Aortic dissection in children: case report and literature review
Highlight box
Key findings
• The clinical symptoms of aortic dissection in children are often atypical compared to those in adults, contributing to misdiagnoses and treatment delays. The prognosis for children with aortic dissection after treatment is generally better than that for adults. Surgical treatment should be promptly performed once the condition is diagnosed. In children with non-traumatic aortic dissection, the underlying condition is likely to be a hereditary connective tissue disorder, and genetic test is recommended.
What is known and what is new?
• There are currently very few reported cases of aortic dissection in children under age 16 and a lack of relevant statistics. No expert consensus has yet been reached on the pathology, clinical presentation, and surgical approach selection.
• The prognosis for children with aortic dissection after treatment is generally better than that for adults.
What is the implication, and what should change now?
• In pediatric emergency settings, persistent chest pain that does not alleviate and cannot be attributed to common illnesses warrants a heightened suspicion of aortic dissection. Early elevation of D-dimers has diagnostic value, and aortic computed tomography angiography significantly aids in diagnosis.
Introduction
Aortic dissection refers to necrosis or degeneration of the middle layer of the aortic wall. When the intima is damaged or ruptured, blood enters the middle layer and forms a hematoma extending distally, causing the vessel wall to tear continuously. While aortic dissection is common in adults (1), it is a comparatively rare but life-threatening disease in children that is often associated with connective tissue disorders, congenital heart disease, and chest trauma (2). Aortic dissection progresses rapidly, and diagnosis depends on imaging examinations, specifically aortic computed tomography angiography (CTA). Once diagnosed, most patients require surgical treatment, and the risk of death can be extremely high without timely management. Researchers have indicated that aortic dissection in young individuals occurs more commonly between the ages of 0 to 5 and 15 to 20 years, with a higher incidence in males and a mortality rate of approximately 13% to 17% (3,4). Here, we reported three cases of aortic dissection treated at our center in 2024. We retrieved 27 reports describing 34 cases of aortic dissection in patients aged ≤16 years published from 1990 to 2024 to analyze and summarize the characteristics of precipitating factors, aortic dissection status, clinical presentations, and so on to enhance the diagnostic awareness of aortic dissection in children with acute chest pain or trauma, avoid delayed diagnosis and the subsequent worsening of clinical outcome. We present this article in accordance with the CARE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-24-460/rc).
Case presentation
Case 1
A 13-year-old boy (65 kg, 166 cm) presented at our hospital with persistent chest pain. He experienced left-sided chest pain after running, which extended to the left side of the lower back and was accompanied by profuse sweating and tachypnea. As the patient’s chest pain progressively worsened, he was transferred to our center. Significantly elevated blood pressure in the boy’s right upper limb was detected with measurements of 113/51 mmHg in the left upper limb, 128/64 mmHg in the left lower limb, 171/114 mmHg in the right upper limb, and 122/66 mmHg in the right lower limb, respectively. The possibility of aortic dissection was soon considered, and a full aortic CTA was performed. The report indicated aortic dissection (Stanford Type A; Figure 1A,1B), and the patient was admitted to the pediatric intensive care unit. The patient received midazolam and dexmedetomidine for sedation, analgesia with dezocine, oxygen therapy, sodium nitroprusside for blood pressure reduction, and esmolol for heart rate control. Nevertheless, his chest pain persisted without relief.

On the second day of hospitalization, the patient underwent emergency descending aortic-covered stent graft placement and ascending aortoplasty with deep hypothermic cardiopulmonary bypass. Intraoperative exploration revealed a hematoma, dilation of the ascending aorta, and significant widening of the descending aorta located at the small curve of the left subclavian artery opening measuring approximately 2.5 cm. The rupture was sutured and reinforced, and a No. 24 covered stent was inserted into the descending aorta to completely cover the aortic rupture. The covered stent was intermittently sutured and secured within the descending aorta. The ascending aorta was reinforced with sutures, and the incision in the descending aorta was closed. Stent selection is usually based on normal vessel diameter. However, considering that the patient’s physical development was similar to that of an adult, a larger stent was chosen to avoid the possibility of future vascular replacement due to relative stent stenosis.
