592609
research-article2015
CPJXXX10.1177/0009922815592609Clinical PediatricsAlsaied et al
Resident Rounds
Asymmetric Pulses in a 5-Year-Old Asian Female: Is It Worth Further Investigations?
Clinical Pediatrics 1–4 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815592609 cpj.sagepub.com
Tarek Alsaied, MD1, Yasasvi Vasili2, Ryan Moore, MD1, Jessica McPhaul, MD1, Pradeep Javarayee, MD1, and Robert Beekman, MD, FAAP1
Case Report A 5-year-old female was seen in the pediatric cardiology clinic following her adoption from China 1 month prior. A cardiovascular assessment was requested due to her pertinent cardiac medical history of ventricular septal defect (VSD), patent ductus arteriosus (PDA), and right aortic arch. She underwent surgical closure of the VSD and ligation of the PDA at age of 2 years in China. Her postoperative echocardiogram in China showed excellent results with no residual lesions. In addition to establishing follow-up cardiology clearance was requested for a rectal biopsy to rule out Hirschsprung’s because of chronic constipation. At the cardiology visit she was described as an active child who is able to keep up well with peers. She had a history of constipation and recurrent abdominal pain. A review of systems was negative for fevers, diaphoresis, emesis, shortness of breath, exercise intolerance, dizziness, syncope, or claudication. Her development was normal and her growth tracked the third percentile for height and weight and was felt to be due to social circumstances. On examination, the patient was found to have a heart rate of 90 beats/min, right upper extremity blood pressure of 130/84 mm Hg, and a right lower extremity blood pressure of 105/57 mm Hg with normal oxygen saturation. The upper extremity pulses were 2+ while both femoral artery pulses were absent, with acyanotic, warm, and well-perfused lower extremities. Auscultation yielded normal S1 and S2 heart sounds with no murmurs, and clear lung fields. Abdominal exam showed mild distension but otherwise was unremarkable. No bruit was appreciated. An electrocardiogram demonstrated normal sinus rhythm. Because of the weak femoral pulses and the significant blood pressure discordance between upper and lower extremities, an echocardiogram was obtained which demonstrated a right aortic arch without thoracic coarctation, a moderately dilated aortic root and ascending aorta, and a blunted abdominal aorta spectral Doppler profile suggestive of abdominal
aortic obstruction; there was no residual shunting from the previously repaired VSD or the PDA. Because of these concerning findings, a magnetic resonance imaging and angiography (MRI/MRA) study of the chest and abdomen was obtained. The MRI/MRA demonstrated severe asymmetric luminal narrowing in the upper abdominal aorta just superior to the celiac and renal arteries and a moderately dilated ascending aorta. It also showed circumferential aortic wall thickening with increase contrast enhancement suggestive of aortic wall inflammation (Figure 1). These findings were most consistent with aortitis and the patient was admitted to the inpatient cardiology service.
Hospital Course Rheumatology and infectious disease services were consulted. An extensive workup was initiated and yielded low inflammatory markers with an erythrocyte sedimentation rate of 4 mm/h and C-reactive protein of 15 mm Hg (53%), vascular bruits (53%), and extremity claudication (48%).10 This study also reported that in 23% of the TA patients laboratory studies were within normal limits, as in our case, even in the presence of active disease.10 Furthermore, 46% of patients had discordance between acute-phase reactant levels and evidence of disease on magnetic resonance imaging; these individuals may have had high levels of reactants but no evidence of active disease, or low levels of reactants and imaging evidence of active disease.10 Treatment for TA is primarily medical management, with intervention when appropriate for localized, symptomatic lesions. Medical management in the pediatric population uses glucocorticoids and methotrexate, with the addition of infliximab or cyclophosphamide for refractory cases.9 Furthermore, use of antiplatelet therapy (aspirin) may be warranted because of the increased presence of inflammatory and thrombogenic factors in patients with TA.9 If intervention is required it can be surgical or endovascular (percutaneous angioplasty with stenting). The endovascular techniques are especially helpful for and have a higher success rate compared with surgery in cases of TA with renal artery stenosis and refractory hypertension.9,11
Conclusion Despite the relative rarity of Takayasu arteritis, it is an important diagnosis to recognize because of the potential for significant morbidity and mortality if left untreated. In our case, simple physical exam findings (diminished and discordant upper and lower extremity pulses and blood pressures) were important indicators of a serious disease entity that warranted further investigation and therapy. This case underscores the importance of a thorough physical examination with vital signs even in an asymptomatic patient. Author Contributions TA is the fellow taking care of the patient and wrote the first draft. YV is the medical student and gathered the data for the case presentation. RM is the imaging fellow who made the diagnosis and helped with the images. JM and PJ are the residents who gathered the references and helped with the discussion part. RB is the attending physician who managed this patient and he saw the patient in clinic. He modified the case significantly as the senior author
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Downloaded from cpj.sagepub.com at University of Sussex Library on July 26, 2015
4
Clinical Pediatrics
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Cronenwett JM, Johnston KW eds. Rutherford’s Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014. 2. Rao PS. Coarctation of the aorta. Semin Nephrol. 1995;15:87-105. 3. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936-941. 4. Gornik HL, Creager MA. Aortitis. Circulation. 2008;117:3039-3051. 5. Wu W, Chaer RA. Nonarteriosclerotic vascular disease. Surg Clin North Am. 2013;93:833-875.
