Pericardial Constriction and Myocardial Restriction in Pediatric Mulibrey Nanism : A Complex Disease With Diastolic Dysfunction

Publisher Copyright: © 2021 The AuthorsBackground: Mulibrey nanism (MUL) is a rare condition with profound growth delay. Congestive heart failure is a major determinant of prognosis. The aim was to delineate pericardial constriction and myocardial functional abnormalities in a pediatric MUL sample. Methods: A total of 23 MUL patients and 23 individually sex- and age-matched healthy control subjects were prospectively assessed in a cross-sectional study with echocardiography. Results: Clinical signs of heart failure were present in 7 MUL patients, with severe congestive heart failure in 2. Significant diastolic dysfunction, mainly related to constriction, was found in MUL patients without pericardiectomy (N = 18)—septal bounce, pronounced hepatic vein atrial reversal and right heart inflow–outflow variations, and decreased inferior vena cava collapse during respiration. The appearance of the pericardium was not different from that of control subjects. Longitudinal diastolic myocardial velocities were similar to those in control subjects, suggesting an absence of significant myocardial restriction. Right ventricular free wall longitudinal systolic strain and bilateral longitudinal myocardial systolic velocities were decreased in MUL patients, indicating mild biventricular systolic dysfunction. Myocardial motion abnormalities and persistent congestive heart failure were common (in 3 of 6) in MUL patients with a history of pericardiectomy. Cardiac dimensions were similar between MUL patients and control subjects when adjusting for body size, except for smaller biventricular volumes. Conclusions: MUL disease presents with significant constriction-related diastolic dysfunction and mild bilateral systolic dysfunction. Constriction–restriction assessments during follow-up could be of benefit in decision-making regarding pericardiectomy in MUL disease. Myocardial abnormalities were prevalent among MUL patients who had undergone pericardiectomy and are consistent with progression of myocardial disease in a significant proportion of patients.Peer reviewe

The Influence of Percutaneous Closure of Patent Ductus Arteriosus on Left Ventricular Size and Function A Prospective Study Using Two- and Three-Dimensional Echocardiography and Measurements of Serum Natriuretic Peptides

ObjectivesWe aimed to evaluate the effect of percutaneous closure of patent ductus arteriosus (PDA) on left ventricular (LV) hemodynamics.BackgroundToday, most PDAs are closed percutaneously. Little is known, however, about hemodynamic changes after the procedure.MethodsOf 37 children (ages 0.6 to 10.6 years) taken to the catheterization laboratory for percutaneous PDA closure, the PDA was closed in 33. Left ventricular diastolic and systolic dimensions, volumes, and function were examined by two-dimensional (2D) and three-dimensional (3D) echocardiography and serum concentrations of natriuretic peptides measured before PDA closure, on the following day, and 6 months thereafter. Control subjects comprised 36 healthy children of comparable ages.ResultsAt baseline, LV diastolic diameter measured >+2 SD in 5 of 33 patients. In 3D echocardiography, a median LV diastolic volume measured 54.0 ml/m2in the control subjects and 58.4 ml/m2(p < 0.05) in the PDA group before closure and 57.2 ml/m2(p = NS) 6 months after closure. A median N-terminal brain natriuretic peptide (pro-BNP) concentration measured 72 ng/l in the control group and 141 ng/l in the PDA group before closure (p = 0.001) and 78.5 ng/l (p = NS) 6 months after closure. Patients differed from control subjects in indices of LV systolic and diastolic function at baseline. By the end of follow-up, all these differences had disappeared. Even in the subgroup of patients with normal-sized LV at baseline, the LV diastolic volume decreased significantly during follow-up.ConclusionsChanges in LV volume and function caused by PDA disappear by 6 months after percutaneous closure. Even the children with normal-sized LV benefit from the procedure

Cryptic temporal changes in stock composition explain the decline of a flounder (Platichthys spp.) assemblage

Unobserved diversity, such as undetected genetic structure or the presence of cryptic species, is of concern for the conservation and management of global biodiversity in the face of threatening anthropogenic processes. For instance, unobserved diversity can lead to overestimation of maximum sustainable yields and therefore to overharvesting of the more vulnerable stock components within unrecognized mixed-stock fisheries. We used DNA from archival (otolith) samples to reconstruct the temporal (1976-2011) genetic makeup of two mixed-stock flounder fisheries in the angstrom land Sea (AS) and the Gulf of Finland (GoF). Both fisheries have hitherto been managed as a single stock of European flounders (Platichthys flesus), but were recently revealed to target two closely related species: the pelagic-spawning P. flesus and the newly described, demersal-spawning P. solemdali. While the AS and GoF fisheries were assumed to consist exclusively of P. solemdali, P. flesus dominated the GoF flounder assemblage (87% of total) in 1983, had disappeared (0%) by 1993, and remained in low proportions (10%-11%) thereafter. In the AS, P. solemdali dominated throughout the sampling period (>70%), and P. flesus remained in very low proportions after 1983. The disappearance of P. flesus from the GoF coincides in time with a dramatic (similar to 60%) decline in commercial landings and worsening environmental conditions in P. flesus' northernmost spawning ground, the Eastern Gotland Basin, in the preceding 4-6 years. These results are compatible with the hypothesis that P. flesus in the GoF is a sink population relying on larval subsidies from southern spawning grounds and the cause of their disappearance is a cessation of larval supply. Our results highlight the importance of uncovering unobserved genetic diversity and studying spatiotemporal changes in the relative contribution of different stock components, as well as the underlying environmental causes, to manage marine resources in the age of rapid anthropogenic change.Peer reviewe

Chronic Disease Burden After Congenital Heart Surgery : A 47-Year Population-Based Study With 99% Follow-Up

