A case of fetal diagnosis of noncompaction cardiomyopathy and coarctation of the aorta.
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ABSTRACT:
SUBMITTER: Jacobs K
PROVIDER: S-EPMC4078169 | biostudies-other | 2014 May
REPOSITORIES: biostudies-other
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Project description:Background:Aortitis is a rare condition that can be caused by inflammatory or infectious aetiologies. The clinical presentation of aortitis includes a heterogeneous range of symptoms and clinical signs. Case summary:We present a 53-year-old man whose medical history included presence of a ventricular septal defect, a bicuspid aortic valve, and coarctation of the aorta. The coarctation was treated with percutaneous stent implantation. One and a half years later, he presented to our hospital with complaints of fatigue, night sweats, and shivers. Physical examination revealed a fever, tachycardia, and hypertension. Imaging studies showed no signs of endocarditis. Positron emission tomography-computed tomography (PET-CT) showed an increase in 18F-fluorodeoxyglucose uptake at the distal end of the stent in the descending aorta. Blood cultures revealed a Streptococcus gordonii and antibiotic treatment was adjusted accordingly. The patients' functional status improved quickly, the fever resolved, and the laboratory markers of inflammation returned to normal. Discussion:Aortitis is extremely rare after stent implantation. Risk factors for aortitis include congenital vascular malformation and stent implantation. Computed tomography is currently the imaging study of choice for aortitis, while PET-CT seems ideal for identification of stent infection. Mortality associated with infectious aortitis ranges from 21% to 44%, with generally higher mortality if managed with antibiotics alone. The differential diagnosis of stent infection should be taken into account in patients presenting with fever and chills after previous stent procedures.
| S-EPMC7319810 | biostudies-literature
Project description:31-year-old male with no past medical history apart from high blood pressure noted by GP one week prior to admission presented with a three-week history of a flu-like illness and symptoms of heart failure with severe global left ventricular dilation and dysfunction on Transthoracic Echocardiography (TTE). Two weeks following admission he complained of left arm pain and CT upper limb confirmed embolic occlusion of the left brachial artery and incidental severe coarctation of the proximal descending aorta after the origin of the left subclavian artery. Follow-up TTE suggested the presence of coarctation of the aorta on a suprasternal view which was not performed at the time of his first TTE. His heart failure and blood pressure responded very well to medical therapy and he has been referred for surgical correction of his aortic coarctation.
| S-EPMC5551473 | biostudies-other
Project description:BackgroundPrenatal diagnosis of coarctation of the aorta (CoA) is challenging for most examiners. The malformation often occurs at the aortic isthmus, which is a short segment between the origin of the left subclavian artery and the insertion of the ductus. We report herein a rare case of CoA with a long, angled, and hypoplastic isthmus. The echocardiographic characteristics and postmortem findings are presented to approach the skill of fetal diagnosis.Case presentationA pregnant women undergone fetal echocardiography at 26 + 3 gestational weeks in our center. Conventional two-dimensional echocardiography (2DE) showed that ascending aorta went straight upward branching three brachiocephalic arteries without the appearance of the arch, suggesting the possibility of an interrupted aortic arch. Three-dimensional echocardiography (3DE) using spatiotemporal image correlation (STIC) and high-definition flow imaging technique was performed to obtain the 3D rendered images, which clearly showed the arch and its angled junction with the slim isthmus in space. Intra-uterine fetal death occurred and an autopsy was performed. The gross findings showed the angled hypoplastic aortic isthmus in detail and thus confirmed the prenatal diagnosis.ConclusionsTraditional 2DE may be limited in showing the angled hypoplastic aortic isthmus, while the 3DE STIC technique can provide additional spatial information to show great arteries in detail, help to find tiny vessels, and thus benefit the examiners to make an accurate diagnosis.
