Intramuscular hematomas on the psoas muscle are rare and usually occur as a result of trauma, iatrogenic etiology during lumbar surgery, rupture of the aortic aneurysm, and hematologic diseases. The incidence of spontaneous psoas muscle hematomas has slowly increased as a result of using anticoagulation and antiplatelet agents. Magnetic resonance (MR) imaging is a more sensitive option compared to computed tomography (CT) when diagnosing a hematoma. Coronal T2-weighted images are more useful. CT imaging is also useful to establish the rapid diagnosis of hematoma. When a prolonged prothrombin time and international normalized ratio and decrease platelet count are noted, psoas muscle hematomas should be considered, if there was no lesion in the spinal canal. Most hematomas resolve spontaneously without clinical complications if the hematoma is not large or it is not compressing the surrounding important structures, irrespective of cause.
Aortic Aneurysm ; Diagnosis ; Hematologic Diseases ; Hematoma ; Incidence ; International Normalized Ratio ; Magnetic Resonance Imaging ; Platelet Aggregation Inhibitors ; Platelet Count ; Prothrombin Time ; Psoas Muscles ; Rupture ; Spinal Canal

PURPOSE: Coronary arterial lesion assessment in children can be difficult, depending on the coronary dominance pattern. Although it is easier to determine coronary dominance with echocardiography in children than in adults, it is still difficult. This study aimed to examine the coronary dominance pattern according to the objective coronary artery (CA) indices. METHODS: The CA diameter, aortic valve annulus, and abdominal aorta of 69 children without any cardiovascular disease were measured with cross-sectional echocardiography at Chungnam National University Hospital. To evaluate the coronary dominance pattern, echocardiography was primarily used; additionally, coronary computed tomographic angiography or coronary angiography (CAG). Coronary dominance was determined according to the status of the CA that gives rise to the posterior descending artery. RESULTS: The mean age was 4.02±2.78 years, and the mean body surface area (BSA) was 0.70±0.22 m². Right dominance was present in 78% and left in 22% of the subjects. In those with left dominance, the CA to aortic valve annulus diameter ratio was 0.125±0.021 in the right coronary artery (RCA) and 0.255±0.032 in the left coronary artery (LCA). In those with right dominance, the corresponding ratio was 0.168±0.028 in the RCA and 0.216±0.030 in the LCA (P<0.05). Significant differences were also found in the diametric ratios of the CA to BSA and abdominal aorta (P<0.05). CONCLUSION: The CA indices showed significant difference according to the coronary dominance pattern in early childhood. It is possible to indirectly determine the coronary dominance pattern with the CA indices in children using echocardiography. The accuracy of coronary artery lesion diagnosis can be improved by taking coronary dominance into account.
Adult ; Angiography ; Aorta, Abdominal ; Aortic Valve ; Arteries ; Body Surface Area ; Cardiovascular Diseases ; Child ; Chungcheongnam-do ; Coronary Angiography ; Coronary Vessels ; Diagnosis ; Echocardiography ; Humans ; Mucocutaneous Lymph Node Syndrome

Endovascular aortic repair is often performed for the treatment of aortic disease because of less invasiveness and fewer complications. Cardiac tamponade is a fatal disease that can lead to death if not treated properly. Cardiac puncture by rigid guide wire used in endovascular aortic repair may cause cardiac tamponade. Rapid diagnosis and treatment are needed when cardiac tamponade occurs. Confirmation of the cardiac tamponade can be accomplished with echocardiography. Continuous echocardiography should be monitor for detection of cardiac complications during endovascular aortic repair.
Aneurysm, Dissecting ; Aortic Diseases ; Cardiac Tamponade ; Diagnosis ; Echocardiography ; Endovascular Procedures ; Punctures ; Rupture

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
Adult ; Aortic Coarctation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Constriction, Pathologic ; Diagnosis ; Female ; Follow-Up Studies ; Heart ; Heart Defects, Congenital ; Humans ; Mitral Valve

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.
Aorta, Thoracic ; Aortic Coarctation ; Arrhythmias, Cardiac ; Arteries ; Birth Rate ; Echocardiography ; Fetus ; Heart Defects, Congenital ; Heart Diseases ; Hypoplastic Left Heart Syndrome ; Parturition ; Prenatal Diagnosis ; Pulmonary Atresia ; Surgeons ; Tetralogy of Fallot

