Sweating deficiency has been reported to represent a cardinal symptom of ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome and ankyloblepharon-ectodermal dysplasia-cleft lip/palate syndrome, two rare p63-associated disorders. According to online resources, hypohidrosis may lead to most life-threatening complications in affected patients. Thus, counseling on the prevention of hyperthermia would be indispensable in case of such syndromes, although detailed information on this issue is missing in the literature. We investigated 14 individuals with ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome (age range 2-48 years) and 9 individuals with ankyloblepharon-ectodermal dysplasia-cleft lip/palate syndrome (0.5-60 years of age) by confocal laser scanning microscopy to determine their palmar sweat duct density and by quantification of pilocarpine-induced sweating. Genotype-phenotype correlations were assessed. In 12 of 23 patients (52%), a normal amount of sweat ducts was detected. These individuals (9 with ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome, 3 with ankyloblepharon-ectodermal dysplasia-cleft lip/palate syndrome) produced sufficient sweat volumes (≥ 20 μl) in response to pilocarpine. All other patients had clearly reduced sweating ability and fewer sweat glands, but no anhidrosis. Alteration of a specific proline residue (Pro590) of p63 was consistently linked to impaired perspiration.Conclusion: Hypohidrosis in p63-associated syndromes is less common and potentially less severe than previously thought and may be attributable to certain genotypes. What is Known: • Hypohidrosis which has been listed as a cardinal symptom of AEC and EEC syndromes may lead to life-threatening hyperthermia. What is New: • Patients with EEC and AEC syndromes often can sweat normally. • Hypohidrosis seems to be attributed to certain TP63 genotypes.
Radial access is recommended for percutaneous coronary intervention (PCI), but rotational atherectomy remains frequently performed via femoral access. Analyzing the procedural parameters, success rate and complications of rotational atherectomy, performed via radial in comparison to femoral access. We retrospectively analyzed 427 consecutive patients undergoing rotational atherectomy. Procedural parameters and outcome were determined in 171 patients, scheduled for radial and compared to 256 patients with femoral access use. In the radial access group (74 ± 9 years, 84% male), the LAD was most frequently treated (49%). Sheath size was 7F in 59% and 6F in 41%, burr size was 1.5 mm in 46% and 1.25 mm in 14% of patients. A temporary pacemaker was inserted in 14%. Procedural success rate stood at 97%. Access site complications occurred in 4% of patients, which was significantly less frequent than in in 256 patients treated via femoral access (13% p = 0.003). Compared to radial access, femoral access was associated with the use of larger sheaths (p < 0.001), more frequent treatment of non-LAD vessels (58.2% vs. 44.4%, p = 0.013) and a higher rate of temporary pacemaker use (27%; p = 0.001). No differences could be seen in procedural success (p = 0.83) and burr size (p = 0.51). Femoral access (OR 3.33; 95% CI 1.40–7.93), and female sex (OR3.40 95% CI 1.69–6.63) were independent predictors for access site complications. For coronary rotational atherectomy, radial access has a high success rate with overall use of smaller sheaths, but of equally sized burrs as well as a significant lower rate of access site-related complications than femoral access.
Background The accuracy of CT-derived FFR (FFRCT) has been repeatedly reported. However, the influence of lesion location on accuracy is unknown. Therefore, we evaluated the diagnostic accuracy of FFRCT to detect lesion-specific ischemia and determined the influence of lesion location (proximal vs. distal vessel segments) compared to invasively measured FFR in patients with suspected CAD. Methods A total of 136 vessels in which “Dual-Source”-CT coronary angiography had been performed due to suspected CAD and who were further referred for invasive coronary angiography with invasive FFR measurement within three months of the index CT examination were retrospectively identified and screened for inclusion in this analysis. Patients with either left main coronary artery stenoses, bifurcation or ostial stenoses were excluded. Invasive FFR was measured using a pressure wire (CERTUS®, St. Jude Medical, Minnesota, USA or Verrata®, Volcano, San Diego, USA). FFRCT was calculated using an on-site prototype (cFFR Version 3.0, Siemens Healthineers, Forchheim, Germany). All vessels were analyzed by an experienced observer blinded to the results of invasive FFR. Stenoses with invasively measured FFR ≤0.80 were classified as hemodynamically significant. We evaluated the diagnostic accuracy of FFRCT in proximal vs. non-proximal vessel segments. Proximal lesions included stenoses located in segment one, six, eleven and twelve. All other stenoses were categorized as distal lesions. Results Out of 136 coronary stenoses, 47 (35%) were located in proximal segments and 89 (65%) lesions were located in distal segments. Compared to invasive FFR, the