Persistent pulmonary hypertension (PPHN) remains a severe complication of the transition to extra-uterine life with significant morbidity and mortality in the newborns. Dehydroepiandrosterone (DHEA) ...represents a new pharmacological agent with vascular effects, including improvement of PPHN in several animal models. We hypothesized that DHEA could decrease pulmonary vascular resistance (PVR) in the pulmonary circulation of fetal sheep. We studied the effect of intravenous infusion of DHEA in fetal lambs using chronically instrumented sheep at 128 days of gestation. PVR was computed before and after intravenous infusion of increasing doses of DHEA. We assessed pre-treatment by L-nitroarginine, an inhibitor of NO production. Blood gases and doses of DHEA were measured in both sheep and fetus before/after DHEA infusion. Intravenous infusion of DHEA had a vasodilator effect with a significant decrease in PVR (respectively -11%, -14% and -36% after infusion of 6, 12 and 24 mg DHEA, p<0.01) without damaging effects on systemic circulation or on blood gases. The inhibitory effect of pre-treatment with L-nitroarginine resulted in a significant increase in PVR. We demonstrated a potent vasodilator effect of DHEA on fetal pulmonary circulation without deleterious effects. DHEA might represent a new treatment for PPHN.
(1) Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is becoming more attractive and effective with the development of new occluders. The aim of this study was to report a ...single-center experience in PmVSD closure using the Lifetech Konar-multifunctional occluder (MFO). (2) From March 2019 to October 2022, 43 consecutive patients were enrolled in the study. Among them, 13 had multifenestrated PmVSD including 5 Gerbode-type defects. (3) There were 23 males/20 females, and the median age was 17 years (range 2–68 years). Trivial aortic regurgitation was noticed in 19 patients. Implantation was successful in all patients under general anesthesia. A retrograde approach was used in 35 patients (81%). The retrograde approach was associated with a lower radiation dose (p = 0.042) and shorter fluoroscopy time (p = 0.002) compared to the antegrade approach. Full occlusion was observed immediately in 12 patients (28%) and in 33 patients (77%) at a median follow-up of 11 months. There were no complications such as embolization, complete atrioventricular block, device dislocation, new onset above grade I, or progression of tricuspid or aortic valve regurgitation. Seven of the thirteen patients with a multifenestrated defect had no residual shunt. The persistent shunts were all trivial intra-prosthetic leaks. (4) MFO is effective and safe for PmVSD closure including multifenestrated/Gerbode-type defects with no complication. However, a longer follow-up remains warranted to establish the safety of this technique.
Background
In multisystem inflammatory syndrome in children (MIS-C), diagnostic delay could be associated with severity. This study aims to measure the time to diagnosis in MIS-C, assess its impact ...on the occurrence of cardiogenic shock, and specify its determinants.
Methods
A single-center prospective cohort observational study was conducted between May 2020 and July 2022 at a tertiary care hospital. Children meeting the World Health Organization MIS-C criteria were included. A long time to diagnosis was defined as six days or more. Data on time to diagnosis were collected by two independent physicians. The primary outcome was the occurrence of cardiogenic shock. Logistic regression and receiver operating characteristic curve analysis were used for outcomes, and a Cox proportional hazards model was used for determinants.
Results
Totally 60 children were assessed for inclusion, and 31 were finally analyzed 52% males, median age 8.8 (5.7–10.7) years. The median time to diagnosis was 5.3 (4.2–6.2) days. In univariable analysis, age above the median, time to diagnosis, high C-reactive protein, and high N-terminal pro-B-type natriuretic peptide (NT-proBNP) were associated with cardiogenic shock odds ratio (OR) 6.13 (1.02–36.9), 2.79 (1.15–6.74), 2.08 (1.05–4.12), and 1.70 (1.04–2.78), respectively. In multivariable analysis, time to diagnosis ≥ 6 days was associated with cardiogenic shock adjusted OR (aOR) 21.2 (1.98–227). Time to diagnosis ≥ 6 days had a sensitivity of 89% and a specificity of 77% in predicting cardiogenic shock; the addition of age > 8 years and NT-proBNP at diagnosis ≥ 11,254 ng/L increased the specificity to 91%. Independent determinants of short time to diagnosis were age < 8.8 years aHR 0.34 (0.13–0.88), short distance to tertiary care hospital aHR 0.27 (0.08–0.92), and the late period of the COVID-19 pandemic aHR 2.48 (1.05–5.85).
