-
Journal of Cardiothoracic Surgery Sep 2021Persistent fifth aortic arch (PFAA) is a rare anomaly often associated with aortic coarctation or interruption, and various surgical techniques for this anomaly have...
BACKGROUND
Persistent fifth aortic arch (PFAA) is a rare anomaly often associated with aortic coarctation or interruption, and various surgical techniques for this anomaly have been reported. Herein, we show a case of an infant with PFAA and severe aortic coarctation.
CASE PRESENTATION
A 41-day-old female infant was admitted for sustained fever. Initially, the patient was diagnosed with bacterial meningitis, and echocardiography showed PFAA with severe aortic coarctation. Because the patient presented progressive oliguria and metabolic acidosis, she was transferred for emergency cardiac surgical intervention. The aortic arch was reconstructed using end-to-side anastomosis between the fifth aortic arch and the descending aorta without any artificial conduit or patching material.
CONCLUSIONS
PFAA with aortic coarctation can be repaired by various surgical methods. Among them, our surgical approach is easy and effective, has growth potential, and an additional surgery is not needed.
Topics: Anastomosis, Surgical; Aorta, Thoracic; Aortic Coarctation; Cardiac Surgical Procedures; Echocardiography; Female; Humans; Infant
PubMed: 34583714
DOI: 10.1186/s13019-021-01664-y -
Current Cardiology Reviews Aug 2013Aortic coarctation represents a distinct anatomic obstruction as blood moves from the ascending to the descending aorta and can present in a range of ages from infancy... (Review)
Review
Aortic coarctation represents a distinct anatomic obstruction as blood moves from the ascending to the descending aorta and can present in a range of ages from infancy to adulthood. While it is often an isolated and discrete narrowing, it can also be seen in the more extreme scenario of severe arch hypoplasia as seen in the hypoplastic left heart syndrome or in conjunction with numerous other congenital heart defects. Since the first description of an anatomic surgical repair over sixty years ago, an evolution of both surgical and transcatheter therapies has occurred allowing clinicians to manage and treat this disease with excellent results and low morbidity and mortality. This review focuses on the current state of both transcatheter and surgical therapies, paying special attention to recent data on long-term follow-up of both approaches. Further, current thoughts will be explored about future therapeutic options that attempt to improve upon historical long-term outcomes.
Topics: Anastomosis, Surgical; Angioplasty, Balloon; Aortic Coarctation; Heart Valve Prosthesis Implantation; Humans; Hypoplastic Left Heart Syndrome; Long-Term Care; Stents; Surgical Flaps
PubMed: 23909637
DOI: 10.2174/1573403x113099990032 -
Biomedical Engineering Online May 2020Coarctation of the aorta is a common form of critical congenital heart disease that remains challenging to diagnose prior to clinical deterioration. Despite current...
BACKGROUND
Coarctation 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 RESULTS
Over 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.
CONCLUSIONS
We 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.
Topics: Aortic Coarctation; Female; Humans; Male; Oximetry; Postoperative Period; Signal Processing, Computer-Assisted
PubMed: 32408879
DOI: 10.1186/s12938-020-00775-2 -
BMC Cardiovascular Disorders Feb 2022Middle aortic coarctation (MAC), also known as middle aortic syndrome, is an atypical aortic coarctation characterized by narrowing of the distal thoracic aorta and...
BACKGROUND
Middle aortic coarctation (MAC), also known as middle aortic syndrome, is an atypical aortic coarctation characterized by narrowing of the distal thoracic aorta and proximal abdominal aorta. MAC is a rare disease commonly diagnosed by computed tomography angiography (CTA). In this paper, we present a case of long-segmental MAC first diagnosed by transthoracic echocardiography (TTE) and further evaluated by CTA.
CASE PRESENTATION
A 14-year-old girl, with dyspnea and fatigue on exertion for 2 months and refractory hypertension for 6 months, was referred by the local clinic to our hospital. Physical examination showed blood pressure up to 176/100 mmHg measured in the arms despite dual antihypertensives, a marked pressure gradient between her arms and legs, and weak pulses in both dorsal pedes arteries. TTE revealed a segmental narrowing in the descending thoracic aorta below the level of the atrioventricular sulcus, with a calcified plaque in the stenotic region. Abdominal vascular ultrasound revealed the segmental narrowing extending to the descending abdominal aorta (5.7 mm in diameter) above the level of the superior mesenteric artery. Subsequently, CTA verified a long-segment narrowing in the descending aorta from the level of T8 to L2 vertebra, with a calcified plaque in the stenotic aorta, right renal artery involvement, and a rich network of collateral vessels between the pre-and post-stenotic region. The patient was referred for cardiovascular surgery in which a successful ascending aorta-abdominal aorta bypass was performed.
