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Management of severe hypertension in childhood Takayasu's arteritis
L S Milner1, D W Jacobs, P D Thomson
1Department of Pediatrics and Pediatric Surgery, Baragwanath Hospital, University of Witwatersrand, South Africa.
Insights
Takayasu's arteritis in children can cause severe hypertension, often due to renal artery narrowing. Surgical interventions show mixed results, and ACE inhibitors require caution, with a guarded long-term prognosis for extensive vascular disease.
Area of Science:
- Pediatric Cardiology
- Vascular Surgery
- Rheumatology
Background:
- Takayasu's arteritis (TA) is a rare, chronic inflammatory disease affecting large arteries, primarily the aorta and its branches.
- In pediatric populations, TA can manifest with severe hypertension, often secondary to renal artery stenosis or aortic coarctation.
- Diagnosis is supported by clinical presentation, imaging, and immunological markers, such as a positive purified protein derivative (PPD) test.
Purpose of the Study:
- To describe the clinical presentation and management outcomes of severe hypertension in children with Takayasu's arteritis.
- To evaluate the efficacy and limitations of various surgical interventions for renovascular hypertension in this cohort.
- To highlight potential therapeutic challenges and long-term prognostic considerations.
Main Methods:
- Retrospective case series analysis of six children diagnosed with Takayasu's arteritis and severe hypertension.
- Review of clinical data, including presenting symptoms, diagnostic findings, and treatment strategies.
- Assessment of surgical outcomes, including renal artery bypass grafts, renal autotransplantation, and aortic patch grafting.
Main Results:
- Four patients had bilateral renal artery narrowing, and two presented with coarctation syndrome, leading to severe hypertension.
- Surgical interventions like renal artery bypass grafts provided temporary blood pressure normalization but failed long-term due to clotting.
- Renal autotransplantation had variable success, while aortic patch grafting was successful in one case.
- Two patients experienced hypertensive encephalopathy, and four had congestive cardiac failure.
Conclusions:
- Severe hypertension in pediatric Takayasu's arteritis presents significant management challenges, often linked to renovascular complications.
- Surgical revascularization strategies have limited long-term efficacy, with graft failure being a common issue.
- Caution is advised when using angiotensin-converting-enzyme inhibitors due to the risk of renal insufficiency in cases of bilateral renal artery stenosis.
- The long-term prognosis for children with extensive vascular involvement from Takayasu's arteritis remains guarded.
Abstract:
Six children presented with severe hypertension caused by Takayasu's arteritis (TA), of whom four had bilateral renal artery narrowing and two coarctation syndrome. Two presented with hypertensive encephalopathy and four with congestive cardiac failure. All had a strongly positive skin reactions to purified protein derivative of mycobacterium tuberculosis. Bilateral renal arterial bypass grafts performed in two children resulted in prolonged normalization of their blood pressures, but the grafts clotted 12-18 months later. Primary renal autotransplantation was unsuccessful in two children, one with bilateral renal arterial narrowing and iliac vessel involvement and one with a long coarctation. Secondary renal autotransplantation was successful in a third child with localized aortitis. A successful aortic patch graft was performed in one child with coarctation of the aorta. Angiotensin-converting-enzyme inhibitors should be used with caution in treating the hypertension caused by TA, since bilateral renal arterial narrowing is common and their administration may result in renal insufficiency. The long-term prognosis is guarded in severely hypertensive children with extensive vascular disease due to TA.