6. Terao C, Yoshifuji H, Mimori T. Recent advances in Takayasu arteritis. Int J Rheum Dis. 2014;17:238-247. 7. Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu’s arteritis. Lancet. 2000;356:1023-1025. 8. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120:919-929. 9. Szugye HS, Zeft AS, Spalding SJ. Takayasu arteritis in the pediatric population: a contemporary United States– based single center cohort. Pediatr Rheumatol Online J. 2014;12:21. 10. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009. 11. Perera AH, Youngstein T, Gibbs RG, Jackson JE, Wolfe JH, Mason JC. Optimizing the outcome of vascular intervention for Takayasu arteritis. Br J Surg. 2014;101: 43-50.
Downloaded from cpj.sagepub.com at University of Sussex Library on July 26, 2015
research-article2015
CPJXXX10.1177/0009922815592609Clinical PediatricsAlsaied et al
Resident Rounds
Asymmetric Pulses in a 5-Year-Old Asian Female: Is It Worth Further Investigations?
Clinical Pediatrics 1–4 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815592609 cpj.sagepub.com
Tarek Alsaied, MD1, Yasasvi Vasili2, Ryan Moore, MD1, Jessica McPhaul, MD1, Pradeep Javarayee, MD1, and Robert Beekman, MD, FAAP1
Case Report A 5-year-old female was seen in the pediatric cardiology clinic following her adoption from China 1 month prior. A cardiovascular assessment was requested due to her pertinent cardiac medical history of ventricular septal defect (VSD), patent ductus arteriosus (PDA), and right aortic arch. She underwent surgical closure of the VSD and ligation of the PDA at age of 2 years in China. Her postoperative echocardiogram in China showed excellent results with no residual lesions. In addition to establishing follow-up cardiology clearance was requested for a rectal biopsy to rule out Hirschsprung’s because of chronic constipation. At the cardiology visit she was described as an active child who is able to keep up well with peers. She had a history of constipation and recurrent abdominal pain. A review of systems was negative for fevers, diaphoresis, emesis, shortness of breath, exercise intolerance, dizziness, syncope, or claudication. Her development was normal and her growth tracked the third percentile for height and weight and was felt to be due to social circumstances. On examination, the patient was found to have a heart rate of 90 beats/min, right upper extremity blood pressure of 130/84 mm Hg, and a right lower extremity blood pressure of 105/57 mm Hg with normal oxygen saturation. The upper extremity pulses were 2+ while both femoral artery pulses were absent, with acyanotic, warm, and well-perfused lower extremities. Auscultation yielded normal S1 and S2 heart sounds with no murmurs, and clear lung fields. Abdominal exam showed mild distension but otherwise was unremarkable. No bruit was appreciated. An electrocardiogram demonstrated normal sinus rhythm. Because of the weak femoral pulses and the significant blood pressure discordance between upper and lower extremities, an echocardiogram was obtained which demonstrated a right aortic arch without thoracic coarctation, a moderately dilated aortic root and ascending aorta, and a blunted abdominal aorta spectral Doppler profile suggestive of abdominal
aortic obstruction; there was no residual shunting from the previously repaired VSD or the PDA. Because of these concerning findings, a magnetic resonance imaging and angiography (MRI/MRA) study of the chest and abdomen was obtained. The MRI/MRA demonstrated severe asymmetric luminal narrowing in the upper abdominal aorta just superior to the celiac and renal arteries and a moderately dilated ascending aorta. It also showed circumferential aortic wall thickening with increase contrast enhancement suggestive of aortic wall inflammation (Figure 1). These findings were most consistent with aortitis and the patient was admitted to the inpatient cardiology service.