sensitivity of FFRCT to correctly identify/exclude hemodynamically significant stenoses in proximal vessel segments was 93% (95% CI: 68–99.8%) and the specificity was 100% (95% CI: 89–100%), compared to a sensitivity of 72% (95% CI: 46.5–90%) and a specificity of 87% (95% CI: 77–94%) for FFRCT in distal lesions. The positive predictive value was 100% and the negative predictive value was 97% (95% CI: 82.8–99.5%) compared to a positive predictive value of 59% (95% CI: 42–93.9%) and a negative predictive value of 93% (95% CI: 85.4–96.3%) for proximal vs. distal vessel segment, respectively. This corresponds to an accuracy of 98% vs. 84%, respectively (p=0.02). ROC-Curve analysis showed a slightly higher – albeit non-significant – area under the curve for FFRCT to detect hemodynamic relevance in proximal lesions compared to distal lesions (AUC 0.95, p<0.001 vs. AUC: 0.86, p<0.001, respectively, p=0.2). Conclusion FFRCT obtained using an on-site prototype shows overall a high diagnostic accuracy for detecting lesions causing ischemia as compared to invasive FFR with a trend towards better diagnostic performance in proximal vessel segments. Funding Acknowledgement Type of funding source: None
Introduction Leaflet thrombosis can frequently be identified by computed tomography angiography (CTA) in patients after transcatheter aortic valve implantation (TAVI). Oral anticoagulation is assumed to lead to resolution of thrombosis. We analyzed the resolution of leaflet thrombosis after TAVI by anticoagulant therapy in serial CTA and assessed the influence of prosthesis type. Methods Consecutive TAVI patients who underwent CTA follow-up were screened and individuals with leaflet thrombosis on CTA (defined by the presence of hypo-attenuated leaflet thickening, HALT) in whom oral anticoagulation was initiated and who underwent follow-up CTA were included. The type of anticoagulation was according to physicians' discretion. We assessed the resolution of HALT and compared patients with and without resolution of HALT regarding prosthesis type, prosthesis diameter and type of anticoagulation. Results Out of 395 patients screened for participation, 36 patients (mean age 80±7, 67% men) with leaflet thrombosis underwent follow-up CTA at a medial interval of 3 months (IQR: 3; 5.75 months) after anticoagulation was initiated. 36 patients received either vitamin-K antagonists (n=28, 78%) or Factor-Xa Inhibitors (n=8, 22%). A total of 22 (61%) balloon-expandable and 14 (39%) self-expandable transcatheter aortic valves were implanted. Nominal prosthesis diameter was 23, 25, 26, 27 and 29 mm in 7 (19%), 1 (3%), 10 (28%), 7 (19%) and 11 (31%) patients, respectively. 30 patients (83%) with anticoagulation showed resolution of HALT, whereas persistent HALT was detected in 6 patients (17%), of whom 1 patient with balloon-expandable and 5 patients with self-expandable valve. No difference was seen in duration of anticoagulation between patients with and without resolution of HALT (p=0.984). In univariate analysis, prosthesis type (balloon-expandable vs. self-expandable valves) showed a significant association of self-expandable valves with lack of resolution of leaflet thrombosis (p=0.017). In multivariable logistic regression analysis, this association persisted (p=0.043) and was independent of the type of anticoagulation (p=0.660) and prosthesis diameter (p=0.942). Conclusion Persisting leaflet thrombosis despite anticoagulation is not infrequent and seems to be associated with prosthesis-type rather than small valve diameter or type of anticoagulation. Further research is necessary to identify structural aortic valve determinants for this finding. Funding Acknowledgement Type of funding source: None
Objectives and Background: This study aims to evaluate whether the high correlation and classification agreement of the instantaneous wave-free ratio (iFR) and the resting distal coronary to aortic pressure ratio (P d /P a ) with the fractional flow reserve (FFR) can be confirmed in stent-jailed side branches (J-SB).Methods: Consecutive patients (n = 49) undergoing provisional stenting were prospectively enrolled and a physiological assessment of the J-SB (n = 51) was performed. FFR, iFR, and P d /P a were measured and the hemodynamic relevance was determined using cutoff values of ≤0.80, ≤0.89, and ≤0.92, respectively.Results: Both iFR (r = 0.75) and P d /P a (r = 0.77) correlated closely with FFR.Classification agreement with FFR was 78% for iFR (81% sensitivity, 77% specificity) and 75% for P d /P a (63% sensitivity and 80% specificity). However, angiographic diameter stenosis and pressure indices correlated poorly. For a threshold of ≥70% stenosis, agreement concerning hemodynamic relevance was found in 59% for FFR, 69% for iFR, and 61% for P d /P a . Conclusion:As reported for other lesion types, FFR and the adenosine-independent pressure indices iFR and P d /P a show close correlation and a high classification agreement of approximately 75%-80% in J-SB. Therefore, iFR can be regarded as a recommendable alternative to FFR for the guidance of provisional stenting in bifurcation lesions.
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