On postoperative day (POD) 0, the patient exhibited signs of acute kidney injury (AKI) including oliguria and elevated creatinine levels. Both urine output and creatinine levels improved after seven days of continuous renal replacement therapy (CRRT). On POD 3, follow-up CTA of the large vessels indicated that the blood flow within the stent was smooth and that the false lumen had decreased (Figure 1C,1D). On POD 7, the patient was weaned off the ventilator before being transferred to the pediatric cardiovascular surgery ward on POD 14. The patient was discharged on POD 30. At the 6-month follow-up, the patient’s renal function had returned to normal, and he was currently receiving amlodipine to control his blood pressure. Whole-exome sequencing indicated a mutation in PKD1 at nucleotide position NM_001009944.2:c.6899G>A(p.Cys2300Tyr), correlating with a diagnosis of polycystic kidney disease type 1.
Case 2
A 9-year-old boy (35 kg, 135 cm) was admitted to a local hospital following a traffic accident. He presented with multiple injuries throughout the body, clouding of consciousness, agitation, and inability to respond normally. The patient was transferred to our hospital for additional treatment due to his critical condition. Aortic CTA showed a localized irregular bulging and dilation with nodular protrusions in the aortic arch (distal to the left subclavian artery opening) and the lumen in the proximal descending aorta. Incomplete membrane-like shadows were observed at both ends of the affected vessels. A traumatic localized aortic dissection was suspected (Figure 2A,2B). Esmolol was used to control the heart rate, and sodium nitroprusside was used for blood pressure management. Treatment also included anti-infection measures, organ protection, fluid resuscitation, blood transfusion therapy, hemostasis, electrolyte balance, and maintenance of homeostasis.

Given the high risk of a large aneurysm rupture, the patient underwent emergency descending abdominal aortic replacement with prosthetic vascular graft. During the surgery, the left subclavian artery-left femoral artery bypass was established as an extracorporeal temporary conduit to ensure blood supply to the lower body, and it was immediately removed after the surgery. Both ends of the thoracic aortic pseudoaneurysm were separately occluded and distal perfusion was performed via the femoral artery. An incision was made at the enlarged section of the thoracic aorta, revealing a traumatic pseudoaneurysm at the origin of the thoracic aorta with partial dissection that was shaped like a gourd and measured approximately 6 cm in length. Scattered thrombi were observed within the walls and removed. Considering the potential for relative stenosis of the artificial vessel in the future owing to the child’s growth and development, we selected the largest caliber of the artificial vessels within the safe range based on the current vascular conditions to avoid or postpone the possibility of another vascular replacement. A No. 16 artificial vessel was then anastomosed end-to-end with both ends of the thoracic aorta to complete thoracic aortic replacement. The patient’s postoperative treatment included hemostasis, anti-infection measures, sedation, and analgesia. The patient was extubated on POD 3 and transferred to the pediatric surgical ward on POD 7. The patient was discharged on POD 27, because he underwent two orthopedic surgeries after the heart surgery. At his 6-month follow-up, the patient’s general condition was good, and no complications related to vascular transplantation were observed after the surgery. He is undergoing rehabilitation training (Figure 2C,2D).
Case 3
An 11-year-old boy (30 kg, 146 cm) presented to a local hospital with persistent chest pain after running. The symptoms were accompanied by pallor, tachypnea, and vomiting. He had never undergone echocardiography before this incident and denied a history of heart disease. He was treated with “nitroglycerin” at a local hospital, but the symptoms showed no significant improvement. Owing to his critical condition, the patient was transferred to our hospital. The patient’s blood pressure was normal on examination. He exhibited a distinctive appearance with a noticeably increased distance between the orbits and bifid uvula. He was slim with slender limbs and a funnel chest. No typical heart murmurs were auscultated on admission. Echocardiography showed significant widening of the aortic sinuses and ascending aorta with an aneurysm at the aortic root and membranous echoes within the aorta, suggesting the possibility of dissecting aneurysm. A patent ductus arteriosus and a small pericardial effusion were also detected. Aortic CTA showed pericardial effusion, aneurysmal dilation of the aortic sinuses and root of the ascending aorta, and localized dissection at the aortic root (Figure 3A,3B). Sodium nitroprusside was administered to control blood pressure, esmolol to manage the heart rate, dexmedetomidine for sedation, and dezocine for analgesia.

