Narrowing Of Aorta Research Articles

Prenatal diagnosis, ultrasound findings and pregnancy outcome of 7q11.23 deletion and duplication syndromes: what are the fetal features?

ObjectiveAnalyze the ultrasound findings, single nucleotide polymorphism array (SNP-array) results, and pregnancy outcomes of fetuses with 7q11.23 deletions and duplications in the second and third trimesters. Investigate the prenatal ultrasound characteristics and follow up information of these fetuses.MethodsSeven fetuses were diagnosed with 7q11.23 deletion and six with 7q11.23 duplication via SNP-array at the prenatal diagnosis center of a single Chinese tertiary medical center from January 2017 to May 2024. Maternal demographics, ultrasound findings, SNP-array results, pregnancy outcomes, and follow-up information were comprehensively reviewed and analyzed.ResultsThe copy number variations (CNVs) ranged from 1.43 Mb to 1.78 Mb in cases of 7q11.23 deletions and from 1.42 Mb to 1.68 Mb in cases of 7q11.23 duplications. These CNVs encompassed 29 OMIM-listed genes, including ELN, DNAJC30, GTF2IRD1, and GTF2I. Among the seven cases of 7q11.23 deletion syndrome, six exhibited ultrasound abnormalities. The main clinical phenotypes included three cases of intrauterine growth restriction and four cases of cardiovascular system abnormalities, specifically two cases with ventricular septal defects, one case with aortic narrowing, and one case with supravalvular pulmonary stenosis. One case was particularly notable, exhibiting complex multi-organ structural malformations. Out of six cases of 7q11.23 duplication syndrome, five exhibited ultrasound abnormalities. These included two cases of cardiovascular abnormalities: one case with a widened left ventricle and another case with a shortened fetal humerus length. One case revealed complex multi-organ structural malformations, including hydronephrosis, a microgallbladder, and a cleft lip and palate. All seven cases of 7q11.23 deletions and three cases of 7q11.23 duplications opted for termination of the pregnancy. The remaining three cases of 7q11.23 duplications chose to continue the pregnancy. One case underwent surgical treatment for a ventricular septal defect after birth, and the prognosis was favorable. Another case involved a full-term delivery, this child was followed up at the age of 4 and exhibited a phenotype of poor language expression ability.ConclusionOur study broadened the clinical phenotype spectrum of fetuses with 7q11.23 deletions and duplications. Additionally, it conducted a preliminary evaluation of prenatal ultrasound findings and postnatal clinical phenotypes in follow-up cases. The clinical phenotype of fetuses with 7q11.23 deletion and duplication syndromes involves multiple systems and is relatively complex. Cardiovascular abnormalities and intrauterine growth restriction are the most common clinical manifestations observed in prenatal 7q11.23 deletion syndrome. Fetuses with 7q11.23 duplications exhibit a wide range of clinical phenotypes that lack specificity.

Anesthetic Management of Middle Aortic Syndrome in an Adult: A Case Report.

Middle aortic syndrome (MAS) is a rare disorder characterized by narrowing of the aorta. Depending on the location and extent of aortic narrowing, surgical management may be necessary to prevent long-term sequelae, such as stroke and congestive heart failure. We report a case of MAS and discuss the unique intraoperative anesthetic considerations including hemodynamic monitoring proximal and distal to the aortic narrowing, minimizing the risk of spinal cord ischemia, and utilization of intraoperative dobutamine stress testing to guide surgical management.

Prenatal Diagnosis and Postnatal Outcomes of Persistent Left Superior Vena Cava Associated With Mild Narrow Aorta: A Cohort Study.