| S-EPMC8045221 | biostudies-literature
Project description:Coarctation of aorta(CoA), complicated by endarteritis in a children is very rare. Here we present a case of endarteritis in an unoperated CoA in a four year old boy. CoA had been diagnosed in the referring hospital, yet the diagnosis of endocarditis distal to CoA, was made in the tertiary center using modified transthoracic echo windows or focused views. After six weeks of intravenous antibiotic treatment, a coarctectomy and end-to-end anastomosis was performed and he recovered clinically well. This case report concludes that echocardiography remains as the standard diagnostic method for identifying intracardiac manifestations of infective endocarditis/endarteritis. Last but foremost, it delineates the importance of modified transthoracic echo windows or focused views in identifying the unusual position of endocarditis.
| S-EPMC6771818 | biostudies-other
Project description:BackgroundWe report on an unusual case of a 3 year-old girl with coarctation of the aorta complicated by mycotic pseudoaneurysm and infected with Streptococcus pneumoniae.Case summaryThe only symptoms and signs were fever and weak femoral pulses. The echocardiography confirmed a localised isthmic's coarctation. In order to complete the evaluation, a CT scan was performed. This proved crucial in terms of the diagnosis and decision to perform emergency surgery. The diagnosis was confirmed surgically. An aortic rupture was contained by the parietal pleura. Bacteriological analysis of surgical specimens revealed bacterial DNA that tested positive for Streptococcus pneumoniae. The post-operative course was free from any cardiovascular or neurological complications after six weeks of antibiotic therapy.DiscussionSurgical repair of coarctation of the aorta is frequently performed in children. However, complicated forms are less common with a potentially fatal outcome. Indeed, there are no recommendations concerning the management and surgical timing of mycotic pseudoaneurysm. These rare complications should be kept in mind. Although short- and medium-term follow-up of these children is good, caution should be exercised with long-term follow-up because of complications in childhood and adulthood.
| S-EPMC6439392 | biostudies-literature
Project description:BACKGROUND:Iatrogenic aortic stenosis is a serious complication and potentially fatal due to erosion of the aortic wall. Timely management is necessary to prevent complications. CASE PRESENTATION:A 2-year-old boy underwent surgery to remove part of an Amplatzer occluder after patent ductus arteriosus (PDA) device embolization in the thoracic aorta. He exhibited moderate to severe obstruction with erosion of the intimal layer of the aorta caused by the device, part of which was retrieved surgically with restructuring of the thoracic aorta segment and occluder remnant. The patient's postoperative course was uneventful. CONCLUSIONS:When possible, retrieving only part of an embolized device can be advocated because it reduces the risk of aortic and pulmonary artery damage.
| S-EPMC6969450 | biostudies-literature
Project description:BackgroundCoarctation of the aorta is a common form of critical congenital heart disease that remains challenging to diagnose prior to clinical deterioration. Despite current screening methods, infants with coarctation may present with life-threatening cardiogenic shock requiring urgent hospitalization and intervention. We sought to improve critical congenital heart disease screening by using a novel pulse oximetry waveform analysis, specifically focused on detection of coarctation of the aorta.Methods and resultsOver a 2-year period, we obtained pulse oximetry waveform data on 18 neonates with coarctation of the aorta and 18 age-matched controls hospitalized in the cardiac intensive care unit at Children's Healthcare of Atlanta. Patients with coarctation were receiving prostaglandin E1 and had a patent ductus arteriosus. By analyzing discrete features in the waveforms, we identified statistically significant differences in the maximum rate of fall between patients with and without coarctation. This was accentuated when comparing the difference between the upper and lower extremities, with the lower extremities having a shallow slope angle when a coarctation was present (p-value 0.001). Postoperatively, there were still differences in the maximum rate of fall between the repaired coarctation patients and controls; however, these differences normalized when compared with the same individual's upper vs. lower extremities. Coarctation patients compared to themselves (preoperatively and postoperatively), demonstrated waveform differences between upper and lower extremities that were significantly reduced after successful surgery (p-value 0.028). This screening algorithm had an accuracy of detection of 72% with 0.61 sensitivity and 0.94 specificity.ConclusionsWe were able to identify specific features in pulse oximetry waveforms that were able to accurately identify patients with coarctation and further demonstrated that these changes normalized after surgical repair. Pulse oximetry screening for congenital heart disease in neonates may thus be improved by including waveform analysis, aiming to identify coarctation of the aorta prior to critical illness. Further large-scale testing is required to validate this screening model among patients in a newborn nursery setting who are low risk for having coarctation.
