Jyothsna Akam Venkata1,2, Mohamed Abdelghafar Hussein1,3, Joshua Greer1, Robert Jaquiss4, Gerald Greil1, Jeanne Dillenbeck1, Surendrenath R Veeram Reddy 1, Jenifer Hernandez1, and Tarique Hussain1
1Dept of Pediatrics, Division of Pediatric Cardiology, UT Southwestern Medical Center, Dallas, TX, United States, 2University of Mississippi Medical Center, Jackson, MS, United States, 3Kafrelsheikh University, Kafr Elsheikh, Egypt, 4Department of Pediatric Cardiothoracic Surgery, UT Southwestern Medical Center, Dallas, TX, United States
Synopsis
Pre-Fontan cardiac
assessment with CMR in conjunction with cardiac catheterization has
increasingly become the standard of care. Cardiac catheterization and
CMR performed for pre-Fontan assessment at a single institution between
April 2017 and June 2020 were analyzed. Our cohort consisted
of 43 patients with median age of 3.7 years(range 1.8-14) at the
time of CMR. Median age at Fontan operation was 4 years (2.4-14). We found significant
correlation between CMR derived ventricular end diastolic, end systolic
volume, ejection fraction and cardiac catheterization derived ventricular end-diastolic and mean superior vena cava pressure, and duration of post-operative
hospital stay after Fontan operation.
Introduction
Pre-Fontan cardiac
assessment with CMR in conjunction with cardiac catheterization has
increasingly become the standard of care. CMR is increasingly comprehensive including
assessment of systemic ventricular volumes, systolic function, anatomy, atrioventricular
valve regurgitation (AVVR), branch pulmonary artery anatomy with flow
distribution, lymphatic system abnormalities, burden of collateral blood flow
and sternal adhesions. We sought to evaluate the incremental value of novel CMR
evaluation for post-operative Fontan outcomes.Methods
Cardiac catheterization
and CMR exams performed for pre-Fontan assessment at a single institution between
April 2017 and June 2020 were analyzed. Sequences of CMR included (i) balanced 2D Steady-state free precision
cine imaging to calculate ventricular end-diastolic, end-systolic volume, ejection
fraction, (ii) phase-contrast imaging to estimate flow to the pulmonary and
systemic circulation (Qp:Qs), (iii) T2 weighted turbo-spin echo sequence to
assess the lymphatic system1 (iv) 3D-SSFP (v) 3D-black blood VISTA to assess the
anatomy of the pulmonary arteries, aorta, systemic and pulmonary veins, and (vi)
real-time cine with free-breathing to assess for retro-sternal adhesions.
Lymphatic abnormality was classified into grades 1-4 according to Dori et al2.
Maldistribution of pulmonary blood flow was classified as flow discrepancy greater
than 70: 30% to both the lungs. Pulmonary vascular resistance (PVR) was
calculated from cardiac catheterization derived transpulmonary gradient divided
by phase-contrast derived pulmonary blood flow. Atrioventricular valve
regurgitation was estimated as the ratio of (systemic ventricular stroke volume-
aortic or neo-aortic valve forward flow)/ventricular stroke volume3.