To explore prenatal ultrasonic features and prognosis of the persistent left superior vena cava (PLSVC) complicated with mild narrow aorta. A retrospective study was conducted involving 1348 fetuses diagnosed with PLSVC prenatally between January 2016 and December 2019. Forty-five fetuses with PLSVC associated with mild narrow aorta were selected from the cohort as the study group and 79 fetuses with isolated PLSCV were recruited randomly as the control group. All clinical and ultrasound results, including images and parameters of cardiac structures, were reviewed retrospectively. General conditions, ultrasound (US) measurements, and fetal prognosis were compared between the groups. Aorta valve diameter (AOD), Z-score of aorta valve (AODz-score), aortic isthmus diameter (AOIsD), and pulmonary diameter (PAD)/AOD were significantly different in study group than control group no matter in the second or third trimester. Thirty-eight fetuses in study group were born with favorable outcomes after long-term follow-up. A total of 13.16% (5/38) remain mild narrow aorta and 3 of them showed smaller left ventricle after 3 years follow up. Prenatal AODz-score in infants remains mild narrow aorta after 2 years aged was higher than ones' aorta return to normal (P = .01), especially when AODz-score >1.725. Moreover, when prenatal ratio of AOIsD/left subclavian artery was <1.12, it was more likely that the aorta would remain mildly narrow at age 2. Fetuses diagnosed with PLSVC with mild narrow aorta had favorable prognosis. AODz-score and AOIsD/left subclavian artery may be two predictors that reveal the risk of a mildly narrowed aorta remaining after birth.

Mini-access ascending aorto-bifemoral bypass surgery for the treatment of aortic steno-occlusive disease

ObjectivesMid-aortic syndrome is a rare condition characterized by severe aortic narrowing, leading to high upper body blood pressure and organ hypoperfusion, necessitating surgical intervention. Although central bypassing is considered ideal, it involves extensive incisions. To overcome these limitations, less-invasive approaches have been developed. This study aims to introduce a mini-access approach using video-endoscopy and to evaluate the feasibility and outcomes of mini-access ascending aorto-bifemoral bypass surgery. MethodsFrom November 2020 to May 2022, we performed ascending aorta to bifemoral artery bypass operations on 7 patients to treat steno-occlusive diseases in the downstream aorta. A Y-graft was created, and procedures were conducted under general anesthesia using video-endoscopy with limited skin incisions. ResultsIntraoperatively, there were no major complications, and none of the patients required cardiopulmonary bypass support. Furthermore, there were no postoperative mortalities or major complications. Postoperatively, the mean ankle-brachial index significantly improved from 0.59/0.59 to 0.96/0.92 (P = .004), and the mean glomerular filtration rate increased from 61.1 to 85.3 mL/min/1.73 mm2 (P = .012). Additionally, symptoms of claudication resolved in all patients. ConclusionsVideoscope-assisted mini-access aortic bypass surgery not only provides favorable early postoperative outcomes but also represents a technically feasible alternative to traditional surgical approaches for the treatment of steno-occlusive aortic diseases.

Impaired Aortic Growth in Neuroblastoma Patients After Intensive Treatment

PurposeThis study evaluated the abdominal aortic diameter in high-risk neuroblastoma (NB) patients and the risk of aortic narrowing following intensive treatment. MethodsWe measured the aortic diameter at four specific levels of the abdominal aorta (diaphragmatic crus, celiac axis, and the root of the superior (SMA) and inferior (IMA) mesenteric arteries) on contrast CT scans. The control group consisted of 56 children with non-oncologic disorders, while the NB group included 35 patients with high-risk abdominal NB. We used regression analysis of age and aortic diameter to determine the regression formula for each level in each group and performed intergroup comparisons using t-test. ResultsWe evaluated a total of 160 contrast-enhanced CTs performed in the 35 eligible cases. The aortic diameter of pretreated NB patients was not significantly different from the controls. After receiving any treatment, the aortic diameter was significantly smaller in the NB group (p < 0.01 each). Patients who underwent radical surgery, particularly gross total resection (n = 26), had smaller aortic diameters at all levels compared to controls (p < 0.01 each). Patients treated with radiotherapy (RT) had smaller aortic diameters than controls. External beam radiotherapy (EBRT) patients (n = 24) had smaller aortic diameters at all levels except the celiac axis (crus, SMA, IMA; p < 0.01 each), and intraoperative radiotherapy (IORT) ± EBRT patients (n = 5) had smaller aortic diameters at all levels (p < 0.01 each). ConclusionPatients with NB may experience impaired development of the abdominal aorta after multimodal therapy, particularly after RT. Close observation and long-term follow-up is essential to monitor for catastrophic vascular complications. Level of EvidenceLEVEL III.