CONCLUSIONS
Although MAC is usually diagnosed by CTA, it may also be first diagnosed by TTE in some patients whose longitudinal axis view of the thoracic descending aorta could be shown. Careful TTE scan can improve the diagnostic rate of MAC, especially for some hypertension patients whose marked pressure gradient between arms and legs was ignored by the physician.
Topics: Adolescent; Aorta, Thoracic; Aortic Coarctation; Computed Tomography Angiography; Echocardiography; Female; Humans; Rare Diseases
PubMed: 35120452
DOI: 10.1186/s12872-022-02475-2 -
Congenital Heart Disease Sep 2006Understand anatomical and clinical correlatives to coarctation in right aortic arch.
OBJECTIVES
Understand anatomical and clinical correlatives to coarctation in right aortic arch.
BACKGROUND
Coarctation of the aorta is rare in patients with a functional right aortic arch. We reviewed a single institutional experience, examining associated diagnoses, diagnostic methodology, and surgical approaches.
METHODS
A retrospective study was performed of our echocardiographic, magnetic resonance imaging, catheterization, and surgical databases from 1988 to 2001.
RESULTS
Of 240 patients with right aortic arch, 10 (4.1%) had coarctation, constituting 1.9% of all native coarctations (n = 524). Nine (90%) had long-segment hypoplasia. Six (60%) had an aberrant left subclavian artery or retroesophageal diverticulum, 3 (30%) had mirror image branching, and 1 (10%) had a double arch with an atretic left arch. Other congenital heart defects were seen in 6 (60%) comprising 3 with ventricular septal defects, and one each with double-outlet right ventricle, cor triatriatum, and pulmonary valve abnormality. No patients with long-segment hypoplasia had bicuspid aortic valve. Six (60%) had vascular rings, and 5 (50%) had other associated syndromes. Magnetic resonance imaging and/or echocardiography successfully diagnosed all of these patients. Although long-segment right aortic arch coarctation courses behind the trachea posteriorly, only 2 needed an extra-anatomic (jump) graft; the remainders were repaired with patch angioplasty.
CONCLUSION
Coarctation with right aortic arch is rare, constituting 4.1% of all patients with right aortic arch, compared with 5-8% of patients with left aortic arch and congenital heart disease. Nearly all had long-segment hypoplasia without bicuspid aortic valve, and half were part of other syndrome complexes. This association can be diagnosed noninvasively and can often be repaired by patch angioplasty.
Topics: Aorta, Thoracic; Aortic Coarctation; Cardiac Catheterization; Child; Child, Preschool; Comorbidity; Echocardiography, Doppler; Echocardiography, Three-Dimensional; Female; Heart Diseases; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Retrospective Studies
PubMed: 17330153
DOI: 10.1111/j.1747-0803.2006.00038.x -
The Annals of Thoracic Surgery Nov 2005
Topics: Aortic Coarctation; Diagnostic Errors; Humans; Infant; Infant, Newborn; Sensitivity and Specificity; Ultrasonography
PubMed: 16242434
DOI: 10.1016/j.athoracsur.2005.07.011 -
Archivos de Cardiologia de Mexico Apr 2022Aortic coarctation is the most frequent structural anomaly out of congenital heart diseases. This congenital defect is an important cause of death worldwide. We sought...
OBJECTIVES
Aortic coarctation is the most frequent structural anomaly out of congenital heart diseases. This congenital defect is an important cause of death worldwide. We sought to determine the prevalence of aortic coarctation in Colombia and whether new policies have had an impact on its diagnosis.
METHODS
In this study information from the Bogotá birth defect surveillance program during the years 2001-2018 from 63 hospitals was used. 537,026 live births of any weight and stillbirths of any weight were analyzed. The information was stored in a database on the servers of the Health Secretariat and the Pontificia Universidad Javeriana. We analyzed the presence of aortic coarctation according to the newborn's sex, weight, size, mother's age, and gestational age at the time of birth and when coarctation is accompanied by other types of congenital malformations.
