Multiple Coronary Artery/Ventricular Microfistulas: A Late Complication of Fontan Surgery?
Bibliographic record
Abstract
A 16-year-old girl with a history of Fontan surgery for single right ventricle, common atrioventricular valve, common atrium, and pulmonary stenosis at 4 years of age was admitted because of Fontan circulatory failure (FCF). Angiography performed at 9 years of age revealed left coronary artery tortuosity and coronary artery/ventricular microfistulas (CAMFs) with a hypoplastic right coronary artery and an ejection fraction of 53% (Fig. 1A ; Video 1 , view video online). During treatment of FCF, she suffered a brain hemorrhage caused by a dural arteriovenous fistula of the transverse–sigmoid sinus. Despite extensive treatment, she died of sepsis. An autopsy revealed multiple dilated blood vessels with unique undulating shapes surrounded by sparse fibrosis, both grossly and histologically, within the ventricular myocardium from the subendocardium to the midwall (Fig. 1B-E), with an opening into the ventricular chamber (Fig. 1F). Vessel diameter varied from several hundred micrometers to 1 millimeter (approximately the same size as small arteries or veins); however, smooth muscle cells and elastic fibers were sparse in the vessel walls (Supplemental Fig. S1, A-D). Immunohistochemically, the vessels were lined by CD31+ endothelial cells, and were negative for D2-40 (Supplemental Fig. S1, E,F). These pathological features were consistent with those of residual sinusoids, which cause CAMFs. The prognosis of children with congenital heart disease has dramatically improved in recent years owing to advances in medical and surgical treatments. At present, the management of late complications in patients surviving until adulthood is a major concern. Several cases of CAMFs in univentricular circulation, with or without history of Fontan surgery, have been reported; however, their frequency and pathogenesis remain unclear.1Baffa J.M. Chen S.L. Guttenberg M.E. Norwood W.I. Weinberg P.M. Coronary artery abnormalities in right ventricular histology in hypoplastic left heart.J Am Coll Cardiol. 1992; 20: 350-358Crossref PubMed Scopus (79) Google Scholar,2Finn D. Walsh K. Roberson D. McMahon C.J. Myocardial fistulisation and coronary arterial ectasia in children with univentricular circulation: an under-recognised problem.Pediatr Cardiol. 2018; 39: 254-260Crossref PubMed Scopus (3) Google Scholar To the best of our knowledge, this is the first case of CAMF identified by both angiography and pathological examination. Sinusoidal connections may develop and cause CAMFs late after Fontan surgery, which can affect FCF. No funding sources were provided for this article.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".