КЛИНИЧЕСКИЙ СЛУЧАЙ ДИАГНОСТИКИ КОАРКТАЦИИ АОРТЫ В ПОЖИЛОМ ВОЗРАСТЕ

Abstract. Introduction. Aortic coarctation is a congenital narrowing or complete rupture of aorta in the region of its arch, isthmus, lower thoracic part, or abdominal region. Predominantly (in over 90% of cases), the coarctation occurs in a “typical” place, i.e., around the aortic isthmus. This defect is often combined with other defects, such as bicuspid aortic valve, aortic stenosis, patent ductus arteriosus, and ventricular septal defect. Aim: To actualize knowledge about aortic coarctation and its potential clinical manifestations. Materials and Methods. Female patient N., 65 years old, was admitted in the Department of Cardiology of City Clinical Hospital 7, Kazan in an urgent manner, complaining of fatigue, chest burning that could not be reversed by taking nitroglycerin, mixed dyspnea at the minimum exercise load, heart activity disorders, and rapid uneven heartbeat for a long time. The patient was examined using general clinical, laboratory and instrumental methods. In writing this article, the authors used source medical records (history of the present disease). Results and Discussion. The defect manifested in the patient clinically at her age of 25 as her systemic hypertension was first detected, accompanied by headache, dizziness, seeing dark spots in her vision, and later rapid heartbeat and short of breath on exertion. According to literature, in about 10% of cases, early development of aortic atherosclerosis is observed especially at the site of narrowing, as well as atherosclerosis of the coronary arteries, which caused unstable angina pectoris and the development of atrial fibrillation in our patient. In view of clinical manifestations of acute coronary syndrome, the patient was prescribed coronary angiography, during which aortic coarctation was suspected. Echocardiography detected congenital heart disease that often accompanies aortic coarctation (up to 60% of cases according to official sources), namely bicuspid aortic valve, as well as atrial septal aneurysm, reduction of descending aorta, and moderate aortic stenosis with aortic insufficiency. Further, to verify the diagnosis, X-ray computed tomography of the aorta was performed, which is the gold standard in detecting aortic coarctation. This method allowed us to identify the extent of the lesion, the diameter of the narrowing of the aorta, pre- and post-stenotic areas, and the presence and condition of collaterals. Conclusions. These findings are necessary for practicing physicians when choosing a treatment strategy for patients with aortic coarctation.

Anesthesia Management for Asymptomatic Aortic Narrowing

An 80-year-old patient admitted to the emergency department for a fracture of the lower extremity of the femur. The clinical examination reveals a stable patient with normal hemodynamic, respiratory, and neurological status. There are no medical history records. The pre-anesthetic evaluation is unremarkable with normal test results. The patient is transported to the operating room, and after spinal anesthesia, experiences hemodynamic instability due to a tight asymptomatic mitral stenosis observed during the clinical examination.

A case of stent-assisted balloon-induced intimal disruption and relamination of distal remaining aortic dissection after the ascending aorta and aortic arch replacement for acute aortic dissection

BackgroundThe early survival rate of patients with acute type A aortic dissection (TAAD) has improved remarkably over the past two decades. However, a false lumen may remain after proximal aortic repair and is a potential risk factor for aortic diameter enlargement, aortic rupture, and death in the chronic phase. In the stent-assisted balloon-induced intimal disruption and relamination of aortic dissection (STABILISE) technique, a stent graft is implanted at the proximal part of the aortic dissection, followed by bare-metal aortic stent placement over the distal aortic dissection. Next, an aortic balloon catheter is dilated inside the stent graft and the bare stent to disrupt the intima and collapse the false lumen, immediately restoring uniluminal thoracoabdominal aortic flow. This report describes our experience with the STABILISE technique for a residual aortic dissection after an ascending aorta, and partial aortic arch replacement for acute TAAD resulted in complications.Case presentationA 60-year-old man presented to our hospital with chest pain, paresthesia, and left lower-limb paralysis. He was diagnosed with acute type A aortic dissection (TAAD) and malperfusion of the left lower limb by computed tomography (CT), and an emergent ascending aorta and partial aortic arch replacement were performed. Immediately after surgery, the left femoral arterial pulse improved, and left lower limb revascularization was deferred. The postoperative course was good; however, back pain and intermittent claudication recurred on the 9th day after surgery. A CT examination revealed persistent antegrade false lumen flow in the descending thoracic aorta through the left subclavian artery dissection and retrograde false lumen flow from the thoracoabdominal segment, resulting in aortic diameter enlargement and narrowing of the true lumen. The symptoms did not improve, and the STABILISE technique was performed on the 12th day after the initial aortic surgery. The patient’s subsequent course was good; the back pain and intermittent claudication resolved. The patient was discharged on the 8th day after the STABILISE technique.ConclusionsThe STABILISE technique can obliterate the false lumen and resolve symptoms associated with a residual false lumen after proximal aortic repair for acute TAAD.