Surgical details and the immediate post-operative outcomes such as adverse
outcomes, duration of hospital stay, chylous effusion, re-admissions were
reviewed. Adverse outcomes included cardiac arrest, extracorporeal membranous
oxygenation support, the takedown of Fontan circulation, mechanical support (VAD-ventilator
assist device), heart transplantation, or death. CMR and hemodynamic parameters
were compared between groups with adverse and favorable outcomes. The study was
approved by the institutional review board. SPSS v.22 was used for Statistical
analysis.Results
A total of 56 CMR exams
were performed for pre-Fontan assessment, of which 13 patients are awaiting Fontan
operation. Our final cohort consisted of 43 patients (29 males) with a median age
of 3.7 years (range 1.8-14) at the time of CMR. The median age at Fontan operation
was 4 years (2.4-14). 40 patients underwent extra-cardiac conduit placement (39
fenestrated). 2 Patients underwent biventricular conversion. 6 patients had
adverse outcomes (including 2 undergoing transplantation or VAD prior to
Fontan). Pre-Fontan systemic ventricular ejection fraction (EF), (r=-0.56, p=0.004), ventricular end diastolic
volume (EDV) (r=+0.74,p<0.001), end systolic volume(ESV) (r=+0.8,
p<0.001), systemic ventricular end-diastolic pressure (EDP) (r=+0.611,
p<0.002) and mean superior vena cava (SVC) pressure (r=+0.69, p<0.001)
significantly correlated with duration of post-Fontan hospital stay. Patients
with adverse outcomes had a tendency for higher collateral blood flow (median
22.5%, range 3-40 vs 8.5%,0-27, p-0.06) but no factors significantly predicted
adverse outcome. Factors such as AVVR, aortic arch patency, the ratio of pulmonary
to systemic arterial blood flow (Qp: Qs), maldistribution of pulmonary blood
flow, pulmonary vascular resistance (PVR), the grade of lymphatic abnormality were not
predictive of the length of postoperative stay. There was no significant
correlation between lymphatic classification and post-operative chylous
effusion (p=0.786) or readmission (p=0.277). Grade 4 (n=1) lymphatic
abnormality was associated with death after Fontan. Retrosternal adhesions were
present in 87% of the patients.Discussion
This CMR approach
provides a comprehensive assessment of the single ventricle physiology and
anatomy in combination with cardiac catheterization data to assess ventricular
function, branch pulmonary artery anatomy, flow distribution, lymphatic
abnormalities, retro-sternal adhesions, and bleeding risk in the pre-operative
Fontan assessment. We found a significant correlation between the CMR derived
ventricular end-diastolic, end-systolic volume, ejection fraction, and cardiac
catheterization derived ventricular end-diastolic pressure and mean superior
vena cava pressure and duration of postoperative hospital stay after Fontan
operation. Those with adverse outcomes tended to have a higher collateral burden
compared to those with favorable outcomes. We did not find significant
correlation between the ratio of pulmonary to systemic blood flow (Qp: Qs),
maldistribution of pulmonary blood flow, pulmonary vascular resistance, the grade of lymphatic abnormality, AV valve regurgitation, and postoperative
outcomes. Conclusion
Pre-Fontan CMR derived systemic ventricular EF,
ventricular EDV and ESV along with cardiac catheterization derived ventricular EDP
and mean SVC pressure are useful measures
in predicting the duration of post-Fontan hospital stay.Acknowledgements
No acknowledgement found.References
- Pamarthi
V, Pabon-Ramos WM, Marnell V, Hurwitz LM. MRI of the Central Lymphatic System:
Indications, Imaging Technique, and Pre-Procedural Planning. Top Magn Reson
Imaging. 2017 Aug;26(4):175-180. doi: 10.1097/RMR.0000000000000130. PMID:
28665889; PMCID: PMC5548502.
- Biko
DM, DeWitt AG, Pinto EM, Morrison RE, Johnstone JA, Griffis H, O'Byrne ML,
Fogel MA, Harris MA, Partington SL, Whitehead KK, Saul D, Goldberg DJ, Rychik
J, Glatz AC, Gillespie MJ, Rome JJ, Dori Y. MRI Evaluation of Lymphatic
Abnormalities in the Neck and Thorax after Fontan Surgery: Relationship with
Outcome. Radiology. 2019 Jun;291(3):774-780. doi: 10.1148/radiol.2019180877.
Epub 2019 Apr 2. PMID: 30938628; PMCID: PMC6542623.
- Garg
P, Swift AJ, Zhong L, Carlhäll CJ, Ebbers T, Westenberg J, Hope MD,
Bucciarelli-Ducci C, Bax JJ, Myerson SG. Assessment of mitral valve
regurgitation by cardiovascular magnetic resonance imaging. Nat Rev Cardiol.
2020 May;17(5):298-312. doi: 10.1038/s41569-019-0305-z. Epub 2019 Dec 9. PMID:
31819230; PMCID: PMC7165127.