Hospital Course Rheumatology and infectious disease services were consulted. An extensive workup was initiated and yielded low inflammatory markers with an erythrocyte sedimentation rate of 4 mm/h and C-reactive protein of 15 mm Hg (53%), vascular bruits (53%), and extremity claudication (48%).10 This study also reported that in 23% of the TA patients laboratory studies were within normal limits, as in our case, even in the presence of active disease.10 Furthermore, 46% of patients had discordance between acute-phase reactant levels and evidence of disease on magnetic resonance imaging; these individuals may have had high levels of reactants but no evidence of active disease, or low levels of reactants and imaging evidence of active disease.10 Treatment for TA is primarily medical management, with intervention when appropriate for localized, symptomatic lesions. Medical management in the pediatric population uses glucocorticoids and methotrexate, with the addition of infliximab or cyclophosphamide for refractory cases.9 Furthermore, use of antiplatelet therapy (aspirin) may be warranted because of the increased presence of inflammatory and thrombogenic factors in patients with TA.9 If intervention is required it can be surgical or endovascular (percutaneous angioplasty with stenting). The endovascular techniques are especially helpful for and have a higher success rate compared with surgery in cases of TA with renal artery stenosis and refractory hypertension.9,11
Conclusion Despite the relative rarity of Takayasu arteritis, it is an important diagnosis to recognize because of the potential for significant morbidity and mortality if left untreated. In our case, simple physical exam findings (diminished and discordant upper and lower extremity pulses and blood pressures) were important indicators of a serious disease entity that warranted further investigation and therapy. This case underscores the importance of a thorough physical examination with vital signs even in an asymptomatic patient. Author Contributions TA is the fellow taking care of the patient and wrote the first draft. YV is the medical student and gathered the data for the case presentation. RM is the imaging fellow who made the diagnosis and helped with the images. JM and PJ are the residents who gathered the references and helped with the discussion part. RB is the attending physician who managed this patient and he saw the patient in clinic. He modified the case significantly as the senior author
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Downloaded from cpj.sagepub.com at University of Sussex Library on July 26, 2015
4
Clinical Pediatrics
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Cronenwett JM, Johnston KW eds. Rutherford’s Vascular Surgery. 8th ed. Philadelphia, PA: Elsevier Saunders; 2014. 2. Rao PS. Coarctation of the aorta. Semin Nephrol. 1995;15:87-105. 3. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936-941. 4. Gornik HL, Creager MA. Aortitis. Circulation. 2008;117:3039-3051. 5. Wu W, Chaer RA. Nonarteriosclerotic vascular disease. Surg Clin North Am. 2013;93:833-875.
6. Terao C, Yoshifuji H, Mimori T. Recent advances in Takayasu arteritis. Int J Rheum Dis. 2014;17:238-247. 7. Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu’s arteritis. Lancet. 2000;356:1023-1025. 8. Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern Med. 1994;120:919-929. 9. Szugye HS, Zeft AS, Spalding SJ. Takayasu arteritis in the pediatric population: a contemporary United States– based single center cohort. Pediatr Rheumatol Online J. 2014;12:21. 10. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56:1000-1009. 11. Perera AH, Youngstein T, Gibbs RG, Jackson JE, Wolfe JH, Mason JC. Optimizing the outcome of vascular intervention for Takayasu arteritis. Br J Surg. 2014;101: 43-50.
Downloaded from cpj.sagepub.com at University of Sussex Library on July 26, 2015