Anesthesia Management for Asymptomatic Aortic Narrowing

An 80-year-old patient admitted to the emergency department for a fracture of the lower extremity of the femur. The clinical examination reveals a stable patient with normal hemodynamic, respiratory, and neurological status. There are no medical history records. The pre-anesthetic evaluation is unremarkable with normal test results. The patient is transported to the operating room, and after spinal anesthesia, experiences hemodynamic instability due to a tight asymptomatic mitral stenosis observed during the clinical examination.

MRI visualization of aortic recoarctation in the long-term period after surgery (case report)

Case study. Aortic coarctation refers to well-known and well-studied congenital malformations of the cardiovascular system. The success of cardiovascular surgery significantly increased the survival rate of patients, but, unfortunately, did not reduce the risks of serious cardiovascular complications in the long-term postoperative period. One of the significant complications remains recoarctation – repeated narrowing in the area of surgery, which can initiate other complications.The purpose of the study: to present the features of blood flow in the aorta in a patient with aortic coarctation in the long-term postoperative period on the clinical example of a complex MR examination.Material and methods. A 12-year-old patient, at the age of 6, she underwent balloon angioplasty of aortic coarctation. Echocardiographic data revealed a residual gradient at the isthmus of the aorta and expansion of the descending aorta. To clarify the diagnosis, MRI angiography of the heart was performed with intravenous contrast and the use of a cardiopackage for 4D flow analysis (4D flow).Results and discussion. According to the MRI study, a narrowing of the distal aortic arch with aortic dilation after the departure of the left subclavian artery was revealed, which is confirmed by the obtained absolute values of blood flow indicators. MR-angiography of the aortic arch made it possible to visually determine the “gothic” shape of the aortic arch and present 3D reconstructions. 4D flow maps showed acceleration of blood flow to the systole in the area of aortic narrowing, additional vortex flow below the area of aortic narrowing and spiral flow in the descending aorta, which persisted throughout the diastole.One explanation for the abnormal spiral flow in the descending thoracic aorta may be the presence of a “gothic” aortic arch. Also, residual hypoplasia of the aortic arch and narrowing of the isthmus are the leading parameters affecting the pathophysiology of changes in blood pressure during exercise.Conclusion. The 4D-flow MR package in vivo makes it possible to study the flow geometry and blood flow parameters in detail, to obtain a detailed picture of the aortic condition, which gives potential advantages in a comprehensive examination of patients with aortic coarctation under dynamic observation.

Middle aortic syndrome in children and adolescents.

Middle aortic syndrome is a rare form of renovascular disease that may present with severe hypertension during childhood. Narrowing of the abdominal aorta is often associated with narrowing of the renal and/or other visceral arteries and may be secondary to specific genetic syndromes. Following the optimization of blood pressure control, significant aortic narrowing often requires invasive management, including endovascular and surgical intervention. In younger children, endovascular therapy may be attempted in the first instance to acutely reduce the pressure gradient across the narrowing; however, a sustained benefit is rare. Once the child has grown to accommodate a graft of an adequate size, surgical therapy is indicated for patients in whom medical and/or endovascular management has not resulted in adequate blood pressure control. It is critical that individuals with middle aortic syndrome be managed by an experienced multidisciplinary team that includes medical, endovascular, and surgical expertise that can provide long-term care to monitor for recurrent hypertension and evidence of end-organ damage.

Does a Change in Device Design Alter Device Size Selection? A Comparison of Conventional and Occlutech Duct Occluder Designs.

