PMID- 11178633
OWN - NLM
STAT- MEDLINE
DCOM- 20010412
LR  - 20191104
IS  - 1093-5266 (Print)
IS  - 1093-5266 (Linking)
VI  - 4
IP  - 2
DP  - 2001 Mar-Apr
TI  - Congenital alveolar capillary dysplasia with misalignment of pulmonary veins 
      associated with hypoplastic left heart syndrome.
PG  - 167-74
AB  - Three full-term infants died in the first month of life with hypoplastic left 
      heart syndrome (HLH) and persistent pulmonary hypertension (PPH). At postmortem 
      examination, they were found to have alveolar capillary dysplasia with 
      misalignment of pulmonary veins (ACD with MPV). The association of HLH syndrome, 
      and ACD with MPV with intestinal malrotation and/or obstruction, is unique. 
      Decreased blood flow in the ascending aorta in fetuses with left outflow tract 
      obstruction might cause vasoconstriction of pulmonary arterioles to maintain 
      cerebral perfusion. Vasoconstriction early during embryogenesis might lead to 
      decreased growth and development of alveolar capillaries and pulmonary veins. 
      This results in pulmonary hypertension, and the arterial blood is forced to 
      bypass the deficient capillary bed and can drain only via the anomalous bronchial 
      veins.
FAU - Rabah, R
AU  - Rabah R
AD  - Department of Pathology, Children's Hospital of Michigan and Wayne State 
      University School of Medicine, Detroit 48201-2196, USA.
FAU - Poulik, J M
AU  - Poulik JM
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Pediatr Dev Pathol
JT  - Pediatric and developmental pathology : the official journal of the Society for 
      Pediatric Pathology and the Paediatric Pathology Society
JID - 9809673
SB  - IM
MH  - Capillaries/pathology
MH  - Fatal Outcome
MH  - Female
MH  - Gestational Age
MH  - Humans
MH  - Hypertension, Pulmonary/congenital/pathology
MH  - Hypoplastic Left Heart Syndrome/*pathology
MH  - Infant, Newborn
MH  - Intestinal Obstruction/congenital/pathology
MH  - Male
MH  - Pulmonary Alveoli/blood supply/*pathology
MH  - Pulmonary Veins/*abnormalities/*pathology
RF  - 34
EDAT- 2001/02/15 11:00
MHDA- 2001/04/17 10:01
CRDT- 2001/02/15 11:00
PHST- 2001/02/15 11:00 [pubmed]
PHST- 2001/04/17 10:01 [medline]
PHST- 2001/02/15 11:00 [entrez]
AID - 10.1007/s100240010125 [pii]
AID - 10.1007/s100240010125 [doi]
PST - ppublish
SO  - Pediatr Dev Pathol. 2001 Mar-Apr;4(2):167-74. doi: 10.1007/s100240010125.