| S-EPMC7227302 | biostudies-literature
Project description:Our objectives are to compare speckle-tracking peak global longitudinal (pGLS) and regional strain values in neonates with coarctation of aorta (CoA) and control groups. Echocardiographic parameters measured by speckle-tracking were studied in a retrospective single-center study. A comparison of pGLS and segmental deformation between neonates with CoA and control group was performed using a three-way mixed ANOVA model. There was a significant difference in the means of segmental strain values between CoA and control group at the apical (p = 0.018) and basal segments (p = 0.031) of the interventricular septum and at the apical segment (p = 0.026) of the left ventricle (LV). After correcting for multiple comparisons, the results had a tendency toward statistical significance (adjusted-p < 0.10). There was significant difference in the mean values of pGLS [F(1, 39) = 7.61, p = 0.009, adjusted p = 0.018] between the studied groups. The results of ROC analysis showed that a cut-off value of -16.60% for pGLS provided an estimated sensitivity of 92.31% (95% CI: [63.97, 99.81]) and 71.43% specificity (95% CI: [51.33, 86.78]) for the diagnosis of CoA in neonates (AUC = 0.794, 95% CI: [0.66, 0.93]). pGLS can be regarded as a feasible and reproducible parameter reflecting LV dysfunction in newborns with CoA when compared to newborns with a false-positive diagnosis.
| S-EPMC8509133 | biostudies-literature
Project description:Neurofibromatosis type 1 (NF1) is a relatively common autosomal dominant genetic disorder with a prevalence of 1 in 3,000 (0.03%) at birth. Clinical features are café-au-lait macules, intertriginous freckling, dermal neurofibroma, iris hamartoma (Lisch nodules), and learning disability. NF1 vasculopathy is a serious but underrecognized complication involving the cerebrovascular and cardiovascular systems. The incidence of hypertension in patients with NF1 is around 1% and is associated mainly with renal artery stenosis in children. Only a few cases of thoracic aortic coarctation in association with hypertension and neurofibromatosis have been reported. Here we describe the case of a 4-year-old girl who presented with NF1 and hypertension due to atypical coarctation of the thoracic aorta. The diagnosis of coarctation of the thoracic aorta at the Th5-to-Th6 level was made following catheterization with a pressure gradient of 40?mmHg. The patient underwent surgery comprising resection of the coarctation of the thoracic aorta and graft interposition. On the basis of our findings, annual assessment of blood pressure is advised for patients with NF1.
| S-EPMC3603612 | biostudies-literature
Project description:AimsSeveral coarctation of aorta (COA) severity indices are used for timing of COA intervention, and to define severity of residual coarctation post-intervention. However, it is unclear how many of these COA indices are required in order to recommend intervention, and what degree of residual coarctation results in suboptimal recovery of the left ventricle (LV). Our aim was to assess the correlation between different COA indices and effects of chronic LV pressure overload (LV hypertrophy, diastolic, and systolic dysfunction), and to determine the effect of residual coarctation on LV reverse remodelling after COA intervention.Methods and resultsCOA severity indices were defined as Doppler COA gradient, systolic blood pressure (SBP, upper-to-lower-extremity SBP gradient, aortic isthmus ratio. LV remodelling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodelling was defined as the difference between indices obtained pre-intervention and 5-year post-intervention (delta LVMI, e', E/e', LVGLS).Of the COA indices analysed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β ± standard error -28.3 ± 14.1, P < 0.001), LVGLS (1.51 ± 0.42, P = 0.005), e' (3.11 ± 1.10, P = 0.014), and E/e' (-13.4 ± 6.67, P = 0.008). Residual aortic isthmus ratio also had the strongest correlation with LV reverse remodelling, and residual aortic isthmus ratio <0.7 was predictive of suboptimal LV reverse remodelling post-intervention.ConclusionConsidering the known prognostic implications of LV remodelling and reverse remodelling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention.
| S-EPMC8516517 | biostudies-literature