The Occlutech duct occluder (ODO) with a novel design has been available for closure of patent ductus arteriosus (PDA) since 2011. Available data on initial experience with the ODO have shown that operators continue to choose device sizes based on their experience with conventional duct occluders (CDO). This study postulated that occlusion of the pulmonary arterial (PA) end of the ductus should achieve satisfactory PDA closure without additional complications. Accordingly, the size of the ODO was chosen such that the larger PA diameter in the new design exceeded the PA end of the ductus by 2-3 mm and 2-4 mm for normotensive and hypertensive ducts, respectively. It sought to examine the feasibility and safety of such an approach, and compared ODO and CDO devices with respect to device sizes deployed, to ascertain if the newer design had any advantages. This prospective study enrolled 105 infants, children and a few adults with various duct morphologies and PA pressures for ductal closure using the ODO from 2018-2020. The control group consisted of 105 ducts closed with CDO. A comparison of the two groups with respect to duct diameter and implanted device sizes was performed using appropriate statistical software. The study found that 40% of the ducts had moderate-to-severe pulmonary hypertension. Most ducts measured between 3-4 mm in both groups. The mean weight of the subjects in both groups was 12 kg. Although there was no change in the way that duct occluders were chosen in both groups (2-3 mm over the pulmonary artery diameter), ODO could be significantly downsized in most ducts measuring between 2.5-6.4 mm in diameter (p<0.05). This study found successful closure of relatively larger PDA in infants and children using smaller ODO compared with CDO, without embolisation or aortic narrowing. It also demonstrated that a different device size selection technique may be safely employed with the ODO.

Abstract 12157: 4d Flow Mri Demonstrates Hemodynamic Normalization After Coarctation Repair in Pediatric Patients

Background: Coarctation of the aorta (COA) is a focal aortic narrowing, and intervention is required for patients with severe hemodynamic impairment. Following intervention, patients remain at risk for long-term re-coarctation. We used 4D flow MRI to assess aortic hemodynamics pre- and post- COA repair in children compared to age matched healthy controls. Methods: Aortic 4D flow MRI was performed in 5 pediatric COA patients (baseline age 6-14 yrs, 1F) pre- and post COA repair (4 native COA, 1 re-intervention) and 5 age-matched controls (ages 9-17 yrs, 2 female). Peak systolic velocities were quantified with velocity maximum intensity projections (MIP) ROIs in the ascending aorta (AAo), aortic arch, distal to the coarctation, and descending aorta (DAo) (Figure 1A). For flow quantification, nine 2D planes were placed along the aorta. Regional net flow was determined by averaging the 2D plane flows by region. The ratio between DAo to AAo (DAo/AAo) net flow was used to assess collateral flow. Results: Velocities were generally higher and more consistent across the aorta following surgery (Figure 1A). Plots highlighting changes in DAo/AAo flow ratio are shown in Figure 1B. Compared to controls, DAo/AAo flow ratio was significantly lower prior to COA repair (0.38±0.11 vs 0.61±0.05, p = 0.0004), indicating flow obstruction at the COA and presence of substantial collateral flow. Post COA repair DAo/AAo flow ratio was similar compared to age matched controls (0.59±0.06 vs 0.61±0.05, p = 0.51), indicating normalization of aortic flow. Patient 2, who underwent COA repair via stent placement showed the greatest improvement and normalization of flow consistency (DAo/AAo) across the aorta after intervention. Conclusion: A ortic 4D flow MRI shows normalization of aortic flow velocities, decreased collateral flow following COA repair, and has potential for long term surveillance and quantification of pre- and post-COA repair hemodynamic changes in children.

Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)-Implications for Surgery and Multi-Modal Imaging.

Abnormal aortic arches (AAAs) cover a spectrum of malformations, including abnormal laterality, branching patterns, and flow-limiting narrowing, which themselves vary from tubular hypoplasia, through discrete coarctation, to complete interruption of the arch. Neonatal surgery within the first days of life is necessary for most of these morphologies. Patch aortoplasty is widely used as it can offer a good haemodynamic result, being tailored to each combination of presenting pathologies. Our study hypothesis was that arch malformations are frequent in DORV and exhibit a plethora of phenotypes. We reviewed 54 post-mortem heart specimens from the UCL Cardiac Archive, analysing morphological features that would potentially influence the surgical repair, and taking relevant measurements of surgical importance. AAAs were found in half of the specimens, including 22.2% with aortic arch narrowing. In total, 70% and 30% of narrow arches had a subpulmonary and subaortic interventricular defect, respectively. Z-scores were significantly negative for all cases with tubular hypoplasia. We concluded that arch malformations are a common finding among hearts with DORV. Surgery on the neonatal aortic arch in DORV, performed in conjunction with other interventions that aim to balance pulmonary to systemic flow (Qp/Qs), should be anticipated and form an important part of multi-modal imaging.