After prompt preoperative preparation, an emergency Bentall procedure was performed with deep hypothermic cardiopulmonary bypass. Intraoperative exploration revealed a hemopericardium, significant dilation of the ascending aorta, and an approximately 10 cm horizontal intimal tear in the ascending aorta. After resecting the affected vessel, a synthetic valved conduit was used to replace the ascending aorta and aortic valve, with anastomosis of the left and right coronary artery openings to the synthetic conduit. The patient received anticoagulation therapy with warfarin beginning on POD 2, and was extubated on POD 3. On POD 4, follow-up vascular CTA revealed changes after the synthetic graft replacement. The patient was transferred to the general ward on POD 7. The patient’s overall condition was stable (Figure 3C,3D). Whole-exome sequencing indicated a mutation in TGFBR1 at nucleotide position NM_004612.2:c.683_685delAAG(p.Glu228del), correlating with a diagnosis of Loeys-Dietz syndrome (LDS) type 1.
Ethical considerations
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patients’ legal guardians for publication of this case report and accompanying images. A copy of the written consent was available for review by the editorial office of this journal.
Discussion
Aortic dissection is an acute, critical, and severe condition, with an incidence of 5–6 per 100,000 individuals and a high mortality rate. There is a paucity of literature regarding pediatric cases, and only approximately half of the affected children have undergone surgical interventions (2). There are currently very few reported cases of aortic dissection in children under the age of 16 years, and there is a lack of relevant statistics. No expert consensus has yet been reached regarding pathology, clinical presentation, and surgical approach selection. To better understand the disease, we systematically reviewed relevant literature published from 1990 to 2004. We searched the Medline database using PubMed and platforms such as Springer Link and Google Scholar with the search terms “aortic dissection” and “pediatric” or “children”. A total of 27 articles that presented 34 reported cases of aortic dissection in pediatric patients were included in the study (Table 1).
Table 1
Author | Publication year | Age | Gender | Precipitating factor | Aortic dissection status | Clinical presentations | Underlying condition | Treatment | Complications/outcomes |
---|---|---|---|---|---|---|---|---|---|
Aru (5) | 2024 | 16 years | Female | Unclear | Stanford Type B | – | LDS | Endovascular fenestration and stenting | Renal failure, survival |
Perez (6) | 2023 | 14 years | Male | Physical activity | Stanford Type A | Tachypnea, headache, sweating | LDS | Aortic replacement | Survival |
Mangold (7) | 2022 | 4 years | Female | Traffic injuries | Abdominal aorta | Unclear | None | Patch repair | Survival |
Rathnayake (8) | 2022 | 15 years | Male | Unclear | Stanford Type A | Chest pain, dyspnea, and sweating | Autism spectrum disorder, obesity | Bentall procedure | Survival |
Liang (9) | 2021 | 10 years | Female | Infection | Stanford Type A | Headache, nausea, chest pain, pallor, tachypnea | Infective endocarditis | Ascending aortic replacement | Survival |
Datta (10) | 2022 | 3 years | Male | Infection | Ascending aorta | Cough, vomiting | Arteritis | Conservative treatment | Death |
Dueppers (11) | 2022 | 9 years | Male | Unclear | Stanford Type B | Chest pain | LDS | Aortic replacement | Survival |
Baturone (12) | 2021 | 12 years | Male | Unclear | Abdominal aorta | Abdominal pain | None | Abdominal aortic replacement | Renal failure, survival |
Matsushita (13) | 2021 | 11 years | Male | Postprandial | Stanford Type B | Abdominal pain | Suspicious LDS | Descending aortic replacement | Renal impairment, survival |
Comentale (14) | 2021 | 11 months | Male | Unclear | Stanford Type A | Unclear | LDS | Bentall procedure | Survival |
Huang (15) | 2020 | 11 years | Male | Hypertension | DeBakey Type I | Chest pain | Hypertension | Treatment discontinuation | Lost to follow-up |
Kazimierczak (16) | 2018 | 11 years | Female | Hypertension | Stanford Type B | Chest and abdominal pain | Hypertension | Stent implantation | Renal failure, survival |
Wadia (17) | 2015 | 15 years | Male | Unclear | Stanford Type B | Fatigue, weakness, dyspnea | Suspicious connective tissue disease | Conservative treatment | Death |