Conclusions
Time to diagnosis ≥ 6 days was independently associated with cardiogenic shock in MIS-C. Early diagnosis and treatment are crucial to avoid the use of inotropes and limit morbidity, especially in older children.
To evaluate bare-metal Optimus and polytetrafluoroethylene (PTFE)-covered Optimus-CVS balloon-expandable, cobalt-chromium, hybrid cell-designed stents in congenital heart disease (CHD) interventions.
...Retrospective multicentre review of patients with CHD receiving Optimus stents. Stent mechanical behaviour, clinical indications and outcomes were assessed.
183 stents (49.2% XXL/15-ZIG, 33.3% XL/12-ZIG, 17.5% L/9-ZIG) were implanted (98.9% success rate, 2.3% serious procedural complication rate) in 170 patients (57.6% men, 64.1% adults), median age 23.6 years (IQR, 15.2-39.2) and weight 63.5 kg (IQR, 47-75.7). Indications were right ventricular outflow tract stand-alone stenting or before revalvulation (62.4%), aortic coarctation treatment (15.3%), Fontan-circuit fenestration closure (12.4%) and miscellaneous (10%). 86/170 (50.6%) patients had PTFE-covered stenting (50% prophylactic). In 86/170 (50.6%) patients with stenotic lesions, median percentage of achieved stent expansion was 93.4% (IQR, 85.5%-97.7%), median gradient decreased from 28 mm Hg (IQR, 19-41) to 5 mm Hg (IQR, 1-9) (p<0.001), median vessel diameters increased from 13 mm (IQR, 7.9-17) to 18.9 mm (IQR, 15.2-22) (p<0.001) and percentage of vessel expansion was 45.2% (IQR, 19.8%-91.3%). In 30/36 (83.3%) patients with graft, median dilation of 2 mm (IQR, 2-5) above nominal diameter was achieved. Median stent shortening was 10.9% (IQR, 6.1-15.1) and was associated only with expansion diameter (OR: 0.66, 95% CI: 0.38 to 0.93). No clinically relevant fracture, stent embolisation or dysfunction occurred on a median follow-up of 9 (IQR, 4-14) months.
Optimus stents are effective tools for transcatheter treatment of simple and complex CHD. Optimus stents' reliable mechanical behaviour and particular covering design can promote widespread use.
Percutaneous pulmonary valve implantation (PPVI) with a SAPIEN 3 valve is effective for treating treat right ventricle outflow (RVOT) dysfunction. A modified technique was developed without ...prestenting using a protective valve delivery method. We aimed to compare the procedural results of the modified technique group (MTG) to those of patients in a conventional technique group (CTG).
We designed a matched before-after study. All consecutive PPVI with SAPIEN 3 performed in the MTG over 9 months were matched, based on the RVOT type and size, to consecutive procedures performed previously with SAPIEN 3.
A total of 54 patients were included, equally distributed in the two groups. The sizes of the SAPIEN 3 valves were 23 mm (n = 9), 26 mm (n = 9), 29 mm (n = 36). The two groups were similar regarding demographic data, RVOT type, and pre-procedure hemodynamics. PPVI was performed in a single procedure in all patients of the MTG, whereas six (22.2%) patients of the CTG group underwent prestenting as a first step and valve implantation later (
= 0.02). The procedures were successful in all cases. Stent embolization was reported in two patients (7.4%) in the CTG, which were impacted in pulmonary arteries. In one case (3.7%), in the MTG, an unstable 29 mm SAPIEN 3 valve was stabilized with two stents and additional valve-in-valve implantation. The hemodynamics results were good in all cases, without significant differences between the two groups. The procedures' durations and fluoroscopy times were significantly reduced in the MTG (48.1 versus 82.6 min,
< 0.0001; 15.2 versus 29.8 min,
= 0.0002). During follow-up, neither stent fracture nor valve dysfunction was noticed in either group.
PPVI without prestenting and with a protective delivery method of the SAPIEN 3 valve significantly reduces the procedure's complexity, the duration, and the irradiation while maintaining excellent hemodynamics results in selected cases.