Background Postoperative morbidity is an increasingly important outcome measure of patients who have undergone congenital heart surgery (CHS). We examined late postoperative morbidity after CHS on the basis of patients' government-issued medical special reimbursement rights. Methods and Results Between 1953 and 2009, 10 635 patients underwent CHS at Conclusions Chronic cardiac and noncardiac sequelae are common after CHS regardless of the severity of the defect, underscoring the importance of long-term follow-up of all patients after CHS.Peer reviewe

Inkjet-printed flexible silver electrodes on thiol-enes

Flexible and conductive silver electrodes were fabricated by inkjet printing on several different compositions of thiol-ene polymers. Conductive electrodes with resistivity down to 30 ??cm and good adhesion of the electrodes were obtained by optimizing the printing parameters. The maximum printing resolution was 100 ?m lines and 80 ?m gaps between the lines. Printing on top of cross-linked off-stoichiometric thiol-ene polymer was tested for compositions ranging from 30 % thiol excess to 5 % allyl (?ene?) excess. The roughness off the thiol-ene surfaces was shown to greatly improve the quality of the printed electodes: consistently high yield of conductive electrodes was obtained on rough surfaces (roughness ?1 ?m), whereas on smooth surfaces the electrodes were often cracked. The lowest resistivity values were obtained on electrodes printed on near stoichiometric thiol-ene substrates. The conductivity of the electrodes was retained after 5 % linear strain and after repeated bending with 1 mm radius of curvature, showing the potential for flexible sensors. The electrodes were also applied to electrical impedance-based monitoring of cell growth on thiol-ene surfaces, which showcased that the electrodes survive stressed cell culture conditions for at least 36 h.Peer reviewe

Lapsena sydänleikkauksella hoidettujen ennuste on parantunut

Lievissä sydänvioissa elinajanodote vastaa normaaliväestön tasoa. Sydämen vajaatoiminta ja äkkikuolemat ovat vähentyneet merkittävästi vuoden 1990 jälkeen leikatuilla potilailla. Aortan koarktaation leikkauksen jälkeisiä pitkäaikaisongelmia ovat kohonnut verenpaine sekä rekoarktaatio. Fallot’n tetralogian ja yksikammioisen sydämen vuoksi leikatuilla potilailla yleisiä ovat takyarytmiat ja sydämen vajaatoiminta. Valtasuonten transpositiossa eteistunnelointiin liittyvät rytmihäiriöt ja sydämen vajaatoiminta ovat vähentyneet merkittävästi uuden valtasuonten vaihtoleikkauksen myötä. Potilaat tarvitsevat sydämen seurantaa sekä tehokasta liitännäissairauksien primaarista ja sekundaarista ehkäisyä.Peer reviewe

Vascular structure and stiffness in pediatric Mulibrey nanism using ultra-high frequency ultrasound

Mulibrey nanism (MUL) is a disorder with growth delay and congestive heart failure determining prognosis. We aimed to delineate arterial and venous morphology, and arterial stiffness in a representative pediatric MUL cohort. Twenty-three MUL and 23 individually sex and age-matched healthy controls were prospectively assessed in a cross-sectional study with ultra-high frequency ultrasound (48-70 MHz). Heart failure was present in 7 MUL patients, with severe congestive heart failure in 2. Pericardiectomy had been performed in 6 MUL. Arterial lumen diameters and arterial wall layer thickness (intima-media thickness and adventitia thickness) were smaller in MUL patients, but appropriate for body size when compared with controls. Systolic and diastolic blood pressure, aortic and carotid compliance, stiffness as well as central aortic pulsed wave velocity were all similar in MUL compared with controls. Plasma pro-BNP levels were variably elevated (>300 ng/L) in 9/23 MUL patients and in 4/18 MUL patients older than 5 years of age. Internal jugular vein (mean difference 0.054 mm, CI95% 0.024-0.084) and cubital vein (0.046 mm, CI95% 0.013 - 0.078) total wall thickness was elevated in MUL compared with controls. There were no statistically significant relations between vascular parameters and clinical or laboratory signs of heart failure or pericardiectomy. Arterial lumen, wall layer thickness and stiffness are appropriate for body size in MUL, and like healthy controls. Mild venous wall thickening in the upper body region may be due to increased venous pressures related to remodelling caused by diastolic heart failure

Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis

ObjectiveDespite improved survival and neurodevelopmental outcome, children with hypoplastic left heart syndrome and other forms of univentricular heart remain at increased risk for cognitive, motor, and other neurologic deficits.MethodsWe examined 27 children with hypoplastic left heart syndrome or other forms of univentricular heart at a median age of 5.70 years (range 4.99–7.51 years) and performed brain computed tomography or magnetic resonance imaging on 20. Possible risk factors were correlated with outcome.ResultsMean full-scale IQ among patients with hypoplastic left heart syndrome was 86.7; that among patients with other forms of univentricular heart was 89.1, with both differing significantly from the expected population mean (P = .015 and P = .029, respectively). Cerebral palsy was diagnosed in 1 of 7 patients with hypoplastic left heart syndrome and 2 of 20 with other forms of univentricular heart. Brain computed tomography or magnetic resonance imaging revealed ischemic changes and infarcts or atrophy in 5 of 8 patients who had undergone the Norwood procedure and in 2 of 12 of those who had not (P = .062). Abnormal computed tomographic findings correlated significantly with lower full-scale IQ (P = .045) and verbal IQ (P = .02). In the multiple linear regression model, diuresis the third day after the primary operation and cardiopulmonary bypass time in the bidirectional Glenn operation correlated significantly with the primary outcome of full-scale IQ.ConclusionIn children with univentricular heart, intellectual and neurologic deficits are common. Perioperative and postoperative risk factors related to the primary phase and bidirectional Glenn operation contribute to these deficits