Congenital Heart Disease
Petter Frieberg, MD, MSc
Physician
Lund University, Skåne University Hospital, Lund, Sweden
Lund, Skane Lan, Sweden
Petter Frieberg, MD, MSc
Physician
Lund University, Skåne University Hospital, Lund, Sweden
Lund, Skane Lan, Sweden
Erik Hedström, MD, PhD
Associate professor, consultant
Lund University, Skåne University Hospital, Lund, Sweden
Lund, Sweden
Petru Liuba, MD, PhD
Associate professor
Lund University, Skåne University Hospital, Lund, Sweden
Lund, Sweden
Marcus Carlsson, MD, PhD
Professor, Head of Department
Karolinska Institute, Clinical Physiology, United States
CMR data from eighteen Fontan patients with total cavo-pulmonary connection (age 3–14 years, 8 females) was used to measure the ratio of right-to-left pulmonary vein flow, and to create computational fluid dynamics (CFD) models used to calculate the fraction of hepatic blood to the left lung. CFD was also used to calculate the patient-specific left-to-right ratio of pulmonary vascular resistance (PVR) required to match the observed pulmonary artery flow distribution.
In a simplified circuit with pulmonary flow and PVR, the fraction of right-to-left pulmonary vein flow was expected to be proportional to the left-to-right fraction of PVR (Figure 1).
Peripheral oxygen saturation at rest at the time of CMR examination, and diagnosis of PAVMs were noted. The fraction of hepatic blood to the left lung was correlated to the right-to-left fraction of pulmonary vein flow and to the left-to-right fraction of PVR using log/linear regression. The fraction of hepatic blood to the left lung was also correlated to peripheral oxygen saturation at rest using quadratic regression to capture low saturation at the extreme ends of the hepatic flow distribution.
Results: In the studied cohort, a strong relationship was found between the hepatic flow distribution and the right-to-left pulmonary vein flow ratio (r = 0.74; p< 0.001). Also, a strong relationship was found between the hepatic flow distribution and the left-to-right ratio of PVR (r = 0.73; p< 0.001). This also demonstrated the association between pulmonary vein flow and PVR as shown in Figure 1. A strong correlation was also found between the hepatic flow distribution and peripheral oxygen saturation at rest (r = 0.75; p< 0.001). Only two of the patients were diagnosed with PAVMs.
Conclusion:
The distribution of hepatic blood to the lungs has a continuous effect on the pulmonary blood flow, PVR and oxygen saturation, even without clinical manifestations of PAVM’s. These findings may help predict changes in these parameters during planning of surgical or endovascular interventions affecting the hepatic flow distribution in Fontan patients. CMR combined with CFD comprises a powerful tool to discover pathophysiological relationships.