Hypnoanalgesia is a promising non-pharmacologic adjunct technique in paediatric interventions. Its safety, efficiency, and impacts on paediatric cardiac catheterisation (CC) are unknown. Methods: In ...a prospective study, patients aged <16 years who underwent CC under hypnoanalgesia from January to December 2021 were included. Pain and anxiety were assessed using the analgesia nociception index (ANI) and the visual analogue scale (VAS). Results: Sixteen patients were included; the mean age was 10.5 years, and the mean weight was 37 kg. Catheterisations were interventional in 10 patients (62.5%). Hypnoanalgesia indications were general anaesthesia (GA) contraindication in four patients (25.0%), the need for accurate pressure measurements in three patients (18.7%), and interventionist/patient preferences in nine (56.3%). CC was accomplished in 15 patients (93.7%), even in complicated cases. In one case, pulmonary artery pressures were normalised compared to previous catheterisation under local anaesthesia alone. The VAS score was under 5/10 for all patients. The ANI remained above 50 (no painful zone) for all but one patient. There was no significant decrease in the ANI during the intervention compared to the baseline (p = 0.62). No complications were reported. Conclusion: Paediatric CC is feasible and safe under hypnoanalgesia, even in complicated cases. Hypnoanalgesia was efficient in managing pain and stress, and it ensures more reliable pressure measurements.
Persistent pulmonary hypertension of the newborn (PPHN) causes significant morbidity and mortality in neonates.
-3 Poly-unsaturated fatty acids have vasodilatory properties in the perinatal lung. We ...studied the circulatory effects of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in fetal sheep and in fetal pulmonary arterial rings.
At 128 days of gestation, catheters were placed surgically in fetal systemic and pulmonary circulation, and a Doppler probe around the left pulmonary artery (LPA). Pulmonary arterial pressure and LPA flow were measured while infusing EPA or DHA for 120 min to the fetus, to compute pulmonary vascular resistance (PVR). The dose effects of EPA or DHA were studied in vascular rings pre-constricted with serotonin. Rings treated with EPA were separated into three groups: E+ (intact endothelium), E- (endothelium stripped) and LNA E+ (pretreatment of E+ rings with l-nitro-arginine).
EPA, but not DHA, induced a significant and prolonged 25% drop in PVR (
= 8,
< 0.001). Incubation of vascular rings with EPA (100 µM) caused a maximum relaxation of 60% in the E+ (
= 6), whereas vessel tone did not change in the E- (
= 6,
< 0.001). The vascular effects of EPA were significantly decreased in LNA E+ (
= 6). Incubation with DHA resulted in only a mild relaxation at the highest concentration of DHA (300 µM) compared to E+.
EPA induces a sustained pulmonary vasodilatation in fetal lambs. This effect is endothelium- and dose-dependent and involves nitric oxide (NO) production. We speculate that EPA supplementation may improve pulmonary circulation in clinical conditions with PPHN.
Bronchopulmonary dysplasia (BPD) is one of the most common complications of prematurity, occurring in 30% of very low birth weight infants. The benefits of dietary intake of polyunsaturated fatty ...acids ω-3 (PUFA ω-3) during pregnancy or the perinatal period have been reported. The aim of this study was to assess the effects of maternal PUFA ω-3 supplementation on lung injuries in newborn rats exposed to prolonged hyperoxia. Pregnant female Wistar rats (n = 14) were fed a control diet (n = 2), a PUFA ω-6 diet (n = 6), or a PUFA ω-3 diet (n = 6), starting with the 14th gestation day. At Day 1, female and newborn rats (10 per female) were exposed to hyperoxia (O₂, n = 70) or to the ambient air (Air, n = 70). Six groups of newborns rats were obtained: PUFA ω-6/O₂ (n = 30), PUFA ω-6/air (n = 30), PUFA ω-3/O₂ (n = 30), PUFA ω-3/air (n = 30), control/O₂ (n = 10), and control/air (n = 10). After 10 days, lungs were removed for analysis of alveolarization and pulmonary vascular development. Survival rate was 100%. Hyperoxia reduced alveolarization and increased pulmonary vascular wall thickness in both control (n = 20) and PUFA ω-6 groups (n = 60). Maternal PUFA ω-3 supplementation prevented the decrease in alveolarization caused by hyperoxia (n = 30) compared to PUFA ω-6/O₂ (n = 30) or to the control/O₂ (n = 10), but did not significantly increase the thickness of the lung vascular wall. Therefore, maternal PUFA ω-3 supplementation may protect newborn rats from lung injuries induced by hyperoxia. In clinical settings, maternal PUFA ω-3 supplementation during pregnancy and during lactation may prevent BPD development after premature birth.
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