4D flow MRI derived aortic hemodynamics multi-year follow-up in repaired coarctation with bicuspid aortic valve

PurposeThe purpose of this study was to investigate the relationships between hemodynamic parameters and longitudinal changes in aortic dimensions on four-dimensional (4D) flow magnetic resonance imaging (MRI) in patients with bicuspid aortic valve (BAV) and repaired coarctation. Materials and methodsThe study retrospectively included patients with BAV and childhood coarctation repair who had at least two cardiothoracic MRI examinations including 4D flow MRI at baseline and follow-up. Analysis included the calculation of aortic peak velocities, wall shear stress (WSS), pulse wave velocity (PWV), aortic dimensions and annual growth rates. Differences between examinations were assessed using paired t-test or Wilcoxon signed rank test. Relationships between growth rate and 4D flow metrics were assessed using Pearson or Spearman correlation tests. ResultsThe cohort included 15 patients (mean age 35 ± 8 [SD] years, 9 men) with a median follow-up time of 3.98 years (Q1: 2.10; Q3: 4.96). There were no significant differences in aortic mean WSS, peak velocities, and PWV between baseline and follow-up values. Greater baseline peak velocities at the site of the coarctation were strongly associated with aortic narrowing (follow-up vs. baseline diameter) at coarctation zone (r = -0.64; P = 0.010) and moderately in descending aorta (r = -0.53; P = 0.042). In addition, increased baseline WSS in the aortic arch was strongly related with narrowing of the coarctation zone at follow-up (r = -0.64, P = 0.011). ConclusionMeasures of aortic hemodynamics and aortic WSS are stable over time in patients with BAV with coarctation repair. Increased peak velocity was associated with a progressive narrowing at the site of the coarctation repair.

Successful endovascular repair of iliac artery aneurysms with unsuitable anatomy by combining unibody bifurcated endograft and iliac branch systems to preserve hypogastric artery blood flow: a report of two cases

BackgroundTo overcome the anatomical limitation of a narrow aorta and short length from the renal artery to the terminal aorta, unibody endograft AFX2 and iliac branch endoprosthesis (IBE) can be combined.Case presentationCase 1: The first patient was an 89-year-old woman who had a right saccular common iliac artery (CIA) aneurysm (38 mm); the abdominal aorta was not aneurysmal (diameter, 19 mm). The right CIA’s origin was 10 mm in diameter. A bifurcated AFX2 was placed in an ordinary manner. Then, IBE was inserted in the right leg of the AFX2. Case 2: The second patient was an 87-year-old man diagnosed with an abdominal aortic aneurysm (55 mm), right dissecting CIA aneurysm (20 mm), and right hypogastric artery aneurysm (22 mm) extending to the bifurcation of the superior and inferior gluteal arteries. The length between the renal artery and terminal aorta was 107 mm. The beginning of the right CIA was segmentally stenotic (13 mm). A bifurcated AFX2 was placed in the infrarenal aorta; IBE was advanced to the origin of the right limb of the AFX2. To control the type 1b endoleak, the right superior gluteal artery was embolized with coils and internal iliac components were deployed toward the inferior gluteal artery. Satisfactory results were obtained in both cases.ConclusionThe AFX2 main body and IBE could be combined to preserve hypogastric blood flow and overcome anatomical limitations.

Left ventricular function assessment after aortic and renal intervention in Takayasu arteritis by speckle tracking echocardiography: A pilot study

