mortality/aging
• lethality between E16.5 and 3 months with 100% penetrance
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cardiovascular system
• venous angiograms are abnormal with small-vessel pruning and absence of peripheral vascular blush
• however, mice show normal coronary artery/vein configuration and luminal diameter and coronary arteries and veins appear normal
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• venule intimal thickening is correlated with cellular proliferation
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• mice develop lethal pulmonary veno-occlusive disease, showing occlusion and luminal narrowing of postcapillary pulmonary venules
• pulmonary venule occlusion is widespread
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• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice
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• variable pulmonary capillary hemorrhage
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• postnatally, mice develop elevated right ventricular systolic pressures
• however, mice exhibit normal left ventricular systolic function, thickness, inner diameter, and fractional shortening
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• pulmonary hypertension from venous obstruction in mice that survive past 4 weeks
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• pulmonary venous endothelial cells (PVECs) show increased growth rates at perconfluence and increased proliferation
• however, pulmonary arterial endothelial cells (PAEC) growth rate and proliferation are normal
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hematopoietic system
pancytopenia
(
J:227456
)
homeostasis/metabolism
• mice challenged with the pulmonary vasodilator epoprostenol develop life-threatening pulmonary edema
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respiratory system
• variable pulmonary capillary hemorrhage
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• mice challenged with the pulmonary vasodilator epoprostenol develop life-threatening pulmonary edema
|
cellular
• pulmonary venous endothelial cells (PVECs) show increased growth rates at perconfluence and increased proliferation
• however, pulmonary arterial endothelial cells (PAEC) growth rate and proliferation are normal
|
muscle
• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice
|
growth/size/body
• ratio of right ventricle weight to left ventricle and septum is increased in 4 week old mice
|