This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.
This is a preview of subscription content, log in to check access.
Buy single article
Instant unlimited access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Brann S, Brawn WJ, Faro R, Sreeram N (1995) Neonatal pulmonary vascular disease in hypoplastic left heart syndrome. Ann Thorac Surg 60:433–434
Canter CE, Moorhead S, Huddleston CB, Spray TL (1993) Restrictive atrial septal communication as a determinant of outcome of cardiac transplantation for hypoplastic left heart syndrome. Circulation 88(Suppl 2):456–460
Feinstein JA, Benson DW, Dubin AM et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59:1–42
France NE, Brown RJK (1971) Congenital pulmonary lymphangiectasis: report of 11 examples with specific reference to cardiovascular findings. Arch Dis Child 46:528–532
Glatz JA, Tabbutt S, Gaynor JW, Rome JJ, Montenegro L, Spray TL, Rychik J (2007) Hypoplastic left heart syndrome with atrial level restriction in the era of prenatal diagnosis. Ann Thorac Surg 84:1633–1638
Luciani G, Pessotto R, Mombello A, Mazzucco A (1999) Hypoplastic left heart syndrome with restrictive atrial septal defect and congenital pulmonary lymphangiectasis. Cardiovasc Pathol 8:49–51
Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL (1999) The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology, and outcome. J Am Coll Cardiol 34:554–560
Stasik C, Goldberg C, Bove E, Devaney E, Ohye R (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cariodvasc Surg 131:412–417
About this article
Cite this article
D’Souza, M., Vergales, J. & Anitha Jayakumar, K. Hypoplastic Left Heart Syndrome and Pulmonary Veno-occlusive Disease in an Infant. Pediatr Cardiol 34, 1949–1951 (2013). https://doi.org/10.1007/s00246-012-0498-y
- Hypoplastic left heart syndrome (HLHS)
- Total anomalous pulmonary venous return (TAPVR)
- Pulmonary veno-occlusive (PVOD)