Native Aortic Root Thrombus in 3-Year-Old Fontan Patient with Hypoplastic Left Heart Syndrome: Presentation and Echocardiographic Findings of This Life-Threatening Complication

Segar, David E.; Amidon, Matthew; Scott, J. Paul; Frommelt, Peter C.

doi:10.1016/j.case.2021.07.007

Cited by 1 publication

(15 citation statements)

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“…From seventeen studies initially selected, two articles were excluded for uncertain diagnosis and one because it was a post mortem analysis. Twelve case reports [ 1 , 2 , 3 , 5 , 6 , 7 , 8 , 9 , 11 , 12 , 13 , 14 , 15 ] and two original articles [ 10 , 11 ] met the criteria established and were selected for the analysis. In all 32 cases, native aortic thrombosis in HLHS was described, including our case ( Table 1 ).…”

Section: Literature Reviewmentioning

confidence: 99%

“…Age at diagnosis ranged from 13 days [ 7 ] to 18 years [ 5 , 10 ]. Clinical presentation varied from chest pain and/or ECG abnormalities (seventeen cases) [ 1 , 2 , 5 , 6 , 10 , 11 , 14 ] to incidental findings (eight cases) [ 2 , 7 , 11 ], cardiac arrest (three cases) [ 7 , 8 , 10 ], transient ischemic attack (TIA) (one case) [ 9 ], tachycardia (two cases) [ 7 , 10 ], and bradycardia (two cases) [ 1 , 12 ].…”

Section: Literature Reviewmentioning

confidence: 99%

“…Diagnosis was usually made with transthoracic echocardiography [ 1 , 2 , 6 , 7 , 9 , 10 , 12 ], while in a few cases, echocardiographic suspicion was confirmed at cardiac catheterization (five cases) [ 4 , 5 , 8 , 14 ] or at a computed tomography (CT) scan (three cases) [ 5 , 14 ].…”

Section: Literature Reviewmentioning

confidence: 99%

“…Most of the patients (nineteen cases, e.g., 59.4%) [ 2 , 5 , 7 , 10 , 11 , 14 , 15 ] were treated with anticoagulation, surgical thrombolysis was performed in six (18.7%) [ 4 , 6 , 7 , 8 , 10 ], direct thrombolysis in cardiac catheterization theater in four (12.5%) [ 4 , 5 , 7 , 8 ], and systemic thrombolysis in three patients [ 1 , 12 ]. Transplant-free survival was 56.2% (e.g., 18 out of 32 cases).…”

Section: Literature Reviewmentioning

confidence: 99%

“…Native aortic thrombosis in hypoplastic left heart syndrome (HLHS) is a serious and underrecognized complication that may occur at different stages of Fontan palliation and lead to left ventricular (LV) failure, arrhythmias, and death [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ]. Over the past two decades, only small case series of similar complications have been described [ 2 , 3 , 6 , 7 , 8 , 9 , 13 , 14 , 15 ], while in the past two years, a couple of works [ 10 , 11 ] systematically reviewed single-center experience over Fontan palliation to assess the incidence of such complications.…”

Section: Introductionmentioning

confidence: 99%

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Native Aortic Root Thrombosis in Hypoplastic Left Heart Syndrome: An Unusual Presentation (Soon after Atrial Septal Stenting) of a Relatively Unusual Complication—Experience and Literature Review with an Outlook to Diagnosis and Management

Cantinotti,

Marchese,

Assanta

et al. 2023

JCM

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We started with the experience of thrombus formation in the native aorta of a 3-year-old male child with hypoplastic left heart syndrome (HLHS) and severely hypoplastic but patent mitral and aortic valves after Glenn palliation, which occurred soon after left heart decompression by percutaneous stenting of the atrial septum. The diagnosis was incidental, with the child completely asymptomatic, and progressively subsided in a few days with heparin infusion and chronic warfarin therapy. We reviewed the incidence, diagnosis, and management of native aortic thrombosis in HLHS after different stages of Fontan palliation through a systematic literature search. In all 32 cases, native aortic thrombosis in HLHS was found. The HLHS anatomic subtypes included mitral stenosis/aortic stenosis (fourteen cases or 45.2%), mitral stenosis/aortic atresia (eleven cases or 35.5%), and mitral atresia/aortic atresia (four cases or 12.9%). The age at diagnosis ranged from 13 days to 18 years. Clinical presentation varied from incidental findings, chest pain and/or electrocardiographic abnormalities, cardiac arrest, and transient ischemic attack. Diagnosis was feasible in most of the cases with only transthoracic echocardiography. Mostly (59.4%), patients were treated with anticoagulation, while others underwent surgical (18.7%), direct (12.5%), or systemic (9.3%) thrombolysis. Transplant-free survival was 56.2%, and fatal events occurred in 25%. Major events occurred in 26.3% of those treated with anticoagulation, in 33.3% of patients treated with surgical/systemic thrombolysis, and in 100% of patients treated with direct thrombolysis. In summary, native aortic thrombosis in HLHS may occur at different ages, with a wide spectrum of presentation from incidental finding to a sudden major event. Diagnosis is feasible with transthoracic echocardiography, and management with anticoagulation is effective despite the incidence of major events remaining high.

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