Papazoglou (18) | 2015 | 9 years | Male | Traffic injuries | Abdominal aorta | Abdominal pain, paraplegia | None | Stent implantation | Paraplegia, survival |
Parrish (19) | 2015 | 12 years | Male | Traffic injuries | Abdominal aorta | Pain, coma | None | Conservative treatment | Paraplegia, survival |
Besli (20) | 2015 | 14 years | Male | Unclear | Stanford Type B | Abdominal pain, dyspnea, and sweating | Obesity, familial thoracic aortic aneurysm and dissection | Conservative treatment | Death |
Okawa (21) | 2013 | 12 years | Male | Unclear | Stanford Type A | Shoulder and back pain, pallor | None | Hemiarch replacement | Survival |
Geiger (22) | 2011 | 17 days | Male | Cardiac surgery | Stanford Type B | Unclear | Congenital heart disease, aortopulmonary window | Conservative treatment | Survival |
Eun (2) | 2011 | 11 years | Male | Physical activity | Stanford Type B | Chest pain, shortness of breath, and sweating | Obesity, IgA nephropathy | Conservative treatment | Death |
Heck (23) | 2009 | 1 year | Male | Traffic injuries | Abdominal aorta | Unclear | None | Conservative treatment | Survival |
Civilibal (24) | 2008 | 9 years | Female | Infection | Abdominal aortic dissection | – | Takayasu arteritis | Conservative treatment | Survival |
Khanna (25) | 2007 | 7 years | Male | Traffic injuries | Abdominal aorta | Abdominal pain | None | Conservative treatment | Survival |
Hibino (26) | 2006 | 3 years | Male | Post-cardiac surgery | Ascending aorta | Unclear | Atrial septal defect | Conservative treatment | Survival |
Laporte-Turpin (27) | 2005 | 13 years | Male | Hypertension | Stanford Type B | Chest pain | Ehlers-Danlos syndrome, hypertension | Aortic replacement | Hemorrhage, death |
Zalzstein (28) | 2003 | 1 day | Male | Post-cardiac surgery | Thoracic aorta | Intestinal ischemia | Post-TOF | Conservative treatment | Death |
2 years | Male | Post-cardiac surgery | Ascending aorta | Intestinal ischemia | Post-TOF | Conservative treatment | Death | ||
8 months | Female | Post-cardiac surgery | Thoracic aorta | Lower limb ischemia | PDA, extracorporeal circulation | Conservative treatment | Survival | ||
15 years | Male | Unclear | Ascending aorta | None | Marfan syndrome | Surgery | Survival | ||
12 years | Male | Trauma | Ascending aorta | Chest pain | Trauma, no underlying conditions | Surgery | Survival | ||
7 years | Female | Traffic injuries | Ascending aorta | Coma | Road traffic injury, no underlying conditions | Conservative treatment | Death | ||
14 years | Male | Traffic injuries | Abdominal aorta | Lower limb ischemia | Road traffic injury, no underlying conditions | Surgery | Survival | ||
8 years | Female | Unclear | Ascending aorta | Chest pain | Aortic coarctation | Unclear | Death | ||
Vogt (29) | 1999 | 13 years | Male | Hypertension | Abdominal aorta | Renal dysfunction | Renal diseases, hypertension | Stent implantation | Death |
Griggs (30) | 1990 | 15 years | Male | Physical activity | DeBakey Type III | Abdominal and lower back pain | Renal diseases | Conservative treatment | Hemorrhage, death |
IgA, immunoglobulin A; LDS, Loeys-Dietz syndrome; PDA, patent ductus arteriosus; TOF, tetralogy of Fallot.
Among the reported cases, the mean age was 109±60.5 months, and the youngest patient was a newborn. Eight (23.5%) were female. The mortality rate was 32.4% (11/34). The most common type of aortic dissection was Stanford type B, with the abdominal aorta being the most frequently affected site. Adult cases are often associated with hypertension, hyperlipidemia, and atherosclerosis (2), whereas pediatric cases are primarily associated with congenital cardiovascular diseases, hereditary connective tissue disorders, and genetic conditions, such as aortic diseases, LDS, Marfan syndrome, and Ehlers-Danlos syndrome, and some may also occur as a result of severe trauma or iatrogenic injury (3). Aortic dissection associated with hypertension in children accounted for approximately 8.8% (four cases) of the reported cases, which was significantly lower than the 18% reported in adults studies (3). These findings supported the argument that the causes of aortic dissection differ between adults and children. However, the annual increase in the incidence of pediatric hypertension may lead to an increased prevalence of related aortic dissections. Accordingly, relevant guidelines recommend regular blood pressure monitoring of children starting at age three and earlier examinations and treatment for those at high risk (31).
