Right ventricular enlargement in a patient with hereditary haemorrhagic telangiectasia: a rare case of pulmonary arteriovenous malformations with concomitant atrial septal defect
Bibliographic record
Abstract
We describe a rare case (32-year-old woman) with hereditary haemorrhagic telangiectasia and concomitant atrial septal defect (ASD). Transthoracic saline contrast echocardiography (TTCE) revealed several microbubbles appearing early (third cardiac cycle) and significant microbubbles appearing later (seventh cardiac cycle) in the left atrium and ventricle (LA/LV) after opacification of the right atrium in the four-chamber view (panel A). Total left to right shunt was calculated at 3.9 L/min (Qp:Qs = 1.8:1). Despite right ventricular (RV) enlargement, search for intracardiac shunting was not undertaken because chest CT confirmed significant pulmonary arteriovenous malformations (PAVMs) in the right lower lobe (arrows in panel B). Following successful transcatheter coil embolization, TTCE demonstrated significant microbubbles within two cardiac cycles (panel C), consistent with intracardiac shunting. Owing to findings of RV dilatation and early positive microbubbles, a transoesophageal echocardiogram (TOE) was performed, identifying a secundum ASD in the bicaval view (panel D) and three-dimensional (3D) TOE en-face view of the interatrial septum from the LA aspect (arrow in panel E). Using a 3D cross-sectional area of 0.48 cm2, the shunt across the ASD was estimated at 2.9 L/min (Qp:Qs = 1.6:1), implying intrapulmonary PAVMs flow of 1.0 L/min prior to closure. Coexistence of intracardiac and extracardiac shunts should be considered when early appearance of microbubbles is seen with TTCE even with known PAVMs. Furthermore, TOE should be performed in patients with RV dilatation of uncertain aetiology despite known extracardiac shunting. Intracardiac and extracardiac shunts are likely to co-exist when TTCE shows early appearance of microbubbles even if maximal microbubbles appear later. Conflict of interest: none declared. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 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 source (direct Gemma or distilled Codex), 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".