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Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Cyanotic congenital heart disease the coronary arterial circulation
1The Ahmanson/UCLA Adult Congenital Heart Disease Center, David Geffen School of Medicine at UCLA, Los Angeles, California, USA. josephperloff@earthlink.net
Insights
Cyanotic congenital heart disease (CCHD) causes coronary artery dilation and increased basal flow, but preserves flow reserve through microcirculation remodeling. Coronary arteries remain atheroma-free due to factors like hypoxemia and hypocholesterolemia.
Area of Science:
- Cardiovascular Physiology
- Pediatric Cardiology
- Vascular Biology
Background:
- Cyanotic congenital heart disease (CCHD) significantly impacts coronary circulation.
- Key components include extramural arteries, basal blood flow, flow reserve, microcirculation, and atherogenesis.
Purpose of the Study:
- To investigate the characteristics of coronary circulation in adults with CCHD.
- To analyze coronary artery structure, blood flow dynamics, and the presence of atherosclerosis.
Main Methods:
- Analysis of coronary arteriograms in 59 adults with CCHD.
- Histological examination of dilated coronary arteries.
- N-13 positron emission tomography for basal and hyperemic coronary blood flow assessment.
- Microcirculatory morphometric analysis using immunostaining.
- Cholesterol levels were retrieved and categorized based on cyanotic status and surgical intervention.
Main Results:
- Mild to moderate dilation (ectasia) of extramural coronary arteries observed in 49/59 patients.
- Histology revealed smooth muscle loss, increased collagen, and internal elastic lamina duplication.
- Basal coronary blood flow was increased, while hyperemic flow remained comparable to controls.
- Microcirculation remodeling, characterized by changes in arteriolar densities, was noted.
- Absence of coronary atherosclerosis in both arteriograms and necropsy specimens.
Conclusions:
- Extramural coronary arteries in CCHD dilate due to endothelial factors and mural attenuation.
- Increased basal flow with normal hyperemic flow suggests preserved flow reserve.
- Microcirculatory remodeling is crucial for maintaining adequate coronary blood flow.
- Atheroma-free coronaries are attributed to hypocholesterolemia, hypoxemia, nitric oxide, low platelets, and hyperbilirubinemia.
Background:
The coronary circulation in cyanotic congenital heart disease (CCHD) includes the extramural coronary arteries, basal coronary blood flow, flow reserve, the coronary microcirculation, and coronary atherogenesis.
Methods:
Coronary arteriograms were analyzed in 59 adults with CCHD. Dilated extramural coronaries were examined histologically in six patients. Basal coronary blood flow was determined with N-13 positron emission tomography in 14 patients and in 10 controls. Hyperemic flow was induced by intravenous dipyridamole pharmacologic stress. Immunostaining against SM alpha-actin permitted microcirculatory morphometric analysis. Non-fasting total cholesterols were retrieved in 279 patients divided into four groups: Group A---143 cyanotic unoperated, Group B---47 rendered acyanotic by reparative surgery, Group C---41 acyanotic unoperated, Group D---48 acyanotic before and after operation.
Results:
Extramural coronary arteries were mildly or moderately dilated to ectatic in 49/59 angiograms. Histologic examination disclosed loss of medial smooth muscle, increased medial collagen, and duplication of internal elastic lamina. Basal coronary flow was appreciably increased. Hyperemic flow was comparable to controls. Remodeling of the microcirculation was based upon coronary arteriolar length, volume and surface densities. Coronary atherosclerosis was absent in both the arteriograms and the necropsy specimens.
Conclusions:
Extramural coronary arteries in CCHD dilate in response to endothelial vasodilator substances supplemented by mural attenuation caused by medial abnormalities. Basal coronary flow was appreciably increased, but hyperemic flow was normal. Remodeling of the microcirculation was responsible for preservation of flow reserve. The coronaries were atheroma-free because of the salutory effects of hypocholesterolemia, hypoxemia, upregulated nitric oxide, low platelet counts, and hyperbilirubinrmia.
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