Background: Partial anomalous venous connections (PAPVC) are associated with left to right shunting and right heart dilatation. Identification of PAPVC has increased with widespread use of cross-sectional imaging modalities. However, management strategies are mostly based on expert opinion given the scarcity of data from large series. We aimed to define types and significance of isolated and atrial septal defect (ASD) associated PAPVC detected by cardiovascular magnetic resonance. Methods: We retrospectively reviewed our cardiovascular magnetic resonance database from 2002 to 2018 to identify isolated or ASD-associated PAPVC cases. Results: A total of 215 patients (median age 46 years; range, 6–83) with isolated or ASD-associated PAPVC were identified among 102 135 clinical cardiovascular magnetic resonance studies. Of these, 104 were isolated and 111 were associated with an ASD. Anomalous connection of right upper pulmonary vein was the most common single venous anomaly (99/215), but in the isolated PAPVC group there were more anomalous left than right upper pulmonary veins (39 versus 34). The Qp/Qs was significantly higher for isolated anomalous single right upper pulmonary vein than left upper pulmonary vein (1.6 versus 1.4 respectively; P =0.01) as were right ventricular end-diastolic volumes (113.7±30.9 versus 90 [57–157] mL/m ² , P =0.004). In the PAPVC with an ASD group, sinus venosus ASDs (82%) were associated with right-sided PAPVCs while both right and left-sided venous anomalies were seen in secundum ASDs (18%). In a substantial number of patients (30 out of 91) with sinus venosus ASDs, PAPVCs were more complex and involved more than a single anomalous right upper pulmonary vein; and in 5 patients with ASD, PAPVC was identified only after the ASD closure. Conclusions: This large series provides descriptive and hemodynamic features for isolated and ASD-associated PAPVCs. Anomalous isolated right upper pulmonary vein may cause a significant shunt (Qp/Qs >1.5). PAPVC associated with sinus venosus and secundum ASDs might be more complex than a single anomalous pulmonary vein and missed before ASD correction.

OBJECTIVES: The interstage mortality rate after a Norwood stage 1 operation remains 12–20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome. METHODS: A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure. RESULTS: Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood–Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock–Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan–Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan–Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant. CONCLUSIONS: In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.

This review, which is intended for both radiologists and clinical cardiologists, highlights the use of cardiac MRI in pediatric and adult patients after completion of the Fontan procedure and provi... Cardiac MRI has become a widely accepted standard for anatomic and functional assessment of complex Fontan physiology, because it is noninvasive and suitable for comprehensive follow-up evaluation ...

Cardiovascular magnetic resonance (CMR) is valuable for the detection of cardiac involvement in neuromuscular diseases (NMDs). We explored the value of 2D- and 3D-left ventricular (LV) myocardial strain analysis using feature-tracking (FT)-CMR to detect subclinical cardiac involvement in NMD. The study included retrospective analysis of 111 patients with NMD; mitochondrial cytopathies (n = 14), Friedreich’s ataxia (FA, n = 27), myotonic dystrophy (n = 27), Becker/Duchenne’s muscular dystrophy (BMD/DMD, n = 15), Duchenne’s carriers (n = 6), or other (n = 22) and 57 age- and sex-matched healthy volunteers. Biventricular volumes, myocardial late gadolinium enhancement (LGE), and LV myocardial deformation were assessed by FT-CMR, including 2D and 3D global circumferential strain (GCS), global radial strain (GRS), global longitudinal strain (GLS), and torsion. Compared with the healthy volunteers, patients with NMD had impaired 2D-GCS (P < 0.001) and 2D-GRS (in the short-axis, P < 0.001), but no significant differences in 2D-GRS long-axis (P = 0.101), 2D-GLS (P = 0.069), or torsion (P = 0.122). 3D-GRS, 3D-GCS, and 3D-GLS values were all significantly different to the control group (P < 0.0001 for all). Especially, even NMD patients without overt cardiac involvement (i.e. LV dilation/hypertrophy, reduced LVEF, or LGE presence) had significantly impaired 3D-GRS, GCS, and GLS vs. the control group (P < 0.0001). 3D-GRS and GCS values were significantly associated with the LGE presence and pattern, being most impaired in patients with transmural LGE. 3D-FT CMR detects subclinical cardiac muscle disease in patients with NMD even before the development of replacement fibrosis or ventricular remodelling which may be a useful imaging biomarker for early detection of cardiac involvement. Synopsis of the study design, key findings and conclusions.