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.
Aneurysm ; Aorta ; Aortic Aneurysm ; Arteries ; Bile Ducts ; Connective Tissue ; Connective Tissue Diseases ; Diagnosis ; Dilatation ; Early Diagnosis ; Ehlers-Danlos Syndrome ; Exons ; Hernia, Hiatal ; Humans ; Infant, Newborn ; Joint Instability ; Korea ; Loeys-Dietz Syndrome ; Magnetic Resonance Angiography ; Marfan Syndrome ; Mass Screening ; Parents ; Sequence Analysis, DNA ; Vascular Malformations ; Vena Cava, Inferior

PURPOSE: Acute aortic diseases, such as aortic dissection and aortic aneurysm, can be life-threatening vascular conditions. In this study, we compared outcomes before and after the implementation of a critical pathway (CP) for patients with acute aortic disease at the emergency department (ED). MATERIALS AND METHODS: This was a retrospective observational cohort study. The CP was composed of two phases: PRE-AORTA for early diagnosis and AORTA for prompt treatment. We compared patients who were diagnosed with acute aortic disease between pre-period (January 2010 to December 2011) and post-period (July 2012 to June 2014). RESULTS: Ninety-four and 104 patients were diagnosed with acute aortic disease in the pre- and post-periods, respectively. After the implementation of the CP, 38.7% of acute aortic disease cases were diagnosed via PRE-AORTA. The door-to-CT time was reduced more in PRE-AORTA-activated patients [71.0 (61.0, 115.0) min vs. 113.0 (56.0, 170.5) min; p=0.026]. During the post-period, more patients received emergency intervention than during the pre-period (22.3% vs. 36.5%; p=0.029). Time until emergency intervention was reduced in patients, who visited the ED directly, from 378.0 (302.0, 489.0) min in the pre-period to 200.0 (170.0, 299.0) min in the post-period (p=0.001). The number of patients who died in the ED declined from 11 to 4 from the pre-period to the post-period. Hospital mortality decreased from 26.6% to 14.4% in the post-period (p=0.033). CONCLUSION: After the implementation of a CP for patients with acute aortic disease, more patients received emergency intervention within a shorter time, resulting in improved hospital mortality.
Acute Disease ; Adult ; Aged ; Aneurysm, Dissecting/diagnosis/mortality/*surgery ; Aorta ; Aortic Aneurysm/diagnosis/mortality/*surgery ; Aortic Diseases/diagnosis/mortality/*surgery ; *Critical Pathways ; Emergency Service, Hospital/*organization & administration ; Female ; Hospital Mortality ; Humans ; Male ; Middle Aged ; Outcome and Process Assessment (Health Care) ; Postoperative Complications/mortality ; Republic of Korea/epidemiology ; Retrospective Studies ; Time Factors ; Treatment Outcome ; Vascular Surgical Procedures/*methods

Doctors are often faced with difficult decisions and uncertainty when patients need a certain treatment. They routinely rely on the scientific literature, in addition to their knowledge, experience, and patient preferences. Clinical practice guidelines are created with the intention of facilitating decision-making. They may offer concise instructions for the diagnosis, management (medical or surgical treatments), and prevention of specific diseases or conditions. All information included in the final version are the result of a systematic review of scientific articles and an assessment of the benefits and costs of alternative care options. The final document attempts to meet the needs of most patients in most circumstances and clinicians, aware of these recommendations, should always make individualized treatment decisions. In this review, we attempted to define the intent and applicability of clinical practice guidelines, expert consensus documents, and registry studies, focusing on the management of patients with thoracic aortic disease.
Aorta ; Aortic Diseases* ; Consensus* ; Cost-Benefit Analysis ; Diagnosis ; Evidence-Based Practice ; Guidelines as Topic ; Humans ; Intention ; Patient Preference ; Registries* ; Uncertainty

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.
Antihypertensive Agents ; Aorta, Abdominal ; Aortic Coarctation ; Blood Pressure ; Child ; Child, Preschool* ; Constriction, Pathologic ; Diagnosis ; Early Diagnosis ; Humans ; Hypertension ; Hypertension, Malignant* ; Hypertension, Renovascular ; Hypertrophy, Left Ventricular ; Korea ; Lower Extremity ; Male* ; Physical Examination ; Renal Artery Obstruction ; Upper Extremity ; Vascular Diseases

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.
Abnormalities, Multiple ; Aortic Coarctation ; Aortic Valve Stenosis ; Cause of Death ; Counseling ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Genetic Testing ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Hypoplastic Left Heart Syndrome ; Intellectual Disability ; Mitral Valve Stenosis ; Prevalence ; Retrospective Studies