Coarctation of the aorta from fetus to adult: curable condition or life long disease process?
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ABSTRACT:
SUBMITTER: Rosenthal E
PROVIDER: S-EPMC1769162 | biostudies-literature | 2005 Nov
REPOSITORIES: biostudies-literature
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Coarctation of the aorta from fetus to adult: curable condition or life long disease process?
Heart (British Cardiac Society) 20051101 11
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Project description:Abstract Background Coarctation of the aorta accounts for 5–7% of congenital defects of the heart and great vessels. It requires treatment in the form of open surgical or percutaneous repair. Common long-term complications include re-stenosis and aneurysm formation. The formation of a false aneurysm is a complication with a significant morbidity and mortality. Case summary We reviewed six cases of late false aneurysm after repair of a coarctation of the aorta. Our six cases developed a false aneurysm after an open surgical repair of a coarctation more than 30 years after initial surgical repair. All aneurysms were located at the aortic repair site. Discussion The symptoms or risk factors in the described cases are not uniform and are difficult to include in a general follow-up protocol. Guidelines recommend frequent evaluation, but do not specify duration or intervals of imaging follow-up. Our cases support the necessity of life-long follow-up in patients with open aortic repairs irrespective of symptomatology.
| S-EPMC8881378 | biostudies-literature
Project description:Surgical treatment of childhood intermittent exotropia (XT) is associated with high recurrence rates. In addition, the natural history of intermittent XT has not been rigorously studied and, anecdotally, some cases resolve without surgery. We compared long-term cure rates in children with surgically and non-surgically managed intermittent XT. Children undergoing surgery for intermittent XT who had 5 years follow-up were retrospectively identified. A non-surgical cohort of comparable children was selected by matching each surgical patient for age at onset and age at the 5-year examination. Cure was defined as no manifest tropia on examination or by history, no new monofixation (stereoacuity subnormal for age), and no additional surgery. Each group had 33 children (total follow-up from presentation 7.2±2.6 years in the surgical group vs 6.8±2.3 years). There were no significant differences between groups for age at onset, age at presentation, or distance or near angle of deviation at presentation (all P≥0.4). The cure rate at 5 years was 30% in the surgical group and 12% in the non-surgical group (P=0.1; difference 18%, 95% CI -1 to 37%). Only a small proportion of surgical and non-surgical patients met our definition of cure, with the vast majority demonstrating a constant or intermittent manifest deviation after an average of 7 years follow-up. In childhood intermittent XT, long-term cure is difficult to achieve with surgical intervention, and in some patients managed non-surgically the intermittent XT will spontaneously resolve.
| S-EPMC4330281 | biostudies-literature
Project description:Background Abnormal aortic elastic properties are major notable vasculopathy involved in coarctation of the aorta (CoA). However, there are no reports on aortic wall elastic characteristics in fetuses with CoA. Methods and Results Fifty-six fetuses with CoA and 56 normal controls were included in this prospective case-control study. The dimensions of the cardiac chamber, the size of the aorta, left ventricular myocardial performance indexes, and aortic elastic properties, including the global circumferential strain, fractional area change and mean longitudinal strain, were measured serially in utero. The global circumferential strain, fractional area change, and mean longitudinal strain in fetuses with CoA were smaller than those in the normal group at both the first and last examinations (18.50% versus 37.73% for global circumferential strain, 38.90% versus 57.55% for fractional area change, 6.61% versus 11.81% for mean longitudinal strain at first scan, 16.62% versus 42.05% for global circumferential strain, 36.54% versus 59.7% for fractional area change, 6.2% versus 11.46% for mean longitudinal strain at last scan, all P<0.001). There were negative correlations between aortic elastic properties and left ventricular myocardial performance indexes in fetuses with CoA (P<0.01). Aortic elastic properties were correlated positively with aortic isthmus size in fetuses with CoA (P<0.01). Conclusions Aortic strain and the fractional area change were decreased in fetuses with CoA. Impairments of these aortic elastic properties were associated with diminished heart function and aortic isthmus size in utero. Further large-scale longitudinal studies are required to confirm the potential predictive value of cardiovascular morbidity (ie, hypertension) in fetuses with CoA.
| S-EPMC9939075 | 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: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:Graphical abstract Highlights • Congenital Gerbode defect remains a rare phenomenon.• CoA can worsen shunting due to left ventricular obstruction.• Assess for coexisting congenital defects that may alter management.
| S-EPMC10307586 | biostudies-literature
Project description: Not available
| S-EPMC11584194 | biostudies-literature
Project description:Adults with repaired coarctation of the aorta (CoA) suffer reduced long-term survival compared with the general population, in part due to coronary artery disease (CAD). There is conflicting evidence as to whether or not CoA is an independent risk factor for CAD. The primary aim was to determine if CoA is independently associated with premature myocardial infarction (MI) in the contemporary era. The secondary aim was to determine if CoA is independently associated with early coronary intervention. In a cross-sectional study using the National Inpatient Sample database from 2005 to 2014, we compared the age at MI and the age at coronary intervention (coronary artery bypass grafting or percutaneous coronary intervention, in the absence of MI diagnosis) in patients with and without CoA using weighted linear regression. Among 5,472,416 observations with a primary diagnosis of MI, 174 had a diagnosis of CoA. Patients with CoA had MI 7.2 years younger than those without CoA, after adjusting for potential confounders (95% CI -11.3, -3.1, p = 0.001). Among 3,631,718 patients without a diagnosis of MI who underwent coronary artery bypass grafting or percutaneous coronary intervention, 279 had a diagnosis of CoA. Patients with CoA who underwent coronary intervention were 15.6 years younger than those without CoA, after adjusting for potential confounders (95% CI -18.3, -12.9, p < 0.001). In conclusion, patients with CoA have MI at a slightly younger age and undergo coronary intervention at a significantly younger age than those without CoA in the contemporary era. Our findings support continued close surveillance for and treatment of modifiable risk factors for CAD.
| S-EPMC6260783 | 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
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