The cases presented in the literature mainly presented with chest and abdominal pain and dyspnea, and the documented symptoms were consistent with those of the three cases we treated. Most pediatric cases of spontaneous aortic dissection typically present with thoracoabdominal pain as an initial symptom and are frequently precipitated by physical activities. During physical activities, sympathetic nervous system activation leads to an increased heart rate and elevated blood pressure, which may cause rupture in the aorta weakened by previous lesions, resulting in aortic dissection. Therefore, when evaluating pediatric patients with chest pain, especially those presenting after physical activities, relevant auxiliary examinations should be promptly conducted if persistent symptoms cannot be adequately explained by patient history and physical examination. Simultaneously, timely measurement of blood pressure in the extremities should be performed to assess any discrepancies. If there are blood pressure inconsistencies in the extremities or signs of ischemia in certain organs, aortic dissection should be considered.
The coagulation mechanisms in all cases at our center exhibited varying degrees of abnormalities, primarily characterized by elevated D-dimers. However, most cases reported in the literature did not report laboratory results. Eight cases (23.5%) had relatively comprehensive statistics, of which five (14.7%) had varying degrees of elevated D-dimers, most of which were >500 µg/L. The test results were also consistent with research reports on adults, and could be promoted as a preliminary screening criteria standard for children with acute chest pain (32).
The majority of the cases reported in the literature were diagnosed as aortic dissection using CTA. Among them, five cases also showed aortic dilation and dissection using echocardiography; however, this type of lesion could only be detected when the ascending aorta was involved. Several patients were diagnosed using digital subtraction angiography (DSA; three cases), magnetic resonance angiography (MRA; two cases), or transesophageal echocardiography (TEE; one case). CTA demonstrated sensitivity in diagnosing aortic dissection and could clearly identify the type, extent of involvement, and even ruptures. However, for certain suspected cases of aortic dissection, angiography could provide definitive clarification (33). Echocardiography could also be used as a preliminary screening method in pediatric patients with lesions in the ascending aorta (10).
Currently, there are no specific guidelines for the treatment of aortic dissection in children, and its management largely relies on adult treatment protocols, including pain management, heart rate control, and blood pressure reduction. Timely surgical intervention is necessary when indications for surgery arise (1). Surgical approaches, including aortic replacement, endovascular repair, and covered stent implantation, should be selected based on the clinical classification of pediatric patients. Generally, children with aortic dissection who undergo surgical treatment have a better prognosis than those who receive conservative management, and the results from our center were consistent with the findings reported in the literature (34). Compared with adults, children with aortic dissection have a higher survival rate and better prognosis, which may be related to the differences in pathological changes and etiological factors between adults and children (35). According to the scientific statement on pediatric aortic disease published by Morris et al., even if aortic dissection was treated in time during the acute phase, patients still needed long-term follow-up and management, including regular imaging examinations, to ensure that the dissection does not recur or to promptly detect any new lesions. Additionally, blood pressure monitoring, lifestyle management, and psychological support should be further consideration included (36).
Conclusions
In summary, aortic dissection is a rare condition in children. Although the clinical symptoms in children are often atypical compared with those in adults, contributing to misdiagnoses and treatment delays, the prognosis for children with aortic dissection after treatment is generally better than that for adults. In pediatric emergency settings, persistent chest pain that does not alleviate and cannot be attributed to common illnesses warrants a heightened suspicion of aortic dissection. Early elevation of D-dimers has diagnostic value, and aortic CTA significantly aids in diagnosis. DSA can be performed in complex cases. Surgical treatment should be promptly performed once diagnosed. In children with non-traumatic aortic dissection, the underlying condition is likely to be a hereditary connective tissue disorder, and genetic test should be further consideration.
Acknowledgments
The authors thank the resident doctors, chief residents, and nurses at Shanghai Children’s Medical Center Guizhou Hospital, Shanghai Jiao Tong University, School of Medicine, for their care of the patients in the Department of Pediatric Critical Care and Department of Pediatric Cardiovascular Surgery.
Footnote
Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-24-460/rc
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Funding: This study was supported by
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-24-460/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patients’ legal guardians for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
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