BackgroundOvert left ventricular (LV) dysfunction and congestive heart failure are known entities in Takayasu arteritis (TA). Subclinical LV dysfunction may develop in these patients despite normal LV ejection fraction (LVEF). Moreover, effect of treatment of aortic or renal artery narrowing in such patients is unknown. MethodsThis study included 15 angiographically confirmed TA patients undergoing aortic and/or renal intervention. A comprehensive clinical, biochemical and echocardiographic (2-dimensional, speckle tracking and tissue doppler imaging) evaluation were done at baseline, 72 h, and six months post intervention. ResultsSix patients (40%) had reduced LVEF (<50%) at baseline while rest 9 (60%) patients had reduced global longitudinal strain (GLS) but normal EF. Diastolic filling pattern was abnormal in all the patients. In patients with baseline reduced EF, mean EF improved from 24.62 ± 12.14% to 45.6 ± 9.45% (p = 0.001), E/e’ ratio decreased from 15.15 ± 3.19 to 10.8 ± 2.56 (p = 0.005) and median NT pro BNP decreased from 1673 pg/ml (970–2401 pg/ml) to 80 pg/ml (40–354 pg/ml) (p = 0.001) at 6 months after interventional procedure. In patients with baseline normal EF, median NT pro BNP decreased from 512 pg/ml (80–898.5 pg/ml) to 34 pg/ml (29–70.8 pg/ml) (p < 0.01), mean GLS improved from −8.80 ± 0.77% to −16.3 ± 0.78% (p < 0.001) and mean E/e’ decreased from 12.93 ± 2.63 to 7.8 ± 2.73 (p = 0.005) at 6 months follow up. ConclusionLV dysfunction is common in patients with TA and obstructive lesions in aorta or renal arteries. GLS can be used to assess subclinical systolic dysfunction in these patients. Timely intervention can improve LV dysfunction and can even reverse the subclinical changes.

Early myocardial functional abnormalities in primary dyslipidemia: clinical and echocardiographic observations in young children from a highly consanguineous population.

Background Dyslipidemia is a major health problem among children and adolescents worldwide due to its significant association with cardiovascular disease. Primary dyslipidemias are commonly familial syndromes that can be completely asymptomatic.Purpose Apart from the risk of coronary artery disease (CAD), limited data are currently available on the direct effects of dyslipidemia on myocardial function in children.Methods We recruited 25 children with primary dyslipidemia (14 with isolated hypercholesterolemia, 4 with isolated hypertriglyceridemia, and 7 with combined dyslipidemia). Relevant clinical manifestations and laboratory and radiological investigations were evaluated. Pulsed-wave Doppler and tissue Doppler imaging echocardiography were performed for all recruited patients and the results were compared with those of 15 age- and sex-matched healthy children.Results The median age of the dyslipidemic children was 8 years (range, 1.5–16 years). A family history was documented in 13 cases (52%), while 18 (72%) had consanguineous parents. None of the dyslipidemic children had a personal history or clinical manifestations of CAD. In contrast, echocardiographic findings differed in several diastolic function parameters of both right and left ventricles in dyslipidemic children compared to controls. Based on normalized z scores, aortic valve narrowing was detected in 7 patients (28%), while narrowing of the aortic sinus (sinus of Valsalva) was detected in 15 patients (60%).Conclusion Different types of primary dyslipidemia produce functional myocardial abnormalities early in childhood. Biochemical and echocardiographic screening of high-risk children is advised to minimize the incidence of serious cardiovascular complications.

Fetal cardiac evaluation in HIV-positive women under HAART therapy in a Romanian hospital.

Human immunodeficiency virus (HIV) is an RNA (ribonucleic acid) virus that causes acquired immunodeficiency syndrome (AIDS). This disease has a major socioeconomical impact. The aim of the present study was to assess the fetal heart structure and function in HIV-positive pregnant women on highly active antiretroviral therapy (HAART). This is a prospective study conducted between January, 2018 and December, 2019. The hearts of fetuses from 14 HIV-positive pregnant women on HAART were assessed. Statistically significant sex-related cardiac changes in HIV-exposed uninfected fetuses of pregnant women on HAART were found. A narrow aorta was detected in female fetuses and a smaller left ventricular transverse diameter in male fetuses. Overall, the myocardial performance index remained unaltered. The impact of antiretroviral therapy on fetal heart seems to be sex-related. Accurate and complete information about the risks and benefits of antiretroviral treatment and management strategies to improve fetal outcomes may also ensure better compliance of the mothers to this treatment.

Fatal hepatotoxicity due to sevoflurane use in a paediatric patient after aortic repair: reality or fiction? A case of pharmacovigilance

Hepatotoxicity secondary to exposure of volatile anaesthetics is an exceptional finding, but its clinical interest depends on their frequent use, unpredictable appearance and potential severity. Halothane is the volatile anaesthetic...