RESULTS
The prevalence of aortic coarctation in Bogotá during the years 2001-2018 found in this study was 1.25 in 10,000 live births. We also found that prevalence of aortic coarctation in Bogotá changes throughout the years having a significant increase in the year 2018 with 6.57 cases in 10,000 live births.
CONCLUSIONS
This prevalence is higher than the one found in a study with data from 2001 to 2014, which suggests an improvement in the country's epidemiological surveillance and medical training. However, the prevalence found in Bogotá is still lower compared to the prevalence worldwide and from other continents, the prevalence for Latinamerica was significantly lower as compared to those in Asia, Europe, and United States so we emphasize the importance of continuing with improvements, such as standardizing screening methods and sensitivity of said methods in a local scale as well as a continental scale.
Topics: Aortic Coarctation; Colombia; Gestational Age; Heart Defects, Congenital; Humans; Infant, Newborn; Prevalence
PubMed: 34320622
DOI: 10.24875/ACM.20000515 -
Heart (British Cardiac Society) Sep 2003
Topics: Aortic Coarctation; Aortic Valve; Humans
PubMed: 12922988
DOI: 10.1136/heart.89.9.965 -
Journal of Cardiothoracic Surgery Jan 2016Patients with functional aortic interruption of the descending thoracic aorta at the isthmus due to severe coarctation in association with atretic lumen are extremely... (Review)
Review
BACKGROUND
Patients with functional aortic interruption of the descending thoracic aorta at the isthmus due to severe coarctation in association with atretic lumen are extremely rare in the adult population. The management is challenging and carries high morbidity and mortality.
CASE PRESENTATION
We describe successful percutaneous reconstruction using a covered stent in a similar patient who is doing well two-years after intervention. A literature search was done to explore management strategies and their long-term outcomes for better understanding.
CONCLUSIONS
This report is an attempt to highlight the role of minimal invasive approach in the management of rare, severe coarctation of the aorta in adult patients to avoid morbidity and mortality associated with more invasive procedures.
Topics: Adult; Aortic Coarctation; Cardiac Catheterization; Dilatation; Humans; Male; Radiography; Stents
PubMed: 26781635
DOI: 10.1186/s13019-016-0395-1 -
Journal of the American Heart... Apr 2019Background The long-term prognosis of patients with repaired aortic coarctation is characterized by high rates of cardiovascular and cerebrovascular disease related to...
Background The long-term prognosis of patients with repaired aortic coarctation is characterized by high rates of cardiovascular and cerebrovascular disease related to hypertension, the basis of which remains unclear. To define potential underlying mechanisms, we investigated aortic and carotid arterial biomechanics and wave dynamics, and determinants of aortic systolic blood pressure, in young adults after coarctation repair. Methods and Results Aortic arch and carotid biomechanics, wave intensity and wave power, and central aortic blood pressure, were derived from echocardiography and brachial blood pressure in 43 young adults after coarctation repair and 42 controls. Coarctation subjects had higher brachial and central systolic blood pressure ( P=0.04), while aortic compliance was lower and characteristic impedance (Z) higher. Although carotid intima-media thickness was higher ( P<0.001), carotid biomechanics were no different. Carotid forward compression wave power was higher and was negatively correlated with aortic compliance ( R=0.42, P<0.001) and distensibility ( R=0.37, P=0.001) in coarctation subjects. Aortic wave power and wave reflection indices were no different in control and coarctation patients, but coarctation patients with elevated aortic Z had greater aorto-carotid transmission of forward compression wave power ( P=0.006). Aortic distensibility was the only independent predictor of central aortic systolic blood pressure on multivariable analysis. Conclusions Young adults following coarctation repair had a less compliant aorta, but no change in carotid biomechanics. Reduced aortic distensibility was related to greater transmission of aortic forward wave energy into the carotid artery and higher central aortic systolic blood pressure. These findings suggest that reduced aortic distensibility may contribute to later cardiovascular and cerebrovascular disease after coarctation repair.
Topics: Adult; Aorta; Aortic Coarctation; Arterial Pressure; Biomechanical Phenomena; Carotid Arteries; Echocardiography; Female; Humans; Male; Vascular Stiffness
PubMed: 30929595
DOI: 10.1161/JAHA.118.011411