti, Dennis C. Crowley, Thomas R. Lloyd, Raymond T. Fedderly, Robert H. Beekman IIIUniversity of Michigan Medical Center.United States CirculationCirculation 1993; 88: II210-II217DOI: Not Available AbstractBackground: Conventional surgical or balloon dilation therapy for pulmonary artery or vein stenosis has been unsatisfactory in many patients. Balloon-expandable stents offer a

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Percutaneous pulmonary artery and vein stenting: a novel treatment for mediastinal fibrosis

Thomas P. Doyle, James E. Loyd, Ivan M. RobbinsVanderbilt University School of Medicine.United States American Journal of Respiratory and Critical Care MedicineAm J Repir Crit Care Med 2001; 164: 657-660DOI: 10.1164/ajrccm.164.4.2012132 AbstractMediastinal fibrosis is a rare consequence of infection with the fungus Histoplasma capsulatum that can lead to occlusion of large pulmonary arteries and veins and

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Case report: Unilateral pulmonary artery agenesis and Kommerell’s diverticulum in 1-year old girl

Valdone Miseviciene, Gintare Liakaite, Jurgita Zaveckiene, Ausra SnipaitieneLithuanian University of Health Sciences.Lithuania Frontiers in PediatricsFront Pediatr 2023; 11DOI: 10.3389/fped.2023.1212341 AbstractBackground: Unilateral pulmonary artery agenesis (UPAA) and Kommerell’s diverticulum (KD) are two rare embryologically unrelated congenital vascular malformations rarely diagnosed in children. This is the first report of our knowledge of the unique combination for a child as

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Staged Hybrid Approach for Ductal Origin of the Pulmonary Artery in a Low Birth Weight Neonate

Sudesh Prabhu, Shreesha Maiya, Riyan Shetty, Keshava Murthy, Prakash RamachandraNarayana Institute of Cardiac Sciences.India World Journal of Pediatric and Congenital Heart SurgeryWorld J Pediatr Congenit Heart Surg 2021; 12: 429-432DOI: 10.1177/2150135118822302 AbstractEarly recruitment of a discontinuous branch pulmonary artery will give the best long-term outcome for congenitally disconnected pulmonary arteries. This may be achieved using a

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